II-4-1-6.Federal Old-Age, Survivors and Disability Insurance and Supplemental Security Income; Listing of Impairments—Respiratory System (Final Rules; 58 FR 52346, October 7, 1993)
DEPARTMENT OF HEALTH AND HUMAN SERVICES
Social Security Administration
20 CFR Parts 404 and 416
[Regulations No. 4 and 16]
Federal Old-Age, Survivors and Disability Insurance and Supplemental Security Income; Listing of Impairments - Respiratory System
AGENCY: Social Security Administration, HHS.
ACTION: Final rules.
SUMMARY: These amendments revise the criteria in the Listing of Impairments (the listings) that we use to evaluate respiratory impairments for adults and children who claim Social Security benefits or supplemental security income (SSI) payments based on disability under title II and title XVI of the Social Security Act (the Act). The revisions reflect advances in medical knowledge, treatment, and methods of evaluating respiratory impairments.
DATE: These rules are effective October 7, 1993.
FOR FURTHER INFORMATION CONTACT: Cassandra Bond or Alicia Matthews, Legal Assistants, 3-B-1 Operations Building, 6401 Security Boulevard, Baltimore, MD 21235, (410) 965-1794 or 965-1713.
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 became 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 payments 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 which can be expected to result in death or which has lasted or can be expected to last for a continuous period of at least 12 months.
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 statutory 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) does not meet or equal in severity any listing criteria, no conclusion regarding disability is made. Rather, 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 which are set out as appendix 1 to subpart P of part 404 of our regulations. 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. 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).
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 the 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 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.
When we revised the listings on December 6, 1985 (50 FR 50068), we indicated that medical advancements in disability evaluation and treatment and program experience would require that the listings periodically be reviewed and updated. Accordingly, we specified termination dates ranging from 4 to 8 years for each of the specific body system listings. These dates currently appear in the introductory paragraphs of the listings. One extension for the termination date for part A of the respiratory system appeared in the Federal Register of November 27, 1991 (56 FR 60059), and extended the original date on which the part A listings would no longer be effective from December 6, 1991, to December 7, 1992. A second extension for the termination date for part A of the respiratory system appeared in the Federal Register of December 7, 1992 (57 FR 57665), and extended the termination date to June 7, 1993. A third extension for part A of the respiratory system appeared in the Federal Register of June 7, 1993 (58 FR 31906), and extended the date on which the listings would no longer be effective to December 6, 1993. We are now updating the respiratory system listings in both 3.00 (part A) and 103.00 (part B), and extending the effective date of these revised listings for 7 years from the date of their publication. Therefore, 7 years after publication of these final rules, these regulations will cease to be effective, unless they are extended by the Secretary or revised and promulgated again.
We published these regulations in the Federal Register (56 FR 52231) as a Notice of Proposed Rulemaking (NPRM) on October 18, 1991. Interested persons, organizations, Government agencies, and other groups were given 60 days to comment. The comment period ended December 17, 1991. We received letters from 11 commenters. These comments are addressed below.
Explanation of the Final Rules
We have updated these final rules to provide criteria for evaluating respiratory impairments at the listing level of severity reflecting state-of-the-art medical science and technology. The basic approach underlying the final respiratory system listings is to place less emphasis on the diagnosis of disease, and to emphasize the impact of the impairment(s) on a person's ability to perform work-related activities or, in the case of a child applying for SSI payments based on disability, on the child's ability to function independently, appropriately, and effectively in an age-appropriate manner.
We revised the respiratory system listings with information we received from individuals recommended by various medical professional groups, including the American Medical Association, the American Thoracic Society, the American Lung Association, the American College of Chest Physicians, the American Society of Internal Medicine, the American College of Physicians, the Cystic Fibrosis Foundation, and the American Nurses Association. We also received information from individual Federal and State representatives who have expertise in evaluating disability claims involving respiratory impairments. We also obtained information from individual pediatric experts to revise the part B listings.
The final listings revise the criteria to encompass disorders for which specific criteria have not been previously published (e.g., sleep-related breathing disorders) and to include state-of-the-art evaluative techniques. All of the techniques (tests, procedures, etc.) cited in the regulations are generally available and are included in the cumulative list of New or Improved Diagnostic Techniques, as published in the Federal Register on May 9, 1990, at 55 FR 19357.
The following is a summary of the provisions of the final rules and the changes we have made to the text of the NPRM. Finally, we have made a number of minor editorial changes throughout the rules to correct errors in the NPRM, to make the rules internally consistent, and to conform the style of these listings to our other listings.
A. Revisions to Part A of Appendix 1
In a technical correction, we are deleting the opening statement after the heading because it was redundant. The sentence repeated, almost verbatim, the statement in the third paragraph of the introductory text to appendix 1 that the respiratory system listings will no longer be effective 7 years after publication of the final rules unless extended by the Secretary or revised and promulgated again. In addition, our inclusion of the statement in 3.00 was inconsistent with all of the other listings sections except 12.00, the only other section in part A and part B that repeated the expiration date in its opening text.
We have modified the first paragraph of final 3.00A and made conforming changes to the first paragraph of 103.00A so that both paragraphs explain that the listings for respiratory impairments describe impairments based on symptoms, physical signs, laboratory test abnormalities, and response to a regimen of treatment which may have been prescribed by a treating source. The proposed rules referenced a regimen of therapy; however, for clarity the word "therapy" has been changed to the broader term "treatment" in this paragraph and throughout these regulations. No substantive change is intended by this technical correction.
In addition, as a result of public comments, we deleted the last sentence of the first paragraph of proposed 3.00A which indicated that the functional evaluation of the severity and duration of a respiratory disorder should be performed only after prescribed therapy has been instituted and sufficient time has elapsed for the results to be evaluated. We have, however, added a new second paragraph to 3.00A and 103.00A to indicate that, when there is evidence of medically prescribed treatment, the clinical record must include a description of the treatment prescribed by the treating source and response, in addition to information about the nature and severity of the impairment. This paragraph notes that it is important to document the prescribed treatment and response because this medical management may have improved the individual's functional status.
We added the third paragraph in response to comments which pointed out that some people do not receive treatment, and that some do not have an ongoing relationship with the medical community. The new paragraph explains that an individual who does not receive treatment may or may not be able to show the existence of an impairment that meets the listings. Even if an individual does not show that he or she has an impairment that "meets" the criteria of the listings, the individual may still have impairments that are "equal" in severity to a listed impairment or he or she may be found disabled at the last step of the sequential evaluation process. To be consistent with the adult rules, we also added a third paragraph to final 103.00A of the preface to the childhood listings; the paragraph contains provisions similar to the adult rules.
In the third sentence of the fourth paragraph when we discuss the evidence needed to establish the presence of chronic pulmonary disease, we have added other appropriate imaging techniques after x-ray to recognize there are now other technologies used for imaging. We have made a similar change in the first paragraph of 3.00F1, and in 3.00F3.
We also have included in the sixth paragraph a discussion of the documentation which may be needed with a diagnosis of diffuse interstitial fibrosis or clinical findings suggesting cor pulmonale, such as cyanosis or secondary polycythemia. This documentation may require measurement of carbon monoxide diffusing capacity or arterial blood gases at rest and infrequently during exercise in addition to evidence of the clinical evaluation and chest x-ray or other appropriate imaging techniques and spirometry. We also clarified the discussion of pulmonary vascular disease with its specific documentation requirements to state that right heart catheterization with angiography and/or direct measurement of pulmonary artery pressure may have been done. We further state that when performed, the results should be obtained because the results may be useful in evaluating impairment severity.
The final paragraph of 3.00A is a new paragraph that we added to clarify that the listings can never be used to deny claims. It is a reminder that claimants whose impairments do not "meet" any listing may still be found to have impairments that are "equal" in severity to a listing, and that the listings can only be used to find a person disabled, but not to find a person "not disabled." The new paragraph stresses the importance of an individualized residual functional capacity assessment, which may or may not result in a finding of disability, whenever a person's severe impairment(s) does not meet or equal the requirements of a listing. To be consistent with the adult rules, we also added a final paragraph to 103.00A of the preface to the childhood listings emphasizing the importance of equivalence determinations and individualized functional assessments when childhood impairments do not meet a listing.
3. Mycobacterial, Mycotic, and Other Chronic Persistent Infections of the Lung
The final rules in 3.00B include other chronic persistent infections of the lung, in addition to mycobacterial and mycotic infections. We removed, without replacement, the proposed last sentence of this section, which indicated that chronic pulmonary infection occurring in the setting of immunodeficiency should be evaluated under the criteria for the appropriate underlying disorder because we believe it to be unnecessary. Our adjudicators know that we have separate criteria for the evaluation of impairments of the immune system, including human immunodeficiency virus.
4. Episodic Respiratory Disease
In 3.00C, we specify the documentation requirements for episodic respiratory disease. Documentation must include information relating to the adherence to a prescribed therapeutic regimen, thus, again, serving to emphasize the importance of treatment that the claimant is expected to have received. The documentation requirements define the severity of an attack fulfilling the intensity component of the listing criteria, and define hospital admissions as inpatient hospitalizations for longer than 24 hours. Based upon a public comment which questioned the need for spirometric results between attacks of episodic respiratory diseases (asthma, cystic fibrosis, and bronchiectasis), we clarified that this rule pertains only to cases of asthma, as explained in more detail in the public comments section.
5. Cystic Fibrosis
Paragraph D is being added to the introductory material in 3.00 to recognize the increasing number of children with cystic fibrosis surviving to adulthood. The adult listings, therefore, explicitly recognize this condition, and in the first sentence, we indicate that this disorder may affect either the respiratory or digestive body systems or both. We provide a description of cystic fibrosis, including information concerning the proper diagnostic approach. In response to public comments, we added a reference to the medical literature which describes the diagnostic test (i.e., the quantitative pilocarpine iontophoresis sweat test). We also add that there are two methods of sweat collection, the Gibson-Cooke and Wescor Macroduct systems; however, to establish the diagnosis of cystic fibrosis, the sweat sodium or chloride content must be analyzed quantitatively using an acceptable laboratory technique. The pilocarpine method, the specifications for which are published by the Cystic Fibrosis Foundation, is an acceptable testing procedure. The pilocarpine method has the lowest false positive and false negative results of any sweat collection technique that is currently available to confirm the diagnosis of cystic fibrosis. The test is available in all Cystic Fibrosis Centers affiliated with the Cystic Fibrosis Foundation, as well as in major university medical centers. In addition, we have identified and included another test for diagnosing cystic fibrosis. It is the genetic test for homozygosity of the cystic fibrosis gene and is known as "CF gene mutation analysis."
In the penultimate sentence, in response to a public comment, we reference the digestive body system as the other nonpulmonary body system which could be affected by cystic fibrosis. We clarified the last sentence to indicate that the combined effects of the involvement of the respiratory and digestive body systems must be considered in case adjudication.
6. Documentation of Pulmonary Function Testing
For consistency with the terminology used throughout these regulations, in the title and in the second sentence, we have changed "ventilatory" function testing to "pulmonary" function testing. We have made a similar change in 103.00B. We expanded 3.00E, designated as 3.00D in our prior rules, to include more specific requirements for documentation of pulmonary function tests. The revision is consistent with published standards for test performance approved by the American Thoracic Society in the "American Review of Respiratory Disease," Vol. 136, No. 5: pp. 1285-1298, 1987, and the National Institutes of Health Epidemiology Standardization Project. Criteria are outlined specifying what constitutes an acceptable study. These criteria include the use of the back-extrapolation technique for zero time, the requirement for early onset of peak flow rates, and specific criteria for reproducibility. Performance standards require that at least three acceptable spirograms be obtained, two of which are reproducible. Comprehensive standards are provided for calibration of instruments other than those that generate the spirogram by direct pen linkage to a mechanical displacement-type spirometer. Three levels of calibrations are required for primary flow sensing devices because some of these devices are not linear over the wide range of flow rates which occur in the spirogram. These requirements for accuracy and calibration are consistent with the standards for spirometers endorsed by the American Thoracic Society.
The maximum voluntary ventilation requirement in our prior rules was deleted as a criterion because it was considered to be effort-dependent, infrequently used in clinical practice, and poorly standardized among laboratories. In the prior rules, the major usefulness of the maximum voluntary ventilation requirement was that it provided confirmation of the degree of impairment indicated by the one second forced expiratory volume (FEV1) when the test was of less than optimal quality. These final listings incorporate specific requirements to ensure the quality of forced expiratory spirometry studies.
We added two new sentences to the second paragraph of 3.00E and 103.00B to indicate first that pulmonary function studies should not be performed unless the claimant's clinical status is stable. The second sentence indicates that wheezing per se does not necessarily preclude performance of a pulmonary function test because wheezing is common in asthma, chronic bronchitis, or chronic obstructive pulmonary disease. Also, in the last sentence of this paragraph, we defined "most stable state of health" as "any period in time except during or shortly after an exacerbation."
In response to a public comment, we added language to the last sentence in the fifth paragraph of 3.00E and 103.00B to explain that the calibration tracings, as well as the spirograms, must be presented in a volume-time format at a speed of at least 20 mm/sec and a volume excursion of at least 10 mm/liter to permit independent evaluation.
7. Documentation of Chronic Impairment of Gas Exchange
In 3.00F1, we include a detailed description of the specifications for performance of the diffusing capacity of the lungs for carbon monoxide study to evaluate impairment of gas exchange. These listings place greater emphasis on the use of diffusing capacity of the lungs for carbon monoxide study as a measure of gas exchange prior to consideration of purchasing arterial gas analysis and exercise testing. The use of a diffusing capacity of the lungs for carbon monoxide study to estimate gas exchange impairment is based on state-of-the-art information that diffusing capacity is an important indicator of gas exchange and work capacity, and is an important predictor of mortality in lung disorders. The performance criteria for the test have been standardized by the American Thoracic Society in the "American Review of Respiratory Disease," Vol. 136, No. 5: pp. 1299-1307, 1987, and the standardization of criteria has been incorporated into the listings.
In the last sentence of the first paragraph of 3.00F1, we clarified the language on purchase of a diffusing capacity of the lungs for carbon monoxide study to indicate that purchase of a diffusing capacity of the lungs for carbon monoxide study may be appropriate when there is a question of whether an impairment meets or is equivalent in severity to the listing, and the claim cannot otherwise be favorably decided.
We provide criteria in 3.00F2 for resting arterial blood gas studies. In 3.00F2 and 3.00F3, we also specify that before purchase of arterial blood gas studies at rest or during exercise, a program physician, preferably one with experience in the care of patients with pulmonary disease, must review all clinical and laboratory data in the record to determine whether obtaining the test would present a significant risk to the individual. In 3.00F2, we also state that arterial blood gas studies at rest or on exercise should be purchased only from a laboratory certified by a State or Federal agency.
In the second paragraph of 3.00F2 and 103.00C1, we clarified our language on purchase to indicate that purchase of resting arterial blood gas studies may be appropriate when there is a question whether an impairment meets or is equivalent in severity to a listing and the claim cannot otherwise be favorably decided. To further clarify our intent on the purchase of resting arterial blood gas studies, we added a sentence to the second paragraph of 3.00F2 that indicates if the results of a diffusing capacity of the lungs for carbon monoxide study are greater than 40 percent of predicted normal but less than 60 percent of predicted normal, purchase of resting arterial blood gas studies should be considered.
In 3.00F3, we reorganized the first paragraph as it appeared in the NPRM to indicate that arterial blood gas studies with exercise may be appropriate in cases in which there is documentation of chronic pulmonary disease, but full development, short of exercise testing, is not adequate to establish if the impairment meets or is equivalent in severity to a listing, and the claim cannot otherwise be favorably decided. As a result of this reorganization, we deleted the fourth sentence of the proposed rule because it was redundant in light of the revision to the first sentence. Exercise testing with blood gas analysis should only be done after the diffusing capacity of the lungs for carbon monoxide study has been estimated and the documentation has been found to be inadequate for the evaluation of the severity of functional impairment. In 3.00F3, we deleted the language regarding a FEV1 greater than 2.5 liters from the second paragraph of the proposed rule because it was medically inaccurate in that this finding is not a sensitive marker for absence of a gas exchange impairment. We have, however, retained language that when an individual has a diffusing capacity of the lungs for carbon monoxide greater than 60 percent of predicted normal, an exercise blood gas study generally would not be needed.
In 3.00F4, we revised the methodology for performance of an exercise test to include use of measurements at two exercise levels if the individual can satisfactorily complete the lower level of exercise at approximately 5 METs. In the third sentence, we reflect the weight in both pounds and kilograms for clarity. We expanded the last sentence of the fifth paragraph to indicate that the statement the laboratory provides concerning why the claimant failed to complete 4 to 6 minutes of steady state exercise, provides information which may be useful for determining whether effort was limited by lack of effort or another impairment which affected the claimant's ability to exercise.
8. Chronic Cor Pulmonale and Pulmonary Vascular Disease
In 3.00G, we provide a more detailed description of chronic cor pulmonale and pulmonary vascular disease for which specific new criteria are provided in 3.09. We clarified that the establishment of an impairment attributable to irreversible cor pulmonale secondary to chronic pulmonary hypertension requires documentation by signs and laboratory findings of right ventricular overload or failure. This was the intent of the language which appeared in the NPRM. We also clarified that hypoxemia may accompany heart failure and is also a cause of pulmonary hypertension and also that polycythemia with an elevated red blood cell count and hematocrit may be found in the presence of chronic hypoxemia.
9. Sleep-Related Breathing Disorders
We are also adding 3.00H to provide a description of sleep-related breathing disorders, which are not addressed in the prior listings. Reference criteria are provided for the evaluation of these disorders in 3.10. In response to a public comment to provide more detailed information on the effect of sleep apnea on an individual, we have added a sentence recognizing that because daytime sleepiness can affect memory, orientation, and personality, a longitudinal treatment record may be needed to evaluate mental functioning. We also added a sentence to clarify that chronic hypoxemia due to episodic apnea may cause pulmonary hypertension. Daytime somnolence may be associated with disturbance in cognitive vigilance. Impairment of cognitive function may be evaluated under organic mental disorders (12.02). If the sleep disorder is associated with gross obesity, evaluation will be under the applicable obesity listing.
1. Category of Impairments, Respiratory System
a. Chronic Pulmonary Insufficiency
Paragraph A of 3.02 and table I now only require one-second forced expiratory volume values rather than both FEV1 and maximum voluntary ventilation results. The FEV1 values are substantively unchanged from our prior rules. Table I, however, is constructed so that rounding of reported volume values will not be necessary. For clarity, the heights are shown in centimeters and inches rather than just inches. In addition, in paragraph A, for clarity, we have made a nonsubstantive change from the NPRM and added "due to any cause" after the opening phrase, "Chronic obstructive pulmonary disease." This phrase is also in our prior rules. For consistency, we included it in 103.02A. For clarity, we also added, in parentheses, the cross reference to 3.00E for guidance on determining the height of an individual with marked spinal deformity.
In the first sentence of 3.02B, we added "due to any cause" after "chronic restrictive ventilatory disease" and in 103.03B. In final 3.02, paragraph B has been clarified by adding a cross reference to 3.00E for cases of marked spinal deformity rather than repeating, as we did in the NPRM, the same information that is already contained in the introductory material in 3.00E. Table II is modified so that rounding of reported forced vital capacity values will not be necessary and the heights are shown in both inches and centimeters.
Final 3.02C, provides criteria for evaluating a gas exchange abnormality based on a diffusing capacity of the lungs for carbon monoxide study, arterial blood gas studies at rest or exercise testing with measurement of arterial blood gas studies. The diffusing capacity of the lungs for carbon monoxide criteria have been modified from the values in our prior rules (less than 9 ml/min/mm Hg, or less than 30 percent of the predicted normal value) to less than 10.5 ml/min/mm Hg, or less than 40 percent of the predicted normal value. This revision represents updated information from the American Thoracic Society correlating these values for diffusing capacity of the lungs for carbon monoxide with work capacity and mortality. ("American Thoracic Society Statement on Evaluation of Impairment/Disability Secondary to Respiratory Disease", "American Review of Respiratory Disease," Vol. 133, No. 6, pp. 1205-09, 1986.) Because of the variation in published predicted values, the listing criteria specify that the source of the predicted values should be reported or, if not published, should be submitted in the form of a table or nomogram.
Paragraph C2 of 3.02 provides criteria for resting arterial blood gas studies. Resting arterial hypoxemia is associated with increased mortality and development of pulmonary hypertension and cor pulmonale. Based upon a public comment that there could be misapplication of this listing, we have included again tables III-A, III-B, and III-C from the prior listing 3.02 to provide for the evaluation of hypoxemia at three altitudes. We have deleted the proposed criteria for evaluating hypoxemia at two altitudes (i.e., less than 4000 feet and 4000 feet and greater). The correction for hypoxemia for carbon dioxide tension is contained in tables III-A, III-B, and III-C. The requirement in paragraph C2 that the two measurements of resting arterial oxygen tension (PO2) be separated by an interval of three or more weeks within a six-month period in order to meet the requisite level of hypoxemia is based upon the degree of technical and biologic variability of arterial oxygen levels and is consistent with standards used in studies on mortality and performance in persons with chronic lung disease.
Paragraph C3 has been revised based upon public comments to reflect arterial blood gas values of PO2 and simultaneously determined PCO2 during steady state exercise with a parenthetical statement defining the exercise level and referencing to tables III-A, III-B, and III-C.
In 3.03, paragraph B provides that hospitalization for longer than 24 hours for control of asthma counts as two asthma attacks for purposes of meeting the frequency requirement of at least one attack every 2 months, or at least six times a year. Paragraph B also states that medical evidence of record over a period of at least 12 consecutive months must be used to determine the frequency of attacks.
c. Cystic Fibrosis
Listing 3.04 has been added to establish criteria for evaluating cystic fibrosis in adults because an increasing number of individuals with this disease are now living into adulthood. The listing provides new criteria for the evaluation of the pulmonary manifestations of this disorder. Based upon public comments, we revised 3.04A to include a separate table (table IV) to be used only for the evaluation of cystic fibrosis in adults, as is done in the childhood rules in 103.04A. We also added 3.04C to include those criteria shown in 103.04C because some adults could manifest the findings shown.
Listing 3.06 is a cross-reference listing by which pneumoconiosis demonstrated by appropriate imaging techniques can be evaluated under the appropriate criteria in listing 3.02.
Bronchiectasis, 3.07, is somewhat similar to the clinical aspects of asthma and cystic fibrosis in that the course may be characterized by exacerbations and remissions. Therefore, a new paragraph B is provided to make the criteria for episodes of bronchitis or pneumonia or hemoptysis or respiratory failure consistent with 3.03 (asthma) and 3.04 (cystic fibrosis). Paragraph B indicates that episodes of bronchitis or pneumonia or hemoptysis or respiratory failure requiring physician intervention at least six times a year will meet the requirements of the listing.
f. Mycobacterial, Mycotic, and Other Chronic Persistent Infections of the Lung
In response to a public comment, we have combined proposed 3.08 and 3.09 into one reference listing and retitled it as shown above.
g. Cor Pulmonale Secondary to Chronic Pulmonary Vascular Hypertension
Because we combined proposed 3.08 and 3.09 into final 3.08, we have renumbered this listing as 3.09. More specific criteria are provided requiring the establishment of an impairment due to chronic cor pulmonale. Additional criteria to those in 4.02 are provided to facilitate adjudication when resting arterial blood gases have been measured and hypoxemia fulfills 3.02C2. Also, we have clarified paragraph B for arterial hypoxemia to cross-refer to 3.02C2 for the evaluation of arterial hypoxemia. We have revised 3.09C to merely reference 4.02 so that this listing will remain current even if future revisions are made to the 4.02 criteria.
h. Sleep-related Breathing Disorders
Based upon the reorganization discussed previously, this listing is now designated 3.10 (3.11 in the NPRM). This is a new listing for sleep-related breathing disorders and refers to the persistent functional consequences of this condition. When a sleep-related breathing disorder limits or precludes work activity, the physiologic basis for the impairment may be chronic cor pulmonale, or a disturbance in cognitive performance. Some individuals with this disorder are morbidly obese. Reference listings are provided for the evaluation of these manifestations.
C. Revisions to Part B of Appendix 1
Consistent with our discussion of revisions to part A of appendix 1, we have deleted the language we proposed in the NPRM which repeated in 103.00 the sunset provision for the respiratory listings in part B. This statement in the childhood rules would not only have been redundant of the opening text, but would also have made 103.00 inconsistent with all other listings sections except 12.00, the only other section that repeats the sunset provision in its opening text.
In addition to the changes explained in 3.00A, we added a final sentence to the first paragraph to indicate that reasonable efforts should be made to ensure evaluation of the child's claim by a program physician specializing in childhood respiratory impairments or a qualified pediatrician. We also added introductory material in the third paragraph of 103.00A to provide a discussion that evaluation should include consideration of the adverse effects of respiratory disease in all relevant body systems, and especially on the child's growth and development or mental functioning.
3. Documentation of Pulmonary Function Testing
We have retitled 103.00B to be consistent with 3.00E. We removed the reference to 103.10 contained in the NPRM because there was no such listing in the NPRM or in these final regulations. The updated standards for pulmonary function testing and the rationale for including them are essentially the same as those under 3.00E. A statement describing the child's ability to understand directions and the child's effort and cooperation in the testing should still be included as part of the report. Criteria are outlined specifying what constitutes an acceptable study. These criteria include the use of the back-extrapolation technique for zero time, the requirement for early onset of peak flow rates, and specific criteria for reproducibility. Performance standards require that at least three acceptable spirograms be obtained, two of which are reproducible. Criteria are included for when spirometry should be repeated after administration of an aerosolized bronchodilator. Comprehensive standards are provided for calibration of instruments other than those that generate the spirogram by direct pen linkage to a mechanical displacement-type device. Three levels of calibrations are required for primary flow sensing devices because some of these devices are not linear over the wide range of flow rates which occur in the spirogram.
As a result of a public comment, we eliminated the discussion on rounding and constructed the tables similar to the adult tables.
In response to a public comment, we have added a new seventh paragraph to explain that performance of a pulmonary function test is appropriate only when the child is capable of performing reproducible forced expiratory maneuvers. This capability usually occurs around age 6.
4. Documentation of Chronic Impairment of Gas Exchange
This section has been redesignated 103.00C (it was proposed 103.00B2) because we decided to reorganize these rules to be consistent with the rules in part A. In 103.00C1, "Arterial blood gas studies (ABGS)," we provide the documentation requirements for chronic impairment of gas exchange and guidance regarding the performance of blood gas studies. In 103.00C2, we provide guidance on the use of pulse oximetry as a substitute for arterial blood gas testing in children under age 12. The proposed rules had age 3 as the cut-off point, but based upon a public comment and advances in medical science, we increased the age to 12. Based on a public comment, we also added a statement at the end of the first paragraph to indicate that the report of the pulse oximetry test should include a statement on the child's cooperation during the test. The pulse oximetry provision for documenting gas exchange abnormalities is necessary because arterial blood samples are difficult to obtain in children under 12 years of age, and many children being evaluated will require oxygen periodically, during which times blood gas determinations cannot be made. Further, invalid values can result because the procedure is painful and frequently causes crying. Pulse oximetry has been established as a safe and relatively painless procedure. It is more repeatable than ABGS and more accurate than transcutaneous techniques. It is becoming the state-of-the-art procedure for determining gas exchange abnormalities in young children and is as available in hospitals and clinics as are blood gas determinations.
For clarity, in the last sentence of 103.00C1, we indicated that the program physician who determines whether to purchase a resting arterial blood gas study should preferably be one experienced in the care of children (the NPRM indicated patients) with pulmonary disease.
For consistency with the adult rules and based upon a public comment, we added a new paragraph to 103.00C1 and C2 which discusses when purchase of a resting ABGS or oximetry may be appropriate (i.e., when there is a question of whether an impairment meets or is equivalent in severity to a listing, and the claim cannot otherwise be favorably decided).
5. Cystic Fibrosis
This section has been redesignated 103.00D (it was 103.00C in the NPRM) because of the reorganization discussed above. Based upon a public comment, we revised the first sentence to indicate that this disorder can affect either the respiratory or digestive body systems or both, as well as impact the child's growth and development. In response to public comments, we have provided additional guidance on tests which can be used for the diagnosis of cystic fibrosis. The quantitative pilocarpine iontophoresis procedure for collection of sweat content must be utilized. We note that the Gibson-Cooke and Wescor Macroduct methods are acceptable. The sweat sodium or chloride content must be analyzed quantitatively using an acceptable laboratory technique. This pilocarpine method, published by the Cystic Fibrosis Foundation, is an acceptable testing procedure as it has the lowest false positive and false negative results of any sweat collection technique that is currently available to confirm the diagnosis of cystic fibrosis. The test is available in all Cystic Fibrosis Centers affiliated with the Cystic Fibrosis Foundation, as well as in major university medical centers. In addition, we are including another diagnostic technique which is a genetic test for homozygosity of the cystic fibrosis gene and is called "CF gene mutation analysis." Most children with suspected cystic fibrosis are referred to these centers for the diagnosis, evaluation, and ongoing management of this disorder. We clarified the last sentence contained in 103.00C of the NPRM to indicate that the combined effects of the involvement of the respiratory and digestive body systems, as well as the impact of the disorder on a child's growth and development should be considered in case adjudication.
6. Bronchopulmonary Dysplasia (BPD)
This section has been redesignated 103.00E (it was 103.00D in the NPRM) because of the reorganization above. We added new material to the introduction in 103.00E to provide an explanation of bronchopulmonary dysplasia (BPD) and the requirements for establishing the diagnosis. BPD was first reported in 1967 and had a very high mortality rate. Improvements in technology and patient management have resulted in increased survival and recovery of premature infants. Although the majority of infants with BPD do recover, some progress to chronic pulmonary disease, resulting in severe respiratory and related functional impairments.
7. Category of Impairments, Respiratory System
The revisions to the part B respiratory listings maintain structural and content comparability with part A to the extent applicable. As in the sections of 103.00, there were a few instances in which we were able to incorporate language from the adult rules into the childhood listings language we proposed in the NPRM, or to make the same or similar revisions in both parts for even greater consistency between parts A and B.
a. Chronic Pulmonary Insufficiency
In a technical, nonsubstantive change, we have redesignated the listing we proposed as 103.14 in the NPRM as final 103.02 and revised the final listing in order to preserve structural and content comparability with part A. Thus, final 3.02 and 103.02 now both list impairments that fall under the heading of chronic pulmonary insufficiency, including chronic obstructive and chronic restrictive pulmonary impairments.
We revised final 103.02A from the language in proposed 103.14A to confine its criteria to chronic obstructive pulmonary disease due to any cause, in order to maintain structural and content comparability with 3.02A. For the same reason, we moved table I from 103.03 of the NPRM to final 103.02A. Table I is constructed so that rounding of reported volume values will not be necessary. For clarity, the heights are shown in centimeters and inches. We also added a statement, like the statement in the corresponding adult rule, that the height in the table refers to height without shoes and added a cross reference to 103.00B for the discussion of measurement of children who have marked spinal deformity. Similarly, we revised the first column heading in table I to indicate that the height is without shoes. None of these revisions is substantive. We have merely incorporated the guidance that was already in proposed 103.00B directly into the listing and the table in order to make them clearer and consistent with the analogous adult rule. Finally, we made minor editorial changes to the language of the provision for clarity and consistency with other provisions.
Final listing 103.02B is now applicable to chronic restrictive ventilatory disease due to any cause, and corresponds to adult 3.02B. As in final 103.02A, we incorporated the guidance on height and a cross reference to 103.00B. Because of the restructuring of the rules, we redesignated table III of proposed 103.14 as table II in final 103.02B and modified it so that rounding of reported forced vital capacity values will not be necessary. The heights are shown in centimeters and inches.
Final 103.02A and B, as in the NPRM, still provide a means for evaluating chronic pulmonary impairments not covered under 103.03 and 103.04.
Because we divided proposed 103.14A into two listings (final 103.02A and B), we redesignated the subsequent paragraphs of the rule. Paragraphs B through G of the proposed rules are, therefore, paragraphs C through H in the final listing. Paragraphs C, D, and E of final 103.02 (paragraphs B, C, and D of proposed 103.14) were developed specifically for the evaluation of children who have experienced respiratory distress in the neonatal period and subsequently develop chronic lung disorders such as BPD. However, other conditions such as congenital or acquired defects of the larynx and trachea, neuromuscular disorders and spinal cord injuries affecting respiration, and central nervous system mediated hypoventilation can also be evaluated under these listings.
We made a technical correction to listings 103.02C and 103.02E5 to make these regulations internally consistent. In these regulations, we changed the word "continuous" to "frequent."
In paragraphs 103.02E6 and F2, we clarified the language as a result of a public comment that we should define "poor weight gain." We revised final listings 103.02E6 and F2 to provide criteria for the evaluation of involuntary weight loss or failure to gain weight at an appropriate rate for age. In this way, we not only include children who have stopped gaining weight but also children who do not gain enough weight. We also explicitly provide for the possibility that a child will actually lose weight. Paragraphs F and G of final 103.02 (paragraphs E and F of proposed 103.14) specify other criteria for assessing manifestations of a pulmonary impairment in children, such as recurrent lower respiratory infection, acute respiratory distress, chronic hypoventilation or chronic cor pulmonale. In paragraph F, we clarified that the two hospital admissions within a 6-month period must be required and each must be for more than 24 hours.
Paragraph H of final 103.02 (paragraph G of proposed 103.14) provides criteria for evaluating interference with linear growth as a manifestation of lung disease. In a minor change from the NPRM, we revised the reference from a reference to 100.02 to a more general reference to the growth impairment listings in 100.00. In this way, 103.02H will remain current regardless of any revisions we may make to the listings in 100.00.
Paragraphs A and B, of 103.03 ensure greater structural and content relationship with paragraphs A and B of 3.03 in part A. Paragraph A of 103.03 and the values specified in table I (following final 103.02A) provide for the use of pulmonary function testing, and paragraph B of 103.03 provides the clinical criteria for assessing pulmonary insufficiency. These listings are intended to represent the childhood counterparts of the proposed paragraphs A and B of 3.03 in part A. Because we moved table I from proposed 103.03 into final 103.02A, we have revised the text of final 103.03A to indicate the location of table I.
Paragraph C of 103.03 recognizes that some children have persistent symptomatology related to bronchial asthma that may not meet or equal in severity the listing criteria in 103.03A and B, in part because of their drug regimen. In response to a public comment, we revised 103.03C. We deleted proposed 103.03C2 (barrel or pigeon chest deformity related to underlying pulmonary dysfunction) because these were merely descriptive anatomical deformities subject to various interpretations, and we renumbered proposed 103.03C3 to 103.03C2. We also revised 103.03C to now require one of the two criteria.
Paragraph D to 103.03 provides that a growth impairment related to asthma should be evaluated under the criteria in 100.00.
c. Cystic Fibrosis
Paragraph A of 103.04 incorporates and revises the criteria in paragraphs A and B of 103.13 of our prior rules, and revises the FEV1 values. The values in our rules now being updated were set at 50 percent of predicted normal, and the final values have been set at 60 percent of predicted normal. The higher values in the final rules are more consistent with studies that show good correspondence between clinical scoring systems based on the manifestations of lung disease and FEV1 values set at the higher standard. Such scoring systems primarily reflect pulmonary status as measured by physical pulmonary findings, including radiographic evidence, nutritional status and level of activity. As a result of this change, forced expiratory pulmonary testing will permit more reliable evaluation of a functional impairment. Consistent with the revision of the FEV1 threshold necessary to establish a disabling impairment, the extent of clinical evidence needed under paragraph B of 103.04 for children in whom pulmonary function testing cannot be performed will now require that two of three listed manifestations be present, rather than all three as the rules being updated required. Table III is contructed so that rounding of reported volume values will not be necessary and the height is shown in centimeters and inches.
Paragraph C of 103.04 provides for the evaluation of persistent pulmonary infections accompanied by superimposed, recurrent, symptomatic episodes of increased bacterial infection requiring intravenous or nebulization antimicrobial treatment. We added nebulization treatment because that is becoming more widely used.
Paragraph D was added and provides for the evaluation of episodes of bronchitis, pneumonia, hemoptysis, or respiratory failure.
This is a technical change which adds to the childhood listings these manifestations of cystic fibrosis which had been included only in the proposed adult listings.
Paragraph E of 103.04 provides that a growth impairment related to cystic fibrosis should be evaluated under the criteria in 100.00.
D. Public Comments
Following the publication of the NPRM in the Federal Register, we received 11 letters containing comments pertaining to the changes we proposed. The letters came primarily from advocates of the rights of individuals with respiratory disease (including both legal advocates and medical associations).
We have carefully considered all of the comments and have adopted many of the recommendations. These changes are identified in the following discussion of issues that were raised in the comments.
A number of the comments were quite long and detailed. Of necessity, therefore, we have condensed, summarized, or paraphrased them. However, we have tried to express everyone's views adequately and to respond to all of the relevant issues raised. There were also a few comments that we do not address below; this is because they were minor editorial comments pointing out typographical errors, or administrative matters that are not appropriate to the final rules.
For ease of reference, we have 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. The section references in the headings below refer to the final rules. In those instances in which 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.
1. Public-General Issues:
Comment: A commenter thought that the proposed 7-year expiration date for these rules was too long and recommended a 5-year period because rapidly developing medical technologies could make these listings obsolete.
Response: We did not adopt the comment because we are not bound to wait 7 years before promulgating new rules. If there are medical advances that should be reflected in our regulations, we can at any time before the expiration of the 7-year period revise all or part of the rules.
Comment: A commenter indicated that the requirement for an evaluation period of at least 12 consecutive months in order to determine the frequency of episodes for asthma, cystic fibrosis, and bronchiectasis was much too stringent.
Response: We did not modify our requirement in listings 3.03 and 103.03 (asthma), 3.04 and 103.04 (cystic fibrosis) and 3.07 (bronchiectasis) for an evaluation period of at least 12 consecutive months. These listings are intended to be used in the most medically severe cases of asthma, cystic fibrosis, and bronchiectasis. Severity for these listings is predicated on the frequency and intensity of episodes. We need an evaluation period of at least 12 months to establish a longitudinal clinical picture because there are a number of factors which may require this much time in order to evaluate the functional impact of the impairment on the individual. For example, the therapeutic adjustments or modifications made in the prescribed treatment and the response are variable because some individuals may not respond initially to a particular regimen and will later need frequent adjustments to their treatment regimen. We also need information on the optimal benefit which has been achieved from the treatment modalities. This cannot be assessed until an effective regimen has been achieved and enough time has elapsed for the regimen to be effective. In addition, there may be a seasonal component contributing to the frequency of attacks, and we want to determine from the longitudinal record whether this is a temporary factor contributing to the exacerbation of an episodic respiratory disease. Nonetheless, if disability can be established at any point in the claims development process, we will do so.
2. Preface to Part A
Comment: A few commenters were concerned about the requirement that the functional evaluation of the severity and duration of a respiratory disorder should be performed only after prescribed treatment has been instituted and sufficient time has elapsed for the results to be evaluated. They said that many claimants do not have access to this type of treatment or cannot afford medications. These commenters wanted a definition of "sufficient time" because some respiratory impairments respond more quickly to treatment than others and without such a definition, various interpretations could result. Another commenter noted an inconsistency in the preamble language which explained this change and the actual language in 3.00A. The explanation in the preamble indicated that the evaluation of the severity and duration of a respiratory disorder should be performed only after prescribed treatment has been instituted and expected therapeutic benefits have been achieved. The commenter noted that this language difference would require different documentation and evaluation judgments. One commenter wanted to know how to handle situations in which the treating physician advises the patient to stop smoking, yet the claimant continues to smoke.
Response: We deleted the last sentence of the first paragraph of proposed 3.00A and revised the final rules by adding a new second paragraph in final 3.00A, and for consistency, we made the same addition in 103.00A of part B. In the new second paragraph of final 3.00A and 103.00A, we now indicate that many individuals who have "listing-level" impairments will have received the benefit of medically prescribed treatment and that, whenever there is such evidence, the longitudinal clinical record must include a description of the treatment prescribed by the treating source and the response (which would include evidence of the effects of not smoking), in addition to information about the nature and severity of the impairment. Although we agree that people with impairments of lesser severity than those in the listings may not necessarily receive this kind of treatment, we believe that the listing-level impairments are so severe that many, if not most, individuals with such serious impairments will be placed on some sort of medical treatment. We have added a new third paragraph to recognize that there will be some individuals who, despite the existence of a severe impairment, will not have received ongoing treatment. Such an individual may or may not be able to show the existence of an impairment that meets the criteria of a listing. Even if an individual does not show that his or her impairment meets the criteria of a listing, he or she may have an impairment(s) equivalent in severity to one of the listed impairments or be disabled because of a limited residual functional capacity.
We need a longitudinal record to establish the severity and duration of the impairment, especially for those impairments that may be amenable to treatment. Because, as the commenter noted, some respiratory impairments respond more quickly to treatment than others, it is not possible to provide specific criteria as to what is a sufficient period of time. Our rules allow the adjudicator to decide, on a case-by-case basis, if sufficient evidence is available for a determination or decision.
To underscore our policy that we never deny claims because of failure to meet or equal the listings, we added new paragraphs in final 3.00A and 103.00A which stress the necessity for making an equivalence determination when a claimant's impairment(s) does not meet a listing, and for assessing residual functional capacity (or performing an individualized functional assessment) when a claimant's severe impairment(s) neither meets nor equals in severity any listing.
Finally, the rules on failure to follow prescribed treatment are quite complex, but have one simple underpinning: They do not come into play unless an individual's treating source has prescribed treatment for the individual which the individual is not following. If the individual's treating source has not prescribed treatment, or the individual does not have a treating source, the principle does not apply.
If the issue arises in which a physcian advises a claimant to stop smoking, and he or she does not, a judgment will need to be made on a case-by-case basis to determine if the physician's advice was directly related to or part of the treatment regimen for the respiratory impairment.
Comment: A commenter suggested that pulse oximetry or ear oximetry be included in proposed listings 3.02C2 and 3.10B (now renumbered 3.09) for adults and children over the age of 3 as alternative documentation to arterial blood gas studies.
Response: We did not accept the comment to include oximetry results for adults, but we revised the age limit for children to age 12 because, as stated previously, pulse oximetry in children is becoming the state-of-the-art procedure for determining gas exchange abnormalities in young children. We accept pulse oximetry in a child under age 12 because of technical problems with arterial puncture. However, oximetry provides only a single value for measuring the degree to which oxygen is bound to hemoglobin (oxygen saturation). It is not as complete a measure of gas exchange as arterial blood gas studies. Arterial blood gas studies provide information about other factors such as the amount of carbon dioxide in the blood and metabolic status. The evaluation of all these factors provides a more sensitive and specific indicator of a disabling gas exchange impairment.
b. Mycobacterial, Mycotic, and Other Chronic Persistent Infections of the Lung
Comment: A commenter welcomed the broadening of the applicability of the listings to consider chronic persistent lung infections other than mycotic or mycobacterial infections, and recommended that the title of listings 3.08 and 3.09 also include this information.
Response: We agree with the commenter that the title of listings 3.08 and 3.09 should be broadened to include chronic persistent lung infections. In addition, because both these listings contain cross references to listing 3.02 and section 3.00B, we combined them into one listing now numbered 3.08 and retitled it, "Mycobacterial, mycotic, and other chronic persistent infections of the lung."
c. Episodic Respiratory Disease
Comment: Two commenters suggested that we clarify the requirement for information documenting adherence to a prescribed therapeutic regimen. They questioned whether therapeutic blood levels of theophylline or other drugs will be required as documentation of adherence to prescribed treatment. They also felt that the requirement for spirometric results obtained between attacks appears to be unnecessary if the listing is satisfied by attacks meeting the severity and frequency criteria described in proposed listing 3.03B.
Response: Therapeutic blood levels of theophylline or other drugs are not required because theophylline is not prescribed in all cases. Many factors other than compliance with medication affect blood levels through alterations in absorption and metabolism. We believe that the treating source should be able to supply this information because if an individual truly has a listing-level impairment, it would be unusual for him or her not to be receiving ongoing treatment from a treating source who has longitudinal evidence on the prescribed treatment and response.
We revised the last sentence of 3.00C because the requirement for spirometric results between attacks pertains only to asthma. We now require spirometry between asthma attacks because spirometry showing reversible bronchospasm between attacks with improvement in the one-second forced expiratory volume post bronchodilator is needed to document the severity of asthma.
d. Cystic Fibrosis
Comment: One commenter suggested that the pilocarpine techniques should be specified in this section as they are in 103.00C.
Response: We agree and have added appropriate language to 3.00D.
Comment: A commenter requested that we specify that the other body systems which can be affected by cystic fibrosis are the gastrointestinal and cardiovascular systems. This commenter also noted that the references to sweat tests in proposed sections 3.00D and 103.00C were insufficient and suggested we include the Gibson-Cooke method and the Wescor Macroduct system.
Response: We partially agree with the first comment and have modified 3.00D and 103.00D to specifically reference the digestive system. In the childhood rules (103.00D), we also indicate that cystic fibrosis can adversely impact a child's growth and development. We did not reference the cardiovascular body system because the manifestation involved would be cor pulmonale, and that is covered by these listings. We included references to the Gibson-Cooke procedure and the Wescor Macroduct system in 3.00D and 103.00D. We indicate that both methods can be used for sweat collection, but the sweat or chloride content must be analyzed quantitatively using an acceptable laboratory technique.
e. Documentation of Pulmonary Function Testing
Comment: One commenter suggested that we address the acceptability of pulmonary function tests in the presence of bronchospasm.
Response: We agree with the commenter and have added language to the second paragraph of 3.00E to indicate that pulmonary function studies should not be performed unless the individual's clinical status is stable, and the individual is not, for example, suffering an episodic respiratory attack, acute respiratory infection or other chronic illnesses. We also provide that wheezing, per se, does not preclude testing and is commonly found in asthma between attacks, in chronic bronchitis, and in chronic obstructive pulmonary disease.
Comment: One commenter felt that requiring documentation on the manufacturer and model number of the device used to measure and record the spirogram was excessive. The commenter indicated that if the Agency knows about the acceptability of various devices, it should publish such a notice in the Federal Register for notice and comment.
Response: We did not accept the comment because the manufacturer and model number of spirometric devices may be helpful particularly when the adjudicator must review an incompletely labeled or calibrated spirogram, and either through program experience or reference materials available to the adjudicator, he or she can determine if the test results are acceptable. It may then be unnecessary to contact the source which performed the test for additional information if the specific information regarding the spirometer is available. We feel the time required to report the manufacturer and model number is less costly and time consuming than the possible recontact. In addition, as clearly indicated in 3.00E, this evidence "should" be stated. There is not an absolute requirement that the manufacturer and model number be a part of the record in order for the claim to be decided. If all the evidence supports a favorable decision, the claim would not be delayed merely to document this piece of evidence. In addition, this is not a new requirement. It is in section 3.00D of the prior rules, and there have been no problems with this aspect of documentation. We did not accept the comment that if the Agency knows about the acceptability of various devices, we should publish such a list in the Federal Register for notice and comment. We do not certify acceptability of spirometers nor do we maintain a list of acceptable devices. Rather, we provide rules defining what data are acceptable for program purposes, and, in turn, we accept the results of spirometry from any equipment that can provide these data.
Comment: The same commenter felt that the calibration tracings, as well as the spirograms, must have a time scale of at least 20 mm/sec and a volume scale of at least 10 mm/liter to permit independent measurement of the spirogram"
Response: We agree with the comment and have added the language to the last sentence of the fifth paragraphs of 3.00E and 103.00E that the spirogram and the calibrations must be presented in a volume-time format at a speed of at least 20 mm/sec and a volume excursion of at least 10 mm/L to permit independent evaluation.
f. Documentation of Chronic Impairment of Gas Exchange
Comment: One commenter requested clarification as to when to consider purchase of a diffusing capacity of the lungs for carbon monoxide or resting arterial blood gas studies. The commenter noted that this section says these tests should be purchased in cases in which "there is documentation of chronic pulmonary disease, but the existing evidence, including properly performed spirometry, is not adequate to establish the level of functional impairment." The commenter questioned whether chronic pulmonary disease specifically refers to those diseases that may result in significant impairment of gas exchange and whether this will result in an increase in the need to purchase diffusing capacity of the lungs for carbon monoxide and arterial blood gas studies. The commenter questioned whether there is a specific sequence of development that is required. He said proposed section 3.00F3 indicates that an exercise arterial blood gas study should be purchased only if full development is not adequate to establish the level of functional impairment and goes on to note that full development means the results of spirometry and measurement of diffusing capacity of the lungs for carbon monoxide and resting arterial blood gas studies have been obtained. This implies that both these studies may be purchased before exercise arterial blood gas studies.
Response: It is not possible to provide an exact sequence which should always be followed in purchasing tests. Chronic pulmonary insufficiency, as designated in listing 3.02, may be the result of many disorders which may alter pulmonary function, including, but not limited to, chronic obstructive pulmonary disease, emphysema, chronic bronchitis, bronchiectasis, pneumoconiosis, infection, cystic fibrosis, asthma, pulmonary infiltrative disorders, and pulmonary resection. The important issue in documentation of severity in these disorders is to have sufficient information to identify those individuals with disabling functional loss. Because each disease process may manifest itself differently, each case needs to be independently evaluated in light of the pertinent evidence already in file and the likelihood that a particular study will provide additional information for accurate adjudication. We have, however, modified the language in 3.00F1 and 3.00F2 to clarify that purchase of a diffusing capacity of the lungs for carbon monoxide and arterial blood gas studies may be appropriate when there is a question of whether an impairment meets or is equivalent in severity to a listing and the claim cannot otherwise be favorably decided. We may purchase exercise arterial blood gas studies if full development is not sufficient to establish if the claim meets or is equivalent in severity to a listing and the claim cannot otherwise be favorably decided. "Full development" can include spirometry, diffusing capacity of the lungs for carbon monoxide or resting arterial blood gas studies, available either through evidence of record or purchase, and all other available evidence, including but not limited to medical history, physical examination, chest x-ray or other appropriate imaging techniques, electrocardiogram, hematocrit or hemoglobin.
a. Chronic Pulmonary Insufficiency
Comment: One commenter indicated that arterial blood gases are simplified in the proposed rules and was concerned about eliminating carbon dioxide (CO2) tension. The commenter believed that even if two tests are done three weeks apart, the test results can be influenced significantly and easily by such things as a little anxiety, or breath holding. In addition, the commenter explained that a person with a resting PaO2 of 60-65 could be really hypoxic if the individual were hyperventilating. Although the commenter believed that the use of the diffusing capacity of the lungs for carbon monoxide or arterial blood gas studies are appropriate, the commenter suggested that revisions should be made in 3.02 to include CO2-O2 correlations.
Response: We agree with the commenter that the results of an arterial blood gas study could be influenced by such things as a little anxiety or breath holding. To avoid any misapplication, we amended listing 3.02C2 and C3 to delete the values at two altitudes (designated 3.02C2a and b in the NPRM). We revised these listings to include again tables III-A, III-B and III-C from our prior 3.02 listings because these criteria are more sensitive indicators of disabling impairment of gas exchange and include correction for hypoxemia for carbon dioxide tension.
Comment: A commenter recommended that listings 3.02C2 and C3 specify that the values are based on breathing room air.
Response: We agree and have added "breathing room air" to 3.00F2, 3.00F4, 103.00C1 and listings 3.02C2 and C3.
Comment: One commenter was concerned with the requirement in listing 3.02C2 for resting arterial blood gases in a stable state 3 or more weeks apart. The commenter indicated that getting resting arterial blood gas studies performed in stable state on one occasion is difficult, but it will be even harder to get these results on two occasions. The commenter felt that this requirement precludes the use of medical evidence of record.
Response: We did not adopt the comment. This requirement for results on two different occasions is included in these final rules because repeat testing increases the probability that hypoxemia is chronic. This requirement also ensures that inappropriate decisions will not be made utilizing blood gas values during an acute illness which is not expected to result in a chronic gas exchange impairment. We believe that in some cases evidence of record may contain applicable pre-discharge reports from a hospitalization for an intercurrent acute illness, or out-patient records may have the test results that are applicable, particularly when chronic home oxygen therapy is contemplated.
Comment: Two commenters indicated that the heights listed in tables I and II should be listed in either inches or centimeters, and the English and metric system should not be mixed by using tenths of an inch. They also indicated that because the one-second forced expiratory volume cannot be estimated more closely than to tenths of a liter, the listing requirements should not be shown in hundredths of a liter.
Response: We decided that rather than showing the heights in tables I and II in either inches or centimeters, it would be clearer if we indicated both. We have revised the tables accordingly. We do not agree with the commenter that one-second forced expiratory volume results cannot be estimated more closely than to tenths of a liter because it has been our program experience that many times results are reported beyond a tenth of a liter, and that impairment severity assessment can be made more accurately with these results.
Comment: One commenter suggested that we not permit the purchase of arterial blood gases with exercise and clarify that such tests be evaluated only when obtained as medical evidence of record. The commenter did not feel that this test provided information of greater adjudicative value than other testing methods that are more readily available and less risky to the claimant.
Response: We did not accept the comment because these rules provide that exercise testing should only be purchased in infrequent instances in which the other studies may underestimate the degree of functional loss. Further, our rules in 3.00F3 specify that exercise arterial blood gas measurements should only be purchased after careful review of the medical history, physical examination, chest x-ray or other appropriate imaging techniques, spirometry, diffusing capacity of the lungs for carbon monoxide study, electrocardiogram, hematocrit or hemoglobin, and resting blood gas results by a program physician, preferably one with experience in the care of patients with pulmonary disease, to determine whether obtaining the test would present a significant risk to the individual. The purchase of such tests on infrequent occasions is designed to benefit claimants by providing another means by which a disabling impairment can be established.
Comment: Three individuals noted that proposed listing 3.02C2b should read, "At an altitude of 4000 feet or greater...."
Response: We did not accept the comment because we have removed the altitude criteria of proposed listing 3.02C2a and C2b and revised the listing to include tables III-A, III-B, and III-C from 3.02 of the prior rules.
Comment: A physician commented that the separation of ventilatory impairments (chronic obstructive and chronic restrictive diseases) into two separate listings tends to be confusing and implies an importance in establishing a diagnosis. To simplify the process, the commenter suggested we have one listing that provides for either a one-second forced expiratory volume equal to table I or a forced vital capacity equal to table II. This person believed that one listing would suffice because someone with chronic obstructive pulmonary disease could be considered as meeting the listings in either table I or table II. The same would be true if the person has a predominantly restrictive disease.
Response: We did not accept this comment because an individual with a predominantly air obstructive impairment that meets table II would nearly always meet table I. (These tables are contained in listing 3.02.) However, an individual with a restrictive impairment that meets table II may not meet table I. We do not want to construct a listing that will disadvantage anyone. In addition, the designations, "one-second forced expiratory volume" and "forced vital capacity," are used in clinical practice and connote different physiologic deficits. For these reasons, we prefer to incorporate the one-second forced expiratory volume and the forced vital capacity in listing 3.02 as we have constructed it.
b. Cystic Fibrosis
Comment: Several commenters were concerned that the criteria for adults to qualify for benefits with cystic fibrosis are more stringent than the criteria for children with cystic fibrosis. Under the proposed criteria, a child who is 67 inches tall has to have a one-second forced expiratory volume of 1.6 to meet the childhood listings. An adult 67 inches in height would require a one-second forced expiratory volume of 1.45 to meet the adult listing. The commenters noted that the one-second forced expiratory volume values for adults are those established for the evaluation of adults with chronic pulmonary insufficiency while a separate table just for cystic fibrosis has been established for children. A commenter noted that we did not list any manifestation of cystic fibrosis to aid in the evaluation of adults similar to the childhood manifestations. The commenters urged that more realistic criteria, similar to the criteria proposed for children, be provided for adults.
Response: We agree with the commenters that the criteria for adults with cystic fibrosis could be revised. We revised 3.04A to now include a table (table IV) which will be used solely for the evaluation of adults with cystic fibrosis, similar to the approach used in 103.04A of the childhood listings. We have included the criteria from 103.04C of the childhood listing in the adult listings at 3.04C. In turn, we revised the childhood criteria to include the criteria in 3.04B in the childhood listing (now in 103.04D). We believe that these changes will provide a fair and equitable means for evaluating both adults and children.
b. Pulmonale Secondary to Chronic Pulmonary Vascular Hypertension
Comment: A commenter noted that this listing (proposed listing 3.10) requires arterial blood gas studies on at least two occasions, 3 or more weeks apart, but the listing did not set an outside limit for this requirement. The commenter felt a limit should be set or situations may arise when, for example, one study was performed in 1989 and another in 1991, and the listing requirement would be satisfied.
Response: We agree with the comment and have revised the final listing by including a cross reference to listing 3.02C2 which indicates that these studies be within a 6-month period.
d. Sleep-Related Breathing Disorders
Comment: A commenter objected to this listing because it merely provides cross references to other listings for cor pulmonale, obesity, and organic brain syndrome to evaluate the manifestations of sleep apnea. The commenter felt that specific criteria should be developed for the evaluation of this disorder that weigh more heavily on the disabling effects of daytime drowsiness. The commenter suggested that it ought to be possible to develop a listing for sleep apnea that relies upon sleep laboratory studies and suggested the listing could perhaps be based upon either the number of instances of arousal due to sleep apnea or the degree of hypoxemia demonstrated during sleep testing.
Response: We did not accept this comment because we are not aware of any data to support a relationship between the findings of sleep studies (number of apneas, arousals, severity of desaturation during sleep) and the individual's remaining daytime functional capacity. There are considerable data available relating functional limitations due to extreme obesity, impaired cardiopulmonary function and hypoxemia while awake. We acknowledge that excessive daytime drowsiness can lead to cognitive and personality changes, as well as affective disturbances which result in impaired daytime functioning. Individuals with excessive daytime drowsiness may have disturbances in orientation, memory and personality and these manifestations can be considered under listing 12.02. However, to make our intent clearer, we modified 3.00H to elaborate that daytime drowsiness may affect memory, orientation, and personality and that longitudinal treatment records may be needed to evaluate mental functioning.
4. 103.00 Preface to Part B
Comment: One commenter noted that this section emphasizes medical evaluation and does not mention the importance of nonmedical evidence in evaluating impairment severity. The commenter indicated that valuable information about a child's condition can often be given by a parent or other caregiver. This individual also indicated that the requirement that respiratory disorders be evaluated by a specialist at the State agency may send a message that medical evidence provided by non-specialist treating sources will not be considered important.
Response: The listings contain examples of some of the most common impairments in the disability program. The criteria include specific symptoms, signs, and laboratory findings that are considered to characterize impairments severe enough to prevent a child applying for benefits under the SSI program from functioning independently, appropriately, and effectively in an age-appropriate manner. Claimants may be found disabled based on medical factors alone if their impairment(s) meets or equals the medical criteria in a listing. If the severity of a claimant's impairment(s) does not meet or equal medically the severity of an impairment in the listings, we then, in the case of a child under the SSI program, determine whether the impairment(s) functionally equals the listings and, if not, then perform an individualized functional assessment to determine whether the child has an impairment(s) of comparable severity to one that would disable an adult. Thus, following this sequential evaluation process, nonmedical evidence is considered at all appropriate steps in the process—and especially for children at the equals step and in performing an individualized functional assessment. Nonetheless, to clarify this point, we have expanded the fourth paragraph to emphasize the importance of equivalence determinations and individualized functional assessments. In addition, we clarified the language that concerned the commenter, which was that a child's respiratory disorder will be evaluated, if possible, by a pediatrician or by a specialist in respiratory diseases of children. Our intent for this requirement was to recognize the importance of specialization in childhood respiratory disease, not to send a message that evidence provided by a non-specialist treating source will not be considered important. To clarify our intent, we have added a statement at the end of the first paragraph in 103.00A to indicate that reasonable efforts should be made to ensure review by a program physician specializing in the evaluation of childhood respiratory disease or by a qualified pediatrician. This language is adapted from section 1614(a)(3)(H) of the Act and merely reflects our current policy.
b. Documentation of Pulmonary Function Testing and Documentation of Chronic Impairment of Gas Exchange
Comment: A commenter indicated that the proposed rules did not provide enough detailed information on when to purchase the various pulmonary tests. The commenter indicated that the rules should discuss the value of studies performed during or soon after an acute respiratory illness. Two commenters suggested that providing a minimum age for spirometry would be helpful.
Response: We agree with the commenter that our rules should provide more detailed information on when to purchase the various pulmonary tests. Accordingly, we added a new seventh paragraph to 103.00B and a new second paragraph to 103.00C1 and C2 which indicates that purchase may be appropriate when there is a question of whether an impairment meets or is equivalent in severity to a listing and the claim cannot otherwise be favorably decided. In addition, in the seventh paragraph of 103.00B, we indicate that purchase of a pulmonary function test is appropriate only when the child is capable of performing reproducible forced expiratory maneuvers, and that this capability usually occurs around age 6.
Comment: A commenter indicated that in cases of cystic fibrosis, pulmonary functioning may be extremely and progressively impaired most of the time. Therefore, children with cystic fibrosis should be excluded from the requirement stated in 103.00B that the criteria in 103.04 (incorrectly referred to as 103.10 in the NPRM) can only be applied during the child's most stable state of health because these children's most stable state of health may occur only upon discharge from a hospital and thereafter begin to deteriorate.
Response: We have modified the language in this section and 3.00E to define "most stable state of health" to mean any period in time except during or shortly after an exacerbation. In addition, in evaluating a child's claim for disability, we consider all available evidence related to the child's impairment so that we have a longitudinal view of the impact of the impairment on the child.
Comment: A commenter suggested that we consider the use of pulse oximetry for older children, and stated that the protocol for the test should require a statement regarding the child's cooperation or behavior.
Response: We agree with the commenter that the use of pulse oximetry should be extended to older children, and we have amended 103.00C2 to indicate that pulse oximetry may be substituted for arterial blood gases in children under 12 years of age. The use of pulse oximetry to assess oxygenation status (rather than obtaining arterial blood gas samples through arterial puncture) has become the standard of care in the pediatric community. Experience has shown that pediatric patients infrequently have arterial gases drawn unless they already have an indwelling arterial line or are in an emergency intensive care setting. We have also added a statement to 103.00C2 that the report of the test should include information on the child's cooperation in performing the test.
Comment: A few commenters suggested that, rather than having an explanation of rounding in 103.00B, we use the same format for recording height, one-second forced expiratory volume and vital capacity as is used in the adult listings in 3.02A and 3.02B. These same commenters suggested that in proposed 103.02 table I, 103.02 table II, and 103.14 table III we show the heights in inches and centimeters because some facilities report both ways.
Response: We agree and have modified the tables accordingly.
c. Cystic Fibrosis
Comment: A commenter recommended that we consider adding the stool trypsin test for use in the evaluation of cystic fibrosis in young babies and to confirm gastrointestinal involvement. The commenter indicated that the diagnosis of cystic fibrosis may be based on this test in the "neonate period."
Response: We did not accept the comment because the stool trypsin test is not considered an acceptable diagnostic test for cystic fibrosis. The test can be considered only as a gross screening test for pancreatic insufficiency due to any cause and is, therefore, nonspecific for cystic fibrosis. We have, however, added to 103.00D another diagnostic test, the "CF gene mutation analysis."
a. Chronic Pulmonary Insufficiency (Numbered 103.14 in the NPRM and Titled Chronic Obstructive or Restrictive Pulmonary Disease Due to Any Cause)
Comment: One commenter suggested that we should explain what we mean by "poor weight gain" in 103.02E6 and F2 (designated 103.14D6 and E2 in the NPRM) and also specify if loss of weight could meet these listings requirements.
Response: We agree with the commenter and have clarified 103.02E6 and F2. We deleted "poor weight gain," and, to avoid any ambiguity, we now provide criteria for the evaluation of involuntary weight loss or failure to gain weight at an appropriate rate. In this way, we not only include children who have stopped gaining weight but also children who do not gain enough weight. We also provide for the possibility that a child will actually lose weight.
Comment: A commenter indicated that the requirement in 103.02F (designated 103.14E in the NPRM) for two hospital admissions within a 6-month period seemed to be set at a higher level of severity than the requirement in 103.04C of one episode of infection every 6 months. This commenter believed that requiring hospitalization as a criterion of severity discriminates against children who are uninsured or underinsured.
Response: We do not agree with the comment. The requirement of only one increased bacterial infection under 103.04C (103.04B in the NPRM) within a 6-month period is in relation to cystic fibrosis, which is a condition affecting multiple systems associated with progressive irreversible pulmonary morbidity and death during early to mid-adult life. The occurrence of more than one exacerbation for a child with cystic fibrosis during a 6-month period for recurrent respiratory infection is normally associated with progression and a superimposed underlying persistent pulmonary infection and functional impairment. On the other hand, repeated hospital admissions for lower tract respiratory infection in children with other types of chronic pulmonary disease more commonly reflect acute intermittent episodes that are not related to chronic persistent underlying pulmonary infection. These criteria do not discriminate against any class of children, but only provide criteria to quickly allow the most seriously impaired individuals. This is not to imply that children who do not meet or equal in severity a listing cannot be found disabled at the final step of the sequential evaluation process.
Comment: A legal group commented that the requirement in 103.03C that a child must meet two of the three listed criteria seemed excessive because if a child has asthma as severe as described, it should be enough to meet one of the three listed criteria. Another commenter noted that proposed 103.03C and 103.03C3 should include the use of inhaled steroid as well as oral steroid. The commenter also suggested that administration of intravenous fluids should be equated with intravenous administration of a bronchodilator and antibiotics.
Response: We agree with the commenter that the requirement in proposed 103.03C for two of the three listed criteria could be problematic. We, therefore, have deleted the criterion in proposed 103.03C2 because "barrel or pigeon chest" deformity was merely a description of anatomical deformities which may have led to various interpretations. We also revised the listing to require only one of the two remaining criteria. We did not include the use of inhalant steroids in 103.03C1 or 103.03C2 (103.03C3 in NPRM), because this form of treatment does not in itself imply the required level of severity that is intended by this listing. We did not accept the comment to equate administration of intravenous fluids with intravenous administration of a bronchodilator and antibiotics because the need for intravenous fluids generally is not a serious manifestation of chronic disease. The administration of intravenous fluids is merely a treatment modality for an acute episode of asthma.
c. Cystic Fibrosis
Comment: A commenter indicated that the listing for cystic fibrosis is problematic. The commenter indicated that this listing requires very low one-second forced expiratory volume values and totally disregards functioning except in children who cannot have spirometry performed. According to the commenter, the reliance on spirometry results is particularly unwise because of the episodic nature of the progression of the disease over time. The commenter believed that we should evaluate the underlying data rather than relying on proxies which are statistically associated with them. This individual recommended that proposed 103.04A1 should be separated into two different parts and proposed 103.04A2 and A3 be renumbered 3 and 4 (i.e., 1. History of dyspnea on exertion and 2. Accumulation secretions as manifested by repetitive coughing or cyanosis; or xxx). In addition, this person believed that the specification of intravenous treatment in proposed 103.04B (final 103.04C) is unwise given the constantly changing nature of treatment. The commenter said that the same criticism could be made of the decision to rely on the quantitative iontophoretic test for the diagnosis of cystic fibrosis. This individual believed that, not only may there be advances in diagnostic techniques within the next 7 years, but also many SSI eligible children would not have access to the designated test, and no child with an impairment as severe as that required by the criteria in 103.04 should be determined not to meet the listings simply because cystic fibrosis has not been diagnosed by the most accurate technique in existence.
Response: We did not accept the comment about low one-second forced expiratory volume values or proxy values. We use one-second forced expiratory volume results in children with cystic fibrosis because it is the testing procedure that is most widely accepted by pediatric specialists to evaluate pulmonary functional status. The one-second forced expiratory volume value set at 60 percent of predicted normal is not low, but higher than the former value, which was set at 50 percent. The one-second forced expiratory volume value is not a proxy measurement for other underlying impairments but a direct measurement of the respiratory system's ability to perform a vital life function. The 60 percent value selected correlates reasonably well with clinical scoring systems that reflect other manifestations of pulmonary impairment, including activity levels, radiographic findings, and nutritional state. Studies also indicate that very few children with cystic fibrosis and one-second forced expiratory volume values that exceed 60 percent of predicted normal are found to experience significant limitation of activity, malnutrition or other evidence of severe disease. Although medical progress in the management of cystic fibrosis continues, intravenous or inhalant antimicrobial therapy is expected to be a necessary modality for the large majority of children with cystic fibrosis for at least the next 5 years.
As mentioned in our discussion in the comment and response on 3.00D, we have revised the methods for diagnosing cystic fibrosis in 3.00D and 103.00D. We did not accept the comment that 103.04B1 (referred to by the commenter as 103.04A1) be separated into two criteria. The criteria in 103.04B1 refer to a history of signs and symptoms and 103.04B2 and B3 are criteria for clinical and laboratory findings, respectively. If we separated 103.04B into two criteria, a child could then meet the listing based on a history of signs and symptoms with no definitive clinical and laboratory findings. This would be contrary to statutory and regulatory requirements that disability be established based on signs, symptoms, and laboratory findings. We also do not believe that an earlier expiration date for these listings should be set. As previously stated, if there are medical advances which should be reflected in our listing criteria, we can revise all or portions of our listings at any time before the end of the 7-year expiration date. Finally, as we make clear in these listings, because an individual fails to meet the requirements of a listing does not mean the claim is denied. We have explained this longstanding policy in our final rules (3.00A and 103.00A) and discussed it under our explanation of the final rules.
d. Additional Change
In a technical change required by these final rules, we revised § 416.926a(d)(3) and (d)(12) to be consistent with the new listings. Paragraph (3) formerly included mechanical ventilation as an example of a life-sustaining device. Inasmuch as final listing 103.02C1, provides for the frequent need for mechanical ventilation, we are removing mechanical ventilation from this functional equivalence example. In its place, we include central venous alimentation catheter as an example of a life-sustaining device. Paragraph 12 formerly included tracheostomy in a child who had not attained age 3 as an example of a consequence of an impairment that would establish functional equivalence to the listings. Inasmuch as final listing 103.02D (proposed listing 103.14C) incorporates tracheostomy in a child who has not attained age 3 into the listings, equivalence in this situation is no longer an issue.