| 4190-29P |
DEPARTMENT OF HEALTH AND HUMAN SERVICES
Social Security Administration
20 CFR Part 404 (58 FR 36008)
Regulations No. 4
RIN 0960-AC06
Federal Old-Age, Survivors, and Disability Insurance; Determining Disability and Blindness; Revision of Part A and Part B of the Listing of Impairments; Endocrine, and Multiple Body Systems; Immune System
AGENCY: Social Security Administration, HHS
ACTION: Final rules
SUMMARY: These amendments revise the Listing of Impairments in appendix 1 of 20 CFR part 404, subpart P (hereafter referred to as "the listings"). They establish a new listing section called "Immune System" in both part A and part B of appendix 1. The new part A (adult) listings section includes up-to-date criteria for evaluation of connective tissue diseases (previously contained in the "Multiple Body Systems" section) and establishes a listing for the evaluation of human immunodeficiency virus (HIV) infection. The amendments also move the adult listing for obesity from the "Multiple Body Systems" section to the "Endocrine System" section, and change the name of the "Endocrine System" section to "Endocrine System and Obesity." The new part B (childhood) section establishes a listing for the evaluation of human immunodeficiency virus (HIV) infection, includes up-to-date criteria for evaluation of congenital immune deficiency disease (previously contained in the "Multiple Body Systems" section), and adds new criteria for evaluation of connective tissue diseases.
These criteria describe disorders that are severe enough to prevent a person from performing any gainful activity, or in the case of a child under age 18 applying for Supplemental Security Income (SSI) based on disability, severe enough to prevent the child from functioning independently, appropriately, and effectively in an age-appropriate manner.
EFFECTIVE DATE: These rules are effective July 2, 1993.
FOR FURTHER INFORMATION CONTACT: Harry J. Short or Richard M. Bresnick, Legal Assistants, Office of Regulations, Social Security Administration, 6401 Security Boulevard, Baltimore, MD 21235, (410) 965-6242 or 965-1758.
SUPPLEMENTARY INFORMATION:
The Social Security Act (the Act) provides, in title II, for the payment of disability benefits to workers insured under the Act. Title II also provides for the payment of child's insurance benefits for persons who become disabled before age 22 and widow's and widower's insurance benefits based on disability for widows, widowers, and surviving divorced spouses of insured individuals. In addition, the Act provides, in title XVI, for SSI payments to persons who are disabled and have limited income and resources. For workers insured under title II, for children of workers insured under title II who become disabled before age 22, for widows, widowers, and surviving divorced spouses claiming widow's or widower's insurance benefits based on disability under title II, and for adults claiming SSI benefits based on disability, "disability" means inability to engage in any substantial gainful activity. For children under the age of 18 who apply for SSI benefits based on disability, "disability" means that the child's physical or mental impairment(s) is of comparable severity to an impairment that would make an adult (a person age 18 or older) disabled. Under both title II and title XVI, "disability" must be by reason of a medically determinable physical or mental impairment or combination of impairments that can be expected to result in death or that has lasted or can be expected to last for a continuous period of at least 12 months. To the extent that Medicare and Medicaid eligibility are based on title II and title XVI eligibility, these regulations also affect the Medicare and Medicaid programs.
Under the sequential evaluation process, if the evidence shows that an individual is not engaging in substantial gainful activity and has an impairment(s) that meets the duration requirement, is severe, and meets or equals in severity a listing criteria, the individual is disabled. (In the case of a child applying for SSI, this includes consideration of whether the child's impairment(s) is functionally equivalent to a listed impairment, as defined in § 416.926a.) If the impairment(s) (e.g., HIV infection) does not meet or equal in severity any listing criteria, we evaluate all signs, symptoms, laboratory findings, and other evidence to determine whether the person is disabled. For an adult, we assess residual functional capacity and, based on that assessment, determine whether the claimant retains the capacity to perform past relevant work, or, if not, whether he or she retains the capacity to perform any other work considering his or her residual functional capacity, age, education, and work experience. If not, the adult is disabled. For a child under the age of 18 applying for SSI, we individually assess the child's ability to function to determine whether there is a substantial reduction in the child's ability to function independently, appropriately, and effectively in an age-appropriate manner. If there is such a substantial reduction, the child is disabled.
Medical criteria for evaluating disability and blindness at the third step of the sequential evaluation processes for adults and children are found in the listings. The listings include examples of the most commonly occurring medical conditions for persons who file applications for disability benefits. It describes, for each of 13 major body systems, impairments that are considered severe enough to prevent an individual from engaging in any gainful activity, or in the case of a child under the age of 18 applying for SSI, examples of impairments that are severe enough to prevent a child from functioning independently, appropriately, and effectively in an age-appropriate manner. To establish disability under the Act, the impairment must be expected to result in death or last or be expected to last for a continuous period of not less than 12 months. Most of the listed impairments are permanent or are expected to result in death; in some instances, a specific durational requirement is a part of the medical criteria for the impairment (in addition to the 12-month duration requirement that applies to all impairments that are not expected to result in death). If an individual is not performing substantial gainful activity and has an impairment that meets the requirements of one of the listings, or has an impairment or combination of impairments that is equal in severity to one of the listings (and meets the duration requirement), the individual is disabled. If, however, the individual does not have an impairment which meets or equals in severity the requirements of a listing, the claim is not denied and no conclusion on the issue of disability is made. Rather, resolution of the issue of disability depends on other factors. For adults, these other factors are residual functional capacity, and, for adults who are unable to perform their past work, age, education, and work experience. For children whose impairments are severe but do not meet or equal in severity the requirements of a listing, we will do an individualized functional assessment and determine whether the child is able to function independently, appropriately, and effectively in an age-appropriate manner.
Appendix 1 consists of two parts, part A and part B. The criteria in part A apply to the evaluation of impairments of adults but may, in some cases, be appropriate for evaluating impairments in children under age 18. Part B contains medical criteria for the evaluation of impairments in children under age 18 when criteria in part A do not give appropriate consideration to the particular effects of the disease processes in childhood. In evaluating disability for a child under age 18, we use part B first. If the child's impairment(s) does not meet or equal the medical criteria in part B, or the criteria in part B do not apply, then we use the medical criteria in part A, when the criteria are appropriate. To the extent possible, we maintain a structural and content relationship between parts A and B (see §§ 404.1525 and 416.925). When part A criteria are repeated in part B, our intent is to eliminate any question about their application to children.
We revised the listings on December 6, 1985 (50 FR 50068). At that time, as a result of medical advancements in disability evaluation and treatment, and program experience, we indicated that the listings should be reviewed periodically and updated. Accordingly, we specified termination dates for the listings ranging from 4 to 8 years. These final rules revise the listings to: Establish a new listing section called "Immune System" in both part A and part B of appendix 1; establish new listings for the evaluation of HIV infection in both adults and children; update the criteria for evaluation of connective tissue disorders in adults and for congenital immune deficiency disease in children; establish new criteria for evaluation of connective tissue disorders in children; move the adult listing for obesity from the "Multiple Body Systems" section to the "Endocrine System" section; change the name of the adult "Endocrine System" section to "Endocrine System and Obesity"; delete Hansen's disease (leprosy, formerly Listing 10.02) from the listings; and modify the "Multiple Body Systems" section for children to make it effective for 5 years.
We revised the connective tissue disorders criteria with information we received from individuals recommended by various professional groups, including the American College of Physicians, the American College of Rheumatology (formerly the American Rheumatism Association), the Arthritis Foundation, the Lupus Research Institute, the American Society of Internal
Medicine, and from individual Federal and State representatives with expertise in the evaluation of disability claims involving connective tissue disorders. In addition, in developing the proposed part B criteria for children, we received information from individuals with expertise in these areas.
We developed the proposed listings contained in our December 18, 1991, Notice of Proposed Rulemaking (NPRM) (see 56 FR 65702) for HIV infection based in part on information from numerous individuals recommended by or affiliated with various professional organizations, including the Public Health Service's Centers for Disease Control (CDC), the Johns Hopkins Hospital, the State of Maryland AIDS Administration, the Department of Veterans Affairs, and Federal representatives with expertise in the evaluation of disability claims involving HIV infection. A number of individuals who commented on the NPRM expressed concern that we did not consult with experts on the various segments of the population who are infected with HIV, especially women and children. Therefore, in response to the comments, we obtained additional information about women and children and other segments of the population infected with HIV, and about other issues from experts in the Department of Health and Human Services, including experts from the Public Health Service's CDC, Health Resources and Services Administration, and National Institutes of Health. We also obtained additional assistance from individual physicians and other experts involved in the evaluation and treatment of HIV infection—particularly in women and children—in various parts of the country, as indicated below. We obtained additional information from individuals at Albert Einstein College of Medicine, Beth Israel Hospital, Bronx Lebanon Hospital, Columbia Presbyterian Hospital, Harlem Hospital, Montefiore Hospital, St. Luke's/Roosevelt Hospital, and St. Vincent's Hospital in New York; Howard University Hospital in Washington, D.C.; University of Maryland Medical Center and Johns Hopkins Hospital in Baltimore; University of Texas Medical School in Houston; Cook County Hospital and Children's Memorial Hospital in Chicago. Several of the experts were recommended to us by the American Medical Association and the American Academy of Pediatrics. We also received information from the American Medical Association and the HIV Project of the MFY Legal Services Inc., a legal advocacy group in New York City. Therefore, the listings contained in this final rule reflect updated information about HIV infection.
HIV Infection
In 1980, shortly after acquired immunodeficiency syndrome (AIDS) was first identified in the United States, the CDC developed a case definition in order to conduct epidemiologic surveillance. The CDC defined AIDS based on a variety of diseases that encompassed the most severe manifestations observed in infected individuals. In late 1982, we began receiving disability claims from individuals infected with HIV. We used the CDC's surveillance definition of AIDS in developing our initial criteria for determining disability of listing-level severity in people with AIDS. These criteria provided that an individual who had a confirmed diagnosis of AIDS, as manifested by one or more of the conditions identified by the CDC, and who was not engaging in substantial gainful activity, would have an impairment of listing-level severity. As medical knowledge and understanding about HIV infection was continuously refined, and with knowledge derived from adjudicating disability cases involving HIV infection, we updated our policies.
For instance, as early as 1983, clinicians identified a syndrome that for a time was known as AIDS-Related Complex (ARC), although that term was never used or defined by the CDC. Shortly thereafter, we issued instructions stressing our policy that the evaluation of disability in these cases was not limited to the CDC's surveillance definition of AIDS, that claimants with AIDS or ARC, like all disability claimants, must be evaluated on a case-by-case basis, and that an individual need not have fully developed AIDS to be found disabled.
We reminded our adjudicators that individuals who have immune system dysfunction, but who do not have a confirmed diagnosis of AIDS, may still have an impairment that is of listing-level severity, either because of a manifestation that, in and of itself, meets the criteria of a listing, or because an individual's impairment(s) is equal in severity to a listing. We said that, as with any medically determinable impairment, the assessment of severity must take into account all signs, symptoms, and laboratory findings—not only those included in our criteria—and must follow the full sequential evaluation process if an applicant's impairment(s) is severe and does not meet or equal in severity any listing.
As more information about HIV infection became available and as we gained even more adjudicative experience, it became apparent that HIV infection was being manifested by impairments that were not encompassed under ARC, or in the CDC's criteria for AIDS. By 1987, we issued instructions that provided specific criteria beyond those covered in the CDC's surveillance definition for adjudicators to use in evaluating whether a claimant's impairment(s) was of listing-level severity. We have continued to update and refine our instructions to reflect the escalating array of information available on the manifestations of HIV infection.
We have always viewed AIDS (and other symptomatic HIV infection) from a different perspective than the CDC. The CDC defines AIDS for health purposes to enhance its capability for activities such as disease reporting and surveillance, epidemiologic studies, prevention and control activities, and public health policy and planning. Its definition is not intended to determine whether any statutory or legal requirements for disability are met. In evaluating disability claims, our concern is to determine whether an individual's impairment(s) is severe enough to prevent him or her from engaging in any substantial gainful activity (or, in the case of a child under age 18 applying for SSI, substantially reduces the child's ability to function independently, appropriately, and effectively in an age-appropriate manner).
In these final rules, we have made it clear that all our disability evaluation standards apply to cases of HIV infection in the same way they apply to cases involving other impairments. The standards require, on a case-by-case basis, an evaluation of all relevant factors, including the symptoms (such as pain, fatigue, and malaise), signs, and laboratory findings, as well as the effects of medication, on the ability to function, and a determination whether an individual is able to engage in substantial gainful activity or, in the case of a child, able to engage in age-appropriate activities. Although these final rules relate only to one part of the sequential evaluation process, they stress that (as we do for all individuals) we apply the full sequential evaluation process when adjudicating claims from individuals with HIV infection.
HIV Infection in Women and Children
These final rules include specific criteria to take into account the clinical manifestations and course of the disease in women and children.
The criteria in parts A and B recognize that HIV infection can manifest itself differently in women (including female adolescents) than in men. Therefore, the final rules state that it is important when reviewing the claim of a woman with HIV infection that manifestations of HIV infection that affect women (e.g., gynecological conditions) be considered in assessing impairment severity and the degree of functional loss. Similarly, the criteria in part B recognize that the disease process may manifest itself differently in children (especially younger children) than in adults.
Other Immune System Listings
The final rules also include listings for evaluation of other immune system disorders. In preparing these listings, our aim was to put less emphasis on disease labeling or diagnosis, and to place more emphasis on the functional impact on a person's ability to work or, in the case of an SSI claimant under age 18, on the ability to function independently, appropriately, and effectively in an age-appropriate manner.
Endocrine System and Obesity
We revised the adult listing for obesity and moved it from the Multiple Body Systems (10.00) to the Endocrine System (9.00), and renamed the latter Endocrine System and Obesity. Therefore, all listings that were under 10.00, Multiple Body Systems, are now under either 14.00, Immune System, or 9.00, Endocrine System and Obesity. We also converted the weight tables that accompany the obesity listing into metric measurements.
Explanation of the Final Rules
We published an NPRM in the FEDERAL REGISTER on December 18, 1991 (56 FR 65702), and invited interested persons, organizations, and groups to submit comments pertaining to the proposed rules within a period of 60 days from the date of publication of the NPRM. The comment period ended on February 18, 1992.
In response to the NPRM, we received over 6,000 letters containing comments pertaining to the changes we proposed. The majority of the letters were form letters in support of the proposed changes to the listing for systemic lupus erythematosus (final Listing 14.02). We also received a number of other form letters that were sent by multiple individuals. The majority of these letters concerned the proposed HIV infection listings. Many of these were from legal services organizations and advocacy groups, State and city government departments, Members of Congress, and individual lawyers. Some letters came from individuals or Government agencies whose responsibilities require them to make disability determinations involving HIV-related impairments under titles II and XVI of the Act. Other letters came from medical associations, hospitals, physicians and other medical professionals, or from individual private citizens.
The public comments were invaluable to us in drafting these final rules. Some of the commenters pointed out problems or potential problems with the proposed rules, and we adopted or accommodated many of these comments. Other commenters, however, suggested changes that would go beyond the scope of these rules, or even our authority in promulgating regulations. We carefully considered all the comments we received, and adopted the commenters' suggestions whenever possible. In the public comments section of this preamble, we address all the substantive comments and explain how we used the comments (or why we did not use them) in preparing these final rules.
The following is a summary of the listings we are adopting in these final rules, with an explanation of the more important changes we made from the text of the NPRM. We describe other changes in the public comments section of this preamble.
9.00 ENDOCRINE SYSTEM AND OBESITY
9.00 Endocrine System and Obesity
Because we have moved the listing for obesity from former Listing 10.10 to final Listing 9.09, we also moved the prefatory text describing obesity, which was formerly in 10.00B, to the second paragraph of final 9.00. We also revised the paragraph to make it more specific to listing-level determinations. In response to a comment, we added a second paragraph which clarifies that the weight-bearing criterion in Listing 9.09A refers to the lumbosacral spine, not the cervical or thoracic spines.
We changed the headings of 9.00 and 9.01 to include references to obesity. There are no changes in the headings from the NPRM.
9.09 Obesity
We revised the listing for obesity, which was previously Listing 10.10 in the Multiple Body Systems section, and moved it to the Endocrine System section, which we renamed Endocrine System and Obesity. The listing for obesity is now Listing 9.09. In addition, we revised paragraph 9.09A (formerly 10.10A) to clarify that the pain, limitation of motion, and evidence of arthritis required in that section must be in the same joint or in the lumbosacral spine. We clarified the rules based on questions we have received over the years.
In response to a public comment, we revised the language of the NPRM to make clear that the phrase "weight-bearing" applies to both the joints and the spine; the revision adds language describing the lower parts of the spine (the lumbosacral spine), which are the weight-bearing areas. This is not a substantive change, but a clarification of the policy as we have always applied it. In the final rules, we also made a technical correction to the weight tables that accompany the obesity listing. We converted the table values to the metric system. Except for some very minor rounding off necessitated by the conversion, we did not change any of the relative weight and height criteria from the prior rules.
14.00 IMMUNE SYSTEM
We have established a new section 14.00, Immune System. The new section includes criteria for systemic lupus erythematosus (14.02), systemic vasculitis (14.03), systemic sclerosis and scleroderma (14.04), polymyositis or dermatomyositis (14.05), undifferentiated connective tissue disorder (14.06), immunoglobulin deficiency syndromes or deficiencies of cell-mediated immunity, excepting HIV infection (14.07), and HIV infection (14.08). We describe each of these listings below.
Final 14.00 includes all the criteria that were previously in 10.00, Multiple Body Systems, with the exception of former Listing 10.02, Hansen's disease (leprosy), which we have deleted, and former Listing 10.10, "Obesity." We deleted the Hansen's disease listing because new cases of the condition are almost nonexistent in the Western Hemisphere and because the disease can now usually be treated successfully. As already stated, we moved the obesity listing to 9.00. We reserved the entry for 10.00, which no longer contains any listings, for future use.
Although we made a number of editorial changes for clarity and consistency, we made few substantive changes from the NPRM in final Listings 14.02-14.07 and 114.02-114.07; that is, all but the HIV listings. We discuss those listings first, beginning with a summary of final 14.00A-C and 114.00A-C, the sections of the preface that are appropriate to the non-HIV listings. We then provide a summary of the provisions of, and changes to, the rules on HIV in 14.00D and 114.00D, and final Listings 14.08 and 114.08.
THE NON-HIV LISTINGS
14.00 and 114.00 Preface
Final 14.00A-C and 114.00A-C describe impairments of the immune system. We made a number of revisions in the final rules from the NPRM, both in response to comments and for technical reasons. Most of the changes were for consistency. We compared the rules in part A with those in part B and, wherever it was appropriate, added provisions that were lacking in one but were present in the other. We also revised language when both part A and part B contained the same provisions but used different language; the revisions simply make their language the same. As we explain below, none of these changes is substantive: They only improve the consistency and clarity of the rules.
14.00A and 114.00A
In 14.00A and 114.00A, we describe some of the components of the immune system. There are no changes from the NPRM.
14.00B and 114.00B
In final 14.00B and 114.00B, we discuss connective tissue disorders. In a technical correction, and for internal consistency, we changed the proposed phrase "connective tissue disease" in the first paragraph and throughout the preface to "connective tissue disorder." This is a more accurate description of the disorders. Moreover, the two phrases were used interchangeably in the NPRM; the revision now uses only one phrase throughout. We also changed the reference to "The American Rheumatism Association" in proposed 14.00B1 to the current name, "The American College of Rheumatology."
For consistency, we revised the language we had proposed as the second paragraph of 14.00B so that it better reflected the language we had proposed in the first paragraph of 114.00B (now the third paragraph in final 114.00B). We also made minor changes to the third paragraph of final 114.00B so that both paragraphs would say the same thing.
The final language in the second paragraph of 14.00B is almost identical to the proposed language. The most significant difference is that we deleted the proposed opening statement from the NPRM, "Each of these disorders should be differentiated diagnostically * * *," for conformity with the childhood rules. Although the clause was true, we believe that it is inherent in the remaining language of the paragraph, as well as being a basic principle of disability evaluation that need not be repeated in this particular context.
We expanded the third paragraph of final 14.00B from the NPRM to incorporate language that was formerly in proposed Listings 14.02-14.06 and 114.02. We simplified those listings, each of which repeated the same provisions about duration of active disease despite prescribed therapy, by incorporating the language into the preface and replacing the repetitious criteria in the listings with cross-references to the preface. We describe our reasons for this change in greater detail in the public comments section of this preamble. However, the revision is merely editorial, not substantive.
In the fourth paragraph of the final adult rules in 14.00B, we made two technical corrections, but no substantive changes. We replaced the abbreviation "SAL" with "SLE," for systemic lupus erythematosus, and we replaced the phrase "undifferentiated connective tissue disease" with "undifferentiated connective tissue disorder," as already described. We did not change the language of the fifth paragraph of the adult rules from the proposed language in the NPRM.
In the sixth paragraph of final 14.00B, we revised the first sentence to refer to "any gainful activity," which is the standard of severity in the listings, as set forth in §§ 404.1525 and 416.925, instead of the proposed "gainful work activities." This is a technical correction for consistency among the rules. We also added a sentence describing our use of the word "severe" in these listings. We explain this addition, and our reasons for making it, in the public comments section of this preamble. We added the latter sentence to the sixth paragraph of final 114.00B.
We made only minor editorial revisions in final 14.00B1-3 (for example, by deleting the word "Listing" before the listing numbers for consistency with other body system listings). In response to a comment, we revised 14.00B4, Polymyositis or dermatomyositis, to provide more information about the criteria for muscle weakness in final Listing 14.05. We also updated the terminology by replacing the references to SGOT and SGPT with the more current, generic term "aminotransferases."
In response to a comment, we added a discussion of so-called overlap syndromes to final 14.00B5. We also indicate that these syndromes are to be evaluated under Listing 14.06, Undifferentiated connective tissue disorders.
Finally, we made a number of nonsubstantive editorial revisions to the childhood rules in 114.00B in addition to those already mentioned. We divided the first paragraph of the NPRM into three separate paragraphs for clarity and better conformity with the paragraphs in the corresponding adult rules. We added a new fourth paragraph, which is identical to the fifth paragraph of 14.00B, to stress the importance of considering the effects of treatment in connective tissue disorders; the addition is only for consistency between the adult and childhood rules.
We also established a new 114.00C to include allergies (also in conformity with the adult rules). We also moved the second and third paragraphs of proposed 114.00B, dealing with growth impairments and Kawasaki disease, into the new section. We did this because both of these paragraphs provide guidance about cross-referring to other listings: The second paragraph of proposed 114.00B provided that growth impairments could be evaluated under the listings in 100.00, and the third paragraph provided cross-reference listings for Kawasaki disease. Inasmuch as the guidance on allergic disorders refers to evaluation under the appropriate body system listing, we believe that it is clearest to group all three paragraphs together under the same heading.
14.00C and 114.00C
Final 14.00C of part A states that allergic disorders are discussed under the appropriate listing for the affected body system. We made no substantive changes from the NPRM in this paragraph. In a technical clarification, we added the phrase "and evaluated," to the sentence to make it clear that allergic disorders are both discussed and evaluated under the appropriate listing for the affected body system.
Final 114.00C of part B is new. As we have explained, we established the section in order to include the same guidance about allergic disorders in the childhood rules as is in the adult rules. We also moved the paragraphs about growth impairments and Kawasaki disease from proposed 114.00B into this new section for reasons already given. We also added a heading, for clarity. In a technical revision, we revised the provision on Kawasaki disease to better state our original intent. The proposed language could have suggested that Kawasaki disease is not a multisystem impairment when, in fact, all we meant to say was that disease of the coronary arteries is the usual cause of listing-level disease.
Because we added this new section in the childhood rules, we redesignated proposed 114.00C, on HIV infection, to 114.00D. This also makes the lettering of the childhood preface correspond to the lettering in the adult preface.
14.02-14.07 and 114.02-114.07 The Non-HIV Listings
14.02 and 114.02 Systemic Lupus Erythematosus
The final rules move former Listing 10.04 to 14.00, renumber it as Listing 14.02, and change the heading from "Disseminated lupus erythematosus" to "Systemic lupus erythematosus" to conform to the current nomenclature for this disease. They also establish a new Listing 114.02 for systemic lupus erythematosus in children that is nearly identical to the adult rule, but includes criteria for the possible limiting effects unique to children that are not included in the adult rules.
In the final adult rule, we expanded and revised the criteria formerly in Listing 10.04 to focus on and delineate severe functional loss. We also removed the requirement that this disorder be established by a positive lupus erythematosus (LE) preparation, biopsy, or positive anti-nuclear antibody (ANA) test in favor of the currently accepted 1982 criteria of the American College of Rheumatology for classification of this disease (cited in final 14.00B1). Both final Listings 14.02A and 114.02A describe listing-level abnormalities in a single organ or body system, whereas final Listings 14.02B and 114.02B describe disability resulting from functional loss of lesser severity than in Listing 14.02A or 114.02A in two or more organs or body systems, with severe documented constitutional symptoms and signs.
We revised both listings from the proposed language in the NPRM in response to public comments and for technical reasons. For reasons we have already explained, we removed the criteria, "Documented * * * by a longitudinal clinical record of at least 3 months demonstrating active disease despite prescribed therapy during this period with the expectation that the disease will remain active for 12 months," from the proposed language in the first paragraph of Listings 14.02 and 114.02, as well as the similar language in Listings 14.02B and 114.02B; the criteria now appear in the third paragraph of 14.00B and the fifth paragraph of 114.00B and are applicable to all connective tissue disorders.
In final Listings 14.02A and 114.02A, we made minor technical revisions to several of the cross-references to other listings so that the listing now refers to other listing sections, rather than to individual listings. The change makes our method of cross-referencing consistent within the two listings. Moreover, by referring to entire body system listing sections instead of individual listings, we ensure that Listings 14.02 and 114.02 will remain current if the numbering changes as other body system listings are revised.
In response to a comment, we added a new Listing 114.02A3 for muscle involvement. The same criterion already appears in the adult rules at Listing 14.02A2. The addition of the new criterion required us to renumber the subsequent criteria. We also reversed the order of the criteria for endocrine and skin involvement so that they are in the same order that they appear in the cross-referenced listings. In response to a comment, we also added a cross-reference to final listing 14.04D to include listing-level Raynaud's phenomena under final Listings 14.02A5 and 114.02A6.
As we explain in the public comment section of this preamble, we also changed the phrase "severe, documented, incapacitating constitutional symptoms and signs" from proposed Listings 14.02B and 114.02B to "significant, documented, constitutional symptoms and signs" in order to clarify the phrase and make it consistent with language in final Listings 14.03 and 14.04.
14.03 and 114.03 Systemic Vasculitis
The final rules move prior Listing 10.03 to section 14.00, renumber it as Listing 14.03, and change the heading from "Polyarteritis or periarteritis nodosa (established by biopsy)" to "Systemic vasculitis" to correspond with currently accepted medical nomenclature. We also expanded this listing to emphasize the spectrum of vasculitic/arteritic syndromes that can preclude any gainful activity. These syndromes include classical polyarteritis nodosa, aortic arch arteritis, giant cell arteritis, Wegener's granulomatosis, and vasculitis associated with other connective tissue disorders.
The only changes from the NPRM language in final Listing 14.03 are those that we have already described in connection with final Listing 14.02. We deleted the language about documentation of active disease for 3 months despite therapy and the expectation of persistence for 12 months from the opening paragraph of the listing and the similar language in Listing 14.03B, because the provisions are now in the third paragraph of final 14.00B. We also changed the phrase "severe, documented, constitutional symptoms and signs" in proposed 14.03B to "significant, documented, constitutional symptoms and signs" consistent with our revisions in Listings 14.02, 14.04, and 114.02.
The childhood listing for systemic vasculitis, 114.03, is unchanged substantively from the NPRM. We made a minor language change for clarity, but the listing still cross-refers to the adult rules in Listing 14.03, and also includes a criterion for growth impairment.
14.04 and 114.04 Systemic Sclerosis and Scleroderma
The final rules move prior Listing 10.05 to section 14.00, renumber it as Listing 14.04, and change the title from "Scleroderma or progressive systemic sclerosis (the diffuse or generalized form)" to "Systemic sclerosis and scleroderma." We deleted the term "progressive" from the title because it was redundant. The criteria in final Listing 14.04 describe systemic disease of severity that precludes performance of any gainful activity for the requisite duration. The proposed criteria provide greater specificity in describing listing-level severity in the extremities and target organs (i.e., lungs, heart, kidneys).
We changed the NPRM language in the same way we have already described in connection with final Listing 14.02. We deleted the language about documentation of active disease for 3 months despite therapy and the expectation of persistence for 12 months from the opening paragraph of the listing and the similar language in Listing 14.04B, because the provisions are now in the third paragraph of final 14.00B. We also changed the phrase "severe, documented, constitutional symptoms and signs" in proposed 14.03B to "significant, documented, constitutional symptoms and signs" consistent with our revisions in Listings 14.02, 14.03, and 114.02.
In response to a comment, we made a minor revision in final Listing 14.04D. We replaced the word "with" with the phrase "characterized by" to clarify our original intent that the phrase "digital ulcerations, ischemia, or gangrene" describes the severe Raynaud's phenomena in the listing.
We added a separate childhood listing because these disorders may be manifested differently in children than in adults. Moreover, even when the manifestations are similar, the impact on a child's growth, development, and age-appropriate functioning may be more serious than the impact on an adult's ability to perform work-related activity. We revised proposed Listing 114.04 by adding cross-references to the documentation requirements in 14.00B3 and 114.00B in the opening paragraph. The revision is for consistency with the corresponding adult section and is not substantive.
We also revised the cross-references to other listings in final Listings 14.04A and 114.04B so that both listings now refer to entire listing sections, rather than to individual listings. This makes the cross-references consistent with those in final Listings 14.02A and 114.02A, and ensures that the listings will remain current when other body systems are revised.
14.05 and 114.05 Polymyositis or Dermatomyositis
The prior listings formerly codified in 10.00ff did not include listings for polymyositis or dermatomyositis. We added the new adult listing to recognize the potential for a disabling work-related functional deficit in some patients with chronic refractory myopathy. We added a separate childhood listing because these disorders may be manifested differently in children than in adults. Moreover, even when the manifestations are similar, the impact on a child's growth, development, and age-appropriate functioning may be more serious than the impact on an adult's ability to perform work-related activity.
In response to public comments, we added a discussion on evaluating severity of muscle weakness to proposed 14.00B4 and changed the cross-reference in final Listing 14.05A from 11.12B to 14.00B4. In response to another comment, we also revised final Listing 14.05B1 to better describe impairment of swallowing. We describe these changes in greater detail in the public comments section of this preamble.
We made only minor revisions to the final childhood listing. As in final Listing 114.04, we added cross-references to the appropriate documentation requirements in final 14.00B4 and 114.00B. We also revised the remainder of the listing language for consistency with other listings.
14.06 and 114.06 Undifferentiated Connective Tissue Disorders
We added new undifferentiated connective tissue disorders listings in parts A and B because some individuals can be disabled at the listing-level by connective tissue disorders that cannot be classified with an exact diagnosis.
In response to a comment about the NPRM, we added to final Listing 14.06 a cross-reference to Listing 14.04. In response to another comment, we revised final Listing 114.06 to change the cross-reference from Listing 14.06 of the adult listings to Listings 114.02 or 114.04 of the childhood listings.
14.07 Immunoglobulin Deficiency Syndrome or Deficiencies of Cell-Mediated Immunity, Excepting HIV Infection; 114.07 Congenital Immune Deficiency Disease
We added new Listing 14.07 to provide criteria for adults comparable to those we formerly included for children in Listing 110.09 (now final Listing 114.07). The listing provides criteria with which to evaluate immunoglobulin deficiency syndromes and deficiencies of cell-mediated immunity, excepting HIV infection.
In the final rule, we reorganized the language of proposed Listing 14.07 in order to make it consistent with Listing 114.07. The reorganization does not change the criteria.
We moved prior Listing 110.09 to section 114.00, renumbered it as Listing 114.07, and changed the heading from "Immune deficiency disease" to "Congenital immune deficiency disease." As in the foregoing listings and throughout these listings, we revised the language of proposed Listing 114.07A1 to make it consistent with final Listing 14.07. Because of this revision, we have deleted the requirement from proposed Listing 114.07A1 that the episodes of recurrent, severe infections must have occurred in the 5 months prior to adjudication. This additional requirement was not only inconsistent with the adult rules, but would have made the childhood listing more stringent than the adult listing and would have been difficult to implement in our case adjudications at the various levels of appeal.
THE HIV LISTINGS
In response to the many comments we received about the proposed rules for evaluating HIV infection, we have extensively revised the final rules from the NPRM. The following are some of the most important changes in the final rules. Thereafter, we provide a summary of all of the final provisions pertaining to HIV, beginning with final 14.00D and 114.00D of the prefaces.
Reorganization and Simplification
The most obvious change we made to the proposed rules in response to public comments was to reorganize and rewrite the proposed HIV infection listings (14.08 and 114.08). We did so in response to many commenters' concerns about the complexity of the proposed listings, and suggestions that we include additional manifestations of HIV infection in the listings and delete or modify some of the criteria we proposed.
Many commenters pointed out that the proposed listings were unnecessarily complex and repetitive for Social Security disability evaluation purposes. This was primarily because we had included in the listings both the CDC's criteria defining AIDS and other manifestations of symptomatic HIV infection we deemed appropriate for inclusion in our listings, even though they are not AIDS-defining under the CDC surveillance definition. As we have already explained, however, the CDC's criteria are primarily for surveillance purposes, not for the evaluation of disability; therefore, the CDC criteria contain requirements that are not necessary in our program.
For instance, the CDC surveillance definition contains criteria for establishing the diagnosis of AIDS in the presence of documented HIV infection, as well as when infection is not documented. However, both categories include a number of opportunistic infections in common that establish the diagnosis of AIDS; for example, pneumocystis carinii pneumonia and extrapulmonary cryptococcosis are included in both categories and, therefore, are repeated within the CDC's surveillance definition of AIDS. As many commenters pointed out, whereas it may be appropriate for the CDC surveillance definition to be repetitive for surveillance purposes, for Social Security disability purposes we need only be satisfied that a person with HIV infection has experienced one of the manifestations (for example, pneumocystis carinii pneumonia or extrapulmonary cryptococcosis) to conclude that the individual has a listing-level impairment. Therefore, it was unnecessary for us to have listed these infections in two places (proposed adult Listings 14.08A2 and C2, and childhood Listings 114.008A2 and C2 for extrapulmonary cryptococcal infections, and proposed adult Listings 14.08A8 and B2, and childhood Listings 114.08A9 and B2 for pneumocystis carinii pneumonia) when the outcome was the same in both instances. Similarly, the CDC surveillance definition includes several separate criteria for Hodgkin's and non-Hodgkin's lymphomas, which we had listed separately, following the CDC surveillance definition. We had also proposed to include other lymphomas that are not included in the CDC surveillance definition, and listed them separately. The commenters pointed out that we were, in effect, saying that any individual who has HIV ifection and any lymphoma would have an impairment that meets our listing and that there was, therefore, no need to list lymphomas in three separate places as we had proposed (i.e., in proposed adult Listings 14.08A6, I, and J, and childhood Listings 114.08A6, H, and I).
Many commenters pointed out that it was also unnecessary, and could be unfair, to provide specific requirements for the diagnosis of each manifestation of HIV infection in the listings. They pointed out that, at a minimum, we could summarize our criteria for establishing the existence of HIV infection and its manifestations in the prefaces to the listings; i.e., 14.00 and 114.00. (They also offered comments about our rules for establishing these findings, many of which we adopted, and which we describe later in this preamble.)
In the final adult rules, therefore, we combined the criteria in proposed Listing 14.08A with the criteria in proposed Listings 14.08B-L and organized them first by etiology of infection (final Listings 14.08A-D) and then by type of manifestation, regardless of etiology (final Listings 14.08E-N). We also removed the specific documentation requirements for each disease or manifestation from the listings and consolidated all documentation requirements with the general discussion of documentation in final 14.00D3 and D4. We removed duplicative language from the listings and clarified the standards established for many of the diseases. We made the same kinds of changes to the childhood listings in 114.00 and 114.08.
Manifestations of HIV Infection in Women
In response to public comments, final Listing 14.08 now also includes specific criteria for most manifestations of HIV infection that are common in women. Because these conditions are now included in the listings, we deleted the discussion of specific conditions common in women that we proposed in 14.00D of the NPRM, although we retained and augmented that section's general discussion of women's issues in final 14.00D5. The final listing, which we describe in greater detail below, explicitly mentions conditions common to women and provides criteria by which we will determine whether a given manifestation is of listing-level severity. We provide specific responses to the many comments on this issue in the public comments section of this preamble.
Listings 14.08M, 114.08L and 114.08M: The Functional Criteria
We received many public comments on the functional criteria in proposed Listings 14.08M, 114.08L, and 114.08M, the majority of which were unfavorable. The proposed rules had listed several possible manifestations of HIV infection (for example, meningitis, Kaposi's sarcoma, mucosal candidiasis, and oral hairy leukoplakia), and clinical and laboratory findings (for example, anemia, fever, and weight loss) that were not listed as stand-alone medical manifestations but that, in conjunction with functional restrictions, could establish listing-level HIV infection.
The commenters asked us to delete or substantially revise the rules for a number of reasons. Many commenters asked us to delete the rules employing functional criteria because they thought that the proposed medical manifestations were sufficient in themselves to establish listing-level disability. Many of these commenters pointed out that we had already incorporated indicators of medical severity by requiring the conditions in proposed Listings 14.08M2, 114.08L1, and 114.08M2 to be "persistent and/or resistant to therapy," and by requiring a 2-month persistence of at least two of the medical manifestations in proposed Listings 14.08M3, 114.08L2, and 114.08M3. The commenters pointed out that to require functional limitations in addition to these medical requirements seemed excessive and unfair. For instance, a number of commenters thought that diarrhea that had already persisted for 2 months and was unresponsive to treatment should be enough to establish disability, and need not be associated with another medical manifestation and functional limitations.
Some commenters thought that the mere existence of some of the manifestations (for example, pulmonary tuberculosis or vulvovaginal candidiasis) was in itself sufficient to establish disability in HIV-infected individuals and that no indicator of severity—either medical or functional—was necessary. Others offered suggestions for tying some of the manifestations to a test of functioning while making some of the other manifestations stand-alone medical listings without functional criteria. Some offered suggested criteria for describing medical severity for the stand-alone manifestations. The thrust of these suggestions was toward providing medical criteria specific to each different manifestation instead of the more general criteria for persistence and unresponsiveness to treatment we had proposed.
With regard to the functional criteria themselves, most of the comments addressed the adult criteria in proposed Listing 14.08M. Many people said that the criteria were inappropriate and too difficult to meet. Some said that the criteria were originally intended for the evaluation of mental impairments and, therefore, could not be used to evaluate physical impairments, especially HIV infection. (Of these comments, many singled out the criterion of marked limitation of social functioning in proposed Listing 14.08M4b as being especially inapt.) Some thought that this was the first time we had employed functional criteria in the physical listings and said that we should not start with HIV infection.
Many commenters who thought that we should not have the functional criteria at all recommended that, if we must have functional criteria, we should revise the proposed rules so that meeting only one of the functional criteria—instead of two, as we had proposed—would suffice. Some thought that we should incorporate into the listing the two functional criteria we formerly used in our manual instructions, believing the old criteria to be less stringent than the proposed criteria. Some thought that we should revise the language of the functional criteria to make them more specific to HIV infection. Finally, many commenters said that our definitions of the term "marked" in proposed 14.00D with respect to each of the functional criteria set too severe of a standard.
We address the individual comments, and other related comments, in more detail in the public comments section of this preamble. Notwithstanding the comments, however, we have decided to retain listings that permit a showing of disability based on an individualized assessment of the impact of a person's HIV infection on his or her functioning in the broad areas of activities of daily living, social functioning, and concentration, persistence, or pace. However, we have also extensively revised the rules in response to the comments, and we believe that we have addressed many of the commenters' concerns.
We have addressed most of the concerns by adding listings that provide stand-alone medical criteria for most of the manifestations that were in proposed Listings 14.08M, 114.08L, and 114.08M. The medical criteria in the new stand-alone medical listings are specific to the listed manifestations. (For reasons we explain later, we have deleted the criterion for a CD4 (T4) lymphocyte count; therefore, there are no provisions in the final rules corresponding to proposed Listings 14.08M1 or 114.08M1.) By doing this, the functional criteria become simply an alternative way that individuals with most of the manifestations in the proposed rules can establish that they are disabled under the listings, instead of the only way.
In final adult Listing 14.08N, we now describe episodic manifestations. Listing 14.08N thus includes individuals who suffer from the same manifestation periodically but who are not necessarily continuously ill; whose manifestations, though continuously present, wax and wane in severity; or who suffer episodes of different manifestations that, taken together, demonstrate listing-level severity. Final childhood Listing 114.08O (which replaces proposed Listings 114.08L and 114.08M and applies to children from birth to the attainment of age 18) includes all manifestations of HIV infection (both episodic and continuous). Both new listings include people who have manifestations that are listed in the preceding medical listings but that do not meet the medical criteria, as well as manifestations that are not listed in final Listings 14.08A-M and 114.08A-N.
For reasons we explain below, the final adult listing, 14.08N, now includes only three functional criteria—1) activities of daily living; 2) social functioning; and 3) concentration, persistence, and pace—and an individual need only establish marked limitations in one of the three areas to show an impairment that meets this listing. We have also revised our definitions of the term "marked" to clarify its applicability in HIV cases.
As we analyzed the comments, we realized that many of them were based on misconceptions about both the proposed rules and how we evaluate disability in general. Although we agreed with those commenters who expressed concerns that some of the conditions tied to the functional criteria in the proposed rules need not be so tied—and we made appropriate changes—we could not agree with those commenters who stated that the proposed functional criteria would be used to deny disability benefits or to disqualify some individuals.
Our disability evaluation policies do not permit denial of disability benefits on the basis that an individual's impairment(s) does not impose functional limitations at the listing level. We use the listings at the third step of our sequential evaluation processes for adults and children to "screen in," i.e., allow, individuals who are clearly disabled. (See §§ 404.1520, 416.920, and 416.924 for our rules on the sequential evaluation processes.) Under these processes, if an individual's impairment(s) is "severe" but does not meet or equal in severity any listing, we reach no conclusion at all about disability. Rather, we move on to the next step of the process and look at other factors to resolve that ultimate issue. We may use the criteria described in these listings to find that an individual is disabled, but we do not use the criteria to find that an individual is not disabled.
Therefore, the nature of this process is such that any time we include a new listing in appendix 1, no matter what the requirements, this is an advantage to an individual who applies for disability benefits because it adds a new way we may find the individual disabled, without adding a new way to find him or her not disabled.
As in the proposed rules, the functional criteria in these final rules serve a very important purpose—to provide individuals who have what may at first seem like less severe manifestations of HIV infection, or combinations of impairments that would not fit neatly into any of the purely medical listings, with a listing their impairments can meet. The listing, therefore, provides claimants with every opportunity to be found disabled as early in the evaluation process as possible. We believe that the commenters who argued against the functional criteria did not understand this purpose and misinterpreted what we intended to be a very beneficial part of the listing. This was partly because we did not explain it clearly enough. But it was also because—as the commenters correctly pointed out—we need not have limited the functional criteria only to certain specific manifestations, that some of the proposed manifestations were in themselves disabling, that some of the manifestations in the listing were more medically serious than others, and that we could have provided alternative medical criteria for some of the manifestations we had proposed.
The following changes respond to the commenters' concerns that some HIV-related medical conditions were included in the listings only in relation to functional standards. At the same time, they retain the flexibility we need for making favorable disability determinations at the listing level using functional criteria.
Stand-Alone Medical Criteria
We reviewed each of the medical conditions that were tied to functioning in proposed Listings 14.08M, 114.08L, and 114.08M and attempted to draft a medical description of each condition, at listing-level severity, that did not include a functional evaluation. In doing so, we heeded comments pointing out that some of the medical requirements in proposed Listing 14.08M were already extremely severe without the functional criteria. We did not, however, agree with those commenters who thought that the mere existence of each of the manifestations should be enough to establish listing-level severity. Most of the manifestations we had proposed in Listing 14.08M can vary in severity, responsiveness to treatment, and their impact on functioning. Therefore, we believe it is imperative that each manifestation be described by criteria that define listing-level severity if it is to be a stand-alone medical listing.
We were able to draft stand-alone listings for all the manifestations included in proposed Listings 14.08M2, 114.08L1, and 114.08M2: Pulmonary tuberculosis in final Listings 14.08A1 and 114.08A1; Kaposi's sarcoma in final Listings 14.08E2 and 114.08E2; peripheral neuropathy in final Listings 14.08H and 114.08H; and pneumonia, bacterial or fungal sepsis, meningitis, septic arthritis, and endocarditis in final Listings 14.08M and 114.08N. We were also able to include most of the conditions included in proposed Listings 14.08M3, 114.08L2, and 114.08M3: Mucosal candidiasis, including vulvovaginal candidiasis, and dermatological conditions in final Listings 14.08B2 and 14.08F and 114.08B2 and 114.08F; Herpes zoster in final Listings 14.08D3 and 114.08D3; anemia, granulocytopenia, and thrombocytopenia in final Listings 14.08G and 114.08G; diarrhea in final Listings 14.08J and 114.08J; and sinusitis in final Listings 14.08M and 114.08N. In some cases, the new criteria consist of a reference to another listing (e.g., final Listing 14.08G, anemia, as described under the criteria in 7.02). In other cases, the criteria are new (e.g., final Listing 14.08D3, Herpes zoster either disseminated or with multidermatomal eruptions, that are resistant to treatment).
We did not include fever, weight loss, and oral hairy leukoplakia as stand-alone listings. Fever and weight loss are not medical conditions in themselves, but the observable outcome—i.e., signs—of medical conditions. We believe that there are few people whose sole manifestation of HIV infection is a persistent, high fever without any other observable problems; indeed, the individual will likely have other signs and symptoms that may be evaluated together with the fever. Moreover, any stand-alone medical listing that tried to describe listing-level fever would have to be set at a very high level, would rarely apply, and would be subject to the same criticism that we received about some of the manifestations in the proposed functional listings, i.e., that it is too severe. We believe, therefore, that it would be better to evaluate the few individuals who suffer only from persistent fever (of any level) in terms of their functioning; such individuals may be fatigued and weak, have difficulty doing their daily chores, and may even be confined to their homes or even to bed. Final Listings 14.08N and 114.08O also allow for the possibility that the individual's fever is not constant, but recurrent, which we believe is a more realistic possibility than continuous high fever. To underscore these points, we have included fever among the examples of symptoms and signs that may result in the functional limitations in the listing.
Similarly, weight loss is already inherent in the listings for HIV wasting syndrome and growth disturbance (final Listings 14.08I and 114.08I) as well as the aforementioned new listings for diarrhea. Individuals who have unexplained weight loss or weight loss because of loss of appetite may have impairments that are medically equivalent to one of these listings or the new functional listings, or to listings in other body systems; even those whose weight loss is not as serious as in final Listings 14.08I and 114.08I may have symptoms of fatigue and weakness resulting in listing-level functional restrictions. We have also included weight loss among the examples of signs and symptoms that may result in the functional limitations of the listing.
We did not include oral hairy leukoplakia as a stand-alone medical condition because it is generally an asymptomatic condition that may persist for a relatively long time without interfering with the individual's functioning. We believe, therefore, that each case will have to be evaluated to determine the particular effects of the manifestation on the individual under final Listings 14.08N or 114.08O. We also did not include from the proposed childhood rules parotitis, or the clinical findings of splenomegaly, hepatomegaly, and generalized lymphadenopathy. These conditions and clinical findings can vary greatly from child to child in their severity, medical significance, and impact on a child's ability to function. Because of this, it is not possible to define with solely medical criteria, except in the most extreme terms, a level of severity for these conditions and clinical findings that would interfere with most children's ability to engage in age-appropriate activities to the required degree.
Final Listings 14.08N and 114.08O do not list specific impairments. We made this change partly in response to comments suggesting many other possible manifestations of HIV infection for inclusion in the listing and partly because it is logical. We decided that instead of expanding the list of manifestations, we could respond to the commenters' concerns by abandoning the finite list of HIV-related manifestations and referring instead to "manifestations of HIV infection" in general. This allows for consideration of any manifestations, whether identified in the listing or not. We have also added discussions to final 14.00D8 and 114.00D8, the sections of the prefaces that describe the functional criteria, explaining that these listings may be used not only to evaluate manifestations of HIV infection that are not included in Listings 14.08A-M and 114.08A-N, but to evaluate manifestations that are listed in, but do not meet the criteria of, those listings.
The Functional Criteria
Many commenters expressed concern that the functional criteria in proposed Listing 14.08M were based on the functional deficits described in the mental impairment listings for adults in 12.00 of the listings. The commenters were concerned that these criteria, therefore, only related to individuals with mental disorders and were not appropriate measures of severity in the case of individuals with HIV infection. We do not agree. Although adjudicators are most accustomed to applying the functional criteria in 12.00 in the context of mental impairments, those criteria describe broad areas of functioning that are relevant to any individual's ability to work. It does not matter, for example, whether an individual's ability to perform activities of daily living is restricted because of memory loss or hallucinations, or whether it is because of fatigue, headaches, or weakness resulting from a manifestation of HIV infection. In either event, the ability to perform the tasks is compromised.
Nevertheless, we realized from the comments that the proposed rules may not have made application of the functional criteria sufficiently specific to the evaluation of HIV-related impairments. Therefore, we modified proposed Listing 14.08M to more clearly reflect our original intent, which was to expand the way we assess the severity of HIV-related impairments at the listing level beyond the use of strict medical criteria by using broad functional criteria. We had hoped to include in the listings (via proposed Listing 14.08M3) a group of individuals whom we believed would be very difficult to describe in strictly medical terms—individuals who become ill then improve, only to repeatedly become ill again, either with the same manifestation of HIV infection or with different manifestations.
Based on some commenters' questions about the applicability of the proposed functional criteria to physical disorders, we also realized that proposed paragraph 14.08M4d, repeated episodes of decompensation, was not really a measure of functioning at all, but a description of what we were trying to address in this listing. Unlike its purpose in the mental listings (where decompensation can be a measurement of an individual's ability to tolerate stress), when applied to HIV-related illnesses the criterion measured the persistence and frequency of episodes of manifestations of HIV infection; in effect, it distinguished between individuals who develop and recover from only one or two isolated manifestations of HIV, and those who have a pattern of repeated episodes of illness.
Therefore, we removed proposed Listing 14.08M4d from the list of functional criteria, modified it to make it more specific to HIV, and used it as the introductory criterion for final Listing 14.08N. The final listing is for the evaluation of individuals who have repeated manifestations of HIV infection. We also revised the criterion in response to commenters who pointed out that the requirement in proposed paragraph 14.08M4d for the episodes to occur 3 times a year or once every 4 months and to last for at least 2 weeks was unnecessarily inflexible. In the third paragraph of 14.00D8 we have retained the provision that the conditions may occur on an average of 3 times a year, or once every 4 months, and each last at least 2 weeks, and at the same time we provide additional flexibility. Specifically, we now provide that the episodes may also last for less than 2 weeks and occur substantially more frequently than 3 times a year or every 4 months, or that they may occur less frequently than 3 times a year or once every 4 months but last substantially longer than 2 weeks each time.
In response to commenters who asked us to include criteria for some of the more common symptoms and signs of individuals who do not have CDC-defined AIDS, we adopted and expanded language from Listing 14.02B, the listing for systemic lupus erythematosus, about which we received literally thousands of favorable public comments. The language in final Listing 14.08N explains that disability under this listing will result from "significant, documented, symptoms or signs (e.g., fatigue, fever, malaise, weight loss, pain, night sweats)" that cause functional limitations. (Unlike Listing 14.02B, we do not provide that there must be both symptoms and signs in this listing. The constitutional symptoms and signs in Listing 14.02B help to define the syndrome of systemic lupus erythematosus and its severity. In contrast, the criterion in final Listing 14.08N includes any symptoms or signs that can be the cause of the functional limitations because the existence of the impairment has already been established.)
We retained the three remaining functional criteria as the standards for measuring functional deficit. Having more accurately described the individuals to whom we intend the listing to apply, we then agreed with commenters who stated that marked functional restrictions in any one of the categories would be sufficient to demonstrate listing-level severity. Consequently, final Listing 14.08N requires marked limitations in only one of the three broad areas of functioning.
We want to reiterate, however, that we retained a revised version of the proposed Listing 14.08M4d criterion in the final listing. Our intention in modifying and relocating the criterion is to better express our original intent and to recognize that the proposed fourth criterion was not a "functional" criterion in this listing but a medical one. We believe that the result is an improvement over the proposed rule. Individuals with less serious manifestations than several of those we had proposed in Listing 14.08M2 and those who have only one of the manifestations we had proposed in Listing 14.08M3 will now be able to show that they have impairments that meet this listing. Furthermore, even though there is, in effect, no change in the functional severity level of this listing for those people whose impairments would have satisfied one of the criteria in proposed Listing 14.08M4a (activities of daily living), 14.08M4b (social functioning), or 14.08M4c (concentration, persistence, or pace) and the criterion in 14.08M4d—thus satisfying two of the proposed "functional" criteria—we have made the functional criteria more accurate measures of an individual's true functional limitations. No claimant will have to establish that he or she has marked limitations in two of the three true areas of functioning about which so many of the commenters were concerned. In this way, by requiring that a claimant show marked limitations in only one of the three functional areas, the area of social functioning, about which many commenters were concerned, becomes only one way among three available to establish disability at the listing level and can only benefit claimants by providing another area in which to document functional restrictions. However, if social functioning is not markedly limited, a claimant may still show listing-level impairment by demonstrating marked limitations in one of the other areas, activities of daily living or concentration, persistence, or pace.
The Childhood Functional Criteria: Final Listing 114.08O
We also did not adopt the recommendations of commenters who urged us to eliminate the functional criteria for children in proposed Listings 114.08L and 114.08M. These commenters noted that our regulations, in § 416.926a, already allow for a finding of equivalence when the functional limitation(s) resulting from a child's impairment(s) is the same as the disabling functional consequences of a listed impairment. Therefore, they did not believe that it was necessary to restate this previously established policy within the context of this listing.
Although we agree that proposed Listings 114.08L and 114.08M were based on a principle similar to functional equivalence, and we agree that most or all children whose impairments meet the criteria of proposed Listing 114.08L or 114.08M would have been found disabled based on the functional equivalence rule, we did not want to take the chance that our rules would be misinterpreted as being more advantageous to adults. In addition, the concept of functional equivalence applies only to childhood SSI claims under title XVI of the Act. Even though SSI claims constitute the great majority of childhood disability applications, it is possible for individuals under age 18 to apply for disability benefits (both as disabled minor children and as workers) under title II. The rules on functional equivalence do not apply in these cases, and such children could be disadvantaged by removal of the rule.
We did not change the proposed childhood functional criteria the same way we changed the adult criteria. The adult criterion we changed (repeated episodes of decompensation) is not applicable to the evaluation of functioning in children. Further, the childhood functional criteria vary depending on the age of the child. We concluded that the functional criteria in 112.00ff represent the best way to measure broad functional restrictions in children. Consequently, we retained the proposed childhood functional criteria (which cross-refer to Listings 112.02 and 112.12).
CD4 (T4) Count
Another change in the final listings is the elimination of a specific criterion for CD4 lymphocyte count. Proposed Listings 14.08M1 and 114.08M1 used a CD4 count of less than or equal to 200 cells/mm3 as a measure of the severity of immunodeficiency. A number of public commenters questioned why we used this particular criterion to evaluate impairment severity. Some said that individuals with higher CD4 counts than 200 could be just as functionally limited, and suggested that we use a higher CD4 count. Some commenters said that a CD4 count of 200 should, in and of itself, be sufficient to establish listing-level severity, without the need to show functional restrictions. Others stated that using a CD4 count is not appropriate at all because it is not a good indicator of impairment severity.
In light of these comments, we reevaluated the listing and realized that, while a low CD4 count (and especially a rapidly declining CD4 count) is an indicator of a compromised immune system and a valuable tool for determining when to institute prophylactic treatment, there is no consistent correlation between a given CD4 count and how or whether an individual is functionally impaired by HIV infection. Individuals with high CD4 counts may be quite severely limited, while others with very low counts may be able to continue normal activities. One individual who commented on our proposed rules related his own story of living with HIV infection, noting that he continued to feel well and to work until his CD4 count was well below 100. He argued that to base our rules on such an unreliable indicator would be to unfairly stigmatize individuals who are able to function well despite low CD4 counts.
Therefore, we decided not to include a specific CD4 lymphocyte count as a criterion in the listings. For informational purposes, we have also included in final 14.00D3, 14.00D4, and 114.00D4 general statements about the role CD4 counts play in disease susceptibility.
In final 114.00D3, we also retained guidance that permits a finding of the existence of HIV infection in very young children based on a CD4 count. We did this because these tests are helpful in making the difficult diagnosis of HIV infection in infants. However, based on a commenter's suggestion, which was consistent with other information we received, we extended this provision (which we had proposed to apply to children up to 15 months of age) to cover children up to 24 months of age.
Provisions of the Final HIV Rules
14.00D Human Immunodeficiency Virus (HIV) Infection
Final 14.00D introduces the subject of HIV infection and lists some of the information that is important in documenting and evaluating the disease. The section explains what is acceptable evidence of HIV infection and its manifestations. It provides definitions of some of the terms we use in the listings, including the terms associated with the functional listing, 14.08N.
We extensively revised final 14.00D, both substantively and technically, based on public comments. In place of the 23 paragraphs we had proposed for 14.00D in the NPRM, the final rules are now divided into 8 numbered sections. We have also deleted repetitious language and several paragraphs that are no longer necessary in 14.00D because we have included the impairments they described in Listing 14.08.
14.00D1 HIV Infection.
Final 14.00D1, which describes "HIV infection" and "AIDS," corresponds to the first paragraph of proposed 14.00D. We revised the final language to emphasize that an individual need not have CDC-defined AIDS to have an impairment that meets or is equivalent in severity to, the listed impairments in final Listing 14.08.
14.00D2 Definitions.
Final 14.00D2 is a new section we added in response to comments asking us to define some of the terms in the listing. The final section defines the terms "resistant to treatment," "recurrent," "disseminated," and "significant involuntary weight loss." It states that the first three terms have the same general meaning as used by the medical community, but cautions that the precise meaning of the terms will necessarily vary depending on the specific disease or condition in question, the body system affected, the usual course of the disorder and its treatment, and other relevant circumstances. We then provide definitions of the three terms.
For the fourth term, "significant involuntary weight loss," which is used in Listing 14.08I, we explain that the term does not describe a specific minimum amount or percentage of body weight. We still provide that we always consider an involuntary weight loss of 10 percent of baseline to be significant. However, in response to a comment, we now also provide that loss of less than 10 percent of body weight may be significant, especially in a smaller person. To illustrate the principle, we provide examples of two women, showing when weight loss of less than 10 percent of body weight may and may not be significant.
14.00D3 Documentation of HIV Infection; 14.00D4 Documentation of the Manifestations of HIV Infection.
Final 14.00D3 provides our standards for documenting the existence of HIV infection and final 14.00D4 provides our standards for documenting its manifestations. These sections correspond to the provisions we had proposed in the third through seventh paragraphs of proposed 14.00D. However, in response to many comments, we extensively revised these sections.
We revised final 14.00D3 to explain that, even though the medical evidence must include documentation of the existence of HIV infection (which is required by the statute), documentation may be by laboratory evidence or by other generally acceptable methods consistent with the prevailing state of medical knowledge and clinical practice. We adopted the additional language about generally acceptable methods of diagnosis from comments pointing out that many claimants will not have undergone the kinds of testing we had described. Many commenters noted that clinicians do not always perform laboratory testing for HIV because the existence of HIV infection can be inferred, or presumed, based on the existence of certain opportunistic infections. These commenters pointed out that even the proposed rules recognized this practice. Some commenters also pointed out that in many instances where claimants have been tested for the HIV, the test results will not be available because of privacy concerns.
The section is then divided into two parts: 14.00D3a, which describes how HIV infection may be diagnosed definitively, and 14.00D3b, which describes how HIV infection may be diagnosed presumptively—that is, be acceptably documented without the definitive laboratory evidence described in 14.00D3a. In response to comments with which we agreed, we clearly state in final 14.00D3a that when laboratory testing for HIV infection has been performed, every reasonable effort must be made to obtain reports of the results of that testing. We also clarified the language to explain why the results of a positive ELISA screening test are ordinarily verified by a more definitive test for HIV antibodies. In final 14.00D3a(ii), we combined the tests specifically for HIV antigen into one category, and included the laboratory tests named in the proposed listing as examples. We also added cerebrospinal fluid specimens to this category of clinical tests to make the adult rules consistent with the childhood rules; even though such testing is rare in adults, it is not unheard of. In response to a comment, we expanded final 14.00D3a(iii), which was formerly the fourth example in the fourth paragraph of proposed 14.00D, to include other tests that are acceptable methods of detecting HIV and consistent with the prevailing state of medical knowledge.
The third paragraph of final 14.00D3a has been adapted from the second sentence of the third paragraph of proposed 14.00D. In response to comments, and for reasons we have already explained above in the summary of revisions to Listing 14.08N, we clarify that, even though the level or rate of decline of CD4 count correlates with the extent of immune depression, a reduced CD4 count alone does not definitively diagnose the presence of HIV infection or provide information about the severity or functional effects of HIV infection; additional documentation will always be necessary.
Final 14.00D3b describes when documentation of HIV infection is possible without definitive laboratory evidence. It states that HIV infection may be documented by medical history, clinical and laboratory findings, and diagnoses shown in the medical evidence, provided that the documentation is consistent with the prevailing state of medical knowledge and clinical practice, and is consistent with the other evidence. As an example, it states that HIV infection will be documented if the individual has an opportunistic disease predictive of a defect in cell-mediated immunity, and there is no other known cause of diminished resistance to that disease. This is a provision we moved from proposed Listing 14.08A as part of our simplification of the listing.
Final 14.00D4 explains the documentation requirements for opportunistic diseases and other manifestations of HIV infection. It is structured in the same way as final 14.00D3, with a section (final 14.00D4a) describing definitive methods of diagnosis, and a section (final 14.00D4b) describing other acceptable methods of diagnosis. It notes that every reasonable effort should be made to obtain whatever specific laboratory evidence is available. If only hospitalization summaries or treating physician reports are available, this evidence should include details of the clinical findings and the results of the diagnostic or microscopic studies.
As in final 14.00D3, we have added guidance that documentation of manifestations of HIV infection may be by laboratory evidence or documentation which is consistent with the prevailing state of medical knowledge and clinical practice, and consistent with the other evidence. We have also included in final 14.00D4a a discussion of the relevance of CD4 counts, which cross-refers to the discussion in 14.00D3a.
Final 14.00D4b discusses other acceptable documentation of opportunistic diseases and other manifestations of HIV infection. In response to comments, with which we agree, we have clarified the explanation of how opportunistic diseases and manifestations of HIV infection may be documented by medical history, clinical and laboratory findings, and diagnoses indicated in the medical evidence. Though a diagnosis of opportunistic disease or HIV manifestation may not be supported by a definitive test, the diagnosis is acceptable documentation provided that it is consistent with the prevailing state of medical knowledge and clinical practice and is consistent with the other evidence. As a point of clarification, we have also added a discussion about cytomegalovirus (CMV) disease, which presents special documentation issues. Because the CMV is an organism that is present in many individuals who are not ill, a positive serology in itself does not confirm that a person has CMV disease. Therefore, in this circumstance, we require confirmation by biopsy or other generally acceptable methods consistent with the prevailing state of medical knowledge and clinical practice. One such method, which we single out in the new paragraph, is diagnosis by an ophthalmologist of chorioretinitis caused by CMV.
14.00D5 Manifestations Specific to Women.
The two paragraphs of final 14.00D5 replace the tenth through twelfth paragraphs of proposed 14.00D and discuss the evaluation of HIV infection in women. We shortened the discussion of manifestations specific to women contained in proposed 14.00D because we have incorporated the specific diseases mentioned in the proposed prefatory language directly into final Listings 14.08A5 (pelvic inflammatory disease), 14.08D2 (genital herpes), and 14.08F (vulvovaginal candidiasis) as stand-alone medical listings.
In final 14.00D5, we have retained the basic guidance from the NPRM for evaluating HIV infection. Both paragraphs of final 14.00D5 continue to alert adjudicators to give careful consideration and scrutiny to the medical evidence when evaluating HIV infection and its manifestations in women.
The first paragraph of final 14.00D5 corresponds to the tenth paragraph of proposed 14.00D. We have revised it slightly following the publication of the NPRM because most women with severe immunosuppression do, in fact, exhibit the same kinds of manifestations that men do, and the HIV infection need not necessarily be in the end stages for this to happen. However, in addition to these manifestations, HIV infection does have effects in some women that are different from those in men with the disease, sometimes by increasing the frequency and resistance to treatment of conditions, including gynecologic conditions, that occur in women who do not have HIV infection. We have, therefore, revised the last two sentences of the paragraph to say that HIV infection may have different manifestations in women than in men, and that adjudicators must carefully scrutinize the medical evidence and be alert to the variety of medical conditions that are both specific to women and common in the female population, but may be more severe because of the HIV infection.
The second paragraph of final 14.00D5 includes material that was in the remaining two paragraphs of the NPRM and explains the foregoing principles in more detail. Because we have incorporated the most important conditions specific to women directly into the listing, we now no longer state that gynecologic conditions may result only in equivalence determinations under the listings. Instead, we provide that manifestations of HIV infection in women may be evaluated under the specific listing criteria (such as Listing 14.08E, which explicitly lists cervical cancer), under an applicable general listing category (such as final Listing 14.08A5), or under final Listing 14.08N (which considers the specific impact of an impairment(s) that does not otherwise meet a listing on the individual's ability to function). We believe that final Listing 14.08N will be especially useful in cases of women who do not suffer from a continuous, listing-level manifestation of HIV
14.00D6 Evaluation
Final 14.00D6 gathers under one heading the three paragraphs of the NPRM that addressed issues of evaluation: the second, thirteenth, and last paragraphs of proposed 14.00D.
We consolidated the repetitive language in these paragraphs but retained the discussion of the need to evaluate the impact of all impairments in individuals with HIV infection. We changed the second sentence of the first paragraph of final 14.00D6 (the second paragraph of proposed 14.00D) to emphasize that equivalence to other listings must be considered in evaluating an individual's HIV disease or condition. We also revised the subsequent discussion, which was adapted from the thirteenth paragraph of proposed 14.00D, but which was confined to mental manifestations in the proposed rules. The final rule refers to both mental and physical impairments and removes any implication that we did not consider that mental signs and symptoms could be manifestations of HIV infection.
We also explain that some individuals with HIV infection may have impairments that are less than listing-level severity, but still may be disabling. Evaluation of these cases should proceed through the final steps of the sequential evaluation process.
14.00D7 Effect of Treatment.
Final 14.00D7 is an expanded version of the fourteenth paragraph of proposed 14.00D. It discusses the need to evaluate the impact of treatment in individuals with HIV infection. In response to public comments, with which we agreed, we clarified the first and second sentences of the proposed paragraph by specifically referring to both the potential benefits and the potential adverse effects of treatment. We expanded the explanation dealing with individual responses to treatment to further emphasize the importance of evaluating adverse or beneficial consequences of treatment on a case-by-case basis. We also added language that explains why it is important to know that the effects of treatment may be temporary or long-term as a reminder that any decision regarding the impact of treatment should be based on a sufficient period of treatment for an accurate and realistic assessment.
14.00D8 Functional Criteria.
Final 14.00D8 discusses the functional criteria contained in Listing 14.08N. We extensively modified the proposed language in response to comments and to conform with the changes we made in the functional criteria of final Listing 14.08N, already described above.
The first paragraph of final 14.00D8, together with the third paragraph, replaces the twenty-second paragraph of proposed 14.00D, which had described the fourth area of functioning, repeated episodes of deterioration or decompensation in work or work-like settings. This paragraph now explains that the provisions of final Listing 14.08N apply both to manifestations that are listed in Listings 14.08A through M but that do not meet those listings, and to unlisted manifestations. In this way, instead of using a finite list of manifestations as we had proposed, the provision now applies to any type of manifestation.
The second paragraph stresses important considerations in the evaluation of HIV infection. It requires an assessment of the full impact of signs, symptoms, and laboratory findings on an individual's ability to function, and mentions the following specific factors: Symptoms, such as fatigue and pain; characteristics of the illness, such as the frequency and duration of manifestations, or periods of exacerbation and remission; and the functional impact of treatment, including the side effects of medication.
The third paragraph of final 14.00D8 (as well as the first paragraph) replaces the twenty-second paragraph of the NPRM. In the third paragraph, we provide the definition of the term "repeated" as we use it in Listing 14.08, which we have already explained above. Our intent is to provide as much flexibility as possible to include "repeated" manifestations, provided that the episodes are of sufficient frequency or duration as to be at the listing level.
The fourth through eighth paragraphs of final 14.00D8 replace the seventeenth through twenty-first paragraphs of 14.00D in the NPRM. Inasmuch as we require an individual to satisfy only one of the three functional criteria now in Listing 14.08N, we have revised the fourth paragraph accordingly. We have added language that reminds adjudicators that the functional restrictions may result from the impact of the manifestation on mental or physical functioning or both. We have also moved into this paragraph the language in paragraphs 18 through 21 of the NPRM about the importance of considering symptoms (such as depression, fatigue, or pain) and the side effects of medication when assessing functioning.
In response to comments about the seventeenth paragraph of the NPRM, now the fifth paragraph of final 14.00D8, we revised the general guidance definition of the term "marked." The revisions now state that a marked limitation does not represent a quantitative measure of the individual's ability to do an activity for a certain percentage of the time. We also state plainly, in response to many comments, that an individual with a marked limitation is not totally precluded from performing an activity and that the term "marked" does not imply that the individual is confined to bed, hospitalized, or in a nursing home. This has always been our intent in the rules; our reason for including the statement that "marked * * * means more than moderate, but less than extreme" is to illustrate that there is a level of limitation higher than the "marked" level, a situation that would not be possible if "marked" meant complete limitation.
In the sixth, seventh, and eighth paragraphs of final 14.00D8, we revised the descriptions of the three general areas of functioning to make them more specific to people who have HIV infection and to respond to concerns in the public comments. For instance, in the seventh paragraph, we now explain that an individual may be able to communicate with close friends and relatives yet still have a marked limitation of the ability "to engage in social interaction on a sustained basis." This, too, has always been our intent. The ability to communicate effectively with close family and friends is not necessarily indicative of an individual's ability to maintain social contact independently or in a work setting.
Even though all of the foregoing information is basic to the use of the rules we proposed in the NPRM, and would have been understood by our adjudicators, we have included it in the preface to the final listing to make our policy clearer in the regulations.
Finally, we deleted from the preface the material that was in the seventh, eighth, and ninth paragraphs of proposed 14.00D. The seventh paragraph described documentation requirements for Pneumocystis carinii pneumonia, and has been superseded by the new discussions on documentation of the manifestations of HIV infection at the listing-level. We incorporated the provisions of proposed paragraphs eight and nine directly into their respective listings (14.08H1 for HIV encephalopathy, and 14.08I for HIV wasting syndrome).
14.08 Human Immunodeficiency Virus (HIV) Infection
This new listing adds to the regulations our criteria for evaluating HIV infection at the listing level. The listing includes a range of opportunistic diseases, cancers, and other manifestations that are indicative of listing-level severity in an individual with HIV infection. Specific manifestations that are considered indicative of listing-level HIV infection are in final Listings 14.08A-M, grouped by type (e.g., fungal infections, bacterial infections, malignant neoplasms) for ease of reference. Final Listing 14.08N includes any manifestations of HIV infection that cause listing-level functional limitations.
Final Listing 14.08 is significantly different from the proposed listing. We reorganized and changed the proposed listing in the following ways:
To improve the clarity of the final listing criteria, we deleted specific documentation requirements from each HIV manifestation listed in 14.08 and retained only one general cross-reference to the comprehensive discussion of documentation requirements in final 14.00D3 (for documentation of the existence of HIV infection) and 14.00D4 (for documentation of the manifestations of HIV infection). Because we have included a discussion of opportunistic disease predictive of a defect in cell-mediated immunity that document HIV infection in final 14.00D3, we have deleted proposed 14.08A.
14.08A Bacterial Infections
Final Listing 14.08A, Bacterial infections, includes the proposed listings that described bacterial infections. Thus, final Listing 14.08A1, Mycobacterial infection, includes proposed Listings 14.08A7, 14.08D, and 14.08M2b; final Listings 14.08A2, Nocardiosis, and 14.08A3, Salmonella bacteremia, were in proposed Listing 14.08F. We combined the proposed listings for mycobacterial infections, which are a kind of bacterial infection, because we agreed with those commenters who pointed out that the proposed listings (except for 14.08M2b, Pulmonary tuberculosis) resulted in a finding of "meets" regardless of the kind of the mycobacterial infection. (We made a technical correction to the name of one of the three bacteria we listed as examples of causes of mycobacterial infections, M. avium-intracellulare.) We added pulmonary tuberculosis, resistant to treatment, in response to comments that asked us to create a stand-alone medical listing for this condition.
For the same reason, we added syphilis or neurosyphilis to final Listing 14.08A4, and required that the sequelae be evaluated under the criteria for the affected body system. We also added listing criteria for multiple or recurrent bacterial infections, such as pelvic inflammatory disease, in final Listing 14.08A5. The final listing includes criteria for hospitalization or intravenous antibiotic treatment as a measure of severity.
14.08B Fungal Infections
Final Listing 14.08B includes material originally proposed in Listings 14.08A and 14.08C. Final Listing 14.08B2, Candidiasis (at a site other than the skin, urinary tract, intestinal tract, or oral or vulvovaginal membranes; or involving the esophagus, trachea, bronchi, or lungs) combines the criteria of proposed Listings 14.08A1 and 14.08C1. Final Listings 14.08B3, Coccidioidomycosis, and 14.08B5, Histoplasmosis, were both in proposed Listing 14.08C. Final Listing 14.08B4, Cryptococcosis, was in proposed Listings 14.08A2 and 14.08C2; we have also added a reference to cryptococcal meningitis in partial response to the public comments asking us to list the criteria in proposed Listing 14.08M2 as stand-alone listings. (Other forms of meningitis are listed in final Listing 14.08M.) In response to comments, we added aspergillosis to 14.08B1 and mucormycosis to 14.08B6; neither of these manifestations was in the proposed listing.
14.08C Protozoan or helminthic infections
Final Listing 14.08C1 includes manifestations originally proposed as Listings 14.08A3 and 14.08B1 (both for cryptosporidiosis) and 14.08B4 (isosporiasis). In response to comments, we also added microsporidiosis to final Listing 14.08C1. Final Listing 14.08C2, for Pneumocystis carinii pneumonia, includes the manifestations that were in proposed Listings 14.08A8 and 14.08B2. In response to comments, we added extrapulmonary pneumocystis infection to final 14.08C2. Thus, the listing includes all infections with the Pneumocystis carinii organism. We listed both the pneumonia and the extrapulmonary infections (instead of a single description of Pneumocystis carinii infection) because pneumonia is such a common manifestation of HIV infection.
Final Listing 14.08C3, Strongyloidiasis, extra-intestinal, was proposed Listing 14.08B5. Final Listing 14.08C4, Toxoplasmosis, combines proposed Listings 14.08A10 and 14.08B3 into one listing.
14.08D Viral Infections
Final Listing 14.08D1, Cytomegalovirus disease, combines proposed Listings 14.08A4 and 14.08E1. The final criteria for cytomegalovirus disease include a cross-reference to the new discussion of documentation of the disease in 14.00D4, already described.
Final Listing 14.08D2, Herpes simplex, combines proposed Listings 14.08A5 and 14.08E2. We revised the language of those rules slightly for clarity, and divided the listing into three separate criteria. We added to final Listing 14.08D2a, mucocutaneous infection, examples of such infections that may result from Herpes simplex virus.
Final Listing 14.08D3 is a stand-alone medical criterion for Herpes zoster, formerly in proposed Listing 14.08M3h as an impairment that required limited functioning. The listing is met with either disseminated infection or with multidermatomal eruptions that are resistant to treatment. Final Listing 14.08D4, Progressive multifocal leukoencephalopathy, was in proposed Listings 14.08A9 and 14.08E3. We also added a new Listing 14.08D5 for viral hepatitis in response to the public comments.
14.08E Malignant Neoplasms
Final Listing 14.08E consolidates the two proposed listings for neoplastic diseases, Listings 14.08I and 14.08J, and proposed Listing 14.08A6 (which was for primary lymphoma of the brain in individuals less than 60 years old). In the final rules, we use the term "malignant neoplasms" instead of the term "neoplasms" (which was in the proposed rules) to more accurately reflect the nature of these disorders. In response to public comments pointing out that we considered all lymphomas associated with HIV infection to be disabling, we combined all types of lymphomas into one listing, 14.08E3. We now mention primary lymphoma of the brain, Burkitt's lymphoma, immunoblastic sarcoma, other non-Hodgkin's lymphoma, and Hodgkin's disease only as examples. In response to numerous comments, we also added as Listing 14.08E2 stand-alone medical criteria for Kaposi's sarcoma, which was in the proposed functional listing, 14.08M2. Final Listing 14.08E2 recognizes that there is a range of severity to Kaposi's sarcoma and, therefore, provides specific criteria for extensive oral lesions, or involvement of the gastrointestinal tract, lungs, or other viscera, or involvement of the skin or mucous membranes as described in final Listing 14.08F, discussed below.
Final Listing 14.08E1, Carcinoma of the cervix, was proposed Listing 14.08J2. Final Listing 14.08E4, Squamous cell carcinoma of the anus, was proposed Listing 14.08J3.
14.08F Conditions of the Skin or Mucous Membranes
In response to numerous comments, we added a new Listing 14.08F to the final rules. The listing includes criteria for conditions of the skin or mucous membranes, including mucosal candida, such as vulvovaginal candidiasis (which we had proposed with functional criteria in Listing 14.08M3f) and persistent dermatological conditions, such as eczema or psoriasis (proposed with functional criteria in Listing 14.08M3i). We added to this listing examples, including condyloma caused by human papillomavirus and genital ulcerative disease. Because these conditions may range in severity, we have provided criteria for extensive fungating or ulcerating lesions not responsive to treatment. We also include a cross-reference to the skin listings in 8.00ff, in the event they might apply.
14.08G Hematologic Abnormalities
In response to comments, we have added a new final Listing 14.08G with stand-alone medical criteria for the HIV-related hematological abnormalities we had proposed to link with functional criteria (anemia, granulocytopenia, and thrombocytopenia) in proposed Listings 14.08M3a-c. The listing consists of cross-references to existing hematological listings as a measure of severity.
14.08H Neurological Abnormalities
In response to public comments, we have expanded the proposed listing for HIV encephalopathy (proposed Listing 14.08G) into a general listing category for neurological manifestations of HIV infection, final Listing 14.08H. Final Listing 14.08H1 combines the proposed listing for HIV encephalopathy (proposed Listing 14.08G) with its definition in the eighth paragraph of proposed 14.00D. We revised the description from proposed 14.00D to remove superfluous language. We changed the phrase "cognitive and/or motor dysfunction" to "cognitive or motor dysfunction" because either of these findings is sufficient to find that the listing is met; therefore, "and/or" was unnecessary. We also deleted the requirement that the dysfunction progress "over weeks and months in the absence of a concurrent illness." The phrase "over weeks and months" was unclear: If there had already been months of progression, it is self-evident that weeks would have also passed. Moreover, "weeks and months" is an imprecise standard. Similarly, "in the absence of a concurrent illness" is unnecessary because it speaks to the issue of documentation of the existence of the manifestation called HIV encephalopathy.
Final Listing 14.08H2 adds listing criteria for other neurological manifestations of HIV infection, including peripheral neuropathy, which was in proposed Listing 14.08M2. The impairments in final Listing 14.08H2 must be evaluated under the criteria for the neurological listings in 11.00ff.
14.08I HIV Wasting Syndrome
Final Listing 14.08I combines the proposed listing for HIV wasting syndrome (proposed Listing 14.08H) with its definition in the ninth paragraph of proposed 14.00D. We clarified the proposed language to more accurately reflect the criteria for wasting syndrome. We also corrected a typographical error in the proposed rule: We intended to require chronic diarrhea for 1 month, not 2 months as stated in proposed 14.00D.
14.08J Diarrhea
Final Listing 14.08J is new, and includes stand-alone medical criteria for evaluating listing-level chronic diarrhea, which we had proposed with functional criteria in Listing 1408M3j.
14.08K Cardiomyopathy
The final listing for cardiomyopathy in 14.08K now includes a cross-reference to the criteria in 11.04 of the neurological listings. This addition makes the criteria for HIV-related cardiomyopathy consistent with the criteria for cardiomyopathy in the listing of impairments for the cardiovascular system.
14.08L Nephropathy
We modified the proposed listing for nephropathy, proposed 14.08L, to cross-refer to the entire genitourinary system listings section, rather than to specific listings in keeping with similar revisions throughout these final rules.
14.08M
In 14.08M of the final listing, we combined the proposed listings criteria for pneumonia in 14.08M2a, bacterial or fungal sepsis in 14.08M2C, meningitis in 14.08M2d, septic arthritis in 14.08M2e, endocarditis in 14.08M2f, and radiographically documented sinusitis in 14.08M3k into a general group of HIV-related manifestations that are resistant to treatment and that alone meet the listing without consideration of functional criteria. In some cases, specific variants of these conditions are described in other listings (e.g., bacterial sepsis under final Listing 14.08A, cryptococcal meningitis under final Listing 14.08B4). Therefore, we specify that final Listing 14.08M applies only to these infections if they are not listed in 14.08A-14.08L.
14.08N Repeated Manifestations of HIV Infection
Final 14.08N contains criteria (from proposed Listing 14.08M) for evaluation of manifestations of HIV infection based on functional consequences. We have extensively revised the proposed listing, as discussed above.
114.00D Human Immunodeficiency Virus (HIV) Infection
As we have already explained, we added a new 114.00C, Allergies, growth impairments, and Kawasaki disease, to correspond to 14.00C of the adult rules. This required us to redesignate proposed 114.00C to final 114.00D, which also makes all of the final designations in the preface to the childhood rules parallel the adult rules. We also revised most of final 114.00D of the childhood rules in the same way as the adult rules. In place of the 17 paragraphs we had proposed in 114.00C, final 114.00D is now divided into 8 sections with the same headings and organization as in 14.00D, except that we have added references to children in the headings. Of course, we also revised the text as necessary to refer to children. Thus, final 114.00D1, HIV infection, is the same as final 14.00D1, except that we refer to children instead of adults.
Final 114.00D2, Definitions, is the same as final 14.00D2, except that it does not include a paragraph corresponding to the last paragraph of final 14.00D2. The last paragraph in final 14.00D2 defines the term "significant involuntary weight loss" as it is used in final Listing 14.08I. We did not include a similar explanation in the childhood rules because final Listing 114.08I, Growth disturbance, contains three separate criteria for assessing weight loss in children and is, therefore, more precise than the adult rule.
In response to comments, we extensively revised the discussions in final 114.00D3, Documentation of HIV infection in children, and 114.00D4, Documentation of the manifestations of HIV infection in children. Except as noted below, these sections parallel final 14.00D3 and D4.
In final 114.00D, we have revised, clarified, and expanded the guidance in the fourth and fifth paragraphs of proposed 114.00C for establishing the existence of HIV infection in children. For reasons we explain in the public comments section of this preamble, we changed the proposed rules that referred to children up to the age of 15 months to apply to children up to the age of 24 months.
Final 114.00D3a(i) corresponds to the first category in the fourth paragraph of proposed 114.00C. In addition to revising the paragraph in the same way as the corresponding adult rule, we added a new first sentence to clarify that HIV infection is not documented in children under 24 months of age by antibody testing. Inasmuch as any kind of specimen (such as serum, lymphocyte culture, or cerebrospinal fluid) that contains HIV antigen definitively diagnoses HIV infection, we deleted the repetitive references to HIV antigens from the proposed rules (the second and third criteria in the fourth paragraph of proposed 114.00C and the first and second criteria in the fifth paragraph) and provide only one all-inclusive criterion in final 114.00D3a(ii). In final 114.00D3a(iii) we added the immunoglobulin A (IgA) serological assay specific for HIV as another test that documents HIV infection in children. Although this test is not widely available, it is highly accurate for diagnosis of HIV infection.
Final 114.00D3b describes when documentation of HIV infection is possible without definitive laboratory evidence. We have expanded the explanation of why infants may have serum antibodies for HIV but not have HIV infection, formerly in the fifth paragraph of proposed 114.00C, and have extended the age limit from 15 months to 24 months in response to comments and information we received. We also include criteria for situations in which the presence of HIV infection may be presumed in such infants when there are HIV antibodies and other signs of the infection, such as a significantly depressed CD4 count, even though these findings would not definitively diagnose the presence of the disease. In response to comments, we added to these criteria abnormal immunoglobulin G (IgG) and abnormal CD4/CD8 ratio. As in the adult rules, we provide that the presence of HIV infection in children may also be established by medical history, clinical and laboratory findings, and diagnoses consistent with the prevailing state of medical knowledge and clinical practice, as, for example, when the child has an opportunistic disease predictive of a defect in cell-mediated immunity and there is no other known cause of diminished resistance to that disease.
Final 114.00D4 explains the documentation requirements for opportunistic diseases and other manifestations of HIV infection in children. Final 114.00D4a describes the methods of documenting manifestations of HIV infection by definitive diagnosis. It is identical to final 14.00D4a of the adult rules except that we have added a reference to children.
Final 114.00D4b discusses other acceptable documentation of opportunistic diseases and HIV manifestations. It is identical to final 14.00D4b of the adult rules except that we have added a reference to children.
Final 114.00D5 replaces the discussions in the ninth, tenth, and eleventh paragraphs of proposed 114.00C. For reasons we explain in the public comments section of this preamble, we deleted the proposed text on the epidemiology of HIV infection in children, the text discussing the mean age of diagnosis in infants, and the provisions on the course and spectrum of the disease in children age 13 or older. We also deleted the sentence from the ninth paragraph of proposed 114.00C that cross-referred to the adult listing for HIV wasting syndrome; instead, we have provided explicit listing criteria for the evaluation of weight loss in children in final Listing 114.08I.
In final 114.00D5, we continue to acknowledge that HIV infection can manifest itself differently in children than in adults, and have expanded the provisions describing these differences in response to comments. We moved the proposed guidance on HIV encephalopathy and neurologic problems into a separate paragraph because neurological impairments may be more subtle and difficult to detect in children than in adults. We also added two new paragraphs discussing the evaluation of bacterial infections; as part of this guidance, we point out that older female children may have pelvic inflammatory disease, just as women do.
Final 114.00D6, Evaluation of HIV infection in children, replaces the second, twelfth, and sixteenth paragraphs of 114.00C of the NPRM. The first and second paragraphs of the section are identical to the first and second paragraphs of final 14.00D6, except that we have used the word "child" as appropriate. The third paragraph contains the same information as the third paragraph in final 14.00D6, except that it refers to the sequential evaluation process for children in § 416.924 of part 416. As in the adult rules, final 114.00D6 includes a discussion of the need to evaluate the impact of all impairments in children with HIV infection and explains that some children with HIV infection may have severe impairments that are less than listing-level severity, but that may still be disabling. Evaluation of these cases should proceed through the final step of the sequential evaluation process, where an individualized functional assessment is performed.
Final 114.00D7, Effect of treatment, is an expanded version of the thirteenth paragraph of proposed 114.00C. As in final 14.00D7 of the adult rules, it discusses the need to evaluate the impact of treatment in children with HIV infection and refers to both the potential benefits and the potential adverse effects of treatment on a case-by-case basis. The first and third paragraphs of final 114.00D7 are identical to the corresponding paragraphs in final 14.00D7 except that we have used the word "child" as appropriate. The second paragraph is nearly identical to the second paragraph of final 14.00D7 except that we use an example of a childhood infection, otitis media, and the word "child" as appropriate.
Final 114.00D8, Functional criteria, discusses the functional criteria contained in final Listing 114.08O. We modified the proposed language in order to conform with the changes we made in the functional criteria (see "Explanation of the Final Rule," above).
114.08 Human Immunodeficiency Virus (HIV) Infection
This new listing adds to the regulations our criteria for evaluating HIV infection in children at the listing level. The listing includes a range of opportunistic diseases, cancers, and other manifestations that are indicative of listing-level severity in children with HIV infection. A separate listing is necessary for children because children with HIV infection may differ from adults in the mode of infection, clinical manifestation, and course of the disease.
Specific manifestations that are considered indicative of listing-level HIV infection are in final Listings 114.08A-N, grouped by type (e.g., fungal infections, bacterial infections, malignant neoplasms) for ease of reference. Final Listing 114.08O includes any manifestation(s) of HIV infection that causes listing-level functional limitations.
We reorganized the basic structure and presentation of final Listing 114.08 in the same way as the adult rules. Therefore, the final listing is significantly different from the proposed listing. Important differences between the final listing and the NPRM and between the childhood and adult listings follow.
For completeness, we added to the final childhood listing a number of criteria that we had proposed only in the adult listing. Adding these criteria did not change the evaluation of HIV infection in children because our regulations in §§ 404.1525 and 416.925 call for using the adult criteria for children whenever the childhood criteria do not apply. We added these new criteria only to make the childhood listing easier to use.
Final Listing 114.08A addresses bacterial infections. The listing includes the same criteria as in final Listing 14.08A. In addition, we have retained, in final Listing 114.08A5, the criteria we proposed in Listing 114.08F1 for children less than 13 years old who experience certain pyogenic bacterial infections at least twice in 2 years. Although we have deleted from the remainder of the childhood listings all of the previously proposed distinctions between children under age 13 and children age 13 and above, we retained this distinction only in final Listing 114.08A5, where it is medically valid. However, consistent with the adult listings, we also added criteria at final Listing 114.08A6 that apply to multiple or recurrent bacterial infections caused by any bacteria—including pelvic inflammatory disease—and that can be applied to all children.
We revised final Listings 114.08B, Fungal infections, 114.08C, Protozoan or helminthic infections, 114.08D, Viral infections, 114.08E, Malignant neoplasms, 114.08F, Conditions of the skin and mucous membranes, and 114.08G, Hematologic abnormalities, in the same way as the adult rules. The language of these provisions is the same except that we provide cross-references to the appropriate childhood listings where necessary. As in the adult listings, the revisions to these childhood rules also provide stand-alone medical criteria for several of the manifestations we had proposed to tie to a test of functional limitations in proposed Listings 114.08L and 114.08M. We added criteria for carcinoma of the cervix and squamous cell carcinoma of the anus (as in the adult listing) to final Listing 114.08E because these conditions may occur in adolescents.
We have extensively revised final Listing 114.08H (proposed Listing 114.08J), Neurological manifestations, based on public comment. A child with HIV infection may now have a neurological manifestation (for example, HIV encephalopathy or peripheral neuropathy) that meets the listing in any of four ways. In response to a comment pointing out that the criteria in proposed Listing 114.08J3 essentially described impairments that meet the criteria in 111.00 of the listings, we revised final Listing 114.08H3 to provide for only progressive motor dysfunction affecting gait and station or fine and gross motor skills. We also changed the criteria for evaluating motor deficits to eliminate the requirement that they be symmetric. However, inasmuch as some children will have neurologic manifestations that meet the criteria of one of the listings in 111.00, we added to the opening paragraph a criterion that permits a finding of disability by cross-reference to those listings. In final Listing 114.08H1, we also revised the criteria for evaluating loss of previously acquired intellectual ability (which were in proposed Listing 114.08J1) to reflect our intent to include those situations where the child does not lose previous knowledge, but is unable to learn new information; that is, suddenly acquires a new learning disability. We also added a cross-reference in final Listing 114.08H2 to the new discussion in 114.00D5 describing documentation of impaired brain growth.
Final Listing 114.08I addresses growth disturbances. These criteria were previously in proposed Listing 114.08K. Based on numerous public comments, we added weight criteria for evaluating failure to thrive, which are based on a fall from an established growth curve. These criteria recognize that, unlike adults (who have stopped growing), children can be gaining weight yet still be failing to thrive because their weight gain is not commensurate with their growth. Final Listing 114.08I1 describes children who, because of weight loss or failure to gain weight at an appropriate rate for age, have a persistent fall (defined as 2 months or longer) of 15 percentiles from an established growth curve on standard growth charts, irrespective of the actual percentile at which their weight lies. Conversely, final Listing 114.08I2 describes children whose weight, because of an involuntary weight loss or failure to gain weight at an appropriate rate, falls and persists below the third percentile from an established growth curve on standard growth charts, irrespective of the number of percentiles of the fall. A new third criterion, final Listing 114.08I3, provides for an involuntary weight loss greater than 10 percent of baseline that persists for at least 2 months. In final Listing 114.08I4, which incorporates the proposed listing's cross-reference to the growth impairment listings, we changed the cross-reference to the entire section, 100.00, for consistency with the changes we made to other listings.
Final Listing 114.08J, Diarrhea, is the same as the corresponding final adult listing, 14.08J. This condition was previously in proposed Listings 114.08L and 114.08M only in conjunction with the functional requirements. Final Listing 114.08K, Cardiomyopathy, is also the same as final Listing 14.08K, except for the cross-reference to the listings in 104.00; the cross-reference to adult neurological Listing 11.04, however, is correct and consistent with our cardiovascular rules for children. This condition was proposed only in the adult listings. We decided to add it to the childhood listings because it also occurs in children.
Final Listing 114.08L addresses lymphoid interstitial pneumonia/pulmonary lymphoid hyperplasia (LIP/PLH complex). This criterion was previously in proposed Listing 114.08G. We changed the criteria to apply to all children rather than just applying it to children under age 13, as we proposed. We also added criteria for listing-level severity, because these conditions may range widely in their severity and impact on a child's functioning.
Final Listing 114.08M, Nephropathy, is the same as final Listing 14.08L except that the cross-references are to the criteria in 106.00 of the childhood listings. As with cardi