II-4-1-4.Federal Old-Age, Survivors, and Disability Insurance; Determining Disability and Blindness; Revision of Part A and Part B of the Listing of Impairments; Endocrine, and Multiple Body Systems; Immune System (Final Rules; 58 FR 36008, July 2, 1993)

4190-29P

DEPARTMENT OF HEALTH AND HUMAN SERVICES

Social Security Administration

20 CFR Part 404 (58 FR 36008)

Regulations No. 4

RIN 0960-AC06

Federal Old-Age, Survivors, and Disability Insurance; Determining Disability and Blindness; Revision of Part A and Part B of the Listing of Impairments; Endocrine, and Multiple Body Systems; Immune System

AGENCY: Social Security Administration, HHS

ACTION: Final rules

SUMMARY: These amendments revise the Listing of Impairments in appendix 1 of 20 CFR part 404, subpart P (hereafter referred to as "the listings"). They establish a new listing section called "Immune System" in both part A and part B of appendix 1. The new part A (adult) listings section includes up-to-date criteria for evaluation of connective tissue diseases (previously contained in the "Multiple Body Systems" section) and establishes a listing for the evaluation of human immunodeficiency virus (HIV) infection. The amendments also move the adult listing for obesity from the "Multiple Body Systems" section to the "Endocrine System" section, and change the name of the "Endocrine System" section to "Endocrine System and Obesity." The new part B (childhood) section establishes a listing for the evaluation of human immunodeficiency virus (HIV) infection, includes up-to-date criteria for evaluation of congenital immune deficiency disease (previously contained in the "Multiple Body Systems" section), and adds new criteria for evaluation of connective tissue diseases.

These criteria describe disorders that are severe enough to prevent a person from performing any gainful activity, or in the case of a child under age 18 applying for Supplemental Security Income (SSI) based on disability, severe enough to prevent the child from functioning independently, appropriately, and effectively in an age-appropriate manner.

EFFECTIVE DATE: These rules are effective July 2, 1993.

FOR FURTHER INFORMATION CONTACT: Harry J. Short or Richard M. Bresnick, Legal Assistants, Office of Regulations, Social Security Administration, 6401 Security Boulevard, Baltimore, MD 21235, (410) 965-6242 or 965-1758.

SUPPLEMENTARY INFORMATION:

The Social Security Act (the Act) provides, in title II, for the payment of disability benefits to workers insured under the Act. Title II also provides for the payment of child's insurance benefits for persons who become disabled before age 22 and widow's and widower's insurance benefits based on disability for widows, widowers, and surviving divorced spouses of insured individuals. In addition, the Act provides, in title XVI, for SSI payments to persons who are disabled and have limited income and resources. For workers insured under title II, for children of workers insured under title II who become disabled before age 22, for widows, widowers, and surviving divorced spouses claiming widow's or widower's insurance benefits based on disability under title II, and for adults claiming SSI benefits based on disability, "disability" means inability to engage in any substantial gainful activity. For children under the age of 18 who apply for SSI benefits based on disability, "disability" means that the child's physical or mental impairment(s) is of comparable severity to an impairment that would make an adult (a person age 18 or older) disabled. Under both title II and title XVI, "disability" must be by reason of a medically determinable physical or mental impairment or combination of impairments that can be expected to result in death or that has lasted or can be expected to last for a continuous period of at least 12 months. To the extent that Medicare and Medicaid eligibility are based on title II and title XVI eligibility, these regulations also affect the Medicare and Medicaid programs.

Under the sequential evaluation process, if the evidence shows that an individual is not engaging in substantial gainful activity and has an impairment(s) that meets the duration requirement, is severe, and meets or equals in severity a listing criteria, the individual is disabled. (In the case of a child applying for SSI, this includes consideration of whether the child's impairment(s) is functionally equivalent to a listed impairment, as defined in § 416.926a.) If the impairment(s) (e.g., HIV infection) does not meet or equal in severity any listing criteria, we evaluate all signs, symptoms, laboratory findings, and other evidence to determine whether the person is disabled. For an adult, we assess residual functional capacity and, based on that assessment, determine whether the claimant retains the capacity to perform past relevant work, or, if not, whether he or she retains the capacity to perform any other work considering his or her residual functional capacity, age, education, and work experience. If not, the adult is disabled. For a child under the age of 18 applying for SSI, we individually assess the child's ability to function to determine whether there is a substantial reduction in the child's ability to function independently, appropriately, and effectively in an age-appropriate manner. If there is such a substantial reduction, the child is disabled.

Medical criteria for evaluating disability and blindness at the third step of the sequential evaluation processes for adults and children are found in the listings. The listings include examples of the most commonly occurring medical conditions for persons who file applications for disability benefits. It describes, for each of 13 major body systems, impairments that are considered severe enough to prevent an individual from engaging in any gainful activity, or in the case of a child under the age of 18 applying for SSI, examples of impairments that are severe enough to prevent a child from functioning independently, appropriately, and effectively in an age-appropriate manner. To establish disability under the Act, the impairment must be expected to result in death or last or be expected to last for a continuous period of not less than 12 months. Most of the listed impairments are permanent or are expected to result in death; in some instances, a specific durational requirement is a part of the medical criteria for the impairment (in addition to the 12-month duration requirement that applies to all impairments that are not expected to result in death). If an individual is not performing substantial gainful activity and has an impairment that meets the requirements of one of the listings, or has an impairment or combination of impairments that is equal in severity to one of the listings (and meets the duration requirement), the individual is disabled. If, however, the individual does not have an impairment which meets or equals in severity the requirements of a listing, the claim is not denied and no conclusion on the issue of disability is made. Rather, resolution of the issue of disability depends on other factors. For adults, these other factors are residual functional capacity, and, for adults who are unable to perform their past work, age, education, and work experience. For children whose impairments are severe but do not meet or equal in severity the requirements of a listing, we will do an individualized functional assessment and determine whether the child is able to function independently, appropriately, and effectively in an age-appropriate manner.

Appendix 1 consists of two parts, part A and part B. The criteria in part A apply to the evaluation of impairments of adults but may, in some cases, be appropriate for evaluating impairments in children under age 18. Part B contains medical criteria for the evaluation of impairments in children under age 18 when criteria in part A do not give appropriate consideration to the particular effects of the disease processes in childhood. In evaluating disability for a child under age 18, we use part B first. If the child's impairment(s) does not meet or equal the medical criteria in part B, or the criteria in part B do not apply, then we use the medical criteria in part A, when the criteria are appropriate. To the extent possible, we maintain a structural and content relationship between parts A and B (see §§ 404.1525 and 416.925). When part A criteria are repeated in part B, our intent is to eliminate any question about their application to children.

We revised the listings on December 6, 1985 (50 FR 50068). At that time, as a result of medical advancements in disability evaluation and treatment, and program experience, we indicated that the listings should be reviewed periodically and updated. Accordingly, we specified termination dates for the listings ranging from 4 to 8 years. These final rules revise the listings to: Establish a new listing section called "Immune System" in both part A and part B of appendix 1; establish new listings for the evaluation of HIV infection in both adults and children; update the criteria for evaluation of connective tissue disorders in adults and for congenital immune deficiency disease in children; establish new criteria for evaluation of connective tissue disorders in children; move the adult listing for obesity from the "Multiple Body Systems" section to the "Endocrine System" section; change the name of the adult "Endocrine System" section to "Endocrine System and Obesity"; delete Hansen's disease (leprosy, formerly Listing 10.02) from the listings; and modify the "Multiple Body Systems" section for children to make it effective for 5 years.

We revised the connective tissue disorders criteria with information we received from individuals recommended by various professional groups, including the American College of Physicians, the American College of Rheumatology (formerly the American Rheumatism Association), the Arthritis Foundation, the Lupus Research Institute, the American Society of Internal

Medicine, and from individual Federal and State representatives with expertise in the evaluation of disability claims involving connective tissue disorders. In addition, in developing the proposed part B criteria for children, we received information from individuals with expertise in these areas.

We developed the proposed listings contained in our December 18, 1991, Notice of Proposed Rulemaking (NPRM) (see 56 FR 65702) for HIV infection based in part on information from numerous individuals recommended by or affiliated with various professional organizations, including the Public Health Service's Centers for Disease Control (CDC), the Johns Hopkins Hospital, the State of Maryland AIDS Administration, the Department of Veterans Affairs, and Federal representatives with expertise in the evaluation of disability claims involving HIV infection. A number of individuals who commented on the NPRM expressed concern that we did not consult with experts on the various segments of the population who are infected with HIV, especially women and children. Therefore, in response to the comments, we obtained additional information about women and children and other segments of the population infected with HIV, and about other issues from experts in the Department of Health and Human Services, including experts from the Public Health Service's CDC, Health Resources and Services Administration, and National Institutes of Health. We also obtained additional assistance from individual physicians and other experts involved in the evaluation and treatment of HIV infection—particularly in women and children—in various parts of the country, as indicated below. We obtained additional information from individuals at Albert Einstein College of Medicine, Beth Israel Hospital, Bronx Lebanon Hospital, Columbia Presbyterian Hospital, Harlem Hospital, Montefiore Hospital, St. Luke's/Roosevelt Hospital, and St. Vincent's Hospital in New York; Howard University Hospital in Washington, D.C.; University of Maryland Medical Center and Johns Hopkins Hospital in Baltimore; University of Texas Medical School in Houston; Cook County Hospital and Children's Memorial Hospital in Chicago. Several of the experts were recommended to us by the American Medical Association and the American Academy of Pediatrics. We also received information from the American Medical Association and the HIV Project of the MFY Legal Services Inc., a legal advocacy group in New York City. Therefore, the listings contained in this final rule reflect updated information about HIV infection.

HIV Infection

In 1980, shortly after acquired immunodeficiency syndrome (AIDS) was first identified in the United States, the CDC developed a case definition in order to conduct epidemiologic surveillance. The CDC defined AIDS based on a variety of diseases that encompassed the most severe manifestations observed in infected individuals. In late 1982, we began receiving disability claims from individuals infected with HIV. We used the CDC's surveillance definition of AIDS in developing our initial criteria for determining disability of listing-level severity in people with AIDS. These criteria provided that an individual who had a confirmed diagnosis of AIDS, as manifested by one or more of the conditions identified by the CDC, and who was not engaging in substantial gainful activity, would have an impairment of listing-level severity. As medical knowledge and understanding about HIV infection was continuously refined, and with knowledge derived from adjudicating disability cases involving HIV infection, we updated our policies.

For instance, as early as 1983, clinicians identified a syndrome that for a time was known as AIDS-Related Complex (ARC), although that term was never used or defined by the CDC. Shortly thereafter, we issued instructions stressing our policy that the evaluation of disability in these cases was not limited to the CDC's surveillance definition of AIDS, that claimants with AIDS or ARC, like all disability claimants, must be evaluated on a case-by-case basis, and that an individual need not have fully developed AIDS to be found disabled.

We reminded our adjudicators that individuals who have immune system dysfunction, but who do not have a confirmed diagnosis of AIDS, may still have an impairment that is of listing-level severity, either because of a manifestation that, in and of itself, meets the criteria of a listing, or because an individual's impairment(s) is equal in severity to a listing. We said that, as with any medically determinable impairment, the assessment of severity must take into account all signs, symptoms, and laboratory findings—not only those included in our criteria—and must follow the full sequential evaluation process if an applicant's impairment(s) is severe and does not meet or equal in severity any listing.

As more information about HIV infection became available and as we gained even more adjudicative experience, it became apparent that HIV infection was being manifested by impairments that were not encompassed under ARC, or in the CDC's criteria for AIDS. By 1987, we issued instructions that provided specific criteria beyond those covered in the CDC's surveillance definition for adjudicators to use in evaluating whether a claimant's impairment(s) was of listing-level severity. We have continued to update and refine our instructions to reflect the escalating array of information available on the manifestations of HIV infection.

We have always viewed AIDS (and other symptomatic HIV infection) from a different perspective than the CDC. The CDC defines AIDS for health purposes to enhance its capability for activities such as disease reporting and surveillance, epidemiologic studies, prevention and control activities, and public health policy and planning. Its definition is not intended to determine whether any statutory or legal requirements for disability are met. In evaluating disability claims, our concern is to determine whether an individual's impairment(s) is severe enough to prevent him or her from engaging in any substantial gainful activity (or, in the case of a child under age 18 applying for SSI, substantially reduces the child's ability to function independently, appropriately, and effectively in an age-appropriate manner).

In these final rules, we have made it clear that all our disability evaluation standards apply to cases of HIV infection in the same way they apply to cases involving other impairments. The standards require, on a case-by-case basis, an evaluation of all relevant factors, including the symptoms (such as pain, fatigue, and malaise), signs, and laboratory findings, as well as the effects of medication, on the ability to function, and a determination whether an individual is able to engage in substantial gainful activity or, in the case of a child, able to engage in age-appropriate activities. Although these final rules relate only to one part of the sequential evaluation process, they stress that (as we do for all individuals) we apply the full sequential evaluation process when adjudicating claims from individuals with HIV infection.

HIV Infection in Women and Children

These final rules include specific criteria to take into account the clinical manifestations and course of the disease in women and children.

The criteria in parts A and B recognize that HIV infection can manifest itself differently in women (including female adolescents) than in men. Therefore, the final rules state that it is important when reviewing the claim of a woman with HIV infection that manifestations of HIV infection that affect women (e.g., gynecological conditions) be considered in assessing impairment severity and the degree of functional loss. Similarly, the criteria in part B recognize that the disease process may manifest itself differently in children (especially younger children) than in adults.

Other Immune System Listings

The final rules also include listings for evaluation of other immune system disorders. In preparing these listings, our aim was to put less emphasis on disease labeling or diagnosis, and to place more emphasis on the functional impact on a person's ability to work or, in the case of an SSI claimant under age 18, on the ability to function independently, appropriately, and effectively in an age-appropriate manner.

Endocrine System and Obesity

We revised the adult listing for obesity and moved it from the Multiple Body Systems (10.00) to the Endocrine System (9.00), and renamed the latter Endocrine System and Obesity. Therefore, all listings that were under 10.00, Multiple Body Systems, are now under either 14.00, Immune System, or 9.00, Endocrine System and Obesity. We also converted the weight tables that accompany the obesity listing into metric measurements.

Explanation of the Final Rules

We published an NPRM in the FEDERAL REGISTER on December 18, 1991 (56 FR 65702), and invited interested persons, organizations, and groups to submit comments pertaining to the proposed rules within a period of 60 days from the date of publication of the NPRM. The comment period ended on February 18, 1992.

In response to the NPRM, we received over 6,000 letters containing comments pertaining to the changes we proposed. The majority of the letters were form letters in support of the proposed changes to the listing for systemic lupus erythematosus (final Listing 14.02). We also received a number of other form letters that were sent by multiple individuals. The majority of these letters concerned the proposed HIV infection listings. Many of these were from legal services organizations and advocacy groups, State and city government departments, Members of Congress, and individual lawyers. Some letters came from individuals or Government agencies whose responsibilities require them to make disability determinations involving HIV-related impairments under titles II and XVI of the Act. Other letters came from medical associations, hospitals, physicians and other medical professionals, or from individual private citizens.

The public comments were invaluable to us in drafting these final rules. Some of the commenters pointed out problems or potential problems with the proposed rules, and we adopted or accommodated many of these comments. Other commenters, however, suggested changes that would go beyond the scope of these rules, or even our authority in promulgating regulations. We carefully considered all the comments we received, and adopted the commenters' suggestions whenever possible. In the public comments section of this preamble, we address all the substantive comments and explain how we used the comments (or why we did not use them) in preparing these final rules.

The following is a summary of the listings we are adopting in these final rules, with an explanation of the more important changes we made from the text of the NPRM. We describe other changes in the public comments section of this preamble.

9.00 ENDOCRINE SYSTEM AND OBESITY

9.00 Endocrine System and Obesity

Because we have moved the listing for obesity from former Listing 10.10 to final Listing 9.09, we also moved the prefatory text describing obesity, which was formerly in 10.00B, to the second paragraph of final 9.00. We also revised the paragraph to make it more specific to listing-level determinations. In response to a comment, we added a second paragraph which clarifies that the weight-bearing criterion in Listing 9.09A refers to the lumbosacral spine, not the cervical or thoracic spines.

We changed the headings of 9.00 and 9.01 to include references to obesity. There are no changes in the headings from the NPRM.

9.09 Obesity

We revised the listing for obesity, which was previously Listing 10.10 in the Multiple Body Systems section, and moved it to the Endocrine System section, which we renamed Endocrine System and Obesity. The listing for obesity is now Listing 9.09. In addition, we revised paragraph 9.09A (formerly 10.10A) to clarify that the pain, limitation of motion, and evidence of arthritis required in that section must be in the same joint or in the lumbosacral spine. We clarified the rules based on questions we have received over the years.

In response to a public comment, we revised the language of the NPRM to make clear that the phrase "weight-bearing" applies to both the joints and the spine; the revision adds language describing the lower parts of the spine (the lumbosacral spine), which are the weight-bearing areas. This is not a substantive change, but a clarification of the policy as we have always applied it. In the final rules, we also made a technical correction to the weight tables that accompany the obesity listing. We converted the table values to the metric system. Except for some very minor rounding off necessitated by the conversion, we did not change any of the relative weight and height criteria from the prior rules.

14.00 IMMUNE SYSTEM

We have established a new section 14.00, Immune System. The new section includes criteria for systemic lupus erythematosus (14.02), systemic vasculitis (14.03), systemic sclerosis and scleroderma (14.04), polymyositis or dermatomyositis (14.05), undifferentiated connective tissue disorder (14.06), immunoglobulin deficiency syndromes or deficiencies of cell-mediated immunity, excepting HIV infection (14.07), and HIV infection (14.08). We describe each of these listings below.

Final 14.00 includes all the criteria that were previously in 10.00, Multiple Body Systems, with the exception of former Listing 10.02, Hansen's disease (leprosy), which we have deleted, and former Listing 10.10, "Obesity." We deleted the Hansen's disease listing because new cases of the condition are almost nonexistent in the Western Hemisphere and because the disease can now usually be treated successfully. As already stated, we moved the obesity listing to 9.00. We reserved the entry for 10.00, which no longer contains any listings, for future use.

Although we made a number of editorial changes for clarity and consistency, we made few substantive changes from the NPRM in final Listings 14.02-14.07 and 114.02-114.07; that is, all but the HIV listings. We discuss those listings first, beginning with a summary of final 14.00A-C and 114.00A-C, the sections of the preface that are appropriate to the non-HIV listings. We then provide a summary of the provisions of, and changes to, the rules on HIV in 14.00D and 114.00D, and final Listings 14.08 and 114.08.

THE NON-HIV LISTINGS

14.00 and 114.00 Preface

Final 14.00A-C and 114.00A-C describe impairments of the immune system. We made a number of revisions in the final rules from the NPRM, both in response to comments and for technical reasons. Most of the changes were for consistency. We compared the rules in part A with those in part B and, wherever it was appropriate, added provisions that were lacking in one but were present in the other. We also revised language when both part A and part B contained the same provisions but used different language; the revisions simply make their language the same. As we explain below, none of these changes is substantive: They only improve the consistency and clarity of the rules.

14.00A and 114.00A

In 14.00A and 114.00A, we describe some of the components of the immune system. There are no changes from the NPRM.

14.00B and 114.00B

In final 14.00B and 114.00B, we discuss connective tissue disorders. In a technical correction, and for internal consistency, we changed the proposed phrase "connective tissue disease" in the first paragraph and throughout the preface to "connective tissue disorder." This is a more accurate description of the disorders. Moreover, the two phrases were used interchangeably in the NPRM; the revision now uses only one phrase throughout. We also changed the reference to "The American Rheumatism Association" in proposed 14.00B1 to the current name, "The American College of Rheumatology."

For consistency, we revised the language we had proposed as the second paragraph of 14.00B so that it better reflected the language we had proposed in the first paragraph of 114.00B (now the third paragraph in final 114.00B). We also made minor changes to the third paragraph of final 114.00B so that both paragraphs would say the same thing.

The final language in the second paragraph of 14.00B is almost identical to the proposed language. The most significant difference is that we deleted the proposed opening statement from the NPRM, "Each of these disorders should be differentiated diagnostically * * *," for conformity with the childhood rules. Although the clause was true, we believe that it is inherent in the remaining language of the paragraph, as well as being a basic principle of disability evaluation that need not be repeated in this particular context.

We expanded the third paragraph of final 14.00B from the NPRM to incorporate language that was formerly in proposed Listings 14.02-14.06 and 114.02. We simplified those listings, each of which repeated the same provisions about duration of active disease despite prescribed therapy, by incorporating the language into the preface and replacing the repetitious criteria in the listings with cross-references to the preface. We describe our reasons for this change in greater detail in the public comments section of this preamble. However, the revision is merely editorial, not substantive.

In the fourth paragraph of the final adult rules in 14.00B, we made two technical corrections, but no substantive changes. We replaced the abbreviation "SAL" with "SLE," for systemic lupus erythematosus, and we replaced the phrase "undifferentiated connective tissue disease" with "undifferentiated connective tissue disorder," as already described. We did not change the language of the fifth paragraph of the adult rules from the proposed language in the NPRM.

In the sixth paragraph of final 14.00B, we revised the first sentence to refer to "any gainful activity," which is the standard of severity in the listings, as set forth in §§ 404.1525 and 416.925, instead of the proposed "gainful work activities." This is a technical correction for consistency among the rules. We also added a sentence describing our use of the word "severe" in these listings. We explain this addition, and our reasons for making it, in the public comments section of this preamble. We added the latter sentence to the sixth paragraph of final 114.00B.

We made only minor editorial revisions in final 14.00B1-3 (for example, by deleting the word "Listing" before the listing numbers for consistency with other body system listings). In response to a comment, we revised 14.00B4, Polymyositis or dermatomyositis, to provide more information about the criteria for muscle weakness in final Listing 14.05. We also updated the terminology by replacing the references to SGOT and SGPT with the more current, generic term "aminotransferases."

In response to a comment, we added a discussion of so-called overlap syndromes to final 14.00B5. We also indicate that these syndromes are to be evaluated under Listing 14.06, Undifferentiated connective tissue disorders.

Finally, we made a number of nonsubstantive editorial revisions to the childhood rules in 114.00B in addition to those already mentioned. We divided the first paragraph of the NPRM into three separate paragraphs for clarity and better conformity with the paragraphs in the corresponding adult rules. We added a new fourth paragraph, which is identical to the fifth paragraph of 14.00B, to stress the importance of considering the effects of treatment in connective tissue disorders; the addition is only for consistency between the adult and childhood rules.

We also established a new 114.00C to include allergies (also in conformity with the adult rules). We also moved the second and third paragraphs of proposed 114.00B, dealing with growth impairments and Kawasaki disease, into the new section. We did this because both of these paragraphs provide guidance about cross-referring to other listings: The second paragraph of proposed 114.00B provided that growth impairments could be evaluated under the listings in 100.00, and the third paragraph provided cross-reference listings for Kawasaki disease. Inasmuch as the guidance on allergic disorders refers to evaluation under the appropriate body system listing, we believe that it is clearest to group all three paragraphs together under the same heading.

14.00C and 114.00C

Final 14.00C of part A states that allergic disorders are discussed under the appropriate listing for the affected body system. We made no substantive changes from the NPRM in this paragraph. In a technical clarification, we added the phrase "and evaluated," to the sentence to make it clear that allergic disorders are both discussed and evaluated under the appropriate listing for the affected body system.

Final 114.00C of part B is new. As we have explained, we established the section in order to include the same guidance about allergic disorders in the childhood rules as is in the adult rules. We also moved the paragraphs about growth impairments and Kawasaki disease from proposed 114.00B into this new section for reasons already given. We also added a heading, for clarity. In a technical revision, we revised the provision on Kawasaki disease to better state our original intent. The proposed language could have suggested that Kawasaki disease is not a multisystem impairment when, in fact, all we meant to say was that disease of the coronary arteries is the usual cause of listing-level disease.

Because we added this new section in the childhood rules, we redesignated proposed 114.00C, on HIV infection, to 114.00D. This also makes the lettering of the childhood preface correspond to the lettering in the adult preface.

14.02-14.07 and 114.02-114.07 The Non-HIV Listings

14.02 and 114.02 Systemic Lupus Erythematosus

The final rules move former Listing 10.04 to 14.00, renumber it as Listing 14.02, and change the heading from "Disseminated lupus erythematosus" to "Systemic lupus erythematosus" to conform to the current nomenclature for this disease. They also establish a new Listing 114.02 for systemic lupus erythematosus in children that is nearly identical to the adult rule, but includes criteria for the possible limiting effects unique to children that are not included in the adult rules.

In the final adult rule, we expanded and revised the criteria formerly in Listing 10.04 to focus on and delineate severe functional loss. We also removed the requirement that this disorder be established by a positive lupus erythematosus (LE) preparation, biopsy, or positive anti-nuclear antibody (ANA) test in favor of the currently accepted 1982 criteria of the American College of Rheumatology for classification of this disease (cited in final 14.00B1). Both final Listings 14.02A and 114.02A describe listing-level abnormalities in a single organ or body system, whereas final Listings 14.02B and 114.02B describe disability resulting from functional loss of lesser severity than in Listing 14.02A or 114.02A in two or more organs or body systems, with severe documented constitutional symptoms and signs.

We revised both listings from the proposed language in the NPRM in response to public comments and for technical reasons. For reasons we have already explained, we removed the criteria, "Documented * * * by a longitudinal clinical record of at least 3 months demonstrating active disease despite prescribed therapy during this period with the expectation that the disease will remain active for 12 months," from the proposed language in the first paragraph of Listings 14.02 and 114.02, as well as the similar language in Listings 14.02B and 114.02B; the criteria now appear in the third paragraph of 14.00B and the fifth paragraph of 114.00B and are applicable to all connective tissue disorders.

In final Listings 14.02A and 114.02A, we made minor technical revisions to several of the cross-references to other listings so that the listing now refers to other listing sections, rather than to individual listings. The change makes our method of cross-referencing consistent within the two listings. Moreover, by referring to entire body system listing sections instead of individual listings, we ensure that Listings 14.02 and 114.02 will remain current if the numbering changes as other body system listings are revised.

In response to a comment, we added a new Listing 114.02A3 for muscle involvement. The same criterion already appears in the adult rules at Listing 14.02A2. The addition of the new criterion required us to renumber the subsequent criteria. We also reversed the order of the criteria for endocrine and skin involvement so that they are in the same order that they appear in the cross-referenced listings. In response to a comment, we also added a cross-reference to final listing 14.04D to include listing-level Raynaud's phenomena under final Listings 14.02A5 and 114.02A6.

As we explain in the public comment section of this preamble, we also changed the phrase "severe, documented, incapacitating constitutional symptoms and signs" from proposed Listings 14.02B and 114.02B to "significant, documented, constitutional symptoms and signs" in order to clarify the phrase and make it consistent with language in final Listings 14.03 and 14.04.

14.03 and 114.03 Systemic Vasculitis

The final rules move prior Listing 10.03 to section 14.00, renumber it as Listing 14.03, and change the heading from "Polyarteritis or periarteritis nodosa (established by biopsy)" to "Systemic vasculitis" to correspond with currently accepted medical nomenclature. We also expanded this listing to emphasize the spectrum of vasculitic/arteritic syndromes that can preclude any gainful activity. These syndromes include classical polyarteritis nodosa, aortic arch arteritis, giant cell arteritis, Wegener's granulomatosis, and vasculitis associated with other connective tissue disorders.

The only changes from the NPRM language in final Listing 14.03 are those that we have already described in connection with final Listing 14.02. We deleted the language about documentation of active disease for 3 months despite therapy and the expectation of persistence for 12 months from the opening paragraph of the listing and the similar language in Listing 14.03B, because the provisions are now in the third paragraph of final 14.00B. We also changed the phrase "severe, documented, constitutional symptoms and signs" in proposed 14.03B to "significant, documented, constitutional symptoms and signs" consistent with our revisions in Listings 14.02, 14.04, and 114.02.

The childhood listing for systemic vasculitis, 114.03, is unchanged substantively from the NPRM. We made a minor language change for clarity, but the listing still cross-refers to the adult rules in Listing 14.03, and also includes a criterion for growth impairment.

14.04 and 114.04 Systemic Sclerosis and Scleroderma

The final rules move prior Listing 10.05 to section 14.00, renumber it as Listing 14.04, and change the title from "Scleroderma or progressive systemic sclerosis (the diffuse or generalized form)" to "Systemic sclerosis and scleroderma." We deleted the term "progressive" from the title because it was redundant. The criteria in final Listing 14.04 describe systemic disease of severity that precludes performance of any gainful activity for the requisite duration. The proposed criteria provide greater specificity in describing listing-level severity in the extremities and target organs (i.e., lungs, heart, kidneys).

We changed the NPRM language in the same way we have already described in connection with final Listing 14.02. We deleted the language about documentation of active disease for 3 months despite therapy and the expectation of persistence for 12 months from the opening paragraph of the listing and the similar language in Listing 14.04B, because the provisions are now in the third paragraph of final 14.00B. We also changed the phrase "severe, documented, constitutional symptoms and signs" in proposed 14.03B to "significant, documented, constitutional symptoms and signs" consistent with our revisions in Listings 14.02, 14.03, and 114.02.

In response to a comment, we made a minor revision in final Listing 14.04D. We replaced the word "with" with the phrase "characterized by" to clarify our original intent that the phrase "digital ulcerations, ischemia, or gangrene" describes the severe Raynaud's phenomena in the listing.

We added a separate childhood listing because these disorders may be manifested differently in children than in adults. Moreover, even when the manifestations are similar, the impact on a child's growth, development, and age-appropriate functioning may be more serious than the impact on an adult's ability to perform work-related activity. We revised proposed Listing 114.04 by adding cross-references to the documentation requirements in 14.00B3 and 114.00B in the opening paragraph. The revision is for consistency with the corresponding adult section and is not substantive.

We also revised the cross-references to other listings in final Listings 14.04A and 114.04B so that both listings now refer to entire listing sections, rather than to individual listings. This makes the cross-references consistent with those in final Listings 14.02A and 114.02A, and ensures that the listings will remain current when other body systems are revised.

14.05 and 114.05 Polymyositis or Dermatomyositis

The prior listings formerly codified in 10.00ff did not include listings for polymyositis or dermatomyositis. We added the new adult listing to recognize the potential for a disabling work-related functional deficit in some patients with chronic refractory myopathy. We added a separate childhood listing because these disorders may be manifested differently in children than in adults. Moreover, even when the manifestations are similar, the impact on a child's growth, development, and age-appropriate functioning may be more serious than the impact on an adult's ability to perform work-related activity.

In response to public comments, we added a discussion on evaluating severity of muscle weakness to proposed 14.00B4 and changed the cross-reference in final Listing 14.05A from 11.12B to 14.00B4. In response to another comment, we also revised final Listing 14.05B1 to better describe impairment of swallowing. We describe these changes in greater detail in the public comments section of this preamble.

We made only minor revisions to the final childhood listing. As in final Listing 114.04, we added cross-references to the appropriate documentation requirements in final 14.00B4 and 114.00B. We also revised the remainder of the listing language for consistency with other listings.

14.06 and 114.06 Undifferentiated Connective Tissue Disorders

We added new undifferentiated connective tissue disorders listings in parts A and B because some individuals can be disabled at the listing-level by connective tissue disorders that cannot be classified with an exact diagnosis.

In response to a comment about the NPRM, we added to final Listing 14.06 a cross-reference to Listing 14.04. In response to another comment, we revised final Listing 114.06 to change the cross-reference from Listing 14.06 of the adult listings to Listings 114.02 or 114.04 of the childhood listings.

14.07 Immunoglobulin Deficiency Syndrome or Deficiencies of Cell-Mediated Immunity, Excepting HIV Infection; 114.07 Congenital Immune Deficiency Disease

We added new Listing 14.07 to provide criteria for adults comparable to those we formerly included for children in Listing 110.09 (now final Listing 114.07). The listing provides criteria with which to evaluate immunoglobulin deficiency syndromes and deficiencies of cell-mediated immunity, excepting HIV infection.

In the final rule, we reorganized the language of proposed Listing 14.07 in order to make it consistent with Listing 114.07. The reorganization does not change the criteria.

We moved prior Listing 110.09 to section 114.00, renumbered it as Listing 114.07, and changed the heading from "Immune deficiency disease" to "Congenital immune deficiency disease." As in the foregoing listings and throughout these listings, we revised the language of proposed Listing 114.07A1 to make it consistent with final Listing 14.07. Because of this revision, we have deleted the requirement from proposed Listing 114.07A1 that the episodes of recurrent, severe infections must have occurred in the 5 months prior to adjudication. This additional requirement was not only inconsistent with the adult rules, but would have made the childhood listing more stringent than the adult listing and would have been difficult to implement in our case adjudications at the various levels of appeal.

THE HIV LISTINGS

In response to the many comments we received about the proposed rules for evaluating HIV infection, we have extensively revised the final rules from the NPRM. The following are some of the most important changes in the final rules. Thereafter, we provide a summary of all of the final provisions pertaining to HIV, beginning with final 14.00D and 114.00D of the prefaces.

Reorganization and Simplification

The most obvious change we made to the proposed rules in response to public comments was to reorganize and rewrite the proposed HIV infection listings (14.08 and 114.08). We did so in response to many commenters' concerns about the complexity of the proposed listings, and suggestions that we include additional manifestations of HIV infection in the listings and delete or modify some of the criteria we proposed.

Many commenters pointed out that the proposed listings were unnecessarily complex and repetitive for Social Security disability evaluation purposes. This was primarily because we had included in the listings both the CDC's criteria defining AIDS and other manifestations of symptomatic HIV infection we deemed appropriate for inclusion in our listings, even though they are not AIDS-defining under the CDC surveillance definition. As we have already explained, however, the CDC's criteria are primarily for surveillance purposes, not for the evaluation of disability; therefore, the CDC criteria contain requirements that are not necessary in our program.

For instance, the CDC surveillance definition contains criteria for establishing the diagnosis of AIDS in the presence of documented HIV infection, as well as when infection is not documented. However, both categories include a number of opportunistic infections in common that establish the diagnosis of AIDS; for example, pneumocystis carinii pneumonia and extrapulmonary cryptococcosis are included in both categories and, therefore, are repeated within the CDC's surveillance definition of AIDS. As many commenters pointed out, whereas it may be appropriate for the CDC surveillance definition to be repetitive for surveillance purposes, for Social Security disability purposes we need only be satisfied that a person with HIV infection has experienced one of the manifestations (for example, pneumocystis carinii pneumonia or extrapulmonary cryptococcosis) to conclude that the individual has a listing-level impairment. Therefore, it was unnecessary for us to have listed these infections in two places (proposed adult Listings 14.08A2 and C2, and childhood Listings 114.008A2 and C2 for extrapulmonary cryptococcal infections, and proposed adult Listings 14.08A8 and B2, and childhood Listings 114.08A9 and B2 for pneumocystis carinii pneumonia) when the outcome was the same in both instances. Similarly, the CDC surveillance definition includes several separate criteria for Hodgkin's and non-Hodgkin's lymphomas, which we had listed separately, following the CDC surveillance definition. We had also proposed to include other lymphomas that are not included in the CDC surveillance definition, and listed them separately. The commenters pointed out that we were, in effect, saying that any individual who has HIV ifection and any lymphoma would have an impairment that meets our listing and that there was, therefore, no need to list lymphomas in three separate places as we had proposed (i.e., in proposed adult Listings 14.08A6, I, and J, and childhood Listings 114.08A6, H, and I).

Many commenters pointed out that it was also unnecessary, and could be unfair, to provide specific requirements for the diagnosis of each manifestation of HIV infection in the listings. They pointed out that, at a minimum, we could summarize our criteria for establishing the existence of HIV infection and its manifestations in the prefaces to the listings; i.e., 14.00 and 114.00. (They also offered comments about our rules for establishing these findings, many of which we adopted, and which we describe later in this preamble.)

In the final adult rules, therefore, we combined the criteria in proposed Listing 14.08A with the criteria in proposed Listings 14.08B-L and organized them first by etiology of infection (final Listings 14.08A-D) and then by type of manifestation, regardless of etiology (final Listings 14.08E-N). We also removed the specific documentation requirements for each disease or manifestation from the listings and consolidated all documentation requirements with the general discussion of documentation in final 14.00D3 and D4. We removed duplicative language from the listings and clarified the standards established for many of the diseases. We made the same kinds of changes to the childhood listings in 114.00 and 114.08.

Manifestations of HIV Infection in Women

In response to public comments, final Listing 14.08 now also includes specific criteria for most manifestations of HIV infection that are common in women. Because these conditions are now included in the listings, we deleted the discussion of specific conditions common in women that we proposed in 14.00D of the NPRM, although we retained and augmented that section's general discussion of women's issues in final 14.00D5. The final listing, which we describe in greater detail below, explicitly mentions conditions common to women and provides criteria by which we will determine whether a given manifestation is of listing-level severity. We provide specific responses to the many comments on this issue in the public comments section of this preamble.

Listings 14.08M, 114.08L and 114.08M: The Functional Criteria

We received many public comments on the functional criteria in proposed Listings 14.08M, 114.08L, and 114.08M, the majority of which were unfavorable. The proposed rules had listed several possible manifestations of HIV infection (for example, meningitis, Kaposi's sarcoma, mucosal candidiasis, and oral hairy leukoplakia), and clinical and laboratory findings (for example, anemia, fever, and weight loss) that were not listed as stand-alone medical manifestations but that, in conjunction with functional restrictions, could establish listing-level HIV infection.

The commenters asked us to delete or substantially revise the rules for a number of reasons. Many commenters asked us to delete the rules employing functional criteria because they thought that the proposed medical manifestations were sufficient in themselves to establish listing-level disability. Many of these commenters pointed out that we had already incorporated indicators of medical severity by requiring the conditions in proposed Listings 14.08M2, 114.08L1, and 114.08M2 to be "persistent and/or resistant to therapy," and by requiring a 2-month persistence of at least two of the medical manifestations in proposed Listings 14.08M3, 114.08L2, and 114.08M3. The commenters pointed out that to require functional limitations in addition to these medical requirements seemed excessive and unfair. For instance, a number of commenters thought that diarrhea that had already persisted for 2 months and was unresponsive to treatment should be enough to establish disability, and need not be associated with another medical manifestation and functional limitations.

Some commenters thought that the mere existence of some of the manifestations (for example, pulmonary tuberculosis or vulvovaginal candidiasis) was in itself sufficient to establish disability in HIV-infected individuals and that no indicator of severity—either medical or functional—was necessary. Others offered suggestions for tying some of the manifestations to a test of functioning while making some of the other manifestations stand-alone medical listings without functional criteria. Some offered suggested criteria for describing medical severity for the stand-alone manifestations. The thrust of these suggestions was toward providing medical criteria specific to each different manifestation instead of the more general criteria for persistence and unresponsiveness to treatment we had proposed.

With regard to the functional criteria themselves, most of the comments addressed the adult criteria in proposed Listing 14.08M. Many people said that the criteria were inappropriate and too difficult to meet. Some said that the criteria were originally intended for the evaluation of mental impairments and, therefore, could not be used to evaluate physical impairments, especially HIV infection. (Of these comments, many singled out the criterion of marked limitation of social functioning in proposed Listing 14.08M4b as being especially inapt.) Some thought that this was the first time we had employed functional criteria in the physical listings and said that we should not start with HIV infection.

Many commenters who thought that we should not have the functional criteria at all recommended that, if we must have functional criteria, we should revise the proposed rules so that meeting only one of the functional criteria—instead of two, as we had proposed—would suffice. Some thought that we should incorporate into the listing the two functional criteria we formerly used in our manual instructions, believing the old criteria to be less stringent than the proposed criteria. Some thought that we should revise the language of the functional criteria to make them more specific to HIV infection. Finally, many commenters said that our definitions of the term "marked" in proposed 14.00D with respect to each of the functional criteria set too severe of a standard.

We address the individual comments, and other related comments, in more detail in the public comments section of this preamble. Notwithstanding the comments, however, we have decided to retain listings that permit a showing of disability based on an individualized assessment of the impact of a person's HIV infection on his or her functioning in the broad areas of activities of daily living, social functioning, and concentration, persistence, or pace. However, we have also extensively revised the rules in response to the comments, and we believe that we have addressed many of the commenters' concerns.

We have addressed most of the concerns by adding listings that provide stand-alone medical criteria for most of the manifestations that were in proposed Listings 14.08M, 114.08L, and 114.08M. The medical criteria in the new stand-alone medical listings are specific to the listed manifestations. (For reasons we explain later, we have deleted the criterion for a CD4 (T4) lymphocyte count; therefore, there are no provisions in the final rules corresponding to proposed Listings 14.08M1 or 114.08M1.) By doing this, the functional criteria become simply an alternative way that individuals with most of the manifestations in the proposed rules can establish that they are disabled under the listings, instead of the only way.

In final adult Listing 14.08N, we now describe episodic manifestations. Listing 14.08N thus includes individuals who suffer from the same manifestation periodically but who are not necessarily continuously ill; whose manifestations, though continuously present, wax and wane in severity; or who suffer episodes of different manifestations that, taken together, demonstrate listing-level severity. Final childhood Listing 114.08O (which replaces proposed Listings 114.08L and 114.08M and applies to children from birth to the attainment of age 18) includes all manifestations of HIV infection (both episodic and continuous). Both new listings include people who have manifestations that are listed in the preceding medical listings but that do not meet the medical criteria, as well as manifestations that are not listed in final Listings 14.08A-M and 114.08A-N.

For reasons we explain below, the final adult listing, 14.08N, now includes only three functional criteria—1) activities of daily living; 2) social functioning; and 3) concentration, persistence, and pace—and an individual need only establish marked limitations in one of the three areas to show an impairment that meets this listing. We have also revised our definitions of the term "marked" to clarify its applicability in HIV cases.

As we analyzed the comments, we realized that many of them were based on misconceptions about both the proposed rules and how we evaluate disability in general. Although we agreed with those commenters who expressed concerns that some of the conditions tied to the functional criteria in the proposed rules need not be so tied—and we made appropriate changes—we could not agree with those commenters who stated that the proposed functional criteria would be used to deny disability benefits or to disqualify some individuals.

Our disability evaluation policies do not permit denial of disability benefits on the basis that an individual's impairment(s) does not impose functional limitations at the listing level. We use the listings at the third step of our sequential evaluation processes for adults and children to "screen in," i.e., allow, individuals who are clearly disabled. (See §§ 404.1520, 416.920, and 416.924 for our rules on the sequential evaluation processes.) Under these processes, if an individual's impairment(s) is "severe" but does not meet or equal in severity any listing, we reach no conclusion at all about disability. Rather, we move on to the next step of the process and look at other factors to resolve that ultimate issue. We may use the criteria described in these listings to find that an individual is disabled, but we do not use the criteria to find that an individual is not disabled.

Therefore, the nature of this process is such that any time we include a new listing in appendix 1, no matter what the requirements, this is an advantage to an individual who applies for disability benefits because it adds a new way we may find the individual disabled, without adding a new way to find him or her not disabled.

As in the proposed rules, the functional criteria in these final rules serve a very important purpose—to provide individuals who have what may at first seem like less severe manifestations of HIV infection, or combinations of impairments that would not fit neatly into any of the purely medical listings, with a listing their impairments can meet. The listing, therefore, provides claimants with every opportunity to be found disabled as early in the evaluation process as possible. We believe that the commenters who argued against the functional criteria did not understand this purpose and misinterpreted what we intended to be a very beneficial part of the listing. This was partly because we did not explain it clearly enough. But it was also because—as the commenters correctly pointed out—we need not have limited the functional criteria only to certain specific manifestations, that some of the proposed manifestations were in themselves disabling, that some of the manifestations in the listing were more medically serious than others, and that we could have provided alternative medical criteria for some of the manifestations we had proposed.

The following changes respond to the commenters' concerns that some HIV-related medical conditions were included in the listings only in relation to functional standards. At the same time, they retain the flexibility we need for making favorable disability determinations at the listing level using functional criteria.

  1. Stand-Alone Medical Criteria

    We reviewed each of the medical conditions that were tied to functioning in proposed Listings 14.08M, 114.08L, and 114.08M and attempted to draft a medical description of each condition, at listing-level severity, that did not include a functional evaluation. In doing so, we heeded comments pointing out that some of the medical requirements in proposed Listing 14.08M were already extremely severe without the functional criteria. We did not, however, agree with those commenters who thought that the mere existence of each of the manifestations should be enough to establish listing-level severity. Most of the manifestations we had proposed in Listing 14.08M can vary in severity, responsiveness to treatment, and their impact on functioning. Therefore, we believe it is imperative that each manifestation be described by criteria that define listing-level severity if it is to be a stand-alone medical listing.

    We were able to draft stand-alone listings for all the manifestations included in proposed Listings 14.08M2, 114.08L1, and 114.08M2: Pulmonary tuberculosis in final Listings 14.08A1 and 114.08A1; Kaposi's sarcoma in final Listings 14.08E2 and 114.08E2; peripheral neuropathy in final Listings 14.08H and 114.08H; and pneumonia, bacterial or fungal sepsis, meningitis, septic arthritis, and endocarditis in final Listings 14.08M and 114.08N. We were also able to include most of the conditions included in proposed Listings 14.08M3, 114.08L2, and 114.08M3: Mucosal candidiasis, including vulvovaginal candidiasis, and dermatological conditions in final Listings 14.08B2 and 14.08F and 114.08B2 and 114.08F; Herpes zoster in final Listings 14.08D3 and 114.08D3; anemia, granulocytopenia, and thrombocytopenia in final Listings 14.08G and 114.08G; diarrhea in final Listings 14.08J and 114.08J; and sinusitis in final Listings 14.08M and 114.08N. In some cases, the new criteria consist of a reference to another listing (e.g., final Listing 14.08G, anemia, as described under the criteria in 7.02). In other cases, the criteria are new (e.g., final Listing 14.08D3, Herpes zoster either disseminated or with multidermatomal eruptions, that are resistant to treatment).

    We did not include fever, weight loss, and oral hairy leukoplakia as stand-alone listings. Fever and weight loss are not medical conditions in themselves, but the observable outcome—i.e., signs—of medical conditions. We believe that there are few people whose sole manifestation of HIV infection is a persistent, high fever without any other observable problems; indeed, the individual will likely have other signs and symptoms that may be evaluated together with the fever. Moreover, any stand-alone medical listing that tried to describe listing-level fever would have to be set at a very high level, would rarely apply, and would be subject to the same criticism that we received about some of the manifestations in the proposed functional listings, i.e., that it is too severe. We believe, therefore, that it would be better to evaluate the few individuals who suffer only from persistent fever (of any level) in terms of their functioning; such individuals may be fatigued and weak, have difficulty doing their daily chores, and may even be confined to their homes or even to bed. Final Listings 14.08N and 114.08O also allow for the possibility that the individual's fever is not constant, but recurrent, which we believe is a more realistic possibility than continuous high fever. To underscore these points, we have included fever among the examples of symptoms and signs that may result in the functional limitations in the listing.

    Similarly, weight loss is already inherent in the listings for HIV wasting syndrome and growth disturbance (final Listings 14.08I and 114.08I) as well as the aforementioned new listings for diarrhea. Individuals who have unexplained weight loss or weight loss because of loss of appetite may have impairments that are medically equivalent to one of these listings or the new functional listings, or to listings in other body systems; even those whose weight loss is not as serious as in final Listings 14.08I and 114.08I may have symptoms of fatigue and weakness resulting in listing-level functional restrictions. We have also included weight loss among the examples of signs and symptoms that may result in the functional limitations of the listing.

    We did not include oral hairy leukoplakia as a stand-alone medical condition because it is generally an asymptomatic condition that may persist for a relatively long time without interfering with the individual's functioning. We believe, therefore, that each case will have to be evaluated to determine the particular effects of the manifestation on the individual under final Listings 14.08N or 114.08O. We also did not include from the proposed childhood rules parotitis, or the clinical findings of splenomegaly, hepatomegaly, and generalized lymphadenopathy. These conditions and clinical findings can vary greatly from child to child in their severity, medical significance, and impact on a child's ability to function. Because of this, it is not possible to define with solely medical criteria, except in the most extreme terms, a level of severity for these conditions and clinical findings that would interfere with most children's ability to engage in age-appropriate activities to the required degree.

    Final Listings 14.08N and 114.08O do not list specific impairments. We made this change partly in response to comments suggesting many other possible manifestations of HIV infection for inclusion in the listing and partly because it is logical. We decided that instead of expanding the list of manifestations, we could respond to the commenters' concerns by abandoning the finite list of HIV-related manifestations and referring instead to "manifestations of HIV infection" in general. This allows for consideration of any manifestations, whether identified in the listing or not. We have also added discussions to final 14.00D8 and 114.00D8, the sections of the prefaces that describe the functional criteria, explaining that these listings may be used not only to evaluate manifestations of HIV infection that are not included in Listings 14.08A-M and 114.08A-N, but to evaluate manifestations that are listed in, but do not meet the criteria of, those listings.

  2. The Functional Criteria

    Many commenters expressed concern that the functional criteria in proposed Listing 14.08M were based on the functional deficits described in the mental impairment listings for adults in 12.00 of the listings. The commenters were concerned that these criteria, therefore, only related to individuals with mental disorders and were not appropriate measures of severity in the case of individuals with HIV infection. We do not agree. Although adjudicators are most accustomed to applying the functional criteria in 12.00 in the context of mental impairments, those criteria describe broad areas of functioning that are relevant to any individual's ability to work. It does not matter, for example, whether an individual's ability to perform activities of daily living is restricted because of memory loss or hallucinations, or whether it is because of fatigue, headaches, or weakness resulting from a manifestation of HIV infection. In either event, the ability to perform the tasks is compromised.

    Nevertheless, we realized from the comments that the proposed rules may not have made application of the functional criteria sufficiently specific to the evaluation of HIV-related impairments. Therefore, we modified proposed Listing 14.08M to more clearly reflect our original intent, which was to expand the way we assess the severity of HIV-related impairments at the listing level beyond the use of strict medical criteria by using broad functional criteria. We had hoped to include in the listings (via proposed Listing 14.08M3) a group of individuals whom we believed would be very difficult to describe in strictly medical terms—individuals who become ill then improve, only to repeatedly become ill again, either with the same manifestation of HIV infection or with different manifestations.

    Based on some commenters' questions about the applicability of the proposed functional criteria to physical disorders, we also realized that proposed paragraph 14.08M4d, repeated episodes of decompensation, was not really a measure of functioning at all, but a description of what we were trying to address in this listing. Unlike its purpose in the mental listings (where decompensation can be a measurement of an individual's ability to tolerate stress), when applied to HIV-related illnesses the criterion measured the persistence and frequency of episodes of manifestations of HIV infection; in effect, it distinguished between individuals who develop and recover from only one or two isolated manifestations of HIV, and those who have a pattern of repeated episodes of illness.

    Therefore, we removed proposed Listing 14.08M4d from the list of functional criteria, modified it to make it more specific to HIV, and used it as the introductory criterion for final Listing 14.08N. The final listing is for the evaluation of individuals who have repeated manifestations of HIV infection. We also revised the criterion in response to commenters who pointed out that the requirement in proposed paragraph 14.08M4d for the episodes to occur 3 times a year or once every 4 months and to last for at least 2 weeks was unnecessarily inflexible. In the third paragraph of 14.00D8 we have retained the provision that the conditions may occur on an average of 3 times a year, or once every 4 months, and each last at least 2 weeks, and at the same time we provide additional flexibility. Specifically, we now provide that the episodes may also last for less than 2 weeks and occur substantially more frequently than 3 times a year or every 4 months, or that they may occur less frequently than 3 times a year or once every 4 months but last substantially longer than 2 weeks each time.

    In response to commenters who asked us to include criteria for some of the more common symptoms and signs of individuals who do not have CDC-defined AIDS, we adopted and expanded language from Listing 14.02B, the listing for systemic lupus erythematosus, about which we received literally thousands of favorable public comments. The language in final Listing 14.08N explains that disability under this listing will result from "significant, documented, symptoms or signs (e.g., fatigue, fever, malaise, weight loss, pain, night sweats)" that cause functional limitations. (Unlike Listing 14.02B, we do not provide that there must be both symptoms and signs in this listing. The constitutional symptoms and signs in Listing 14.02B help to define the syndrome of systemic lupus erythematosus and its severity. In contrast, the criterion in final Listing 14.08N includes any symptoms or signs that can be the cause of the functional limitations because the existence of the impairment has already been established.)

    We retained the three remaining functional criteria as the standards for measuring functional deficit. Having more accurately described the individuals to whom we intend the listing to apply, we then agreed with commenters who stated that marked functional restrictions in any one of the categories would be sufficient to demonstrate listing-level severity. Consequently, final Listing 14.08N requires marked limitations in only one of the three broad areas of functioning.

    We want to reiterate, however, that we retained a revised version of the proposed Listing 14.08M4d criterion in the final listing. Our intention in modifying and relocating the criterion is to better express our original intent and to recognize that the proposed fourth criterion was not a "functional" criterion in this listing but a medical one. We believe that the result is an improvement over the proposed rule. Individuals with less serious manifestations than several of those we had proposed in Listing 14.08M2 and those who have only one of the manifestations we had proposed in Listing 14.08M3 will now be able to show that they have impairments that meet this listing. Furthermore, even though there is, in effect, no change in the functional severity level of this listing for those people whose impairments would have satisfied one of the criteria in proposed Listing 14.08M4a (activities of daily living), 14.08M4b (social functioning), or 14.08M4c (concentration, persistence, or pace) and the criterion in 14.08M4d—thus satisfying two of the proposed "functional" criteria—we have made the functional criteria more accurate measures of an individual's true functional limitations. No claimant will have to establish that he or she has marked limitations in two of the three true areas of functioning about which so many of the commenters were concerned. In this way, by requiring that a claimant show marked limitations in only one of the three functional areas, the area of social functioning, about which many commenters were concerned, becomes only one way among three available to establish disability at the listing level and can only benefit claimants by providing another area in which to document functional restrictions. However, if social functioning is not markedly limited, a claimant may still show listing-level impairment by demonstrating marked limitations in one of the other areas, activities of daily living or concentration, persistence, or pace.

  3. The Childhood Functional Criteria: Final Listing 114.08O

    We also did not adopt the recommendations of commenters who urged us to eliminate the functional criteria for children in proposed Listings 114.08L and 114.08M. These commenters noted that our regulations, in § 416.926a, already allow for a finding of equivalence when the functional limitation(s) resulting from a child's impairment(s) is the same as the disabling functional consequences of a listed impairment. Therefore, they did not believe that it was necessary to restate this previously established policy within the context of this listing.

    Although we agree that proposed Listings 114.08L and 114.08M were based on a principle similar to functional equivalence, and we agree that most or all children whose impairments meet the criteria of proposed Listing 114.08L or 114.08M would have been found disabled based on the functional equivalence rule, we did not want to take the chance that our rules would be misinterpreted as being more advantageous to adults. In addition, the concept of functional equivalence applies only to childhood SSI claims under title XVI of the Act. Even though SSI claims constitute the great majority of childhood disability applications, it is possible for individuals under age 18 to apply for disability benefits (both as disabled minor children and as workers) under title II. The rules on functional equivalence do not apply in these cases, and such children could be disadvantaged by removal of the rule.

    We did not change the proposed childhood functional criteria the same way we changed the adult criteria. The adult criterion we changed (repeated episodes of decompensation) is not applicable to the evaluation of functioning in children. Further, the childhood functional criteria vary depending on the age of the child. We concluded that the functional criteria in 112.00ff represent the best way to measure broad functional restrictions in children. Consequently, we retained the proposed childhood functional criteria (which cross-refer to Listings 112.02 and 112.12).

  4. CD4 (T4) Count

    Another change in the final listings is the elimination of a specific criterion for CD4 lymphocyte count. Proposed Listings 14.08M1 and 114.08M1 used a CD4 count of less than or equal to 200 cells/mm3 as a measure of the severity of immunodeficiency. A number of public commenters questioned why we used this particular criterion to evaluate impairment severity. Some said that individuals with higher CD4 counts than 200 could be just as functionally limited, and suggested that we use a higher CD4 count. Some commenters said that a CD4 count of 200 should, in and of itself, be sufficient to establish listing-level severity, without the need to show functional restrictions. Others stated that using a CD4 count is not appropriate at all because it is not a good indicator of impairment severity.

    In light of these comments, we reevaluated the listing and realized that, while a low CD4 count (and especially a rapidly declining CD4 count) is an indicator of a compromised immune system and a valuable tool for determining when to institute prophylactic treatment, there is no consistent correlation between a given CD4 count and how or whether an individual is functionally impaired by HIV infection. Individuals with high CD4 counts may be quite severely limited, while others with very low counts may be able to continue normal activities. One individual who commented on our proposed rules related his own story of living with HIV infection, noting that he continued to feel well and to work until his CD4 count was well below 100. He argued that to base our rules on such an unreliable indicator would be to unfairly stigmatize individuals who are able to function well despite low CD4 counts.

    Therefore, we decided not to include a specific CD4 lymphocyte count as a criterion in the listings. For informational purposes, we have also included in final 14.00D3, 14.00D4, and 114.00D4 general statements about the role CD4 counts play in disease susceptibility.

    In final 114.00D3, we also retained guidance that permits a finding of the existence of HIV infection in very young children based on a CD4 count. We did this because these tests are helpful in making the difficult diagnosis of HIV infection in infants. However, based on a commenter's suggestion, which was consistent with other information we received, we extended this provision (which we had proposed to apply to children up to 15 months of age) to cover children up to 24 months of age.

Provisions of the Final HIV Rules

14.00D Human Immunodeficiency Virus (HIV) Infection

Final 14.00D introduces the subject of HIV infection and lists some of the information that is important in documenting and evaluating the disease. The section explains what is acceptable evidence of HIV infection and its manifestations. It provides definitions of some of the terms we use in the listings, including the terms associated with the functional listing, 14.08N.

We extensively revised final 14.00D, both substantively and technically, based on public comments. In place of the 23 paragraphs we had proposed for 14.00D in the NPRM, the final rules are now divided into 8 numbered sections. We have also deleted repetitious language and several paragraphs that are no longer necessary in 14.00D because we have included the impairments they described in Listing 14.08.

14.00D1 HIV Infection.

Final 14.00D1, which describes "HIV infection" and "AIDS," corresponds to the first paragraph of proposed 14.00D. We revised the final language to emphasize that an individual need not have CDC-defined AIDS to have an impairment that meets or is equivalent in severity to, the listed impairments in final Listing 14.08.

14.00D2 Definitions.

Final 14.00D2 is a new section we added in response to comments asking us to define some of the terms in the listing. The final section defines the terms "resistant to treatment," "recurrent," "disseminated," and "significant involuntary weight loss." It states that the first three terms have the same general meaning as used by the medical community, but cautions that the precise meaning of the terms will necessarily vary depending on the specific disease or condition in question, the body system affected, the usual course of the disorder and its treatment, and other relevant circumstances. We then provide definitions of the three terms.

For the fourth term, "significant involuntary weight loss," which is used in Listing 14.08I, we explain that the term does not describe a specific minimum amount or percentage of body weight. We still provide that we always consider an involuntary weight loss of 10 percent of baseline to be significant. However, in response to a comment, we now also provide that loss of less than 10 percent of body weight may be significant, especially in a smaller person. To illustrate the principle, we provide examples of two women, showing when weight loss of less than 10 percent of body weight may and may not be significant.

14.00D3 Documentation of HIV Infection; 14.00D4 Documentation of the Manifestations of HIV Infection.

Final 14.00D3 provides our standards for documenting the existence of HIV infection and final 14.00D4 provides our standards for documenting its manifestations. These sections correspond to the provisions we had proposed in the third through seventh paragraphs of proposed 14.00D. However, in response to many comments, we extensively revised these sections.

We revised final 14.00D3 to explain that, even though the medical evidence must include documentation of the existence of HIV infection (which is required by the statute), documentation may be by laboratory evidence or by other generally acceptable methods consistent with the prevailing state of medical knowledge and clinical practice. We adopted the additional language about generally acceptable methods of diagnosis from comments pointing out that many claimants will not have undergone the kinds of testing we had described. Many commenters noted that clinicians do not always perform laboratory testing for HIV because the existence of HIV infection can be inferred, or presumed, based on the existence of certain opportunistic infections. These commenters pointed out that even the proposed rules recognized this practice. Some commenters also pointed out that in many instances where claimants have been tested for the HIV, the test results will not be available because of privacy concerns.

The section is then divided into two parts: 14.00D3a, which describes how HIV infection may be diagnosed definitively, and 14.00D3b, which describes how HIV infection may be diagnosed presumptively—that is, be acceptably documented without the definitive laboratory evidence described in 14.00D3a. In response to comments with which we agreed, we clearly state in final 14.00D3a that when laboratory testing for HIV infection has been performed, every reasonable effort must be made to obtain reports of the results of that testing. We also clarified the language to explain why the results of a positive ELISA screening test are ordinarily verified by a more definitive test for HIV antibodies. In final 14.00D3a(ii), we combined the tests specifically for HIV antigen into one category, and included the laboratory tests named in the proposed listing as examples. We also added cerebrospinal fluid specimens to this category of clinical tests to make the adult rules consistent with the childhood rules; even though such testing is rare in adults, it is not unheard of. In response to a comment, we expanded final 14.00D3a(iii), which was formerly the fourth example in the fourth paragraph of proposed 14.00D, to include other tests that are acceptable methods of detecting HIV and consistent with the prevailing state of medical knowledge.

The third paragraph of final 14.00D3a has been adapted from the second sentence of the third paragraph of proposed 14.00D. In response to comments, and for reasons we have already explained above in the summary of revisions to Listing 14.08N, we clarify that, even though the level or rate of decline of CD4 count correlates with the extent of immune depression, a reduced CD4 count alone does not definitively diagnose the presence of HIV infection or provide information about the severity or functional effects of HIV infection; additional documentation will always be necessary.

Final 14.00D3b describes when documentation of HIV infection is possible without definitive laboratory evidence. It states that HIV infection may be documented by medical history, clinical and laboratory findings, and diagnoses shown in the medical evidence, provided that the documentation is consistent with the prevailing state of medical knowledge and clinical practice, and is consistent with the other evidence. As an example, it states that HIV infection will be documented if the individual has an opportunistic disease predictive of a defect in cell-mediated immunity, and there is no other known cause of diminished resistance to that disease. This is a provision we moved from proposed Listing 14.08A as part of our simplification of the listing.

Final 14.00D4 explains the documentation requirements for opportunistic diseases and other manifestations of HIV infection. It is structured in the same way as final 14.00D3, with a section (final 14.00D4a) describing definitive methods of diagnosis, and a section (final 14.00D4b) describing other acceptable methods of diagnosis. It notes that every reasonable effort should be made to obtain whatever specific laboratory evidence is available. If only hospitalization summaries or treating physician reports are available, this evidence should include details of the clinical findings and the results of the diagnostic or microscopic studies.

As in final 14.00D3, we have added guidance that documentation of manifestations of HIV infection may be by laboratory evidence or documentation which is consistent with the prevailing state of medical knowledge and clinical practice, and consistent with the other evidence. We have also included in final 14.00D4a a discussion of the relevance of CD4 counts, which cross-refers to the discussion in 14.00D3a.

Final 14.00D4b discusses other acceptable documentation of opportunistic diseases and other manifestations of HIV infection. In response to comments, with which we agree, we have clarified the explanation of how opportunistic diseases and manifestations of HIV infection may be documented by medical history, clinical and laboratory findings, and diagnoses indicated in the medical evidence. Though a diagnosis of opportunistic disease or HIV manifestation may not be supported by a definitive test, the diagnosis is acceptable documentation provided that it is consistent with the prevailing state of medical knowledge and clinical practice and is consistent with the other evidence. As a point of clarification, we have also added a discussion about cytomegalovirus (CMV) disease, which presents special documentation issues. Because the CMV is an organism that is present in many individuals who are not ill, a positive serology in itself does not confirm that a person has CMV disease. Therefore, in this circumstance, we require confirmation by biopsy or other generally acceptable methods consistent with the prevailing state of medical knowledge and clinical practice. One such method, which we single out in the new paragraph, is diagnosis by an ophthalmologist of chorioretinitis caused by CMV.

14.00D5 Manifestations Specific to Women.

The two paragraphs of final 14.00D5 replace the tenth through twelfth paragraphs of proposed 14.00D and discuss the evaluation of HIV infection in women. We shortened the discussion of manifestations specific to women contained in proposed 14.00D because we have incorporated the specific diseases mentioned in the proposed prefatory language directly into final Listings 14.08A5 (pelvic inflammatory disease), 14.08D2 (genital herpes), and 14.08F (vulvovaginal candidiasis) as stand-alone medical listings.

In final 14.00D5, we have retained the basic guidance from the NPRM for evaluating HIV infection. Both paragraphs of final 14.00D5 continue to alert adjudicators to give careful consideration and scrutiny to the medical evidence when evaluating HIV infection and its manifestations in women.

The first paragraph of final 14.00D5 corresponds to the tenth paragraph of proposed 14.00D. We have revised it slightly following the publication of the NPRM because most women with severe immunosuppression do, in fact, exhibit the same kinds of manifestations that men do, and the HIV infection need not necessarily be in the end stages for this to happen. However, in addition to these manifestations, HIV infection does have effects in some women that are different from those in men with the disease, sometimes by increasing the frequency and resistance to treatment of conditions, including gynecologic conditions, that occur in women who do not have HIV infection. We have, therefore, revised the last two sentences of the paragraph to say that HIV infection may have different manifestations in women than in men, and that adjudicators must carefully scrutinize the medical evidence and be alert to the variety of medical conditions that are both specific to women and common in the female population, but may be more severe because of the HIV infection.

The second paragraph of final 14.00D5 includes material that was in the remaining two paragraphs of the NPRM and explains the foregoing principles in more detail. Because we have incorporated the most important conditions specific to women directly into the listing, we now no longer state that gynecologic conditions may result only in equivalence determinations under the listings. Instead, we provide that manifestations of HIV infection in women may be evaluated under the specific listing criteria (such as Listing 14.08E, which explicitly lists cervical cancer), under an applicable general listing category (such as final Listing 14.08A5), or under final Listing 14.08N (which considers the specific impact of an impairment(s) that does not otherwise meet a listing on the individual's ability to function). We believe that final Listing 14.08N will be especially useful in cases of women who do not suffer from a continuous, listing-level manifestation of HIV

14.00D6 Evaluation

Final 14.00D6 gathers under one heading the three paragraphs of the NPRM that addressed issues of evaluation: the second, thirteenth, and last paragraphs of proposed 14.00D.

We consolidated the repetitive language in these paragraphs but retained the discussion of the need to evaluate the impact of all impairments in individuals with HIV infection. We changed the second sentence of the first paragraph of final 14.00D6 (the second paragraph of proposed 14.00D) to emphasize that equivalence to other listings must be considered in evaluating an individual's HIV disease or condition. We also revised the subsequent discussion, which was adapted from the thirteenth paragraph of proposed 14.00D, but which was confined to mental manifestations in the proposed rules. The final rule refers to both mental and physical impairments and removes any implication that we did not consider that mental signs and symptoms could be manifestations of HIV infection.

We also explain that some individuals with HIV infection may have impairments that are less than listing-level severity, but still may be disabling. Evaluation of these cases should proceed through the final steps of the sequential evaluation process.

14.00D7 Effect of Treatment.

Final 14.00D7 is an expanded version of the fourteenth paragraph of proposed 14.00D. It discusses the need to evaluate the impact of treatment in individuals with HIV infection. In response to public comments, with which we agreed, we clarified the first and second sentences of the proposed paragraph by specifically referring to both the potential benefits and the potential adverse effects of treatment. We expanded the explanation dealing with individual responses to treatment to further emphasize the importance of evaluating adverse or beneficial consequences of treatment on a case-by-case basis. We also added language that explains why it is important to know that the effects of treatment may be temporary or long-term as a reminder that any decision regarding the impact of treatment should be based on a sufficient period of treatment for an accurate and realistic assessment.

14.00D8 Functional Criteria.

Final 14.00D8 discusses the functional criteria contained in Listing 14.08N. We extensively modified the proposed language in response to comments and to conform with the changes we made in the functional criteria of final Listing 14.08N, already described above.

The first paragraph of final 14.00D8, together with the third paragraph, replaces the twenty-second paragraph of proposed 14.00D, which had described the fourth area of functioning, repeated episodes of deterioration or decompensation in work or work-like settings. This paragraph now explains that the provisions of final Listing 14.08N apply both to manifestations that are listed in Listings 14.08A through M but that do not meet those listings, and to unlisted manifestations. In this way, instead of using a finite list of manifestations as we had proposed, the provision now applies to any type of manifestation.

The second paragraph stresses important considerations in the evaluation of HIV infection. It requires an assessment of the full impact of signs, symptoms, and laboratory findings on an individual's ability to function, and mentions the following specific factors: Symptoms, such as fatigue and pain; characteristics of the illness, such as the frequency and duration of manifestations, or periods of exacerbation and remission; and the functional impact of treatment, including the side effects of medication.

The third paragraph of final 14.00D8 (as well as the first paragraph) replaces the twenty-second paragraph of the NPRM. In the third paragraph, we provide the definition of the term "repeated" as we use it in Listing 14.08, which we have already explained above. Our intent is to provide as much flexibility as possible to include "repeated" manifestations, provided that the episodes are of sufficient frequency or duration as to be at the listing level.

The fourth through eighth paragraphs of final 14.00D8 replace the seventeenth through twenty-first paragraphs of 14.00D in the NPRM. Inasmuch as we require an individual to satisfy only one of the three functional criteria now in Listing 14.08N, we have revised the fourth paragraph accordingly. We have added language that reminds adjudicators that the functional restrictions may result from the impact of the manifestation on mental or physical functioning or both. We have also moved into this paragraph the language in paragraphs 18 through 21 of the NPRM about the importance of considering symptoms (such as depression, fatigue, or pain) and the side effects of medication when assessing functioning.

In response to comments about the seventeenth paragraph of the NPRM, now the fifth paragraph of final 14.00D8, we revised the general guidance definition of the term "marked." The revisions now state that a marked limitation does not represent a quantitative measure of the individual's ability to do an activity for a certain percentage of the time. We also state plainly, in response to many comments, that an individual with a marked limitation is not totally precluded from performing an activity and that the term "marked" does not imply that the individual is confined to bed, hospitalized, or in a nursing home. This has always been our intent in the rules; our reason for including the statement that "marked * * * means more than moderate, but less than extreme" is to illustrate that there is a level of limitation higher than the "marked" level, a situation that would not be possible if "marked" meant complete limitation.

In the sixth, seventh, and eighth paragraphs of final 14.00D8, we revised the descriptions of the three general areas of functioning to make them more specific to people who have HIV infection and to respond to concerns in the public comments. For instance, in the seventh paragraph, we now explain that an individual may be able to communicate with close friends and relatives yet still have a marked limitation of the ability "to engage in social interaction on a sustained basis." This, too, has always been our intent. The ability to communicate effectively with close family and friends is not necessarily indicative of an individual's ability to maintain social contact independently or in a work setting.

Even though all of the foregoing information is basic to the use of the rules we proposed in the NPRM, and would have been understood by our adjudicators, we have included it in the preface to the final listing to make our policy clearer in the regulations.

Finally, we deleted from the preface the material that was in the seventh, eighth, and ninth paragraphs of proposed 14.00D. The seventh paragraph described documentation requirements for Pneumocystis carinii pneumonia, and has been superseded by the new discussions on documentation of the manifestations of HIV infection at the listing-level. We incorporated the provisions of proposed paragraphs eight and nine directly into their respective listings (14.08H1 for HIV encephalopathy, and 14.08I for HIV wasting syndrome).

14.08 Human Immunodeficiency Virus (HIV) Infection

This new listing adds to the regulations our criteria for evaluating HIV infection at the listing level. The listing includes a range of opportunistic diseases, cancers, and other manifestations that are indicative of listing-level severity in an individual with HIV infection. Specific manifestations that are considered indicative of listing-level HIV infection are in final Listings 14.08A-M, grouped by type (e.g., fungal infections, bacterial infections, malignant neoplasms) for ease of reference. Final Listing 14.08N includes any manifestations of HIV infection that cause listing-level functional limitations.

Final Listing 14.08 is significantly different from the proposed listing. We reorganized and changed the proposed listing in the following ways:

To improve the clarity of the final listing criteria, we deleted specific documentation requirements from each HIV manifestation listed in 14.08 and retained only one general cross-reference to the comprehensive discussion of documentation requirements in final 14.00D3 (for documentation of the existence of HIV infection) and 14.00D4 (for documentation of the manifestations of HIV infection). Because we have included a discussion of opportunistic disease predictive of a defect in cell-mediated immunity that document HIV infection in final 14.00D3, we have deleted proposed 14.08A.

14.08A Bacterial Infections

Final Listing 14.08A, Bacterial infections, includes the proposed listings that described bacterial infections. Thus, final Listing 14.08A1, Mycobacterial infection, includes proposed Listings 14.08A7, 14.08D, and 14.08M2b; final Listings 14.08A2, Nocardiosis, and 14.08A3, Salmonella bacteremia, were in proposed Listing 14.08F. We combined the proposed listings for mycobacterial infections, which are a kind of bacterial infection, because we agreed with those commenters who pointed out that the proposed listings (except for 14.08M2b, Pulmonary tuberculosis) resulted in a finding of "meets" regardless of the kind of the mycobacterial infection. (We made a technical correction to the name of one of the three bacteria we listed as examples of causes of mycobacterial infections, M. avium-intracellulare.) We added pulmonary tuberculosis, resistant to treatment, in response to comments that asked us to create a stand-alone medical listing for this condition.

For the same reason, we added syphilis or neurosyphilis to final Listing 14.08A4, and required that the sequelae be evaluated under the criteria for the affected body system. We also added listing criteria for multiple or recurrent bacterial infections, such as pelvic inflammatory disease, in final Listing 14.08A5. The final listing includes criteria for hospitalization or intravenous antibiotic treatment as a measure of severity.

14.08B Fungal Infections

Final Listing 14.08B includes material originally proposed in Listings 14.08A and 14.08C. Final Listing 14.08B2, Candidiasis (at a site other than the skin, urinary tract, intestinal tract, or oral or vulvovaginal membranes; or involving the esophagus, trachea, bronchi, or lungs) combines the criteria of proposed Listings 14.08A1 and 14.08C1. Final Listings 14.08B3, Coccidioidomycosis, and 14.08B5, Histoplasmosis, were both in proposed Listing 14.08C. Final Listing 14.08B4, Cryptococcosis, was in proposed Listings 14.08A2 and 14.08C2; we have also added a reference to cryptococcal meningitis in partial response to the public comments asking us to list the criteria in proposed Listing 14.08M2 as stand-alone listings. (Other forms of meningitis are listed in final Listing 14.08M.) In response to comments, we added aspergillosis to 14.08B1 and mucormycosis to 14.08B6; neither of these manifestations was in the proposed listing.

14.08C Protozoan or helminthic infections

Final Listing 14.08C1 includes manifestations originally proposed as Listings 14.08A3 and 14.08B1 (both for cryptosporidiosis) and 14.08B4 (isosporiasis). In response to comments, we also added microsporidiosis to final Listing 14.08C1. Final Listing 14.08C2, for Pneumocystis carinii pneumonia, includes the manifestations that were in proposed Listings 14.08A8 and 14.08B2. In response to comments, we added extrapulmonary pneumocystis infection to final 14.08C2. Thus, the listing includes all infections with the Pneumocystis carinii organism. We listed both the pneumonia and the extrapulmonary infections (instead of a single description of Pneumocystis carinii infection) because pneumonia is such a common manifestation of HIV infection.

Final Listing 14.08C3, Strongyloidiasis, extra-intestinal, was proposed Listing 14.08B5. Final Listing 14.08C4, Toxoplasmosis, combines proposed Listings 14.08A10 and 14.08B3 into one listing.

14.08D Viral Infections

Final Listing 14.08D1, Cytomegalovirus disease, combines proposed Listings 14.08A4 and 14.08E1. The final criteria for cytomegalovirus disease include a cross-reference to the new discussion of documentation of the disease in 14.00D4, already described.

Final Listing 14.08D2, Herpes simplex, combines proposed Listings 14.08A5 and 14.08E2. We revised the language of those rules slightly for clarity, and divided the listing into three separate criteria. We added to final Listing 14.08D2a, mucocutaneous infection, examples of such infections that may result from Herpes simplex virus.

Final Listing 14.08D3 is a stand-alone medical criterion for Herpes zoster, formerly in proposed Listing 14.08M3h as an impairment that required limited functioning. The listing is met with either disseminated infection or with multidermatomal eruptions that are resistant to treatment. Final Listing 14.08D4, Progressive multifocal leukoencephalopathy, was in proposed Listings 14.08A9 and 14.08E3. We also added a new Listing 14.08D5 for viral hepatitis in response to the public comments.

14.08E Malignant Neoplasms

Final Listing 14.08E consolidates the two proposed listings for neoplastic diseases, Listings 14.08I and 14.08J, and proposed Listing 14.08A6 (which was for primary lymphoma of the brain in individuals less than 60 years old). In the final rules, we use the term "malignant neoplasms" instead of the term "neoplasms" (which was in the proposed rules) to more accurately reflect the nature of these disorders. In response to public comments pointing out that we considered all lymphomas associated with HIV infection to be disabling, we combined all types of lymphomas into one listing, 14.08E3. We now mention primary lymphoma of the brain, Burkitt's lymphoma, immunoblastic sarcoma, other non-Hodgkin's lymphoma, and Hodgkin's disease only as examples. In response to numerous comments, we also added as Listing 14.08E2 stand-alone medical criteria for Kaposi's sarcoma, which was in the proposed functional listing, 14.08M2. Final Listing 14.08E2 recognizes that there is a range of severity to Kaposi's sarcoma and, therefore, provides specific criteria for extensive oral lesions, or involvement of the gastrointestinal tract, lungs, or other viscera, or involvement of the skin or mucous membranes as described in final Listing 14.08F, discussed below.

Final Listing 14.08E1, Carcinoma of the cervix, was proposed Listing 14.08J2. Final Listing 14.08E4, Squamous cell carcinoma of the anus, was proposed Listing 14.08J3.

14.08F Conditions of the Skin or Mucous Membranes

In response to numerous comments, we added a new Listing 14.08F to the final rules. The listing includes criteria for conditions of the skin or mucous membranes, including mucosal candida, such as vulvovaginal candidiasis (which we had proposed with functional criteria in Listing 14.08M3f) and persistent dermatological conditions, such as eczema or psoriasis (proposed with functional criteria in Listing 14.08M3i). We added to this listing examples, including condyloma caused by human papillomavirus and genital ulcerative disease. Because these conditions may range in severity, we have provided criteria for extensive fungating or ulcerating lesions not responsive to treatment. We also include a cross-reference to the skin listings in 8.00ff, in the event they might apply.

14.08G Hematologic Abnormalities

In response to comments, we have added a new final Listing 14.08G with stand-alone medical criteria for the HIV-related hematological abnormalities we had proposed to link with functional criteria (anemia, granulocytopenia, and thrombocytopenia) in proposed Listings 14.08M3a-c. The listing consists of cross-references to existing hematological listings as a measure of severity.

14.08H Neurological Abnormalities

In response to public comments, we have expanded the proposed listing for HIV encephalopathy (proposed Listing 14.08G) into a general listing category for neurological manifestations of HIV infection, final Listing 14.08H. Final Listing 14.08H1 combines the proposed listing for HIV encephalopathy (proposed Listing 14.08G) with its definition in the eighth paragraph of proposed 14.00D. We revised the description from proposed 14.00D to remove superfluous language. We changed the phrase "cognitive and/or motor dysfunction" to "cognitive or motor dysfunction" because either of these findings is sufficient to find that the listing is met; therefore, "and/or" was unnecessary. We also deleted the requirement that the dysfunction progress "over weeks and months in the absence of a concurrent illness." The phrase "over weeks and months" was unclear: If there had already been months of progression, it is self-evident that weeks would have also passed. Moreover, "weeks and months" is an imprecise standard. Similarly, "in the absence of a concurrent illness" is unnecessary because it speaks to the issue of documentation of the existence of the manifestation called HIV encephalopathy.

Final Listing 14.08H2 adds listing criteria for other neurological manifestations of HIV infection, including peripheral neuropathy, which was in proposed Listing 14.08M2. The impairments in final Listing 14.08H2 must be evaluated under the criteria for the neurological listings in 11.00ff.

14.08I HIV Wasting Syndrome

Final Listing 14.08I combines the proposed listing for HIV wasting syndrome (proposed Listing 14.08H) with its definition in the ninth paragraph of proposed 14.00D. We clarified the proposed language to more accurately reflect the criteria for wasting syndrome. We also corrected a typographical error in the proposed rule: We intended to require chronic diarrhea for 1 month, not 2 months as stated in proposed 14.00D.

14.08J Diarrhea

Final Listing 14.08J is new, and includes stand-alone medical criteria for evaluating listing-level chronic diarrhea, which we had proposed with functional criteria in Listing 1408M3j.

14.08K Cardiomyopathy

The final listing for cardiomyopathy in 14.08K now includes a cross-reference to the criteria in 11.04 of the neurological listings. This addition makes the criteria for HIV-related cardiomyopathy consistent with the criteria for cardiomyopathy in the listing of impairments for the cardiovascular system.

14.08L Nephropathy

We modified the proposed listing for nephropathy, proposed 14.08L, to cross-refer to the entire genitourinary system listings section, rather than to specific listings in keeping with similar revisions throughout these final rules.

14.08M

In 14.08M of the final listing, we combined the proposed listings criteria for pneumonia in 14.08M2a, bacterial or fungal sepsis in 14.08M2C, meningitis in 14.08M2d, septic arthritis in 14.08M2e, endocarditis in 14.08M2f, and radiographically documented sinusitis in 14.08M3k into a general group of HIV-related manifestations that are resistant to treatment and that alone meet the listing without consideration of functional criteria. In some cases, specific variants of these conditions are described in other listings (e.g., bacterial sepsis under final Listing 14.08A, cryptococcal meningitis under final Listing 14.08B4). Therefore, we specify that final Listing 14.08M applies only to these infections if they are not listed in 14.08A-14.08L.

14.08N Repeated Manifestations of HIV Infection

Final 14.08N contains criteria (from proposed Listing 14.08M) for evaluation of manifestations of HIV infection based on functional consequences. We have extensively revised the proposed listing, as discussed above.

114.00D Human Immunodeficiency Virus (HIV) Infection

As we have already explained, we added a new 114.00C, Allergies, growth impairments, and Kawasaki disease, to correspond to 14.00C of the adult rules. This required us to redesignate proposed 114.00C to final 114.00D, which also makes all of the final designations in the preface to the childhood rules parallel the adult rules. We also revised most of final 114.00D of the childhood rules in the same way as the adult rules. In place of the 17 paragraphs we had proposed in 114.00C, final 114.00D is now divided into 8 sections with the same headings and organization as in 14.00D, except that we have added references to children in the headings. Of course, we also revised the text as necessary to refer to children. Thus, final 114.00D1, HIV infection, is the same as final 14.00D1, except that we refer to children instead of adults.

Final 114.00D2, Definitions, is the same as final 14.00D2, except that it does not include a paragraph corresponding to the last paragraph of final 14.00D2. The last paragraph in final 14.00D2 defines the term "significant involuntary weight loss" as it is used in final Listing 14.08I. We did not include a similar explanation in the childhood rules because final Listing 114.08I, Growth disturbance, contains three separate criteria for assessing weight loss in children and is, therefore, more precise than the adult rule.

In response to comments, we extensively revised the discussions in final 114.00D3, Documentation of HIV infection in children, and 114.00D4, Documentation of the manifestations of HIV infection in children. Except as noted below, these sections parallel final 14.00D3 and D4.

In final 114.00D, we have revised, clarified, and expanded the guidance in the fourth and fifth paragraphs of proposed 114.00C for establishing the existence of HIV infection in children. For reasons we explain in the public comments section of this preamble, we changed the proposed rules that referred to children up to the age of 15 months to apply to children up to the age of 24 months.

Final 114.00D3a(i) corresponds to the first category in the fourth paragraph of proposed 114.00C. In addition to revising the paragraph in the same way as the corresponding adult rule, we added a new first sentence to clarify that HIV infection is not documented in children under 24 months of age by antibody testing. Inasmuch as any kind of specimen (such as serum, lymphocyte culture, or cerebrospinal fluid) that contains HIV antigen definitively diagnoses HIV infection, we deleted the repetitive references to HIV antigens from the proposed rules (the second and third criteria in the fourth paragraph of proposed 114.00C and the first and second criteria in the fifth paragraph) and provide only one all-inclusive criterion in final 114.00D3a(ii). In final 114.00D3a(iii) we added the immunoglobulin A (IgA) serological assay specific for HIV as another test that documents HIV infection in children. Although this test is not widely available, it is highly accurate for diagnosis of HIV infection.

Final 114.00D3b describes when documentation of HIV infection is possible without definitive laboratory evidence. We have expanded the explanation of why infants may have serum antibodies for HIV but not have HIV infection, formerly in the fifth paragraph of proposed 114.00C, and have extended the age limit from 15 months to 24 months in response to comments and information we received. We also include criteria for situations in which the presence of HIV infection may be presumed in such infants when there are HIV antibodies and other signs of the infection, such as a significantly depressed CD4 count, even though these findings would not definitively diagnose the presence of the disease. In response to comments, we added to these criteria abnormal immunoglobulin G (IgG) and abnormal CD4/CD8 ratio. As in the adult rules, we provide that the presence of HIV infection in children may also be established by medical history, clinical and laboratory findings, and diagnoses consistent with the prevailing state of medical knowledge and clinical practice, as, for example, when the child has an opportunistic disease predictive of a defect in cell-mediated immunity and there is no other known cause of diminished resistance to that disease.

Final 114.00D4 explains the documentation requirements for opportunistic diseases and other manifestations of HIV infection in children. Final 114.00D4a describes the methods of documenting manifestations of HIV infection by definitive diagnosis. It is identical to final 14.00D4a of the adult rules except that we have added a reference to children.

Final 114.00D4b discusses other acceptable documentation of opportunistic diseases and HIV manifestations. It is identical to final 14.00D4b of the adult rules except that we have added a reference to children.

Final 114.00D5 replaces the discussions in the ninth, tenth, and eleventh paragraphs of proposed 114.00C. For reasons we explain in the public comments section of this preamble, we deleted the proposed text on the epidemiology of HIV infection in children, the text discussing the mean age of diagnosis in infants, and the provisions on the course and spectrum of the disease in children age 13 or older. We also deleted the sentence from the ninth paragraph of proposed 114.00C that cross-referred to the adult listing for HIV wasting syndrome; instead, we have provided explicit listing criteria for the evaluation of weight loss in children in final Listing 114.08I.

In final 114.00D5, we continue to acknowledge that HIV infection can manifest itself differently in children than in adults, and have expanded the provisions describing these differences in response to comments. We moved the proposed guidance on HIV encephalopathy and neurologic problems into a separate paragraph because neurological impairments may be more subtle and difficult to detect in children than in adults. We also added two new paragraphs discussing the evaluation of bacterial infections; as part of this guidance, we point out that older female children may have pelvic inflammatory disease, just as women do.

Final 114.00D6, Evaluation of HIV infection in children, replaces the second, twelfth, and sixteenth paragraphs of 114.00C of the NPRM. The first and second paragraphs of the section are identical to the first and second paragraphs of final 14.00D6, except that we have used the word "child" as appropriate. The third paragraph contains the same information as the third paragraph in final 14.00D6, except that it refers to the sequential evaluation process for children in § 416.924 of part 416. As in the adult rules, final 114.00D6 includes a discussion of the need to evaluate the impact of all impairments in children with HIV infection and explains that some children with HIV infection may have severe impairments that are less than listing-level severity, but that may still be disabling. Evaluation of these cases should proceed through the final step of the sequential evaluation process, where an individualized functional assessment is performed.

Final 114.00D7, Effect of treatment, is an expanded version of the thirteenth paragraph of proposed 114.00C. As in final 14.00D7 of the adult rules, it discusses the need to evaluate the impact of treatment in children with HIV infection and refers to both the potential benefits and the potential adverse effects of treatment on a case-by-case basis. The first and third paragraphs of final 114.00D7 are identical to the corresponding paragraphs in final 14.00D7 except that we have used the word "child" as appropriate. The second paragraph is nearly identical to the second paragraph of final 14.00D7 except that we use an example of a childhood infection, otitis media, and the word "child" as appropriate.

Final 114.00D8, Functional criteria, discusses the functional criteria contained in final Listing 114.08O. We modified the proposed language in order to conform with the changes we made in the functional criteria (see "Explanation of the Final Rule," above).

114.08 Human Immunodeficiency Virus (HIV) Infection

This new listing adds to the regulations our criteria for evaluating HIV infection in children at the listing level. The listing includes a range of opportunistic diseases, cancers, and other manifestations that are indicative of listing-level severity in children with HIV infection. A separate listing is necessary for children because children with HIV infection may differ from adults in the mode of infection, clinical manifestation, and course of the disease.

Specific manifestations that are considered indicative of listing-level HIV infection are in final Listings 114.08A-N, grouped by type (e.g., fungal infections, bacterial infections, malignant neoplasms) for ease of reference. Final Listing 114.08O includes any manifestation(s) of HIV infection that causes listing-level functional limitations.

We reorganized the basic structure and presentation of final Listing 114.08 in the same way as the adult rules. Therefore, the final listing is significantly different from the proposed listing. Important differences between the final listing and the NPRM and between the childhood and adult listings follow.

For completeness, we added to the final childhood listing a number of criteria that we had proposed only in the adult listing. Adding these criteria did not change the evaluation of HIV infection in children because our regulations in §§ 404.1525 and 416.925 call for using the adult criteria for children whenever the childhood criteria do not apply. We added these new criteria only to make the childhood listing easier to use.

Final Listing 114.08A addresses bacterial infections. The listing includes the same criteria as in final Listing 14.08A. In addition, we have retained, in final Listing 114.08A5, the criteria we proposed in Listing 114.08F1 for children less than 13 years old who experience certain pyogenic bacterial infections at least twice in 2 years. Although we have deleted from the remainder of the childhood listings all of the previously proposed distinctions between children under age 13 and children age 13 and above, we retained this distinction only in final Listing 114.08A5, where it is medically valid. However, consistent with the adult listings, we also added criteria at final Listing 114.08A6 that apply to multiple or recurrent bacterial infections caused by any bacteria—including pelvic inflammatory disease—and that can be applied to all children.

We revised final Listings 114.08B, Fungal infections, 114.08C, Protozoan or helminthic infections, 114.08D, Viral infections, 114.08E, Malignant neoplasms, 114.08F, Conditions of the skin and mucous membranes, and 114.08G, Hematologic abnormalities, in the same way as the adult rules. The language of these provisions is the same except that we provide cross-references to the appropriate childhood listings where necessary. As in the adult listings, the revisions to these childhood rules also provide stand-alone medical criteria for several of the manifestations we had proposed to tie to a test of functional limitations in proposed Listings 114.08L and 114.08M. We added criteria for carcinoma of the cervix and squamous cell carcinoma of the anus (as in the adult listing) to final Listing 114.08E because these conditions may occur in adolescents.

We have extensively revised final Listing 114.08H (proposed Listing 114.08J), Neurological manifestations, based on public comment. A child with HIV infection may now have a neurological manifestation (for example, HIV encephalopathy or peripheral neuropathy) that meets the listing in any of four ways. In response to a comment pointing out that the criteria in proposed Listing 114.08J3 essentially described impairments that meet the criteria in 111.00 of the listings, we revised final Listing 114.08H3 to provide for only progressive motor dysfunction affecting gait and station or fine and gross motor skills. We also changed the criteria for evaluating motor deficits to eliminate the requirement that they be symmetric. However, inasmuch as some children will have neurologic manifestations that meet the criteria of one of the listings in 111.00, we added to the opening paragraph a criterion that permits a finding of disability by cross-reference to those listings. In final Listing 114.08H1, we also revised the criteria for evaluating loss of previously acquired intellectual ability (which were in proposed Listing 114.08J1) to reflect our intent to include those situations where the child does not lose previous knowledge, but is unable to learn new information; that is, suddenly acquires a new learning disability. We also added a cross-reference in final Listing 114.08H2 to the new discussion in 114.00D5 describing documentation of impaired brain growth.

Final Listing 114.08I addresses growth disturbances. These criteria were previously in proposed Listing 114.08K. Based on numerous public comments, we added weight criteria for evaluating failure to thrive, which are based on a fall from an established growth curve. These criteria recognize that, unlike adults (who have stopped growing), children can be gaining weight yet still be failing to thrive because their weight gain is not commensurate with their growth. Final Listing 114.08I1 describes children who, because of weight loss or failure to gain weight at an appropriate rate for age, have a persistent fall (defined as 2 months or longer) of 15 percentiles from an established growth curve on standard growth charts, irrespective of the actual percentile at which their weight lies. Conversely, final Listing 114.08I2 describes children whose weight, because of an involuntary weight loss or failure to gain weight at an appropriate rate, falls and persists below the third percentile from an established growth curve on standard growth charts, irrespective of the number of percentiles of the fall. A new third criterion, final Listing 114.08I3, provides for an involuntary weight loss greater than 10 percent of baseline that persists for at least 2 months. In final Listing 114.08I4, which incorporates the proposed listing's cross-reference to the growth impairment listings, we changed the cross-reference to the entire section, 100.00, for consistency with the changes we made to other listings.

Final Listing 114.08J, Diarrhea, is the same as the corresponding final adult listing, 14.08J. This condition was previously in proposed Listings 114.08L and 114.08M only in conjunction with the functional requirements. Final Listing 114.08K, Cardiomyopathy, is also the same as final Listing 14.08K, except for the cross-reference to the listings in 104.00; the cross-reference to adult neurological Listing 11.04, however, is correct and consistent with our cardiovascular rules for children. This condition was proposed only in the adult listings. We decided to add it to the childhood listings because it also occurs in children.

Final Listing 114.08L addresses lymphoid interstitial pneumonia/pulmonary lymphoid hyperplasia (LIP/PLH complex). This criterion was previously in proposed Listing 114.08G. We changed the criteria to apply to all children rather than just applying it to children under age 13, as we proposed. We also added criteria for listing-level severity, because these conditions may range widely in their severity and impact on a child's functioning.

Final Listing 114.08M, Nephropathy, is the same as final Listing 14.08L except that the cross-references are to the criteria in 106.00 of the childhood listings. As with cardiomyopathy, we had proposed to include this condition only in the adult listings, but have added it to the childhood listings because it also occurs in children.

Final Listing 114.08N is identical in substance to final Listing 14.08M. It includes a number of infections that we had proposed to include only with functional limitations: sepsis, meningitis, pneumonia, septic arthritis, endocarditis, and radiographically documented sinusitis.

Final Listing 114.08O addresses any other manifestation(s) of HIV infection that either does not meet the criteria in any of the other childhood HIV listings or is not contained in those listings. We have already described the criteria of the final listing above.

Other Changes

It was apparent to us from some of the comments that it would be helpful and clearer if we used the same or similar language in parts A and B of the listings where we intended the provisions to be analogous. Therefore, we made a number of revisions in parts A and B to make their language consistent. In the majority of cases, these changes were editorial, not substantive. We also made minor editorial changes throughout the rules to correct errors in the NPRM, maintain internal consistency, and conform the style of these listings to our other listings.

As already explained, these final listings move the listings for systemic lupus erythematosus and systemic sclerosis and scleroderma from Listing 10.04 and 10.05 to Listings 14.02 and 14.04. Because of this, we are changing the references in the second sentence of 8.00B of the skin impairment listings to reflect the new designations.

We made several other changes in response to the public comments in addition to those described above. We describe these changes, and explain why we made them, in the following summary of the public comments.

Public Comments

We carefully considered all of the comments and adopted many of the recommendations. The resulting changes are identified in the following discussion of issues that were raised in the comments.

Many of the comments were quite long and detailed. Of necessity, therefore, we had to condense, summarize, or paraphrase them. However, we tried to express everyone's views adequately and respond to all of the relevant issues raised. There were a few comments that we did not address below. This is because they only pointed out minor typographical errors, or were about information in the preamble to the prior rules or administrative matters that are not appropriate to the final rules.

For ease of reference, we organized the comments and responses as follows. We first address general comments, i.e., comments that are either about the rules as a whole or that apply to more than one section of the rules. We then address the remaining comments, which pertain to specific sections of the rules. If we changed the section numbers or headings in the final rules, we provide both the NPRM and final references in the text of the comment and response.

B. 9.00 Endocrine System and Obesity

Hansen's Disease

Comment: A few commenters thought that there are still sufficient cases of Hansen's disease to warrant its retention in the listings.

Response: We did not adopt the comment. The incidence (new cases per year) of Hansen's disease (leprosy) in the United States is very low, less than 135 in the past few years. Moreover, we do not need a separate listing because the dermatological and neurological manifestations of this disorder are addressed in the sections pertaining to those body systems.

9.00 and 9.09 Obesity

Comment: One comment we received said that we should not move the obesity listing to the endocrine system section, but that it should stay with the impairments in the multiple body system section.

Response: We did not adopt the comment. With the exception of obesity and Hansen's disease (which we have deleted), we have moved all the impairments from the adult multiple body systems section to the newly established "Immune System." Rather than keep obesity as the sole impairment in a body system, we believe that the most appropriate location for it would be the endocrine system, which is now titled: "Endocrine System and Obesity."

Comment: A comment asked whether the reference to the "spine" in proposed Listing 9.09A was meant to include the cervical and thoracic regions of the spine. Another comment said that the listing for obesity should state that the history of pain, limitation of motion, and arthritis caused by obesity in any weight-bearing joint or spine need only be minimal to satisfy the listing's requirements.

Response: We agreed with the first comment that the references to the "spine" in former Listing 10.10A and proposed Listing 9.09A could be made clearer. The listing has always applied only to the weight-bearing parts of the spine (i.e., the lumbosacral regions). Therefore we have clarified the language in final Listing 9.09. This is not a substantive change from the prior rules or the NPRM, but a clarification of our intent that the phrase "weight-bearing" modify both "joint" and "spine" in the listing.

We did not adopt the second comment because it is implicit in the language of the rule. The fact that the degrees of pain,limitation of motion, and arthritis in the weight-bearing structures are not quantified indicates the intentional absence of threshold criteria.

Comment: A comment stated that the adult obesity listing is inadequate for assessing obesity in children. The comment also suggested that we consult with pediatricians to develop a childhood obesity listing by which to assess whether a child is functioning independently, appropriately, and effectively in an age-appropriate manner.

Response: We did not adopt the comment because the creation of a childhood obesity listing is beyond the scope of these rules. When we consider revising the childhood endocrine section, we will consider whether we need to add a listing for obesity.

14.00 and 114.00 Immune System—Non-HIV Listings

Connective Tissue Disorders—General Comments

Comment: One comment stated that the term "rheumatic diseases" better describes conditions such as juvenile arthritis, systemic lupus erythematosus, dermatomyositis, and scleroderma than does the term "connective tissue disorders."

Response: We did not adopt the comment. Inflammatory arthritides (which are types of connective tissue disorders), including rheumatoid arthritis, psoriatic arthritis, Reiter's syndrome, and ankylosing spondylitis, are included in the musculoskeletal body system listings in 1.00 and 101.00. Therefore, we prefer the term "connective tissue disorders" because it better describes the disorders in 14.00 and 114.00.

Comment: Another comment said that systemic lupus erythematosus, systemic sclerosis, and polymyositis are rheumatic disorders that should be retained under the multiple body system section, or grouped into a new section titled, "Rheumatic Disorders." The comment added that any listing of the immune system should include multiple sclerosis and myasthenia gravis.

Response: We did not adopt the comment. We agree that all immune system disorders are not included in this listing. There are many disorders of immune regulation that are covered in other body systems, depending on the primary target organs. For example, multiple sclerosis (Listing 11.09) and myasthenia gravis (Listing 11.12) are evaluated under the neurological body system because neurological dysfunction is the primary outcome of these impairments. However, in the immune system listings we have grouped a number of connective tissue disorders that are characterized by autoimmune abnormality.

Comment: A few commenters called for rheumatoid arthritis to be grouped with systemic lupus erythematosus (Listings 14.02 and 114.02), systemic vasculitis (Listings 14.03 and 114.03), systemic sclerosis (Listings 14.04 and 114.04), and polymyositis (Listings 14.05 and 114.05). One of their comments said that the inclusion of rheumatoid arthritis would be consistent with our emphasis on functional aspects rather than labeling or diagnosis, inasmuch as the effects of all of these disorders on joints and internal organs are very similar.

Response: We did not adopt the comment in these final rules. However, we will consider the comment when we consider any revisions to the musculoskeletal body system listings.

Comment: A few commenters called for separate listing subsections for Sjgren's syndrome, sarcoidosis, psoriatic arthritis, Ehlers-Danlos syndrome, Marfan's syndrome, and, in the adult section, congenital immune deficiencies, such as genetic dwarfism. They also stated that consideration of Raynaud's phenomena should not be limited to systemic sclerosis and scleroderma. One of their comments suggested the addition of listings subsections for spondyloarthropathies, reactive arthritides, Bechet's syndrome, familial Mediterranean fever, and inflammatory myopathies other than polymyositis, such as body myositis. The comment also stated that the listings should consider the effects of therapy, which can cause bone thinning, pathologic fractures, and growth failure.

Response: We did not adopt the comments, but we did add guidance to 114.00B of the childhood rules in response to the last comment. The listings are only examples of impairments, not an all-inclusive list, and serve as a screening device by which we can quickly identify individuals who are disabled as a result of commonly occurring impairments. Even though they include many impairments, they have never been intended to include all impairments. It would not be feasible to attempt to provide a listing for every known disease. Generally, when a specific disease is not listed, we use the listing that provides the findings most closely analogous to the findings associated with the unlisted impairment.

Sjgren's syndrome is evaluated under the applicable body system depending on the presenting manifestation (e.g., kerato-conjunctivitis under 2.00 or 102.00, xerostomia under 5.00 or 105.00, arthritis under 1.02 or 101.02, and other connective tissue involvement under 14.00 or 114.00). The most common rheumatic manifestation of sarcoidosis is acute arthritis, which may be evaluated under the musculoskeletal system (Listings 1.02 and 101.02). When chronic arthritis occurs, the predominant impairment is due to involvement of the lungs, spleen, bone marrow, and bone. Hence, sarcoidosis, the cause of which is unknown, should also be evaluated under the applicable body system, depending on the disease manifestations. Psoriatic arthritis and spondylitis may be evaluated under 1.00, 101.00, or 8.00. Raynaud's phenomena are seen in several connective tissue disorders, but are particularly common in systemic sclerosis (Listings 14.04 and 114.04) and undifferentiated connective tissue disorders (Listings 14.06 and 114.06). When they occur in these or other connective tissue disorders and are characterized by digital ulceration, ischemia, or gangrene, equivalence to Listing 14.04 or 114.04 could be found.

Although Ehlers-Danlos syndrome and Marfan's syndrome are connective tissue disorders, they are not immune disorders, but genetic disorders, and, therefore, should not be included in the immune system listings. These syndromes are evaluated under the listings for the affected body system, (e.g., cardiovascular, visual, musculoskeletal, gastrointestinal).

Listings 14.07 and 114.07 provide criteria for immunoglobulin deficiency states and non-HIV cell-mediated immune deficiency. Myositis and myopathy may occur in a wide spectrum of diseases, and should be evaluated under the body system applicable to the primary disorder associated with the myopathy (e.g., 6.00 or 106.00 for hyperthyroidism, 11.00 or 111.00 for myasthenia gravis, or 14.00 or 114.00 for connective tissue disorders). Equivalence to 14.05 or 114.05, polymyositis, may be found when the criteria are applicable but the cause of the myopathy is other than polymyositis. Muscle weakness associated with myopathies may also manifest equivalent severity under the neurological listings. Spondyloarthropathies and "reactive" arthritides may be evaluated under 1.00 or 101.00. Bechet's syndrome is rare, its manifestations diverse, and etiology unknown. The major findings are genital and oral ulcers, skin lesions, and ocular lesions. Evaluation should be under the applicable body system for the manifestation(s). Mediterranean fever is an inherited disorder and not due to immune dysregulation. It is characterized by acute, self-limited attacks of fever, abdominal pain, pleuritic pain and, occasionally, arthritis. Evaluation for equivalence under rules applicable to other episodic illnesses is appropriate.

We do consider the effects of treatment in all cases. The fifth paragraph of 14.00B (both in the NPRM and the final rule) indicates that in addition to the limitations caused by the connective tissue disorder itself, the chronic adverse effects of treatment may result in functional loss. However, even though this principle is fundamental to all disability adjudications, the last comment made us realize that we had stated it explicitly in the preface to the adult rules but not in the preface to the childhood rules. Therefore, in response to the comment, we have added a new fourth paragraph to final 114.00B which is identical to the corresponding paragraph in the adult rules and underscores the need to consider the adverse effects of treatment (such as corticosteroid therapy) when evaluating connective tissue disorders in children.

Comment: We received a comment stating that because of the vast number of rare "orphan diseases," the primary factor that we should use to determine disability should be functional limitations caused by symptoms of any etiology.

Response: As noted previously, the listings are only examples of commonly occurring impairments and are not intended to include all impairments, especially rare ones. Many listings, including final Listings 14.02-14.06 and 114.02-114.06 do include functioning among their criteria; when we use these listings for comparison to evaluate unlisted impairments, we also consider functioning within the context of the listings. Moreover, for children who apply for SSI benefits based on disability, we also provide a "functional equivalence" determination.

Even if the individual's severe impairment(s) does not meet or equal in severity any listing, we still always assess the functional limitations caused by the impairment(s) and use that assessment to determine whether the individual is disabled at the later steps of the sequential evaluation processes for adults and children. As with all claims where the individual has a severe impairment(s) that does not meet or equal the severity of a listed impairment, the individual's claim is evaluated further and residual functional capacity is assessed to determine if he or she has the ability to do past relevant work. If the individual cannot perform his or her past work, we will determine if there are other jobs the individual can perform. In the case of a child under 18 who is applying for SSI, we perform an individualized functional assessment to determine if he or she is able to function independently, appropriately, and effectively in an age-appropriate manner.

Comment: One comment said that, beyond the information needed to make a medical diagnosis, there should be more specific guidelines in the listings on assessing function because of the imperfect relationship between a person's capacity and his or her function.

Response: We did not adopt the comment. We already have very detailed standards on assessing function for all impairments. The instructions address the need to consider the specific effects of each person's impairment(s) on his or her ability to function and recognize that one individual's limitations may differ from another's even though they may have the same impairment(s).

Comment: Another comment suggested adding listing criteria for chronic fatigue syndrome which, the comment said, is an immunological disorder that affects millions of individuals.

Response: We did not adopt the comment. Due to the divergence of medical opinion on chronic fatigue syndrome, we do not believe that it is either possible or appropriate to establish listing criteria. Further, such a listing would be beyond the scope of these rules.

Comment: One comment questioned whether adults who have impairments that would meet the childhood criteria may be found disabled using the part B criteria. The comment also asked if children who have impairments that meet the childhood criteria will remain eligible upon attainment of age 18, or whether they will then have to demonstrate that they have impairments that meet the part A criteria.

Response: As set forth in §§ 404.1525(b)(2) and 416.925(b)(2) of our regulations, the criteria in part B apply only to the evaluation of impairments in persons under age 18. Therefore, the listings in part B may not be used to find an adult disabled.

We do not require children to reestablish disability based on adult criteria when they attain age 18. However, we do periodically review the claims of disabled people to determine whether they are still disabled. When we determine whether disability continues, we apply a medical improvement review standard required by the statute. Under this standard, if a beneficiary who is now an adult was most recently found disabled (or still disabled) because his or her impairment(s) met the childhood criteria, we use those childhood criteria, even after the individual has attained age 18, as a basis of comparison to determine whether there has been any medical improvement in the individual's impairment(s) that is related to the ability to work.

14.00A and B, and 114.00A and B Preface

Comment: One comment said that the discussion on polymyositis and dermatomyositis in 14.00B4 omitted any other inflammatory myopathies and implies that if there is weakness, pain or tenderness in any muscles other than the proximal limb-girdle, cervical, cricopharyngeal, or intercostal muscles or the diaphragm, then one does not meet this criterion.

Response: We accommodated the comment by indicating in final 14.00B4 that the descriptions are only meant to describe the criteria in Listing 14.05. The muscles described in Listing 14.05 and in final 14.00B4 are the ones usually involved in polymyositis or dermatomyositis. If other muscles are involved, the underlying disorder—which may not be polymyositis—should be identified if possible and considered under the appropriate body system listings. If the impairment is found to be severe at the second step of the sequential evaluation processes but does not meet or equal in severity any listing at the third step of the processes, we will do an individualized assessment of its impact on the person's functioning and decide disability at the last steps of the sequential evaluation processes.

Comment: Another comment stated that weight loss as a constitutional symptom, which is recognized in 14.00B of the adult listings, should also be recognized in the childhood listings.

Response: We did not adopt the comment because the proposed childhood listings already included weight loss in the fourth paragraph of proposed 114.00B. That same language appears in the last sentence of final 114.00B.

14.02-14.06 and 114.02-114.06 Connective Tissue Disorder Listings

Comment: One comment noted that the phrase "with the expectation that the disease will remain active for 12 months" appeared repeatedly in the proposed connective tissue disorder listings (in proposed Listings 14.02-14.06 and 114.02) and asked how we make such a prediction. The comment said that unless we describe how physicians are to make the prediction, claimants who have had active disease for 10 or 11 months will be denied benefits.

Response: Even though we disagree with the conclusion that we would deny claims filed by individuals who have had active, listing-level disease for almost a year, we partially adopted the aacomment. We frequently make findings of disability based on an expectation that a disabling impairment(s) is expected to last for at least 12 months. In most cases in which the evidence substantiates a finding of disability, it is readily apparent from the same evidence whether or not the impairment is expected to last 12 months from the onset of disability. When the application is being adjudicated before the impairment has lasted 12 months, the nature of the impairment, the therapeutic history, and the prescribed treatment serve as the basis for concluding whether the impairment is expected to continue to prevent the individual from working for the required 12 months' duration.

However, we are not describing this in the listings because it is longstanding practice that applies to all types of impairments, not just connective tissue disorders.

This comment and others made us realize that the discussions on duration in proposed Listings 14.02-14.06 and 114.02 made the proposed listings unnecessarily complex. More importantly, they only repeated the general listings requirements in §§ 404.1525(a) and 416.925(a). There is, therefore, no reason to repeat the provision in each of these listings. Therefore, in response to this and other comments, we removed the repetitive language from each of the proposed listings and added a single discussion on duration in 14.00B and 114.00B as a reminder of the basic rules. For consistency, we also removed the statements in each of the listings requiring a 3-month longitudinal clinical record, inasmuch as we already make the statement in 14.00B and 114.00B. We also moved the requirement that the disorder remain active, "despite prescribed therapy" into the same sections of the preface. (We also changed the word "therapy" to "treatment" for reasons explained elsewhere in this preamble.) The result is that final Listings 14.02-14.06 and 114.02 are much simpler to read, even though there is no substantive change in the rules as a result of these editorial changes.

Finally, we will not generally find an individual who has had active, listing-level disease for 10 or 11 months to be not disabled. Unless the impairment has significantly improved to the point at which it is no longer disabling at the second, fourth, or fifth steps of the sequential evaluation process for adults (or the second or fourth steps of the sequential evaluation process for children claiming SSI benefits) before the end of 12 months after onset, an allowance would be appropriate. We are confident that our adjudicators understand this principle.

Comment: A comment suggested editorial changes to the statements regarding duration in Listings 14.02 and 14.03, apparently to remove redundancies.

Response: We adopted the comment in part by moving references pertaining to durational requirements from all of the listings that used this language to one location in 14.00B and 114.00B.

Comment: One comment we received said that there were problems with including in Listing 114.02A cross-references to other listings criteria as a means of describing the multiple organ dysfunction of systemic lupus erythematosus. The comment said that the type and pattern of organ involvement in systemic lupus erythematosus is not always the same as in other disorders and that muscle involvement in scleroderma and systemic sclerosis is not necessarily similar or identical to the muscle involvement of polymyositis or dermatomyositis. The comment also questioned the propriety of referencing some of the childhood connective tissue disorders to adult criteria because the disorders are not always identical in children and adults.

Response: We did not adopt the comment. Connective tissue disorders may involve many different organs and body systems. Establishing specific criteria for every organ in each body system would make the listing unnecessarily complicated. Consequently, we believe that cross-references to existing listings are the best solution.

We cross-referenced the childhood systemic lupus erythematosus listing (final Listing 114.02) to other body systems, the scleroderma and systemic sclerosis childhood listing (final Listing 114.04) and polymyositis and dermatomyositis childhood listing (final Listing 114.05) to the corresponding adult rules in final Listings 14.04 and 14.05, and the childhood undifferentiated connective tissue disorders listing (final Listing 114.06) to the childhood listings for systemic lupus erythematosus and systemic sclerosis and scleroderma (final Listings 114.02 and 114.04), because their manifestations can be identical, even though the causes of the problems are not the same. Cross-referencing provides a means to find the existence of a disabling impairment when a single manifestation of disease is at the same level of severity described in the cross-referenced listing.

Comment: A comment asked whether severe fatigue, fever, malaise, and weight loss must all be present to satisfy the criteria in Listings 14.02B, 14.03B, and 14.04B (and, presumably, 114.02B).

Response: We adopted the comment. The parenthetical "e.g." in the proposed rules was an error. We have corrected final Listings 14.02B, 14.03B, 14.04B and 114.02B to show that all four symptoms and signs must be present. However, instead of replacing the proposed "e.g." with "i.e.," as we originally intended, we have revised the sentence to make our intent clearer. The final provisions state that the disorders must be "* * * associated with significant constitutional symptoms and signs of severe fatigue, fever, malaise, and weight loss." We chose the particular symptoms and signs shown in the listings because they are the most common and are most likely to be present.

Comment: Another comment asked that we define the terms "severe" and "moderate" used throughout the listings for connective tissue disorders.

Response: We did not adopt the comment. Even though, as we explain later, we changed the term "severe" in places where it could have been confused with other terms ("incapacitating" and "major"), we retained the terms "severe" and "moderate" where we believe they are appropriate and unambiguous. The terms are widely used to describe relative values on a rating scale, and their meanings are commonly understood. But because their meanings are somewhat nonspecific, use of these terms in Listings 14.02, 14.03, 14.04, and 114.02 unquestionably requires a degree of judgment, as do many other aspects of our disability evaluation process. Our adjudicators are accustomed to making these judgments on a case-by-care bases, and we believe that attempting to devise specific definitions for terms that are, by their nature, non-specific, would only make the listings confusing. However, in response to this comment, we have also provided clarification in 14.00B and 114.00B that we use the word "severe" in these listings in its medical sense, not in the functional sense associated with the second step of our sequential evaluation processes. We explain this provision in a later response, below.

14.02 and 114.02 Systemic Lupus Erythematosus

Comment: One comment noted our statement in proposed 14.00B1 that, "[g]enerally" the medical evidence will show that patients with systemic lupus erythematosus will fulfill the 1982 "Revised Criteria for the Classification of Systemic Lupus Erythematosus" of the American College of Rheumatology (formerly, the American Rheumatism Association). The comment also noted that this implies that an individual can have systemic lupus erythematosus and not fulfill these criteria, and asked why similar latitude is not provided for other conditions.

Response: We did not adopt the comment. We used the word "generally" because the diagnosis is not invariably made strictly according to the criteria. To meet the American College of Rheumatology diagnostic criteria for systemic lupus erythematosus an individual must have four manifestations out of a list of 11 criteria, and the vast majority of people with this disorder will meet these criteria. However, a doctor will occasionally make a diagnosis of systemic lupus erythematosus when an individual has only three out of 11 manifestations, or other findings, when it appears likely that the diagnosis is appropriate.

Latitude is built into all the connective tissue disorder criteria. The guidance in final 14.00B3 for evaluations under Listing 14.04, Systemic sclerosis and scleroderma does not require that any specific pattern of disease manifestations be present to establish the diagnosis. The criteria in Listing 14.04 are similar to those for the other connective tissue disorders, providing references to other listings. As in thoseother listings, it also provides alternative criteria for multisystem manifestations associated with constitutional symptoms and signs. This is also true of polymyositis, 14.00B4 and final Listing 14.05, and undifferentiated connective tissue disorder, 14.00B5 and final Listing 14.06.

Systemic vasculitis, 14.00B2 and final Listing 14.03, comprises several diverse clinical syndromes and is characterized diagnostically by a tissue biopsy showing necrotizing vascular inflammation. Hence, a tissue biopsy or an angiogram showing the characteristic vascular abnormalities is necessary to confirm the clinically suspected diagnosis. However, when the findings of a referenced listing are present or multisystem involvement is evident with constitutional symptoms and signs, listing-level severity may be found even if there has not been a definitive diagnosis. Hence, this listing also provides latitude.

Comment: A few commenters said that they were not sure that the medical community at large is familiar with the 1982 "Revised Criteria for the Classification of Systemic Lupus Erythematosus" of the American College of Rheumatology. They suggested that, instead of referencing it, the material should be included in the listing itself or in a readily available supplement. One of their comments asked why we proposed to use the American College of Rheumatology criteria for systemic lupus erythematosus, but not for the other connective tissue disorders. The comment also said that most of the rheumatic diseases are syndromes and the diagnoses are made by meeting specific criteria.

Response: We did not adopt the first comment because we do not think that it is necessary to publish the diagnostic criteria in the regulations. The American College of Rheumatology diagnostic criteria are widely available and widely known.

Systemic lupus erythematosus is a relatively common disease, the diagnosis of which is based upon the presence of several non-specific clinical and laboratory abnormalities. Because of the lack of a single set of diagnostic findings, individuals may be erroneously diagnosed because of a non-specific laboratory result. It is, therefore, appropriate to refer to the published American College of Rheumatology diagnostic criteria. The vasculitides, on the other hand, are rare and difficult to diagnose clinically. The hallmark for and the diagnosis of vasculitis is almost invariably based upon characteristic clinical findings and tissue biopsy showing necrotizing vascular inflammation. Moreover, there are no published specific diagnostic criteria based upon clinical observations and laboratory tests. Therefore, referral to published diagnostic criteria is not possible.

Comment: A few commenters said that, because the type and pattern of joint involvement in rheumatoid arthritis and juvenile rheumatoid arthritis differs from that seen in systemic lupus erythematosus, the rheumatoid arthritis and juvenile rheumatoid arthritis criteria in Listings 1.02 and 101.02 should not be applied as reference listings to the evaluation of systemic lupus erythematosus under Listings 14.02A1 and 114.02A2. One of their comments noted further that, if there is joint involvement consistent with rheumatoid arthritis or juvenile rheumatoid arthritis in the presence of other findings consistent with systemic lupus erythematosus, then, by our definition, this would be an undifferentiated connective tissue disorder, which should be evaluated under Listings 14.06 and 114.06.

Response: We did not adopt the comment. In referencing proposed Listing 14.02A1 to Listing 1.02, and proposed Listing 114.02A2 to Listing 101.02, we were providing a means to determine the presence of a disabling impairment when a single manifestation of disease is at the same level of severity as that described in the reference listing. We did not mean to imply that systemic lupus erythematosus and rheumatoid arthritis have identical characteristics. To make this point even clearer, we have revised the cross-references in the final rules to the generic body system headings, 1.00 and 101.00, in order to include any musculoskeletal effects of systemic lupus erythematosus that are at the listing level of severity.

A diagnosis of undifferentiated connective tissue disorder is appropriate where the impairment has features suggestive of a connective tissue disorder but not diagnostic of any one disorder. We did not intend to suggest otherwise in Listings 14.02A1 and 114.02A2, which describe properly diagnosed systemic lupus erythematosus.

Comment: One comment noted that the adult listing for systemic lupus erythematosus included a criterion for muscle involvement (Listing 14.02A2), but proposed childhood Listing 114.02 did not.

Response: In response to the comment, we added muscle involvement to final Listing 114.02A3. Because of this addition, we renumbered the subsequent criteria accordingly.

Comment: Another comment suggested that Listing 114.02 include cross-references to criteria in the hemic system and to the listings for depression and Raynaud's phenomena.

Response: We adopted the comment. Although proposed childhood Listings 114.02A8 and 114.02A12 did include cross-references to specific hemic listings (Listings 7.02 and 107.06) and mental disorders listings (Listings 112.02, 112.03, and 112.04), we revised final Listings 114.02A9 and 114.02A13 so that they refer to the hemic and lymphatic and mental "body systems" in general (107.00 and 112.00), instead of to specific listings. In this way, no hemic or mental manifestations will be overlooked and the listing will remain up-to-date should we revise the hemic and mental listings in the future. Even though Raynaud's phenomena are not a primary feature of childhood systemic lupus erythematosus, we added a cross-reference to Listing 14.04D in final Listing 114.02A6 for those situations in which children do have such manifestations at the listing level. For consistency, we also added a cross-reference to Listing 14.04D in the corresponding adult rule, final Listing 14.02A5.

Comment: One comment suggested that in proposed Listing 14.02B the requirement that the individual demonstrate "severe" and "incapacitating" signs and symptoms was extreme, especially when a full 12 months of this level of severity must be anticipated.

Response: We adopted the comment in part. We agree that "incapacitating" is a higher level of severity than is needed to show listing-level severity. Furthermore, the comment made us realize that we had proposed slightly different language (using the terms "severe," "incapacitating," and "major") for corresponding paragraphs in proposed Listings 14.02B, 14.03B, 14.04B, and 114.02B, when we intended to say the same thing in each section. Furthermore, the word "major," which we had proposed in Listing 14.04B, could have caused confusion because it has a particular meaning in the medical community, referring to kinds of infections. Therefore, we replaced all these terms with the word "significant," which conveys the intended meaning consistently throughout these final listings.

We also realized that referring to "severe" symptoms and signs in these listings could have caused confusion because "severe" has a specific meaning when we use the word in the phrase "severe impairment" to describe the functional impact of an impairment(s) (see §§ 404.1520, 404.1521, 416.920, 416.921, and 416.924). For this reason, we have added a sentence at the end of the sixth paragraph of final 14.00B and 114.00B to explain that we use the term "severe" in these listings to describe medical severity and that it does not have the same meaning as it does when we use it in connection with a finding at the second step of the sequential evaluation processes for adults and children.

14.03 and 114.03 Systemic Vasculitis

Comment: One comment said that proposed Listing 14.03 on vasculitis was stricter and more detailed than then-current Listing 10.03, which required only signs of generalized arterial involvement.

Response: Listing 14.03 is more detailed than prior Listing 10.03, but the criteria are not stricter. Rather, they are more medically accurate and reflect state-of-the-art practice. They also now include all forms of systemic vasculitis, and ensure more consistent and valid determinations.

14.04 and 114.04 Systemic Sclerosis and Scleroderma

Comment: A comment suggested that we delete the word "generalized" before "scleroderma" in Listing 14.04C. Another comment questioned why we provided a listing for linear scleroderma for children (Listing 114.04B) but no similar listing for adults, and noted that 14.00B3 omits mention of the differences between limited and diffuse scleroderma.

Response: We have retained the term "generalized" in final Listing 14.04C because adults rarely manifest localized scleroderma; if they do, equivalence to a listing in 1.00 or a residual functional capacity assessment may lead to a finding of disability because of destructive or mutilating lesions of the extremities or the head. We provided criteria for localized scleroderma for children because destructive and mutilating lesions involving the extremities, head, and scalp not only interfere with walking and using the upper extremities, but also with growth and development; scalp and facial lesions in children may also be accompanied by seizures.

"Limited" cutaneous scleroderma is not the same thing as "localized" or "linear" scleroderma, but a systemic form of the disorder. We did not mention the differences between limited and diffuse cutaneous scleroderma in the preface because the differences are not needed for application of the criteria in final Listings 14.04 and 114.04.

Comment: A comment said that, although severe Raynaud's phenomena were included in the proposed Listing 14.04D criteria, they were not defined.

Response: We have clarified the listing in response to the comment. In fact, proposed Listing 14.04D did describe severe Raynaud's phenomena, which are characterized by digital ulcerations, ischemia, or gangrene. However, we realized that the language of the proposed rule, "Raynaud's phenomena with" these findings, was not clear. We have, therefore, changed the word "with" to "characterized by" in the final listing to make clear that the findings of digital ulcerations, ischemia, or gangrene define severe Raynaud's phenomena.

14.05 and 114.05 Polymyositis and Dermatomyositis

Comment: One comment stated that proposed Listing 14.05, for polymyositis and dermatomyositis, was too strict. The comment said that an individual who satisfied the criteria in the opening paragraph of the listing (which required 3 months of active disease, severe proximal muscle weakness despite prescribed treatment, and an expected duration of 12 months) should be found to meet the listing without also having to satisfy the criteria in proposed Listing 14.05A or 14.05B.

Response: We partially adopted the comment. The commenters misunderstood our intent in proposed Listing 14.05. The criteria in proposed Listings 14.05A and 14.05B were not additional criteria, but were meant to define the "severe proximal muscle weakness" in the opening paragraph. However, the comment made us realize that the listing could be made clearer. Therefore, we have clarified the requirements in final Listing 14.05 by removing the opening paragraph, which was redundant of the criteria for documentation, duration, and severity, discussed in other parts of the listings, and which is now in final 14.00B.

Comment: Another comment suggested that we provide more detail about the required severity of proximal muscle weakness. The comment said that proposed Listing 14.05 required shoulder or pelvic muscle weakness as described in Listing 11.12, which pertains only to muscle weakness of the extremities. The comment also questioned how swallowing and impairment of respiration are to be evaluated under Listings 14.05B1 and 14.05B2.

Response: In response to the comment, we deleted the cross-reference to Listing 11.12B in final Listing 14.05A and instead provided a discussion of the intent of the provision in final 14.00B4. We also provided a more detailed description of the criteria in final Listing 14.05B1 for cricopharyngeal weakness. However, we think that proposed Listing 14.05B2 was clear and have made no changes in that final listing.

Comment: Another comment questioned why there was no adult listing corresponding to Listing 114.05B for polymyositis or dermatomyositis with severe multiple joint contracture or diffuse cutaneous calcification, and why swallowing or respiratory difficulties are limited to adult Listing 14.05B1.

Response: Both multiple joint contractures and diffuse cutaneous calcification are extremely uncommon findings in adults with these disorders; however, if an adult has these findings their specific impact on the individual must be assessed. Multiple joint contractures in an adult that are of listing-level severity should be evaluated under the criteria in 1.00ff, the musculoskeletal body system. Listing-level cutaneous calcification may be evaluated under Listing 14.04, Systemic sclerosis and scleroderma.

Swallowing and respiratory difficulties are not limited to Listing 14.05B. Childhood Listing 114.05A indicates that impairment should be evaluated according to Listing 14.05. Therefore, all of the criteria in Listing 14.05 apply to children.

14.06 and 114.06 Undifferentiated Connective Tissue Disorder

Comment: One comment questioned whether the term "undifferentiated connective tissue disorder" used in Listings 14.06 and 114.06 is synonymous with "mixed connective tissue disorder." The comment also questioned why chronic undifferentiated tissue disorder is evaluated by reference to the criteria in Listing 14.02, Systemic lupus erythematosus, and stated that the disorder is either systemic lupus erythematosus or it is not.

Response: We partially adopted the comment. We added a discussion of overlap syndromes to final 14.00B5 (which is also referred to in 114.00B) and noted that these syndromes should be evaluated under Listings 14.06 and 114.06. Although most individuals with undifferentiated connective tissue disorders have features of systemic lupus erythematosus, we recognize that some may have features of systemic sclerosis and scleroderma. Therefore, we added to Listings 14.06 and 114.06 cross-references to Listings 14.04 and 114.04. However, we prefer to confine Listings 14.06 and 114.06 to undifferentiated connective tissue disorders to indicate the lack of a specific diagnosis, with its attendant specific prognosis. We also have retained the title.

Comment: Another comment stated that there is a distinction between the undifferentiated connective tissue disorders (i.e., where a connective tissue disorder is present but unknown) and the overlap syndromes (i.e., where there are elements of more than one connective tissue disorder present). This comment also said that both types should be recognized under the listing and that, because some of these disorders are not undifferentiated, Listings 14.06 and 114.06 should be titled: "Other Connective Tissue Disorders."

Response: We did not adopt the comment, except to the extent that we added the aforementioned discussion about overlap syndromes to final 14.00B5. "Undifferentiated connective tissue disorder" is similar to, but not synonymous with, "overlap syndrome" and "mixed connective tissue disorder," but the latter two classifications depend upon constellations of non-specific features. Undifferentiated connective tissue disorders have the clinical and immunologic features of several connective tissue disorders, none of which satisfies the criteria for any of the disorders described. Overlap syndromes have clinical features of more than one established connective tissue disorder, and mixed connective tissue disorders usually have features of systemic lupus erythematosus, systemic sclerosis, and myositis. Most individuals with mixed connective tissue disorders eventually will be shown to have either systemic lupus erythematosus, systemic sclerosis, or Sjgren's syndrome, but a few remain undiagnosed and should be labeled "undifferentiated."

Comment: A comment stated that the criteria for evaluation of childhood undifferentiated connective tissue disorders in Listing 114.06 were a confusing series of cross-references, noting that Listing 114.06 referred to evaluation under corresponding adult Listing 14.06 which, in turn, referred to Listing 14.02.

Response: We adopted the comment. Final Listing 114.06 now indicates that undifferentiated connective tissue disorders should be evaluated by reference to Listings 114.02 or 114.04.

14.07 and 114.07 Immunoglobulin Deficiency Syndromes or Congenital Immune Deficiency Disease

Comment: One comment said that the criteria for the evaluation of immune deficiency disease in Listings 14.07 and 114.07 are too restrictive because they consider only immunoglobulin deficiency syndromes or deficiencies of cell-mediated immunity, and exclude other immune deficiencies or immune dysregulatory states. The comment also noted our statement in proposed 14.00A that the " * * * disorders include impairments involving deficiency of one or more components of the immune system * * *." The comment said that, although a number of examples are listed in this section, many of the potential immune system impairments are absent from Listings 14.07 and 114.07.

Response: We did not adopt the comment. As we have stated, the listings are only examples of commonly occurring impairments, and are not meant to be all-inclusive. Immunoglobulin deficiency syndromes or deficiencies of cell-mediated immunity are the most common immune deficiencies. Immune deficiency disorders not specified in Listing 14.07 or 114.07, but that are of listing-level severity, may be found equivalent in severity to the listed 14.00 and 114.00 Immune System: General Comments On The HIV Listings

Populations Covered By the Rules

Comment: Various commenters asserted that the proposed rules did not include manifestations of HIV infection that affect women, persons of color, gay and lesbian people, and the poor.

Response: On the basis of information we received from individual medical and other experts who study, treat, and work with people who have HIV infection, as well as our review of the medical literature, we do not agree that the proposed rules excluded these groups of people. Based on our experience since December 17, 1991, using our revised operating procedures, we know that the proposed listings would have included the vast majority of people who were disabled by HIV infection. Nevertheless, as we have already explained above in the summary of the final provisions, we have further revised the final listings to make them even more inclusive. Among the new criteria are several new criteria in both the adult and childhood listings that include more of the manifestations of HIV infection unique to women and girls. We are confident that these final rules provide criteria for evaluating all of the manifestations of HIV infection suffered by various populations.

Comment: One of the comments said that the proposed listing did not recognize the medical conditions that affect drug abusers, and that some chronic conditions were not listed.

Response: This comment did not identify any additional conditions that were not listed. The manifestations in proposed Listing 14.08M2 (with the exception of Kaposi's sarcoma), as well as many others throughout the proposed listing, are conditions that affect drug abusers. The conditions in proposed Listing 14.08M2 are now in the final rules as stand-alone medical listings, without functional requirements.

Comment: Many commenters thought that, despite our assertion to the contrary in the NPRM (56 FR at 65703), the proposed rules had not broken the link to the CDC surveillance definition of AIDS. They said the listings were unfair and discriminatory to women, poor people, those who do not have CDC-defined AIDS, and those with no continuity of health care. They indicated that, although we had proposed to include manifestations that the CDC uses to define AIDS without functional criteria, other illnesses (the kind not associated with CDC-defined AIDS, but frequently found in women, intravenous drug users and others who tend to be poor and have limited access to health care) required that functional criteria be met.

Response: We disagree with the comments, but we have revised the rules in response to these and other comments to explicitly include even more manifestations without a functional requirement. Therefore, even though we have included many of the criteria of the CDC's surveillance definition of AIDS, we have also provided many other criteria for people who have symptomatic HIV infection but who do not meet the CDC surveillance definition.

For example, we added as stand-alone conditions as many HIV-related conditions from proposed Listings 14.08M and 114.08L and 114.08M as possible, including endocarditis, syphilis and neurosyphilis, meningitis, pulmonary tuberculosis, and pneumonia. These manifestations are not stand-alone criteria in the CDC surveillance definition of AIDS but, we believe, can be sufficiently severe to be disabling in an individual with HIV infection. In addition, we created a stand-alone listing that includes pelvic inflammatory disease (final Listings 14.08A5 and 114.08A6) and another that includes vulvovaginal candidiasis (final Listings 14.08F and 114.08F).

Standard of Disability

Comment: Many commenters believed the proposed listings did not take into account the progressive nature of HIV infection in adults or children. They suggested that claimants with HIV infection should be found disabled at commensurately lower levels of severity than claimants with other diseases. A few commenters suggested that we adopt the broadest permissible definition of disability so as to get medical care to as many HIV-infected individuals as early as possible. They said this was important because the degenerative nature of HIV-related conditions guarantees that if someone is nearly disabled today, he or she will become disabled in the near future. One of their comments said that, although our stringent disability standards make sense with impairments that are relatively stable and capable of improvement, such eligibility requirements are less necessary when dealing with rapidly degenerative illnesses such as those associated with HIV. This is because there is little need to consider whether applicants will remain ill long enough to be classified as disabled—those impaired by such illnesses simply do not get better. Another comment noted that, only through a combination of Federal, State and local funding could early treatment and care, including drug trials, be provided, and that tightening the listing criteria would result in the City and State governments bearing the entire responsibility for this continuum of care.

Some commenters cited the rapid deterioration experienced by children with HIV infection, and the fact that few of these children live to adulthood, especially those who acquire the virus from their mothers. The commenters said that our childhood neoplastic listings (i.e., the listings in 113.00) permit a finding of disability before marked functional loss has occurred and thus set a precedent for doing something similar in the case of children with HIV infection.

Response: We believe that these rules provide the broadest permissible definition of listing-level severity, consistent with the definition of disability contained in the Act. Moreover, we do not have the authority to apply a different definition of disability for some people than the standard of disability in the Act. The Act requires that an individual be currently disabled, and does not permit us to find an individual disabled based on a prediction of future disability.

However, these rules are not stricter than our previous criteria. To the contrary, both the proposed rules and these final rules provide more ways in which people with HIV infection may establish that they have listing-level impairments.

Our criteria take into account the unique course and history of HIV disease in both adults and children, including its progressive nature. In cases in which a claimant is experiencing a manifestation(s) of HIV disease that is indicative of a rapid decline in an adult's ability to engage in any gainful activity, or an SSI child claimant's ability to function independently, appropriately, and effectively in an age-appropriate manner, we have defined criteria that do not necessarily require continuous HIV are rapidly degenerative, or that individuals with HIV functional loss following the onset of the initial manifestation. Rather, the manifestation of HIV infection can be found disabling even though it includes periods of improvement. However, even though HIV infection is progressive and ultimately fatal, it is not true that all illnesses or other manifestations associated with infection cannot recover from HIV-related manifestations. Many manifestations are treatable, and many individuals can return to a good level of functioning following a period of severe illness. The impact of HIV and its manifestations is highly individual, and our disability adjudication system, which affords an individualized determination to every claimant, recognizes this.

We believe that this approach is consistent with the approach we take in the neoplastic listings. Neither the HIV listings nor the neoplastic listings describe impairments of lower severity than other listings. Rather, they recognize the medical realities of the conditions in terms of prognosis, overall functioning on a longitudinal basis, and the impact of treatment on functioning.

It is also very important to remember that no individual will be denied benefits simply because his or her impairment(s) does not meet or equal the severity of a listing. If an individual's impairment(s) does not meet or equal the severity of a listing, he or she can be found disabled at later steps of the sequential evaluation processes for adults and children.

Finally, we want to assure the commenters that we share their concerns, and are aware of the poor prognosis for individuals with HIV infection. We believe the promulgation of these listings addresses those concerns.

Comment: Some commenters thought that we should find any individual with symptomatic HIV infection to be disabled.

Response: We do not agree that any individual with symptomatic HIV infection of any type should be found disabled. There are, in fact, many such conditions that are amenable to treatment without significant after-effects, and others that are simply not so severe as to render an individual unable to work or unable to engage in age-appropriate activities. In both instances individuals may continue to function well for long periods, and we believe that it is reasonable to provide regulatory criteria that allow for the individualized assessment of the effects of a person's impairment(s) on him or her, as we have done in final Listings 14.08N and 114.08O.

Comment: A few commenters said that the criteria for HIV infection should recognize that persons who have asymptomatic HIV infection should have the right to treat their condition and prolong their lives through rest and stress reduction, and not be exposed to further compromise of their medical condition in a workplace.

Response: We did not adopt the comment. The standard of disability for adults under the statute is the inability to engage in substantial gainful activity by reason of a medically determinable physical or mental impairment(s), or for children under age 18 who apply for SSI based on disability an impairment(s) of comparable severity to one that would disable an adult. Even though we agree that people who have asymptomatic HIV infection will ultimately become ill, they are not functionally limited until the infection begins to become symptomatic; i.e., until they begin to experience manifestations of the HIV infection. Once individuals do become symptomatic, however, these rules do not require that they be continuously symptomatic. The rules require that their impairments be evaluated on a longitudinal basis in order to form a picture of how the individual is able to function over time. Indeed, we have provided a separate listing, final Listing 14.08N, that includes individuals who suffer periodic manifestations of HIV infection but who may not be continuously limited between the episodes.

We would also like to clarify that we do not require people to work. The Act uses the ability to work as a way of describing the level of severity of impairment that constitutes a "disability." The Act does not say that a person who does not meet the definition of disability must work; it simply says that such a person is not disabled within the meaning of the Act. Indeed, to underscore this point, the statute explicitly excludes from consideration the factors of whether a job exists in the area in which the person lives, whether there are job openings, or whether the person would be hired to do a job. Thus, the listings are not intended for use in determining whether or not an individual should work, but to provide examples of impairments that satisfy the definition of disability in the Act because they are considered severe enough to prevent an adult from engaging in any gainful activity, or an SSI claimant under the age of 18 from functioning independently, appropriately, and effectively in an age-appropriate manner.

Comment: One comment said that any claimant whose physician reports positive HIV infection and a resulting inability to work should be considered disabled, regardless of whether or not the individual presents opportunistic infections.

Response: We did not adopt the comment. As we have said, an individual with asymptomatic HIV infection will not be functionally limited by the impairment. Under our rules for evaluating medical opinion evidence in §§ 404.1527(e) and 416.927(e), the Secretary is responsible for determining whether an individual is "disabled" under the Act; a statement by a medical source that the individual is "unable to work" is not sufficient in itself to establish that an individual is disabled within the meaning of the Act. However, in making our determination, we review all of the medical findings and other evidence in the individual's case record that support a medical source's statement that the individual is disabled and will recontact the source, if necessary, to obtain additional information in support of the opinion.

Comment: A number of commenters suggested that we give special consideration to the "socioeconomic" factors that can affect HIV-infected claimants, such as poor nutrition, limited or no access to ongoing health care, inadequate housing, and adverse family factors.

Response: We did not adopt the comments, but we have revised several provisions in response to these and other comments to make clear that we do consider some of the factors the commenters suggested, though not as "socioeconomic" factors.

The Act requires that disability must be established on the basis of a medically determinable physical or mental impairment(s) that results from anatomical, physiological, or psychological abnormalities which are demonstrable by medically acceptable clinical and laboratory diagnostic techniques. It does not permit direct consideration of "socioeconomic" factors as determinants of disability. For example, under the Act we cannot consider whether a claimant lives in inadequate housing or receives substandard medical care in making a disability determination.

However, we do consider some of the factors the commenters believed to be "socioeconomic" factors because they are medical factors under our program. For example, under our program poor nutrition (i.e., malnutrition) is a medical condition and can be a disabling impairment in and of itself if it is of sufficient severity and duration. Even if it is not of listing-level severity, it can limit an individual's ability to function and cause disability, or combine with other impairments to cause disability. Individuals who receive substandard medical care—especially individuals who have HIV infection—will often be more severely impaired than other individuals because of more frequent illness or failure to adequately recover from infections and other manifestations that might be treatable with proper care, resulting in a generally more severe medical and functional picture.

Thus, it is not necessary for us to consider "socioeconomic" factors, which are ancillary to the determination of how the person is actually affected by his or her impairment(s). For the purpose of deciding disability, we need only know the nature and severity of the individual's impairment(s) and its effects on his or her functioning. We do not use factors like the quality of medical care or housing to determine whether a person is medically disabled, just as we would not use such factors to find a claimant not disabled.

However, in part because we agree with the commenters that many claimants have limited access to ongoing health care, we have clarified the documentation requirements in final 14.00D3 and 14.00D4, and 114.00D3 and 114.00D4 to make it clear that HIV infection or its manifestations may be documented without definitive laboratory evidence if the documentation is consistent with the prevailing state of medical knowledge and current medical practice and is consistent with the other evidence. We do not require specific diagnostic tests in all cases.

Comment: A few commenters proposed that we should also assess the effect of an HIV-infected individual's home situation on the ability to work when the individual is a parent caring for a child or children who also show symptoms of HIV and are, therefore, in need of more intensive care. These commenters observed that, because such children require frequent administration of medication and regular clinic visits and are not allowed to receive day care, they would be a burden on a healthy parent; parents with HIV infection are already required to miss work frequently because of their own conditions, and the situation is exacerbated when the parent must also take time off from work to care for sick children.

Response: We did not adopt the comment. Under the Act, disability must result from the individual's medically determinable impairment(s). We are, therefore, unable to provide for a finding of disability when an adult is unable to attend work because of the need to care for an ailing child and not because of a medically determinable impairment(s). However, when we consider the extent to which an individual's HIV-related manifestations affect his or her ability to function, we consider the individual's ability to care for her or his children.

Organization of the Listings

Comment: Many commenters requested that we simplify the HIV listings. Some commenters thought that it was unnecessary to publish all of the CDC's criteria for diagnosing AIDS in our listings because some of the CDC criteria are redundant. They pointed out, for example, that every manifestation in proposed Listings 14.08A and 114.08A was repeated somewhere in proposed Listings 14.08B-I and 114.08B-H; the only difference was that a person's manifestations would meet Listings 14.08A or 114.08A if the person did not have laboratory evidence confirming the presence of HIV infection, but would meet one of the other listings if he or she did have such evidence. The commenters noted that, whereas the CDC might have good reasons for distinguishing between the two situations, the fact was that we would find an individual who had one of these manifestations to be disabled under the listings whether or not there was laboratory evidence of HIV infection. Therefore, there was no reason for us to list the same manifestation more than once.

Some commenters said that this complexity had led to inconsistencies in the proposed rules. They pointed to various sections of the proposed listings where the same conditions and the same evidence needed to document those conditions were described in slightly different terms, suggesting that the criteria were different even though they clearly were not meant to be. They stated that these discrepancies and other inconsistencies in the language would be confusing to both adjudicators and the general public.

Some commenters pointed out repetitious language that we could delete (for instance, the statement "Documentation of HIV infection as described in 14.00D" at the beginning of every listing under proposed 14.08 and 114.08); others suggested ways to reorganize the listings. One of their comments said that dividing the proposed listing by pathogenic process did not increase the ease of reference and thought the repetition of section headings added clutter. The comment recommended that we combine the proposed listings into a single listing with all of the manifestations arranged alphabetically.

Many commenters objected to the many different proposed criteria for documenting the existence of the various manifestations in the listings. We address the substantive comments in a separate section below, but as pertinent here, the commenters pointed out that the numerous specific criteria for documenting each of the different manifestations made the listings very complex and difficult to use.

Response: We adopted the comments. As we have already explained in the summary of the final provisions, we have simplified the language and organization of the final rules and have eliminated the redundancies of the proposed listings. In response to comments we describe later in this section, we removed all of the various specific requirements for documenting the presence of the manifestations in lieu of the guidance we now provide in final 14.00D4 and 114.00D4. We also revised the language throughout part A and part B to make them both internally consistent and consistent with each other where appropriate.

We chose to retain an organization that lists some of the manifestations under general headings for the type of organism, and others according to the affected body system or type of manifestation. We did not adopt the suggestion that we list all the manifestations alphabetically, although we did try it to see if it would work. We found that an alphabetical list was more cumbersome than the system in these final rules. To begin with, the list was very long; there are over 50 separate named manifestations in final Listing 14.08. In addition, it was sometimes difficult to decide how a given impairment should be of alphabetized (i.e., by specific organism, affected organ, or kind of manifestation), and in some cases, impairments that naturally seemed to group together (for example, cryptosporidiosis, isosporiasis, and microsporidiosis, which are all protozoans that cause diarrhea) were widely separated only because of the alphabetical artifice. Moreover, we believe that the system we decided to use in the final rules carries an advantage that simple alphabetization would not. By grouping impairments according to etiology where possible and, elsewhere, into other logical categories (such as body system or organ affected) we have provided implicit guidance that will be more useful for finding medical equivalence for unlisted manifestations than an alphabetized system would.

Comment: One comment suggested editorial revisions in 14.00D. The comment suggested that we consolidate the definition and description of HIV infection into one location, giving more emphasis to the progressive nature of the disease, that we consolidate all information about evaluation of HIV infection cases under the sequential evaluation process into one location, and that we eliminate superfluous language. The commenter provided alternative language for parts of proposed 14.00D.

Response: Although we have not adopted the specific language suggested in the comment, we have rewritten and reorganized all of the paragraphs in 14.00D of the final rule, and removed repetitious language. In the final rule, we have revised the definition of HIV infection in 14.00D1, consolidated the explanation of how to evaluate individuals with HIV infection under the sequential evaluation process in 14.00D6, and made other changes throughout 14.00D. We discuss these changes in greater detail in the explanation of the final rules and in response to public comments about specific issues addressed in final 14.00D.

Comment: One comment suggested that it was not necessary to list Hodgkin's and non-Hodgkin's lymphomas separately, as we had proposed in Listings 14.08I and 14.08J. The comment noted that, under the proposed rules, any individual with any type of lymphoma would be found to have an impairment that met a listing; therefore, it would be simpler to have one listing that included all lymphomas. Another comment said that we should provide separate criteria for immunoblastic sarcoma, which we had included with the lymphomas in proposed Listing 14.08I2, because there is a controversy over whether it is a true lymphoma.

Response: We adopted the first comment. Final Listing 14.08E3 now includes all lymphomas. In a parenthetical statement, the final listing names some of the various lymphomas we had proposed in the NPRM, but characterizes them as examples to emphasize that all lymphomas are included. This is because all lymphomas in HIV-infected individuals carry a poor prognosis. We made the same revisions in the childhood rules, at final Listing 114.08E3.

Even though we acknowledge that there is a dispute about whether immunoblastic sarcoma is a lymphoma, we did not adopt the second comment. Inasmuch as the prognosis is poor in all such cases with HIV infection and the mere existence of the manifestation establishes listing-level severity, there is no practical reason for establishing a separate listing under our rules.

Documentation

Comment: A number of commenters said that the proposed requirements for documentation of HIV infection were too difficult and burdensome to meet, especially for indigent persons who do not have a primary care physician and have inadequate access to health care. The commenters also said that the tests we required in the proposed rules to document a diagnosis of HIV infection are too expensive for indigent persons to afford, and that proposed 14.00D required individuals to undergo specific laboratory tests or invasive medical procedures to establish a diagnosis or meet the listing. Several commenters also expressed concern that requiring specific laboratory tests, such as an HIV antibody test or a CD4 count, might inappropriately cause denials or the early obsolescence of the criteria for establishing disability related to HIV infection. Several commenters suggested that we consider clinical judgment or generally acceptable means of diagnosis consistent with the current state of medical knowledge. One commenter suggested specific language about the standards for documenting HIV infection without laboratory evidence, and included suggested language for the fourth paragraph of proposed 14.00D to explain why a positive screening test for HIV infection, such as ELISA, needs confirmation by a more definitive test.

Response: We adopted the comments, even though we did not require as much testing as the commenters believed. For instance, in the fifth and sixth paragraphs of proposed 14.00D we explained that a diagnosis of HIV infection could be accepted without laboratory documentation based on the existence of a disease predictive of a defect in cell-mediated immunity with no known cause of diminished resistance to that disease. We also said that, in such cases, the documentation of HIV infection will rely on the clinical history, physical examination, exclusion of other causes for clinical abnormalities, and treating source opinion. We also added language to final 14.00D3a and 114.00D3a to explain why a positive ELISA test must be confirmed by a more definitive test.

However, recognizing the reality of limited access to health care for many individuals, we have revised and expanded the language in final 14.00D3b and 114.00D3b, Other acceptable documentation of HIV infection, to provide that the existence of HIV infection may be documented without definitive laboratory evidence when definitive laboratory evidence is not available. We did not adopt the specific language suggested by one commenter. If no definitive laboratory evidence is available, documentation may be by medical history, clinical and laboratory findings, and diagnoses indicated in the medical evidence, provided that it is consistent with the prevailing state of medical knowledge and clinical practice and is consistent with the other evidence. This would be true, for example, when an individual has an opportunistic disease predictive of a defect in cell-mediated immunity, and there is no other known cause of diminished resistance to the disease (as we provided in the NPRM). We use the clause, "If no definitive laboratory evidence is available," in the final rules to make clear that we include individuals who may have undergone HIV testing anonymously or when there are privacy considerations. Of course, if laboratory tests have been performed and the results are available, we will make every reasonable effort to obtain them.

We have also made other changes in the rules. We made similar revisions to our rules regarding the documentation of manifestations of HIV disease in final 14.00D4b and 114.00D4b. We also no longer include separate listings in final Listings 14.08 and 114.08 for manifestations of HIV with and without documentation.

Comment: Some commenters believed that diagnosis without definitive laboratory evidence should be accepted for every manifestation in the proposed listings. Other commenters requested clarification of which manifestations of HIV infection could be diagnosed without definitive laboratory evidence and which required definitive documentation.

Response: We did not adopt the comments. We cannot make a blanket rule that permits diagnosis of every listed manifestation in Listings 14.08 and 114.08 without definitive laboratory evidence because some of the manifestations, such as Salmonella bacteremia (Listing 14.08A3), lymphoma (Listing 14.08E3), nephropathy (Listing 14.08L), and radiographically documented sinusitis (Listing 14.08M6), will by their very nature require laboratory testing. However, we also do not want to specify exactly which of the manifestations may be diagnosed without definitive laboratory evidence because we want to leave the listings flexible to accommodate future medical practices. For this reason, we provide in final 14.00D3b and 14.00D4b (as well as the corresponding childhood sections) that the diagnosis of HIV and its manifestations may be established by methods of documentation that are "consistent with the prevailing state of medical knowledge and clinical practice and consistent with the other evidence."

Comment: Another comment said that the proposed rule's heavy reliance on documented HIV test results disadvantaged persons who test positive for HIV infection at an anonymous test site before developing HIV-related symptoms. Giving the example of an individual applying for disability benefits under title II after she tested positive for HIV infection at an anonymous test site and subsequently developed an HIV-related condition, the comment recommended that we apply later evidence of HIV infection retroactively to the date when HIV-related symptoms first developed.

Response: We partially adopted the comment. As we explained above, we introduced the clause, "If no definitive laboratory evidence is available," in final 14.00D3 and 14.00D4, and 114.00D3 and 114.00D4, to underscore the fact that we include the situation in which an individual may have undergone HIV testing anonymously.

We did not add explicit rules on determining retroactivity. Our general disability rules already permit us to establish an onset date in the past based on an inference drawn from the medical and other evidence in the case record. This does not mean, however, that we will find all individuals with HIV infection to be disabled from the moment that they tested positive for the HIV. As we have said, individuals with HIV infection who are otherwise asymptomatic and do not yet have any limitations are not disabled under the definition of disability in the Act. On the other hand, it is possible for us to find disabled as of the date the manifestation(s) first occurred, an individual who began experiencing manifestations of HIV infection before she knew that she had HIV infection. As we always do, we will determine an individual's onset date based on the facts of the specific case.

Treatment

Comment: A few commenters said that we did not address the adverse side effects caused by treatment or explain how to evaluate improvement caused by AZT therapy.

Response: Although we did include a general discussion of the need to consider the effects of treatment in the fourteenth paragraph of proposed 14.00D, we have expanded the discussion in antiretroviral agents as an example of a type of treatment that may ameliorate the condition or cause side effects. AZT is a kind of antiretroviral agent; we did not mention it specifically because final 14.00D7, Effect of treatment, in response to the comments. The final section contains three paragraphs. The first paragraph stresses the importance of considering both the positive and negative effects of treatment. In the paragraph, we mention we would like the rules to remain current if new treatments are devised in the future that supplant the use of AZT.

In the second and third paragraphs of the final rule, we provide guidance about how to evaluate the effects of treatment. We stress the need to take into consideration on a case-by-case basis both the positive and negative effects of treatment on the individual's ability to function. In these same paragraphs, we also point out that some individuals may respond to treatment more successfully than others and that the effects of treatment may be temporary or long-term. As in the NPRM, the final section provides that it is essential to obtain a specific description of the drugs or treatment given, and a description of the complications or any other response to treatment.

Equivalence

Comment: A few commenters suggested that we include in the listing preface more instructions to be used in determining when unlisted conditions equal the severity of listed conditions. They said that an applicant would have no way of knowing what he or she would have to prove, because program physicians determine whether an unlisted illness has a level of severity equivalent to a listed impairment. One of their comments suggested that, even though such things are not amenable to exact quantitative measurement, the approximate levels of pain, fatigue, and physical impairment associated with each listed illness, along with any other relevant factors, such as frequency and duration of episodes, could be specified, so any illness that meets the least restrictive of these descriptions could qualify as a disability.

Response: We did not add guidance to the listings about how to determine equivalence, but we have provided more of the kind of detail the commenters requested in the final rules. We do not provide substantive instructions for determining equivalence in any of the listings sections in part A or part B. We have separate rules in §§ 404.1526, 416.926, and 416.926a of our regulations which set forth criteria for determining equivalence. The rules on equivalence include rules in § 416.926a for assessing a child's functional limitations to determine whether they are the same as the disabling functional consequences of any impairment in the listings.

The majority of our listings describe conditions for which medical criteria can be specified that are of such severity that it is unnecessary to consider the kinds of factors mentioned by the commenters. Final Listings 14.08 and 114.08 are no exception; the criteria for all of the manifestations in Listings 14.08A-M and 114.08A-N are met without the need to consider or specify whether there are symptoms or limitations; the levels of fatigue, pain, or "physical impairment" these impairments may cause are implicit in the listings. Only final Listings 14.08N and 114.08O, which employ functional criteria as a measure of severity, require such considerations. As we have already explained, we have extensively revised the functional rules in response to these and other comments, and we believe that we have provided more detail about the kinds of symptoms and the extent of limitations necessary to meet these listings within the listings themselves and in 14.00D and 114.00D.

In addition to symptoms (such as fatigue and pain) and limitations, the commenters also suggested that we better define other factors, such as the frequency and duration of episodes mentioned in various listings. We believe we have responded to this comment as well in the extensive revisions in final 14.00D and 114.00D. In these sections, we have, among other changes, provided definitions of the terms "resistant to treatment" and "recurrent," included language about the need to consider an individual's medical and functional status on a longitudinal basis, and provided explicit guidance (in final 14.00D8) about the meaning of the term "repeated" in final Listing 14.08N. In the listings themselves, we added specific criteria for the frequency, duration, and severity of episodes of manifestations wherever it was relevant. For example, final Listings 14.08A5 and 114.08A6 specify that the multiple or recurrent bacterial infections must require hospitalization or intravenous antibiotic treatment at least 3 times in 1 year.

Finally, an individual does not have to know what he or she has to prove to us in order for us to make a finding of equivalence or any other finding regarding disability. We assist the individual by requesting the evidence we need for our determination. Moreover, an individual does not have to "prove" equivalence to us to be found disabled. If we determine that the individual's impairment(s) is equivalent in severity to a listed impairment, we will find that the individual is disabled. However, if we determine that an individual's impairment or impairments are "severe," but that they are not listed and are not equivalent in severity to a listed impairment, our evaluation will proceed through the final steps of the sequential evaluation process before we make any determination about whether the individual is disabled.

Proposed 14.08M, 114.08L, and 114.08M: The Functional Listings

Proposed 14.08M1-M3, 114.08L1-L2, and 114.08M1-M3: The Medical Criteria

Comment: A number of commenters said that the manifestations of HIV infection in proposed Listings 14.08M2 and 14.08M3, 114.08L1 and 114.08L2, and 114.08M2 and 114.08M3 were severe enough to be disabling without meeting a functional test, or had their own functional ramifications. Some commenters indicated that the manner in which the diseases were ranked did not accurately reflect the true disabling effects of some of the conditions. A number of commenters specifically questioned the need for functional requirements for people with Kaposi's sarcoma in proposed Listing 14.08M2h. Some commenters noted that there was a range of severity for Kaposi's sarcoma. (CD4 lymphocyte count, the other criterion associated with the functional criteria in proposed Listings 14.08M1 and 114.08M1, is addressed in a separate comment and response, below.)

Some commenters thought that our use of the terms "persistent" and "resistant" to describe the severity of the manifestations was confusing. They said we should define the terms.

Response: We adopted many of the comments. As we have already explained in the summary of the final provisions above, we devised stand-alone medical listings for most of the manifestations we had proposed in the functional listings. We also removed all of the specific manifestations we had proposed to list in lieu of more general descriptive rules that include any kind of manifestation of HIV infection, not only those that were in the proposed functional listings. Our changes were based on the public comments, additional medical information received from doctors—including pediatricians and physicians specializing in the treatment of HIV infection in women—and from other professionals with expertise in treating and studying individuals with HIV infection.

We converted all eight HIV manifestations included in proposed Listings 14.08M2, 114.08L1, and 114.08M2 into stand-alone listing criteria. We agreed for the most part with commenters who stated that the first six of the eight listed manifestations would be listing-level impairments if they were "persistent and/or resistant to therapy," as described in the proposed rules, without the need to consider functional deficits. In the final listings, pulmonary tuberculosis is in Listings 14.08A1 and 114.08A1; pneumonia, sepsis, meningitis, septic arthritis, and endocarditis are in final Listings 14.08M and 114.08N.

The final rules require that these conditions be "resistant to treatment" instead of "persistent and/or resistant to therapy." We made this change for a number of reasons. We used the word "treatment" instead of "therapy" only to make the language of the final rules consistent with other sections in the listings; this is merely an editorial change. The phrase "persistent and/or resistant to therapy," however, was redundant and could have been confusing. The phrase "and/or" was unnecessary in that, because of the disjunctive, "or," a person would have had an impairment that met the listings with either persistence or resistance to treatment; therefore, the conjunctive, "and," was superfluous. Also, in most situations, "persistent" would have been redundant of "resistant to therapy" because, if a person was receiving treatment and the manifestation persisted, the manifestation was implicitly resistant to treatment.

Moreover, the word "persistent" was also ambiguous and difficult to define. Some manifestations can respond to treatment without being cured. They can technically "persist" because the organisms that cause them are still present, but not necessarily be disabling. If we said the manifestations had to persist at a disabling level, the individual would have to be in treatment and the manifestation resistant to treatment: Individuals with persistent disabling pneumonia, pulmonary tuberculosis, sepsis, and meningitis require treatment or they will die; septic arthritis is usually a sign of a more pervasive infection and is so debilitating that the individual would also require treatment.

Therefore, we deleted the word, "persistent," from the final rules. However, we also provided alternative criteria for the manifestations in final Listings 14.08M and 114.08N by which individuals whose manifestations respond to treatment only to recur may establish listing-level severity.

We did not agree with the commenters who thought that we could list the remaining two manifestations proposed in Listings 14.08M2, 114.08L1 and 114.08M2, peripheral neuropathy and Kaposi's sarcoma, without some other indication of medical severity. Both Kaposi's sarcoma and peripheral neuropathy vary widely in severity. These disorders, even when not amenable to treatment, may not seriously impair functioning, even in individuals with HIV infection. Therefore, the medical criteria we developed require more than resistance to treatment and are more descriptive of listing-level severity. The final criteria for evaluating Kaposi's sarcoma (final Listings 14.08E2 and 114.08E2) require more than limited superficial lesions; they require extensive oral lesions, or visceral involvement, or skin or mucous membrane lesions of sufficient severity to satisfy the criteria in final Listings 14.08F and 114.08F, Conditions of the skin or mucous membranes. Of course, the condition may also be evaluated under the final functional listings in 14.08N and 114.08O. In the case of peripheral neuropathy (final Listings 14.08H2 and 114.08H), the disorder must be assessed either under the appropriate neurological listings in 11.00 and 111.00 or on the basis of functional limitations under final Listings 14.08N and 114.08O. In order to make clear that HIV-related peripheral neuropathy may be evaluated under the neurological listings, we added cross-references to those listings sections in final Listings 14.08H2 and 114.08H.

In addition, we developed criteria for evaluating most of the other HIV manifestations in proposed Listings 14.08M3, 114.08L2 and 114.08M3. As we did for peripheral neuropathy, we required the three blood disorders now in final Listings 14.08G and 114.08G—anemia (proposed Listings 14.08M3a and 114.08M3a), granulocytopenia (proposed Listings 14.08M3b and 114.08M3b), and thrombocytopenia (proposed Listings 14.08M3c and 114.08M3c)—to meet the criteria of other listings (in 7.00 and 107.00, the listings for the hemic and lymphatic system). Even in the case of an individual with HIV infection, the blood count figures alone do not show how an individual is able to function.

We also developed stand-alone medical listings for mucosal candidiasis, including vulvovaginal candidiasis (proposed Listings 14.08M3f and 114.08M3f, final Listings 14.08F and 114.08F), Herpes zoster (proposed Listings 14.08M3h and 114.08M3h, final Listings 14.08D and 114.08D), dermatological conditions such as eczema and psoriasis (proposed Listings 14.08M3i, 114.08L2g, and 114.08M3i, final Listings 14.08F and 114.08F), diarrhea (proposed Listings 14.08M3j, 114.08L2f, and 114.08M3j, final Listings 14.08J and 114.08J), and radiographically documented sinusitis (proposed Listings 14.08M3k, 114.08L2i, and 114.08M3k, final Listings 14.08M6 and 114.08N6).

Although we agree with the comment that the remaining manifestations in proposed Listings 14.08M3, 114.08L2 and 114.08M3 (fever, weight loss, hepatomegaly, splenomegaly, parotitis, oral hairy leukoplakia, and lymphadenopathy) can have functional ramifications, their effects on an adult's ability to work or a child's ability to function in an age-appropriate manner vary from individual to individual and, thus, listing-level severity cannot be defined in solely medical terms. Therefore, these manifestations, along with other manifestations of HIV infection that do not meet the criteria in final Listings 14.08A-M or 114.08A-114.08N, will continue to be evaluated with functional criteria under final Listings 14.08N and 114.08O.

Comment: Another comment questioned the addition of functional requirements to the criteria for HIV wasting syndrome.

Response: We did not list HIV wasting syndrome in proposed Listing 14.08M3 (or proposed childhood Listing 114.08M3); we proposed a separate Listing 14.08H (final Listing 14.08I) which provided that any person with HIV wasting syndrome had an impairment that met the listing. In the childhood listings, we provided a cross-reference to the proposed adult rule, in the ninth paragraph of proposed 114.00C.

We believe that the commenters misunderstood our intent in proposed Listings 14.08M3 and 114.08M3. HIV wasting syndrome is defined as an involuntary weight loss of more than 10 percent of baseline body weight and either chronic diarrhea or chronic weakness and documented fever greater than 100.4o F (38o C) for the majority of 1 month or longer. Although it is true that in proposed Listings 14.08M3 and 114.08M3 we listed all three of the criteria that may define HIV wasting syndrome (fever, weight loss, and diarrhea), we did not intend to list true HIV wasting syndrome in the functional listing but a lesser manifestation of HIV infection. An individual with true HIV wasting syndrome would have already been found to have an impairment that met the criteria of proposed Listing 14.08H. The individuals who could have met the criteria of proposed Listing 14.08M3 were those who did not have all of the findings needed to define HIV wasting syndrome, but who were nevertheless significantly limited in their functioning because of their manifestations.

For reasons we have already explained in the summary of provisions, we have established separate listings, final Listings 14.08J and 114.08J, to make diarrhea a stand-alone medical condition, but we have not listed fever and weight loss separately, except insofar as they define HIV wasting syndrome. However, these two medical findings, as well as diarrhea of lesser severity than in the stand-alone medical listings, may still be found to be of listing-level severity under final Listings 14.08N and 114.08O.

Comment: Some commenters suggested that we consider additional manifestations of HIV infection in conjunction with the functional standards in proposed Listing 14.08M3. The commenters suggested many specific manifestations, including joint aches, arthritis, or arthralgias, recurrent cystitis, fatigue, chronic headaches, chronic sleep disturbance, chronic shortness of breath or exertional dyspnea, and HIV-related mental disorders. Various comments on the childhood listings also suggested that we add chronic and recurrent otitis media associated with functional limitations.

Response: As we have said, instead of making the lists longer, but still finite, we decided to revise the functional listings so that they would include any possible manifestation of HIV infection. Therefore, we no longer list any manifestations explicitly, only a few examples. The revisions in final Listing 114.08O are sufficient to allow adjudicators to evaluate chronic and recurrent otitis media when it is a manifestation of HIV infection. Additionally, sequelae from otitis media, such as hearing loss or brain abscess, or any other manifestations of HIV infection in children or adults, can be evaluated under the appropriate listing or at the last steps of the sequential evaluation processes.

Comment: A number of commenters believed that the CD4 lymphocyte count required in proposed Listings 14.08M1 and 114.08M1 should be considered enough to establish listing-level severity without the additional requirement to meet the functional criteria. Some commenters stated that the proposed CD4 count less than or equal to 200 cells/mm3 (or 14 percent or less lymphocytes) was too low, especially if we linked it to functioning; others stated that it was too high.

Many suggested various alternatives. At least one comment asked us not to use any particular CD4 count as a measure of disability at all because each individual situation is different; the comment said that it would be unfair to label as disabled all individuals with low CD4 counts, when many such individuals are still functioning well. One commenter suggested a specific description of the standard for using CD4 lymphocyte counts, and suggested specific language to clarify the discussion of CD4 lymphocyte counts in the third paragraph of proposed 14.00D.

Response: We have deleted the CD4 criterion from the final rules. We realize that, although a decreased CD4 count is a gauge of an individual's potential for developing a serious opportunistic infection or other manifestation of HIV, with improved treatment and prophylaxis for certain opportunistic diseases one cannot reliably predict when an individual will develop a disabling manifestation. Further, the laboratory finding does not show whether the individual is functionally limited. There are many cases of individuals with very low CD4 counts (often far below 200) who exhibited few or no functional restrictions, and other individuals with much higher CD4 counts who were seriously impaired. Indeed, we received comments from such an individual, who related his own story of living with HIV infection and working even though his CD4 count was below 100.

We agreed completely with the many commenters who stated that such individuals are at risk of becoming disabled. However, our disability programs require an assessment of whether an individual is disabled currently, without regard to whether the individual may become disabled at some point in the future. Because there is so much variability in the state of health and functioning of individuals with any given CD4 count we could not adopt the suggestions to use a specific CD4 count alone (at any level) as a listing criterion.

Proposed 14.08M4, 114.08L3, and 114.08M4: The Functional Criteria

Comment: Many commenters said that the functional criteria were overly burdensome and restrictive and should be eliminated entirely. Some of these commenters believed that linking manifestations of HIV disease to a functional test ignored the progressive nature of the disease and created a higher level of severity than established by other listings. One commenter suggested extensive revisions in the paragraphs explaining the functional criteria in proposed 14.00D, and provided specific language for such revisions.

Response: For reasons we have given in the explanation of the final rules, we did not eliminate the functional criteria. Our intent in proposing the functional criteria in Listings 14.08M, 114.08L, and 114.08M was to include in the listings many individuals for whom we thought we could not provide solely medical criteria. For instance, the functional listings include a group of individuals who would be very difficult to describe in strictly medical terms—individuals who become ill then improve, only to repeatedly become ill again, either with the same manifestation of HIV infection or with something different. The functional listings also provide a listing for those individuals whose impairments might not be at listing-level severity for all individuals, but that are actually of listing-level severity for the particular individuals given their effects, such as pain, other symptoms, and the consequences of medication, that vary greatly with the individual. They help to ensure a finding of disability for any person whose impairment(s) actually prevents him or her from engaging in any gainful activity, or of any SSI claimant child whose impairment(s) actually prevents him or her from independently, appropriately, and effectively engaging in age-appropriate activities, even though that impairment(s) might not impose similar limitations on other individuals.

Moreover, we believe that, in view of the fact that we have made most of the proposed manifestations into stand-alone medical listings, we have accommodated the comments that asked us to delete the functional listings. The functional criteria now only provide another way to find disabled individuals who have most of the manifestations we proposed in Listings 14.08M, 114.08L, and 114.08M.

Based on other comments, however, we have significantly modified the proposed functional criteria to make them more applicable to cases involving HIV infection and to better express our original intent. We have also revised the paragraphs in the final rules that explain the functional criteria (final 14.00D8). The functional criteria for both adults and children are no longer tied to a finite list of specific medical conditions; any manifestation or combination of manifestations may now be evaluated under this listing. Additionally, the final rules require an adult to demonstrate limitations of functioning in only one of three areas of functioning, rather than the proposed two of four. We describe these rules and our reasons for the changes in subsequent comments.

Comment: Many commenters pointed out that under the functional equivalence policy in § 416.926a(b)(3), any child who has listing-level deficits in the functional domains of the listings in 112.00 is considered disabled regardless of the nature of the impairment. They said it was, therefore, not necessary to include the functional criteria in proposed Listings 114.08L and 114.08M, because these criteria did no more than recodify existing policy.

Response: We disagree. The part B listings are used to evaluate claims filed under both title II and title XVI of the Act if the claimant is under age 18. The functional equivalence policy in § 416.926a(b)(3), however, applies only to claims for SSI filed under title XVI. Even though SSI claims constitute the great majority of childhood disability applications, it is possible for individuals under age 18 to apply for disability benefits (both as disabled minor children and as workers) under title II. Functional equivalence does not apply in these cases, and such children could be disadvantaged by removal of the rule.

Comment: Many commenters said that the proposed HIV listing was the first and only adult physical impairment listing to require a functional test in order to qualify for benefits, and to do so violated various antidiscrimination laws. One comment indicated that the listing should exist solely to provide SSA with medical criteria for the purpose of making disability determinations.

Response: The commenters were not correct. Even though the listing for evaluation of HIV infection is the first to contain functional criteria similar to those in the mental body system, other physical body system listings, such as (but not limited to) several in the neurological and musculoskeletal body systems, include functional criteria among their requirements. We also believe that we have the statutory authority to include functional criteria in the listings because the listings are not intended to include all possible impairments (see, e.g., Sullivan v. Zebley, 493 U.S 521 (1990)) and because our rules ensure that all disabled individuals have an opportunity to establish that they are disabled under the Act. In any case, we have provided stand-alone criteria for most of the manifestations we had proposed to link to functioning, as well as some others that affect women, girls, and other groups of people with HIV infection. Therefore, the final rules do not discriminate against any group of people, but broaden the listings to include more people.

We also do not agree that the listings may include only medical criteria. Functional criteria not only provide an important avenue to allow individuals whose HIV-related conditions impose functional limitations, but, perhaps most importantly, they reflect a true outcome of the illness. Even the strictly medical criteria in the listings have implied functional consequences. By definition, claimants with impairments meeting or equaling listed medical criteria cannot work, and this inability to work—a functional assessment—is the underlying statutory criterion on which the entire disability program is based.

Comment: Several commenters said that requiring functional criteria in the adult listing would prevent adults with HIV infection from establishing their disabilities at the earliest possible point in time. They said that the functional criteria could cause the same delays for gathering and weighing evidence as the commenters believe occur when we assess residual functional capacity when a claimant's severe impairment(s) does not meet or equal in severity any listing. Some commenters said that the requirement for 2 months' persistence of the manifestations in proposed Listings 14.08M3 and 114.08M3 would create a 2-month processing delay.

Response: The effect of the functional criteria may actually be to expedite case processing. The functional criteria do not come into play unless the individual does not have an impairment that meets the requirements of one of the preceding listings. We also follow a general policy in all cases of curtailing development when there is sufficient evidence to properly allow a claim; if the evidence shows medical equivalence to one of the listings, we would not further develop the claim simply to establish whether the individual has an impairment that meets final Listing 14.08N. Therefore, the provision applies only to individuals for whom we would have to assess functioning at later steps of the sequential evaluation process: Individuals who have severe impairments that do not meet the medical listings and for whom we would have to perform a residual functional capacity assessment if we did not have this listing.

The assessment of residual functional capacity is a much more refined evaluation than is required under final Listing 14.08N. Whereas final Listing 14.08N only requires a judgment about whether an individual is markedly impaired in a broad area of functioning, a residual functional capacity assessment is a detailed evaluation of the claimant's ability to do particular physical and mental work-related activities. Both evaluations rely on the same kinds of evidence, so the new listing will not require additional time spent to develop evidence. If anything, individuals who meet this listing may not have to present as much evidence of their ability to function as to function as they would have to for the more detailed residual functional capacity assessment. Furthermore, the actual assessment of functioning under the listing is quicker than the residual functional capacity assessment and does not require evaluation under the medical-vocational rules.

In fact, there has been no evidence that using functional criteria since December 17, 1991, has delayed decisions made on cases involving HIV infection. We updated our procedures for evaluating HIV infection under an interpretive ruling we have been following since December 17, 1991 (Social Security Ruling (SSR) 91-8p, "Titles II and XVI: Evaluation of Human Immunodeficiency Virus Infection," 56 FR 65498, December 17, 1991). The experience we have using SSR 91-8p indicates that claims involving HIV infection are being processed expeditiously. We believe, therefore, that—far from delaying case adjudication—the new listing will speed the processing of many claims and permit more cases to be adjudicated at the listing level than would otherwise be possible.

With regard to the comment about the delays that might have been caused by the criteria requiring 2 months' persistence of the manifestations in proposed Listings 14.08M3 and 114.08M3, we have deleted those rules, as already explained.

Comment: Many commenters said that if we retained functional criteria in the final adult and childhood listings, the requirement should be to demonstrate marked limitations in only one area of functioning (for adults) or one functional domain (for children). They thought that for adults this was equivalent to the threshold we previously used in our operating instructions in effect prior to December 17, 1991. Some commenters were particularly concerned that the proposed rules for adults would be stricter than the rules they would replace by requiring a higher level of functional impairment.

Response: Even though we have changed the standard for adults to require marked limitations in one of the three functional areas, we do not agree that the proposed rules set a higher level of severity than was in our previous operating instructions, nor was that our intent. Indeed, under our prior instructions, an individual needed help with most activity, including climbing stairs, shopping, cooking, and housework, in order to establish a "marked" restriction of activities of daily living. In the nineteenth paragraph of 14.00D of the NPRM, an individual who was unable to perform activities independently most of the time had a "marked" limitation of activities of daily living. We further defined a "marked" limitation in the seventeenth paragraph of the section as arising "when several activities or function are impaired or even when only one is impaired, so long as the degree of limitation is such as to seriously interfere with the ability to function independently, appropriately, and effectively."

More fundamentally, and as we have already explained in the summary of provisions above, the proposed functional criteria for adults effectively permitted a finding of disability based on marked limitations in only one functional area if the individual also suffered episodic bouts of illness. Whereas activities of daily living, social functioning, and concentration, persistence or pace clearly describe functioning, the fourth area, repeated episodes of deterioration or decompensation in work or work-like settings, referred to episodes of illness. (This is not true for people with mental disorders, where the episodes of deterioration or decompensation may result from the stress of the work or work-like setting, but it is true in the context of HIV infection.) Thus, an individual who experienced the required episodes of illness in proposed Listing 14.08M4d and met only one of the three functional criteria in proposed Listing 14.08M4a-c would have had an impairment that met the listing.

This is not to say that all individuals could have met the listing in this way. Some would not have suffered episodic manifestations and, therefore, would have had to meet two of the three functional criteria in proposed Listing 14.08M4a-c. However, it has been our experience, contrary to the beliefs of many commenters, that individuals who are markedly limited in one of the areas of functioning also demonstrate marked limitations in one of the other areas; the requirement for limitations in two areas merely validated the finding of disability. Indeed, we have been using the same procedures under SSR 91-8p and have allowed many cases under this interpretive ruling.

As we have already explained in the summary, and in response to the comments, we revised final Listing 14.08M inasmuch as the fourth proposed functional criterion described the universe of individuals we were trying to capture in the listing. In the final rule, the fourth criterion from the proposed listing is now the threshold criterion for the listing and the individual must meet one of three functional criteria.

A similar change in the number of functional domains that must be limited in a childhood case is not appropriate. The criteria a child has to meet to be considered under the listing (i.e., the child must have a manifestation of HIV infection that does not satisfy any of the criteria in final Listings 114.08A-N) are not repeated in the functional domains, are not analogous to the areas of functioning used in evaluating adult cases, and differ with the age of the child.

Comment: Many commenters thought that the "marked" level of restriction required in the proposed adult functional criteria was too severe. They were particularly critical of the definition of "marked" as occurring "most of the time" in the paragraphs that defined the first three functional criteria. Some commenters suggested that "marked" connoted a level of functional restriction commensurate with almost total incapacitation, i.e., bed confinement or requiring nursing home care, and said that this reflected a higher level of restriction than is required to establish disability under the Act. Some also suggested that individuals would be disabled even if they were not limited "most of the time" but were limited to some lesser extent.

Response: We never intended "marked" to be interpreted as requiring total incapacitation (as, indeed, it does not in the mental body system listings). We proposed language to underscore this intent in the seventeenth paragraph of 14.00D in the NPRM, which, with minor language changes, is now the fifth paragraph of 14.00D8 in the final rules. In that paragraph, we first defined "marked" as being on a continuum between "moderate" and "extreme" to make the point that there is a more severe limitation than a "marked" limitation; that is, an "extreme" limitation. If "marked" meant total debility, it clearly would have left no room on the scale of severity for "extreme" limitation. We then stated that a marked limitation could arise "when several activities or functions are impaired or even when only one is impaired, so long as the degree of limitation is such as to seriously interfere with the ability to function independently, appropriately, and effectively." By indicating that a marked limitation might result from limitations of only several activities, or even only one activity, and by using the phrase "seriously interfere," we again meant to say that the individual need not have been totally debilitated.

We did, however, intend to establish a level of limitation that is higher than is required to establish disability under the Act. This is because all listed impairments in part A and part B define a more severe level of disability than is defined in the Act. The standard of disability in the statute is based on an inability to engage in "any substantial gainful activity" (see sections 216(i), 223(d), and 1614(a) of the Act). Under §§ 404.1525(a) and 416.925(a) of our regulations, however, we explain that the listings describe impairments that are considered severe enough to prevent a person from doing "any gainful activity." Similarly, the regulations defining disability in children provide that "comparable severity" to a disability in an adult means a substantial reduction in the ability to function independently, appropriately, and effectively in an age-appropriate manner (see § 416.924(a)). The listings, however, describe impairments that "prevent" a child from functioning independently,appropriately, and effectively in an age-appropriate manner (see §§ 416.924(e) and 416.926a(a)).

The point is that the listings are meant to be a screening device by which we can decide relatively quickly that an individual is disabled, without the need to proceed to the final steps of the sequential evaluation processes. It is at the final steps of the sequential evaluation processes for adults and children that we determine whether individuals have impairments that meet the statutory definition of disability. Disability under the listings is so severe that we know that there is no need to proceed further because a finding of disability would result even if we proceeded through all the steps of the sequential evaluation processes.

Nevertheless, the comments made us realize two things: First, that we could have more clearly stated that "marked" does not mean total incapacity, and second, that the standard of "most of the time" was unnecessarily inflexible. Consequently, we revised the description of "marked" to explain our intent more clearly. We now state plainly in the fifth paragraph of final 14.00D8 that "an individual need not be totally precluded from performing an activity to have a marked limitation * * *." We also added language in the fifth paragraph that describes "marked" in qualitative terms and makes clear that a "marked" restriction in function is not defined by any frequency of occurrences but by the degree of interference with function. We also state plainly that "marked" is not intended to imply that a person is confined to bed, hospitalized, or in a nursing home. This allows us the flexibility to determine whether a limitation is "marked" on a case-by-case basis. In each of the sixth, seventh, and eighth paragraphs of final 14.00D8 (which define the three functional areas, and correspond to the nineteenth, twentieth, and twenty-first paragraphs of proposed 14.00D) we eliminated the sentences that included the phrase "most of the time" and revised the remaining discussions to be more descriptive of our intent.

We did not simply revise "most of the time" to a shorter period, as some commenters suggested, because we believe the attempt was fraught with the same pitfalls that the commenters pointed out for the phrase we proposed. Furthermore, because we say that "marked" may involve only one activity or several activities, a criterion for less frequent interference could result in unintended variations in severity levels depending on which activities or other functions were limited.

Comment: Many commenters thought that it was inappropriate to use, nearly verbatim, the functional criteria language from the mental listings to describe the functional limitations in proposed Listings 14.08M, 114.08L and 114.08M. Although some commenters said they could appreciate the need to link functional limitations to physical disorders, they thought it was inappropriate to apply mental listing criteria to physical impairments.

Response: We do not agree that it is inappropriate to apply these functional criteria to physical disorders because the criteria are generic; they do not describe mental functions, but broad areas of functioning that are relevant to any adult's ability to work or any child's ability to independently, appropriately and effectively engage in age-appropriate activity. As we have explained in the summary of the final rules, these activities describe what people do and how well they do it on a day-to-day basis. For our purposes, it is immaterial whether an individual has difficulty doing chores or maintaining concentration because of a mental disorder or because of fatigue, weakness, pain, headaches, frequent diarrhea, or any other physical problem; the person still has the limitation that results from a medically determinable impairment(s).

However, as we have also said, we have modified the proposed language in final 14.00D8 to make it even more specific to individuals with HIV infection. As we have previously explained, we also removed the fourth "functional" criterion in proposed Listing 14.08M4d.

We also repeat that, by revising proposed Listings 14.08M, 114.08L and 114.08M to make most of the proposed manifestations into stand-alone medical listings and to broaden the applicability of the final functional listings to include any manifestation or combinations of manifestations, final Listings 14.08N and 114.08O are only advantageous to claimants. They merely provide another means for people to show that they are disabled under the listings.

Comment: A number of commenters specifically commented that the area of social functioning is meant to measure an individual's psychiatric condition and is not appropriate for the evaluation of HIV. They were especially concerned that an individual could be denied disability benefits because he or she "socialized" with family and friends.

Response: The commenters misunderstood our intent; we have, therefore, clarified the rules. We have always recognized that there is a difference between visiting with family and close friends, who may make special allowances for an impaired individual, and independent social functioning. Furthermore, the ability to interact with other people can be affected by a physical impairment. For instance, an individual who is fatigued my have difficulty going out or sustaining conversation. In addition, many individuals with manifestations of HIV infection do have mental findings (such as anxiety, depression, and apathy) that can interfere with their social functioning. Even if the mental findings are not manifestations of HIV infection, or are the only manifestations of the HIV infection, we still consider their effect on the individual's functioning together with any other manifestations.

To make our intent clearer, we have revised the language of the seventh paragraph of final 14.00D8 in response to the comments. Final 14.00D8 states that marked difficulty of social functioning means that an individual "cannot engage in social interaction on a sustained basis (even though he or she is able to communicate with close friends or relatives) * * *." It is also important to note that, under the final listing, social functioning is only one area of functioning among three, each one of which can establish disability at the listing level.

Comment: Several commenters thought that the fourth functional test requirement in the adult listing, i.e., "repeated episodes of decompensation," was too severe and went beyond what is necessary to prevent an adult from working. The commenters suggested that this criterion be revised to more accurately reflect the reality of the exacerbations and remissions in HIV-related illnesses and the need to be absent from work for treatment.

Response: We have already explained how we revised proposed Listing 14.08M in the summary of provisions and the foregoing responses. In the third paragraph of final 14.08D8, we retained the provision for manifestations occurring on an average of 3 times a year, or once every 4 months, and each lasting at least 2 weeks, but changed it to one provision among several alternatives instead of an absolute requirement. We now also provide that the manifestations may last for less than 2 weeks and occur substantially more frequently than 3 times a year or every 4 months, or that they may occur less frequently than 3 times a year or once every 4 months but last substantially longer than 2 weeks each time. We believe this better reflects the variety of patterns of episodic illness experienced by persons with HIV infection.

We do not agree, however, that the proposed criterion was incompatible with the ability to work in and of itself. It described an individual who missed 6 weeks of work during the course of a whole year because of illness. Although we do not mean to suggest that missing work for 2 weeks at a time 3 times in a year is not serious, we do not believe that it is so serious in itself that we could conclude that the individual was disabled for 12 months, as required by the statute. This is why we also require an accompanying indication of marked functional limitations in the final rule.

Comment: Several commenters stated that in setting the adult functional standards, we should evaluate individuals based on both current functional ability and likely future loss of capacity.

Response: The Act requires that an individual be disabled during the period covered by the individual's application. This usually means that the individual must be currently disabled, although we may find disability in the past under title II within the time limits covered by the application. However, we are never permitted to find an individual disabled based on a prediction that the individual will become disabled in the future. There is no provision in the statute that would permit us to overlook a claimant's current favorable level of function because it is expected his or her condition will worsen at some future time. Our policy is to advise individuals to reapply for disability benefits at such time as the condition precludes substantial work activity.

Comment: Numerous commenters suggested that we use functional tests like the Karnofsky Performance Status instead of the proposed functional criteria.

Response: We did not adopt the comments. The Karnofsky Performance Status is not a "functional test," but a physician's estimate of functional status. We do not think, however, that the Karnofsky or other available tests are sufficiently broad or objective to use in place of our functional criteria, as the standard for measuring functional capacity in HIV-related disability claims.

Comment: Several commenters said that we should develop a special form to capture information regarding a claimant's functional limitations and train our Field Office and State agency personnel to properly elicit this information. Some were interested in working with us to develop the form, as well as to develop a national 800-number and telefax service for the dictation of physician narratives and medical documentation.

Response: Developing evidence of functional limitations is not new to Field Office or State agency employees. The disability application forms include basic questions regarding evidence of functional limitations and are sufficient to make a determination in many cases. The State agencies develop additional evidence regarding function from a variety of medical and non-medical sources when that is necessary. Although we appreciate the commenters' offers, at this time we do not believe a special form is needed for either the Field Offices or the State agencies. Also, because medical determinations are made locally, a national telephone/telefax service for physician narratives and medical documentation would not be practical. We believe these kinds of services are best when designed and implemented locally in order to meet the particular needs of the area.

HIV Manifestations Suggested as Additions to the Listings

Comment: A number of commenters suggested adding other manifestations of HIV infection to the listings, such as: anemia, arthritis, oral candidiasis (oral thrush), chronic shortness of breath or exertional dyspnea, chronic sleep disorders, hepatitis (including hepatitis caused by cytomegalovirus), extrapulmonary pneumocystis, fatigue, HIV myositis, leukemia, lymphocytic interstitial pneumonitis, microsporidiosis, mucormycosis, neoplasia, pancytopenia, pulmonary aspergillosis, recurrent giardiasis, renal failure, squamous carcinoma of the genitals, side effects of antiretroviral therapy, syphilis and neurosyphilis. (We discuss other suggested additions to the listings—including several that are specific to women—in subsequent comments and responses).

Some of these commenters suggested specific criteria to be included (e.g., chronic anemia with persistent hemoglobin of less than 10 percent or hematocrit of less than 30 percent, or requiring transfusions more often than twice yearly). Other commenters simply identified the symptoms (e.g., dyspnea) or conditions they thought should be included, without describing any particular level of severity. When a commenter suggested adding a medical condition to the listing but did not include criteria describing impairment severity, we were often unable to discern whether the commenter was asking that we develop listing criteria for that manifestation, or asking that we consider the mere existence of the manifestation in an individual with HIV infection to be listing-level severity. In order to ensure that we considered every comment, we considered both possible interpretations of the comment.

Response: We adopted some of these comments, partially adopted others, and did not adopt others.

In response to the comments, we added the following manifestations of HIV infection to the listing without any qualifying criteria: extrapulmonary pneumocystis carinii infection (final Listings 14.08C2 and 114.08C2); mucormycosis (final Listings 14.08B6 and 114.08B6); and aspergillosis (final Listings 14.08B1 and 114.08B1). An individual with HIV infection and any one of these manifestations has an impairment that meets the listing.

To the extent that the commenters were suggesting that we include any other manifestations in the HIV listings without any qualifying criteria, we did not adopt the suggestions. The information we obtained and the medical literature indicated that, although the other manifestations suggested by the commenters can be disabling, they need not be. Consequently, the assessment of severity must be made based on criteria beyond the mere presence of the manifestation. In order to be responsive to the comments, we attempted to develop a listing-level standard for each suggested addition to the listings, using qualifying criteria to indicate impairment severity.

The listings now include microsporidiosis (final Listings 14.08C1 and 114.08C1), if it results in diarrhea lasting for 1 month or longer; and septic arthritis (final Listings 14.08M4 and 114.08N4) if it is resistant to treatment or requires hospitalization or intravenous treatment 3 or more times in 1 year. These criteria were developed based on the information we obtained.

Some of the HIV manifestations that commenters suggested as additions to the listings may be evaluated under existing listings; consequently, we did not add new criteria for them. These include: oral candidiasis (which is evaluated under final Listings 14.08F and 114.08F, Conditions of the skin or mucous membranes, or 14.08M and 114.08N, for other multiple infection, or under the appropriate body system listing); leukemia (which is evaluated under the criteria in Listing 7.11, 7.12, 13.27, or 107.11); giardiasis (which is evaluated under final Listings 14.08J and 114.08J); and pancytopenia (which is evaluated under final Listings 14.08G and 114.08G or under the criteria in 7.00ff and 107.00ff).

Syphilis and neurosyphilis are also manifestations that may be evaluated under existing listings. However, because of their frequency in individuals with HIV infection, we added Listings 14.08A4 and 114.08A4 to remind adjudicators that HIV infection can make this illness more difficult to treat and to ensure that they look for sequelae of the disease. For the same reason, we added Listings 14.08D5 and 114.08D5 for evaluating viral hepatitis. We did not distinguish in the final listings between CMV hepatitis and other forms; therefore, CMV hepatitis is included under these final listings.

The NPRM included criteria for evaluating various malignant neoplasms. Final Listings 14.08E and 114.08E are expressly for the evaluation of malignant neoplasms. The NPRM also included criteria for renal failure, in proposed Listing 14.08L. The general term "nephropathy" means disease of the kidneys and would, therefore, encompass renal (i.e., kidney) failure. Nephropathy is now included in both the adult and childhood listings at final Listings 14.08L and 114.08M, which are cross-references to the criteria in 6.00ff and 106.00ff.

We did not adopt the suggestions to add listing criteria for the following manifestations of HIV infection because the manifestations are either symptoms, signs, or medical findings that must be evaluated based on the underlying medical condition: dyspnea, sleep disorder, or fatigue.

We did not adopt the suggestion to include criteria for squamous cell carcinoma of the genitals because the condition is not necessarily disabling, even in an individual with HIV infection, and may be evaluated under the listings for malignant neoplasms in 13.00 and 113.00 or as other skin conditions under the criteria in final Listings 14.08F and 114.08F.

Likewise, HIV myositis and arthritis are not necessarily disabling in individuals with HIV infection, and these disorders may be evaluated under existing criteria in 1.00ff. HIV myositis may also be evaluated under the criteria in final Listings 14.05 and 114.05, and septic arthritis under the criteria in final Listings 14.08M and 114.08N.

The NPRM included criteria for lymphocytic interstitial pneumonia (LIP) in children. We did not adopt the suggestion to add criteria for adults because the condition is uncommon in adults, is usually accompanied by other manifestations of HIV infection, and would likely cause respiratory symptoms that could be evaluated appropriately under 3.00ff., or under final Listing 14.08N.

The term "recurrent cystitis" describes many different types of bladder inflammation that occur commonly in individuals who have HIV infection and individuals who do not. Evaluation under the listings will depend on the type of inflammation (e.g., bacterial cystitis may be evaluated under final Listings 14.08A5 and 114.08A6). Separate criteria for cystitis are not warranted because the condition is often not functionally limiting. If it is, and if it does not meet the criteria of any of the stand-alone medical listings, it may still meet the criteria of the functional listings, 14.08N and 114.08O.

In response to the comment about the side-effects of antiretroviral therapy, we supplemented the discussion of the effects of treatment in final 14.00D7 and 114.00D7, to make it clearer that we always consider the effects of treatment when evaluating disability. We have included "antiretroviral agents" as an example of treatment in these sections.

It is important to remember that any severe HIV manifestations not specifically included in the listings (including any of the manifestations discussed above that we declined to add) may still be evaluated based on their functional consequences under final Listings 14.08N and 114.08O, or at later steps of the sequential evaluation processes for adults and children.

Comment: A few commenters questioned whether the HIV infection listing adequately considered the effects of mental disorders such as depression or anxiety, which are common among HIV-infected individuals. They expressed concern that an individual who had HIV infection would nevertheless have to meet a specific mental disorder listing without consideration of the factors of HIV infection and its symptoms. Some commenters suggested that we add depression and anxiety as manifestations of HIV infection.

Response: We agree that many individuals with HIV infection display signs and symptoms of mental disorders, such as anxiety and depression. In some cases, this is a reaction to the condition, similar to that of many individuals afflicted with other serious disorders, such as cancer or heart disease, and may be a mental disorder in itself. In some cases, the mental findings may be manifestations of the underlying HIV infection. For example, mental signs associated with HIV encephalopathy are, of course, manifestations of the illness. Some people who have HIV infection may have mental disorders that are unrelated to the HIV infection but nevertheless contribute to their limitations; for example, individuals who abuse drugs may have a mental disorder related to their use of drugs.

However, regardless of whether the mental findings are signs or symptoms of an underlying disorder, mental impairments in and of themselves, or symptoms of mental impairments, can vary in their severity and impact on each individual's functioning. We, therefore, believe that it is appropriate to evaluate these kinds of mental findings either under our mental listings or under final Listings 14.08N and 114.08O, in both of which we are required to consider their impact on the person's functioning. The mental listings contain criteria not only for the evaluation of depression and anxiety disorders (Listings 12.04, 12.06, 112.04 and 112.06) but other disorders that include these findings among their signs and symptoms. Moreover, Listings 12.02 and 112.02, Organic mental disorders, are listings specifically for people who experience psychological or behavioral abnormalities associated with organic brain dysfunction. Therefore, these listings would include mental manifestations caused by HIV.

We also repeat that the test of disability involves much more than a requirement that an impairment meet (or equal in severity) any listing, and that disability may also be established at the last steps of the sequential evaluation processes.

Comment: Many commenters suggested including listing criteria for genital ulcers or genital herpes. Some suggested specific listing criteria, such as chronic genital ulcers; chronic genital ulcers persisting for more than 1 month; chronic gential ulcers that fail to respond to treatment and persist for more than 4 weeks; chronic genital ulcers caused by a sexually transmitted disease that fail to respond to treatment and persist for more than 4 weeks; recurrent herpes simplex; recurrent herpes with lesions that have not been documented to last 4 weeks, but that recur more often than every 8 weeks or that are incompletely suppressed despite continuous maintenance therapy.

Other commenters simply identified the conditions they thought should be included (e.g., genital herpes), without describing any particular level of severity.

Response: As we noted above, we considered both possible interpretations of these comments; i.e., that the commenters thought the mere existence of the condition was sufficient to establish disability or that the commenters thought we could devise severity criteria. To the extent that the commenters were suggesting that we include these conditions without additional criteria describing impairment severity (such that any individual with HIV infection and genital ulcers would have an impairment that meets the listings), we did not adopt the suggestions. Although genital ulcerative disease can be of disabling severity, it is not necessarily disabling. Consequently, the assessment of severity must be based on criteria beyond the mere presence of the disease.

Some of the comments demonstrated that our proposed criteria for Herpes simplex (proposed Listings 14.08A5, 14.08E2, 114.08A5, and 114.08E2) were not clear. (Many commenters recommended criteria that were essentially the same as the criteria we proposed.) Therefore, we reorganized the proposed listings (which became final Listings 14.08D2 and 114.08D2) to make it clearer that genital ulcers caused by Herpes simplex that persist for 1 month or longer meet the criteria of the listing. We did not adopt the suggestion to include Herpes simplex infection that does not last for 1 month, but recurs, because recurrence alone is not a reliable indicator of impairment severity; an individual with recurrent minor lesions of short duration may be completely unimpaired. Recurrent manifestations of HIV infection may be evaluated based on the functional consequences of the disorder in final Listings 14.08N and 114.08O.

In further response to these and other comments, we also developed general criteria in final Listings 14.08F and 114.08F for conditions affecting the skin and mucous membranes, which include genital ulcerative disease. For reasons we have already given in the explanation of the final rules, the criteria are based on the severity of the resulting lesions ("with extensive fungating or ulcerating lesions") and the response to treatment ("not responding to treatment").

We did not adopt the suggestion to include criteria limiting the evaluation to ulcers caused by a sexually transmitted disease, or the suggestion to require that the conditions be both resistant to treatment and of a specific duration. Adopting these suggestions would have resulted in an unnecessarily restrictive listing.

HIV Manifestations Specific to Women

Comment: Many commenters suggested adding criteria for evaluating pelvic inflammatory disease, often called PID. They suggested various medical criteria for describing listing-level pelvic inflammatory disease, including: pelvic inflammatory disease resulting in severe pain; recurrent or refractory pelvic inflammatory disease; pelvic inflammatory disease that is persistent or resistant to treatment; pelvic inflammatory disease of more than 1 month's duration that does not respond to treatment; pelvic inflammatory disease with a specific number of episodes (e.g., three or more episodes); pelvic inflammatory disease with one episode requiring hospitalization; pelvic inflammatory disease with one episode requiring pelvic surgery; pelvic inflammatory disease with one episode resulting in documented chronic pain syndrome; or some combination of the above.

Response: We responded to these comments by developing stand-alone medical criteria that may be used to evaluate pelvic inflammatory disease in final Listings 14.08A5 and 114.08A6. We included pelvic inflammatory disease in the childhood listings because there are many adolescent girls who have the disease. Although we did not fully adopt any one of the suggestions for specific criteria to describe listing-level severity, we derived our criteria from many of the suggestions.

We did not adopt some of the specific suggestions because they did not represent listing-level severity. For example, we did not include a blanket rule for pelvic inflammatory disease requiring surgery because pelvic inflammatory disease (whether in the general population or in individuals with HIV infection) usually responds to surgical treatment and, therefore, will not always meet the statutory duration requirement. Moreover, a single episode of pelvic inflammatory disease requiring hospitalization is not an accurate predictor of continuing impairment severity because individuals often recover satisfactorily from such an isolated episode.

The criteria in these final rules (i.e., pelvic inflammatory disease requiring hospitalization or intravenous antibiotic treatment 3 or more times in 1 year) are similar to a number of the commenters' suggestions (e.g., recurrent or refractory pelvic inflammatory disease; pelvic inflammatory disease that is persistent or resistant to treatment; pelvic inflammatory disease of more than a month's duration that does not respond to treatment; pelvic inflammatory disease with a specific number of episodes). The criteria are also based on the same premise as those suggestions—that disability from pelvic inflammatory disease can be measured most accurately by the persistence and severity of the infection. We believe that the final rules are less stringent than some of the commenters' suggestions, especially those that require more-or-less continuous disease. The final rules may be used to evaluate claims filed by women and girls who may recover from bouts of infection, but who suffer from repeated infections, or who may have their infections controlled for a time only to suffer exacerbations.

The criteria in final Listings 14.08A5 and 114.08A6 do not apply only to pelvic inflammatory disease, but to any other multiple or recurrent bacterial infections requiring hospitalization or intravenous antibiotic treatment 3 or more times in 1 year. Bacterial infections, including pelvic inflammatory disease, that do not meet these criteria but that may be disabling because of pain, chronic illness, or other symptoms and signs may also be evaluated under the functional criteria in final Listings 14.08N and 114.08O.

Comment: Many commenters recommended that we revise the proposed listing-level criteria for invasive cervical cancer, FIGO stage II, in proposed Listing 14.08J2. Some suggested that we use stage IB because cancer at that stage usually requires the same treatment as cancer at stage II (i.e., surgery and radiation therapy). Other commenters suggested stage I (without indicating IA or IB), or made no specific recommendation.

In addition, some commenters recommended that we allow evaluation of cervical cancer not yet at FIGO stage II under the functional test in proposed Listing 14.08M3.

Response: We did not adopt the recommendations to list cervical cancer less than FIGO stage II as a stand-alone listing. Impairment severity in the case of malignant tumors is assessed by considering the site of the lesion and extent of involvement, histogenesis of the tumor, adequacy of and response to treatment, and any post-therapeutic residuals. We chose FIGO stage II as the listing-level criterion for cervical cancer as a manifestation of HIV infection because that is the minimal point at which the cancer has advanced beyond the cervix. In FIGO stage I, the cancer is confined to the cervix—stage IA indicates cancer that can only be seen microscopically, and stage IB indicates a larger amount, deeper in the tissues of the cervix, but still confined to the cervix. In stage II, however, the cancer has spread beyond the cervix into the uterus or upper vagina. Stage I (including IB) cervical lesions are usuallyamenable to treatment, even in individuals with HIV infection.

The fact that the recommended treatment is the same for stages IB and II may have clinical significance, but it says little about the potential for ongoing functional restrictions.

Our revisions in final Listings 14.08N and 114.08O address the suggestion to evaluate cervical cancer of a severity less than FIGO stage II at the listing level in conjunction with functional restrictions. As we have already explained, final Listing 14.08N allows for a finding that manifestation of HIV infection (including cervical cancer not meeting the criteria in Listing 14.08J) may be found to meet the listing based on the functional consequences of the impairment.

Comment: Many commenters identified other manifestations of HIV infection that they considered disabling to women, and suggested that we include those manifestations in Listing 14.08. They cited many of the same manifestations that commenters suggested as general additions to the adult listings (which we have already discussed above), or as conditions that should not have been tied to the functional criteria in proposed Listings 14.08M, 114.08L, and 114.08M (also discussed above). They also suggested that we add abscess of an internal organ or body cavity, cervical dysplasia, chronic headaches, vulvovaginal candidiasis, human papillomavirus, and vaginal condyloma. (As noted previously, when a comment suggested adding one of these manifestations to the listing but did not include criteria describing impairment severity, we analyzed both possible interpretations of the comment.) One commenter suggested extensive revisions in the tenth, eleventh, and twelfth paragraphs of proposed 14.00D, the proposed paragraphs discussing the evaluation of HIV infection in women. The commenter provided specific language for such revisions.

Response: We have added to the final listings most of the conditions suggested by the commenters by drafting specific criteria describing listing-level severity for a wide range of HIV-related conditions common in women. We could not, however, adopt the suggestions to include these conditions without additional criteria describing impairment severity. None of the conditions suggested are necessarily disabling solely by virtue of being present with HIV infection.

We considered all the criteria the commenters suggested for describing impairment severity, but decided to draft original criteria based on the suggestions and on other information about the severity and consequences of the conditions. In many cases, the criteria we decided to use are similar to the suggested criteria. For example, a comment suggested adding vulvovaginal candidiasis of more than 1 month's duration that does not respond to therapy; we decided to include all skin and mucosal conditions with extensive ulcerating lesions not responding to treatment in final Listings 14.08F and 114.08F. Whenever we decided to use criteria significantly different from that suggested by the commenters, we did so based on what is known about the severity and consequences of the conditions.

Final Listings 14.08F and 114.08F include criteria for vulvovaginal candidiasis and condyloma caused by human papillomavirus. Because these conditions can affect both adults and children, especially adolescent children, we incorporated the criteria into both part A and part B of the listings.

Although abscesses of an internal organ or body cavity are not specifically referred to in the final rules, they may be evaluated under final Listings 14.08A5 and 114.08A6, which apply to multiple or recurrent bacterial infections.

We did not adopt the suggestions to include criteria for cervical dysplasia or headaches. Cervical dysplasia is a clinical finding, a deviation from normal in the cells in the lining of the cervix, which may or may not cause symptoms or progress to a more serious condition. We did not list it as a separate condition because, although clinically meaningful, dysplasia alone does not necessarily result in functional limitation, and evaluation of such a condition will depend on its impact on the individual on a case-by-case basis. Headaches are symptoms that may be associated with a wide range of medical conditions, and should be evaluated according to the underlying condition and our rules for the evaluation of symptoms, including pain, in §§ 404.1529 and 416.929, which we have recently updated and made more detailed.

In the final rules, we deleted the paragraphs the last commenter asked us to edit because vulvovaginal candidiasis, genital herpes, and pelvic inflammatory disease are now specifically included in the final listings as stand-alone medical conditions. Based on these revisions, the additional language suggested by the commenter was not needed. The guidance in final 14.00D5, Manifestations specific to women, is more general and addresses issues of evaluation instead of specific manifestations.

Comment: A comment suggested that we add a discussion of HIV infection in pregnant women to the preface and that we use different listing criteria for pregnant women. The comment said that immunological alterations associated with pregnancy and the fact that the CD4 count typically decreases during pregnancy raise the possibility that HIV infection could be accelerated. For example, pregnant women may develop opportunistic infections when their CD4 counts fall below 300.

Response: We did not adopt the comment. We agree that medical literature reports that the rate of CD4 cell loss in HIV-infected pregnant women is faster than in HIV-negative pregnant women or HIV-infected men. However, as we state in final 14.00D4a and 114.00D4a, a CD4 count in itself is not an indicator of the severity of the HIV infection or its functional effects, or a reliable predictor of when manifestations will occur. If pregnant women develop manifestations of HIV, we will evaluate them in the same way that we do in other women, examining the particular effects of their conditions on a case-by-case basis.

Comment: Another comment noted that we had included gynecological conditions associated with HIV infection and functional limitations in the proposed listings. The comment said that, since the conditions are also prevalent in HIV-negative women, we should add listings for gynecological conditions associated with conditions other than HIV infection, and resulting in functional limitations.

Response: We did not adopt the comment, which was beyond the scope of these rules. However, in evaluating the claim of a woman with or without a compromised immune system under the listings, we will consider whether the medical findings for any gynecological impairment, in combination with other impairments or standing alone, are listed or are medically equivalent in severity to the findings for the most closely analogous listed impairment.

The Childhood Listings: Other Comments

General

Comment: A number of commenters expressed concern that the proposed childhood HIV listings did not adequately reflect the course of the disease in children, but were merely an extension, with minor changes, of the adult HIV listings. One comment recommended that we limit the childhood HIV listings to those aspects peculiar to children that are not covered by the adult HIV listings.

Response: We partially adopted the comments, even though it is not true that the proposed childhood listings were only an extension of the adult listings. It is simply a fact that many of the manifestations of HIV infection in children are the same as those in adults. Although the course of these manifestations may differ somewhat in a child, in most instances the mere existence of a manifestation is sufficient to establish listing-level severity. For that reason, there was no need to provide criteria distinguishing the childhood manifestations from criteria in the adult rules. Where the differences did matter—for instance, in proposed Listing 114.08F (final Listing 114.08A5) (for two pyogenic bacterial infections in 2 years) and proposed Listing 114.08J (final Listing 114.08H) (for HIV encephalopathy)—we proposed criteria that recognized these differences.

However, we agree with the general suggestion to make the childhood listings better reflect the course and manifestations of the disease in children, and have revised the final listings accordingly. We revised the discussion about the course and manifestations of HIV infection in children in final 114.00D5, deleted most cross-references to the adult rules, and provided more listing criteria that describe the unique presentation of some manifestations in children. We describe the listings changes in other comments and responses, below.

The final childhood listings still contain many of the same criteria as the adult listings because they are appropriate to the evaluation of both adults and children. We included these criteria in both listings, as we do in many other body systems, to ensure the public understands the rules and to increase ease and accuracy of adjudication by decisionmakers. Indeed, we have added several new listings to the childhood listings that are the same as adult listings—such as listings describing manifestations that affect women—because we believe that it is not self-evident that many children (especially adolescents) are unfortunately in the same risk groups for HIV infection as many adults and, therefore, suffer from the same manifestations.

Documentation

Comment: One comment stated that the HIV evaluation criteria for children in the proposed rules were too vague to be properly applied.

Response: We have responded to the comment by clarifying 114.00D3 and 114.00D4 of the final rules, the documentation standards for evaluating children with HIV infection, final 114.00D6, Evaluation of HIV infection in children, and final 114.00D7, Effect of treatment.

Evidence of HIV Infection

Comment: We received many comments about our proposal in the fifth paragraph of proposed 114.00C to use CD4 (T4) lymphocyte counts to establish the existence of HIV infection. Some commenters agreed with the proposal that CD4 counts of 1500/mm3 or less or 20 percent or less are evidence of HIV infection for children from birth to age 1. A few commenters believed that a CD4 count of 1000/mm3 or less should by itself be evidence of HIV infection for children 12 to 15 months of age. Other commenters said that CD4 counts of 750/mm3 or less should be evidence of HIV infection for children 12 to 24 months of age.

One comment suggested that we provide language discussing the change in CD4 counts with age.

Some commenters believed that CD4 counts of 750/mm3 or less should be the standard for children 1 to 15 years of age. One comment said that the CD4 counts used in the childhood listings were not consistent with CDC guidelines.

Response: We partially adopted the comments. As we make clear in 114.00D3, antibody testing for HIV infection is not definitive in young children because the mother's antibodies can persist in a child up to 24 months of age, even if the child is not infected. Therefore, we need to include criteria that would help identify when infants who test positive for HIV antibodies are actually infected. CD4 counts alone are generally not used to definitively diagnose HIV infection in children, in part because there is still some debate in the medical community about what the norms for CD4 counts in children should be. However, the CD4 counts in these rules are used by the medical community to begin prophylaxis for Pneumocystis carinii pneumonia, and are sufficiently suggestive in an infant who has tested positive for HIV antibodies to presume the existence of HIV infection.

Because of the continuing debate about the norms in children, we cannot adopt the recommendation to use a higher CD4 count for children age 12 months to 15 months of age. However, even though we have not increased the CD4 count threshold in the final rules, we have extended the age range for CD4 counts of 750/mm3 or less to cover children up to 24 months of age to make them consistent with the CDC guidelines for prophylaxis, in response to some of the comments, and based on other information we received. We also added two additional ways of establishing the presence of HIV infection in response to a comment we summarize below.

We did not extend the use of CD4 counts to aid in the diagnosis of HIV infection in children age 2 years or older because antibody testing is definitive in these children.

Comment: One comment suggested that we find infants who have HIV antibodies automatically eligible until such time as their infection status can be definitively established. Another comment suggested that we establish a listing that would allow for a finding of disability for a child between birth and age 15 months who has HIV antibodies and exhibits failure to thrive, diffuse lymphadenopathy, or any form of candidiasis. The commenters stated that the presence of HIV infection in young children can be difficult to confirm through laboratory testing, which can be expensive and may be inconclusive.

Response: We did not adopt these suggestions because the Act requires that disability be established in order for the claimant to receive benefits. As one medical organization that submitted comments noted, only about one in three infants born with HIV antibodies actually has HIV infection.

However, in response to these and other comments, we revised final 114.00D3 to allow HIV infection to be documented based on medical history, clinical and laboratory evidence (other than the laboratory evidence that definitively diagnoses the impairment), and diagnoses. The documentation must be consistent with the prevailing state of medical knowledge and clinical practice and consistent with the other evidence. Thus, a diagnosis of HIV infection could be established under the final rules for a child who has HIV antibodies and exhibits failure to thrive, diffuse lymphadenopathy, or any form of candidiasis. However, we cannot make a blanket statement that this would, or should, always be the case, because diagnoses of HIV infection in such cases rely on clinical judgment and the documented facts of the individual case. For example, oral candidiasis (oral thrush) is a very common condition in babies. If this were the only finding in an infant with HIV serum antibodies, a doctor would have to make a judgment, based on such factors as the severity, frequency, duration, and response to treatment of the infection, and whether there are other accompanying clinical findings, to decide whether the infection is a routine infection of infancy or a sign of HIV infection.

In addition, even if the suggested signs result in a presumed diagnosis of HIV infection, this alone would not speak to the severity of the manifestations or their effects on the child's ability to function. HIV infection alone, without any serious manifestations, will seldom interfere with a child's ability to function.

Once HIV infection is documented, the child, like any person with HIV infection, can be found disabled if his or her manifestations satisfy, or are equivalent in severity to, the criteria in any of the HIV listings or other listings appropriate for the evaluation of the manifestations. If the impairment(s) of a child claimant for SSI does not meet or equal in severity any listing, the effects of the impairment(s) on the child's ability to function will be evaluated at the last step in the sequential evaluation process for children.

In addition, it is important to remember that we consider all the impairments the child has, whether related to HIV or not. Thus, if the child could be found disabled on some other basis, e.g., a child less than 1 year of age who weighed under 1200 grams at birth, consideration of HIV infection would not be necessary.

Comment: One comment suggested that we include abnormal CD4/CD8 ratios and immunoglobulin G (IgG) levels greater than or less than the normal range for age as laboratory evidence of HIV infection in children.

Response: We adopted the comment in final 114.00D3b(iii) and (iv). These laboratory findings are acceptable documentation of the existence of HIV infection in children up to age 24 months who have serum antibodies for the HIV.

Comment: One comment suggested language to revise the fifth paragraph of proposed 114.00C to expand the discussion about the transmission of HIV antibodies and HIV infection from mother to child and the significance of CD4 counts. The comment suggested adding information about the low prenatal and natal HIV transmission rate to infants, and the duration of HIV antibody persistence, and put the list of laboratory findings in a separate paragraph.

Response: We modified and adopted the suggested language in final 114.00D3a and b.

Comment: Another comment noted that, although proposed 114.00C stated that the mean age of diagnosis of children infected before or shortly after birth is 17 months, various mean ages of diagnosis of HIV infection have been determined and diagnosis is often made earlier.

Response: We have adopted this comment by removing the language concerning the mean age of diagnosis of children infected before or shortly after birth. Final 114.00D3b permits HIV infection to be documented in children from birth to the attainment of 24 months of age based on any of four specific laboratory findings, or based on documentation consistent with the prevailing state of medical knowledge and clinical practice.

Comment: Several commenters said we should delete the language in the ninth paragraph of proposed 114.00C describing how pediatric populations may contract HIV because it was inappropriate and irrelevant to the purpose of disability determination.

Response: We adopted the comment.

Symptoms and Response to Treatment

Comment: One comment said that the criteria incorrectly assumed that children will adequately express and document pain, fatigue, complications and/or reactions to therapy.

Response: We recognize that some children may have a limited ability to report history, symptoms, and other information, but we do not believe that this will have an adverse effect on their claims. Most of the listings in final 114.08 do not include symptoms among their criteria; rather, the criteria consist of clinical signs and laboratory findings that will be documented in the child's medical records. Furthermore, our experience in processing childhood disability claims has shown that a child's symptoms will generally be observed by a parent or other caregiver who will provide this information to the physician and to us.

Although some children may not be able to verbally describe their symptoms, these symptoms may be expressed in other ways, such as otherwise unexplained changes in demeanor, behavior, eating habits, and sleeping habits. These changes would be readily discernible to the child's parents or other caregivers, a physician or other professionals experienced in evaluating and treating children, as well as to other people who see the children, such as relatives, teachers, social workers, and ministers. Older children should be more able to express their symptoms and any adverse effects of treatment, if this information is needed for adjudication.

Adolescents

Comment: Many commenters requested that we eliminate the proposed criteria that distinguished between children under age 13 and over age 13. Many of the commenters questioned our statements in the ninth paragraph of proposed 114.00C that the course and spectrum of disease in children age 13 and older is generally similar to that of adults, and that older children with HIV encephalopathy and HIV wasting syndrome should be evaluated under the appropriate adult listings. One comment asserted that scientific and medical literature point to distinctive differences between the course and spectrum of HIV infection in adolescents and adults, and referred us to the "Journal of Pediatrics," Volume 119, July 1991, Number 1, Part 2, titled "Guidelines for the Care of Children and Adolescents with HIV Infection. Report of the New York State Department of Health AIDS Institute Criteria Committee for the Care of HIV-Infected Children."

Response: We partially adopted the comments. Our statement in the ninth paragraph of proposed 114.00C that the course and spectrum of the disease in children age 13 and older is the same as in adults was correct and was confirmed by various pediatric authorities, including some who specialize in the study and treatment of adolescents. We disagree with the comment suggesting that the scientific and medical literature supports a contrary view. Indeed, the article cited in the comment does not say that there are significant differences between adolescents and adults in the manifestations of HIV infection; it says that there are differences in epidemiology—i.e., the modes of disease transmission. Our disability determination, however, is based on the effects of the disease on a child's ability to function in an age-appropriate manner, not on how the child acquired HIV disease.

Nevertheless, in response to the comments we deleted the statement about the course and spectrum of the disease in adolescents, and revised the statement (now in final 114.00D5) about the manifestations and course of disease in younger children. The proposed statement about the disease in adolescents did not provide guidance that was especially relevant to the determination of disability and, therefore, was superfluous.

Comment: A number of commenters thought that it was more difficult for some children with HIV infection to qualify for disability than it was for children with other impairments. The commenters gave as an example a child over age 13 with HIV encephalopathy. The ninth paragraph of 114.00C of the proposed rules had indicated that such a child should be evaluated under proposed adult Listing 14.08G (which, in turn, cross-referred to criteria in the eighth paragraph of 14.00D), and would have required the child to show progressive motor dysfunction and the absence of a concurrent illness. The commenters suggested that this proposed listing was more severe than the children's neurologic Listing 111.06, which the commenters thought requires only interference with age-appropriate major daily activities.

Response: We do not agree that any of the proposed listings made it more difficult for children with HIV infection to qualify for disability than children with other impairments, for reasons we have already given in an earlier comment and response.

The proposed criteria for HIV encephalopathy for children were not more stringent than Listing 111.06. The criteria in the eighth paragraph of proposed 14.00D, which would have been applied to children, required only that there be HIV encephalopathy "characterized by" cognitive or motor dysfunction that limited function and progressed, and that there not be a concurrent illness that could otherwise explain the neurological findings. Thus, the criteria only defined the syndrome of HIV encephalopathy; that is, how one can tell that a person has HIV encephalopathy without invasive testing. Childhood Listing 111.06, on the other hand, requires more than mere interference with age-appropriate activities; it requires persistent disorganization or deficit of motor function involving two extremities that, despite prescribed therapy, interferes with age-appropriate major daily activities and results in disruption of fine and gross movements or gait and station.

However, as we have already said, we believe that the proposed criteria for evaluating HIV encephalopathy in children could be simplified because they appeared only in proposed 114.00C, not in the listing, and required a cross-reference to an adult listing that itself cross-referred to 14.00D of the adult rules. Therefore, we revised final Listing 114.08H (which replaces proposed Listing 114.08J) to include HIV encephalopathy and criteria specifically for children. We also provided guidance in final 114.00D5 specifically for the evaluation of neurological abnormalities, such as HIV encephalopathy, in children. We also deleted the requirement for ruling out other causes, as we did in the corresponding adult rule.

Comment: Some comments questioned our proposals in Listings 114.08F and 114.08G to limit the criteria for multiple bacterial infections and lymphoid interstitial pneumonia/pulmonary lymphoid hyperplasia to children under age 13. Similarly, some comments questioned the proposal to pair different manifestations with functional requirements, for the two age groups in proposed Listings 114.08L and 114.08M.

Response: We adopted most of the comments. We eliminated the age reference in final Listing 114.08L, Lymphoid interstitial pneumonia/pulmonary lymphoid hyperplasia, so that it now applies to children of all ages. We had proposed the distinction only because the manifestation is quite rare in older children, as it is in adults. However, it is possible that an older child could have the disorder, especially as more and more children who contracted HIV perinatally or early in life are surviving into adolescence. For reasons we have already given, however, we have also revised final Listing 114.08L to better describe listing-level severity.

The functional listing, final Listing 114.08O, which replaces proposed Listings 114.08L and 114.08M, no longer lists specific medical manifestations. Therefore, there is no longer a need to distinguish between adolescents and younger children.

We have retained the age limit in final Listing 114.08A5, Multiple or recurrent pyogenic bacterial infections, because these types of infections are more serious and more indicative of a rapid decline in younger children, and age 13 is medically an appropriate dividing line. Although we could have confined the rule to younger children, we decided to retain the rule because age 13 is fair and consistent with prevailing medical practice, and we want these listings to be as inclusive as possible. Furthermore, unlike the proposed rules, the final rules include a new Listing 114.08A6 under which all children, including adolescents, may establish that they have impairments of listing-level severity as the result of multiple bacterial infections of any type.

Comment: Another comment recommended that we address the special needs of adolescents with HIV infection, including feelings and fears regarding HIV testing, effective ways of counseling adolescents, coping strategies of adolescents with HIV infection, and the role of social support in the lives of adolescents with HIV infection. The comment also recommended that we establish a group of experts within SSA to focus on the specific needs of adolescents with HIV infection.

Response: We share these concerns about the impact of HIV infection on adolescents. However, the recommendations involve areas of social services policy that are beyond our authority under the Act and, thus, cannot be addressed within the context of these rules.

Final 114.08H Neurological Manifestations

Comment: One comment recommended that we add "the sudden acquisition of new learning disabilities" as a fourth criterion in proposed Listing 114.08J (final Listing 114.08H).

Response: We adopted the comment. We added language to the second paragraph of final 114.00D5 and a parenthetical statement in final Listing 114.08H1 (which replaces proposed Listing 114.08J1) to state clearly that the loss of previously acquired, or marked delay in achieving, developmental milestones or intellectual ability, includes "the sudden acquisition of a new learning disability." This addition is only a clarification of our original intent in the proposed rules.

Final 114.08I Growth Disturbance

Comment: Many commenters suggested we clarify our criteria for assessing failure to thrive. Some commenters thought we were using the height criteria specified in Listing 100.02 to assess failure to thrive under proposed Listing 114.08K. The commenters indicated that, because the term "failure to thrive" generally refers to infants and children who fail to gain weight at an appropriate rate or who lose weight, the listing should contain criteria based on weight.

Other commenters stated that the 10 percent weight loss required by proposed Listings 114.08L and 114.08M, which was the same standard used in the adult HIV listings, was too strict. They pointed out that a standard of weight loss can make sense for adults because adults are fully grown and are expected to maintain a static weight. However, because children are growing, it is possible for a child to be gaining weight but falling behind what is normal, so that the resulting impairment would be as severe as a serious weight loss. The majority of these commenters suggested using a 5 percent weight loss as a standard for children. Another suggestion was to base our criteria on a failure to follow age-appropriate growth curves on standard growth charts.

Response: We adopted several of the comments. We revised final Listing 114.08I, which is now headed "Growth disturbance" to include weight criteria for failure to thrive in addition to the height criteria. The first two criteria of final Listing 114.08I describe children who have either lost weight or who have failed to gain weight at an appropriate rate, so that there is persistence of a fall of 15 percentiles on a standard growth chart or persistence of weight below the third percentile on a standard growth chart. We have determined that this approach provides a more accurate method of assessment than basing our criteria solely on a percentage of weight loss because, as the commenters stated, children can, in fact, be gaining weight and still be failing to thrive.

We have, however, also retained the criterion of a 10 percent weight loss in final Listing 114.08I3 (formerly in proposed Listings 114.08L and 114.08M) because in some cases 10 percent weight loss will still be less than 15 percentiles on a standard growth chart or result in a weight above the third percentile. We believe that a 5 percent weight loss would be too small to be a reliable standard in the listings, and that children with this amount of weight loss will have to be evaluated on an individualized basis under the rules for equivalence and the last step of the sequential evaluation process.

We have also retained the rules providing for loss of height or length, as described in the growth impairment listings in 100.00. Both the 10 percent weight loss provision and the cross-reference to the growth impairment listings merely provide alternative criteria by which children may be found disabled under the listings.

Comment: A number of commenters were also concerned about assessing HIV-related growth impairments in children by reference to the criteria of Listing 100.02. The commenters said that Listing 100.02 defines when a growth impairment is disabling in itself and not because HIV infection has interfered with growth. The commenters also questioned whether the longitudinal approach required by Listing 100.02 is appropriate for children with a progressive disease such as HIV infection.

Response: Listing 100.02 is appropriate to use because it is a listing for evaluating growth impairment caused by a known medically determinable impairment, such as HIV infection. It is, thus, a very appropriate listing for evaluating growth impairment caused by HIV infection. (However, we revised the reference to 100.00ff for consistency with our other revisions.) In any event, by expanding final Listing 114.08I, we have made the reference to the growth impairment listing only one alternative among four by which a child's impairment may meet the listing, not the sole criterion as in the NPRM.

We believe the longitudinal approach required by the growth impairment listings is reasonable. Multiple measurements are needed to properly assess the decline in the child's growth and its persistence.

Final 114.08O1: The Functional Criteria for Infants

Comment: One comment objected to the criteria in Listing 112.12, the description of functional deficit we used to describe listing-level severity for infants from birth to age 1 in proposed Listing 114.08L3. The comment stated that our standard of one-half chronological age for these children appeared to be more restrictive than the standard for older children and adults, especially considering how quickly infants change over time. Also, the comment suggested that impairment at the level specified need only be documented at one assessment.

Response: The functional standard for children from birth to the attainment of age 1 (and for many children age 1 to the attainment of age 3), now in final Listing 114.08O, is not more restrictive than the standard used for older children and adults. In the Mental Disorders listings, older children and adults are found disabled at the listing level if their impairments result in marked limitations in two areas of functioning. In Listings 112.12A and B (and in Listings 112.02B1 for children age 1 to 3), however, a young child has an impairment that meets the functional requirements of the listings if he or she has either one "extreme" limitation or two "marked" limitations. An extreme limitation may result when the function or developmental milestone is limited to no more than one-half the child's chronological age, while marked limitations result with less severe limitations—more than one-half but no more than two-thirds of the child's chronological age.

For this reason, we provide two ways for children from birth to the attainment of age 3 to establish listing-level severity. Under the functional criteria in Listings 112.02B1a, b, and c, and in Listings 112.12A and B, children can establish that their impairments are of listing-level severity by showing functioning or delays at no more than one-half of their chronological age. Alternatively, under Listings 112.02B1d and 112.12E, they can establish listing-level severity in the same way that older children and adults do: by showing marked impairment—i.e., functioning at more than one-half but less than two-thirds of chronological age—in two functional areas.

We recognize the problems involved in assessing infants, who do change rapidly over time. Because of this, we cannot state that determinations of disability can always be based on a single evaluation. The amount of evidence needed for each claim has to be determined based on the facts of that specific claim, which include the nature and progression of the impairment, the interventions and treatments available, the response to those interventions and treatments, and—perhaps most importantly—the individual infant's own response to the illness.

Other Comments

Error in Proposed Listing 14.08D

Comment: Several commenters pointed out that the 2-month timeframe set out in the ninth paragraph of proposed 14.00D for chronic diarrhea or documented fever caused by HIV wasting syndrome was longer than the 1 month required by the CDC's surveillance definition.

Response: The criterion in the NPRM was an editorial error. In fact, we have been using a 1-month standard in our operating instructions, consistent with the CDC surveillance definition of HIV wasting syndrome. We have corrected the final rule, which is in final Listing 14.08I.

Administrative Procedure Act

Comment: A few commenters expressed a concern that we had released guidelines on the evaluation of HIV infection in the form of a Social Security Ruling (SSR), in effect implementing the proposed rules in the NPRM in advance of public comments. Some commenters saw this as a breach of faith or a violation of the Administrative Procedure Act (APA).

Response: We have issued SSRs (SSRs 84-19 and 86-20) and manualized instructions concerning HIV infection on various occasions since 1983, as medical and scientific knowledge about this disease became available, to provide guidance to our decisionmakers concerning how claims involving HIV infection could be evaluated within the context of the law and regulations. On December 17, 1991, we published the latest of these instructions, an interpretative ruling, SSR 91-8p, in the FEDERAL REGISTER (56 FR 65498), to announce and to state our criteria for evaluating HIV infection. We have been applying this interpretive ruling in our adjudication of claims filed by of people with HIV infection. Since January 11, 1990, we have published SSRs in the FEDERAL REGISTER pursuant to the provisions of § 422.406(b) of part 422, of title 20 of the Code of Federal Regulations. Statements of policy in SSRs continue to be binding on all components of SSA, just as they have been since before the regulatory change in 1990 that provided for their publication in the FEDERAL REGISTER.

The purpose of these criteria has been to permit our decisionmakers to make findings of disability when a particular AIDS- or HIV-related condition could "meet" or "equal" a listing under the existing regulatory framework. If we had not published them but had waited for these final rules, we would have followed our prior instructions which, as we have stated, were not as inclusive as the criteria we published in SSR 91-8p. The effect would have been only to delay needlessly claims that we have now been able to allow.

Advisory Council

Comment: A number of commenters recommended that we convene a group of experts, an advisory council, or other knowledgeable specialists to evaluate and revise the proposed listings on HIV infection, and to regularly review the listings to keep the criteria for HIV-related diseases current. Some commenters also thought that the proposed rules for evaluating HIV infection in children did not reflect the expertise of childhood medical specialists. They pointed out that no childhood specialty groups, such as the American Academy of Pediatrics (AAP), or public interest advocacy groups were among the list of medical specialty groups listed in the NPRM as providing information in developing the HIV criteria in proposed Part B. They questioned whether any of the experts listed were pediatricians and whether they were independent of SSA. A few commenters also said the implementation of the proposed rules should be delayed until we consult with childhood HIV experts.

Response: We did not adopt the recommendation to establish an advisory council to assist us in preparing these rules. We solicited information from individual medical experts, including pediatricians, in developing the proposed rules. Establishing a separate group of experts following the publication of the NPRM would likely have duplicated many of the steps we had already undertaken and, most importantly, such duplication would have caused unnecessary delay in the publication of these final rules, to the disadvantage of claimants with HIV infection. Moreover, the public comments in response to the NPRM came from a broad spectrum of the medical, legal, and advocacy communities, and, hence, included some of the kind of input recommended by the commenters.

Nevertheless, and partly in response to the comments, we have sought additional information from a wide range of individual medical specialists. Other experts assisted us on an individual basis as we finalized these rules and responded to the comments.

With regard to the proposed rules for evaluating HIV infection in children, although we did not obtain information from the AAP during the development of the proposed rules, we did obtain information from pediatricians at Johns Hopkins Hospital, the Centers for Disease Control, and other Federal agencies, all of which were independent of SSA. Furthermore, during the process of developing the final rules, we obtained information from additional pediatricians and other individuals with knowledge and treatment experience in pediatric HIV infection in all childhood populations, including adolescents. Among these individuals were some recommended by members of the AAP and a physician to whom we were specifically referred by the AAP. Finally, the AAP, as well as other pediatric specialty groups and other children's advocacy groups, have submitted comments on the NPRM expressing their interest or concern about its content and publication. By submitting these comments, these groups have participated in the formulation of the final rules.

Timely Updates

Comment: A number of commenters responded to our request for suggestions on alternatives to our regulatory process consistent with the APA and that would enable us to issue timely updates to the listings for HIV infection (56 FR at 65704). One comment suggested that we develop a decisionmaking protocol, which would be subject to the normal regulatory process, that would establish a procedure for evaluating when changes would be appropriate in the listings. Other comments proposed that we create an ongoing advisory panel composed of a range of experts committed to assisting us in updating and refining these procedures in a timely fashion as medical knowledge on HIV improves.

Response: We appreciate these suggestions, and will give them further study. We will study whether any of the suggestions we received can be used given the constraints of the Act and our regulations. We have always attempted to update the medical listings to reflect advancements in medical technology, disability evaluation and treatment, and changes in knowledge and new disease processes. We monitor the listings on an ongoing basis to ensure that they continue to meet program purposes and, when changes are found to be warranted, the listings for that body system are updated through the normal regulatory process.

We recognize that the HIV listings may need to be changed as we learn more about the course of HIV infection in different populations, and as new tests and treatments are developed. We will update the listings as it becomes necessary, and will issue new instructions to our adjudicators as this becomes necessary.

Excessive Paperwork

Comment: A number of commenters were concerned that the proposed rules were complicated and would require too much paperwork on the part of health care providers and claimants to document a claimant's eligibility. They were also concerned that the proposed rules would not produce timely disability determinations, which would be harmful to individuals affected by HIV infection.

Response: We agree that paperwork and the effort required to establish a disabling impairment should be kept to a minimum. We have made changes in the final listings that will facilitate the documentation and adjudication of HIV claims. These changes include revising the criteria for documenting the existence of HIV infection and its manifestations to permit documentation of HIV infection or its manifestations in the absence of a definitive diagnosis and to permit a finding of "meets" for most of the impairments formerly tied to functional criteria in proposed Listing 14.08M when the medical evidence indicates listing-level severity. In addition, we give these claims priority handling.

Training

Comment: Several commenters expressed the need for extensive training for health care officials, physicians, advocates, Social Security personnel, and the general public.

Response: We agree, and have already begun a public awareness campaign and training initiative with respect to HIV including the design, printing, and distribution of brochures, television and radio public service announcements (in both English and Spanish), and video news releases. We are also working with the medical community, service providers, and advocacy groups to ensure that the important message about the potential for Social Security Disability Insurance (SSDI) and SSI benefits reaches those with HIV infection. We have also provided training to our adjudicators and will continue to provide training as necessary.

Trust Fund

Comment: A few commenters expressed concern about the cost of adding manifestations of HIV infection to the Listing of Impairments on the Social Security and health care financing systems.

Response: These final rules establish a listing for HIV infection to replace the adjudicative criteria we have been using to evaluate manifestations of this disease. These final rules represent only the latest refinement of the criteria we have been using since we began receiving these cases shortly after AIDS was first identified. Consequently, we do not expect their publication to have a significant additional effect on the Federal Disability Insurance Trust Fund or the Federal Hospital Insurance and Federal Supplementary Medical Insurance Trust Funds. It should also be noted that SSI benefits are not paid from the Social Security trust funds, but from the general revenues.

Waiting Period for Cash and Medicare Benefits

Comment: A comment suggested that we waive the 24-month waiting period to qualify for Medicare for individuals with HIV infection, which, the comment indicated, we do for other conditions, such as end-stage renal disease. The comment also noted that the 5-month waiting period requirement for SSDI benefits is inappropriate in HIV-infection cases, in view of the short life expectancy that follows a diagnosis of AIDS.

Response: We certainly empathize with the need for medical care for people who are HIV-infected. The comments are, however, outside the scope of these regulations. More importantly, the waiting periods for Medicare (including the exception for end-stage renal disease) and for SSDI benefits are specified in the Act, and cannot be "waived" without a legislative change.

Critical Payments

Comment: One comment recommended that the regulations require Social Security disability adjudicators to notify claimants of the availability of immediate critical payments at the time they are found eligible for disability benefits.

Response: We did not adopt this comment because it is outside the scope of these regulations and has been dealt with in our operating instructions. One of our goals is to pay all benefits due on time, and in the vast majority of cases we meet this goal through routine processing. However, our operating instructions provide for expedited payment by various means if a claimant has a financial emergency. These methods include the one-time emergency advance payment (EAP) procedure, which can be made in SSI cases in accordance with § 416.520 of our regulations when the individual is presumptively eligible for SSI payments and has a financial emergency. Our Field Offices and processing centers also have the capability to make expedited payments in other critical Social Security and SSI case situations.

Determinations at Steps 4 and 5; Younger Individuals

Comment: A number of commenters supported the philosophy of awarding as many claimants as possible at the listing level. They pointed out that most adult claimants with HIV infection are "younger" individuals (i.e., people under 50 years old) under our rules in §§ 404.1563(b) and 416.963(b). The commenters said that, if these individuals are not found to have an impairment(s) that meets a listing, they would probably be denied at the last step of the sequential evaluation processes. One comment said that we almost never do an equivalence analysis. Other comments said that it was insufficient to rely on the rest of the sequential evaluation process to adjust for the "inadequacies" of the medical standard.

Response: As we have explained, the listings do not represent the standard of disability in the Act, but a higher level of disability, because they are intended only to be a method by which we can quickly pay claims that clearly would be allowed at later steps in the sequential evaluation processes. Indeed, the Act does not require us to have a set of listings at all; the listings are simply a means by which we can process some claims more timely and efficiently.

Therefore, the question is not about any "inadequacies" in the listings, but about whether we will find disabled all individuals who have disabling impairments. We are committed to ensuring that all individuals who are disabled because of HIV infection receive timely and correct determinations under our rules, whether at the listing-level or beyond. This means that we will provide assessments of equivalence and of residual functional capacity (or of a child's functioning) to people who do not have impairments that meet the requirements of any of these listings, and allow those individuals who are disabled within the meaning of the Act.

But the fact that the great majority of people disabled with HIV infection are found to have listing-level impairments also attests to two things: that HIV infection is a terrible disease and that we have made our listing criteria broad enough to include most people who are disabled by HIV infection. We believe that some of the changes in the final rules—the listings for manifestations that affect women and girls, the new stand-alone medical criteria and other new medical criteria we have added, and the improvements to the functional criteria—will include even more disabled people at the listing level.

Beyond that, we can only say that we are as concerned about people with HIV infection as the commenters are. It is never acceptable to deny an individual who is disabled, even more so when the individual has an illness like HIV infection. Nevertheless, we are bound to follow the statute, there are many individuals who have HIV infection and are not yet disabled under the statute, and we have a responsibility to ensure that only individuals who are disabled receive benefits.