[Federal Register Volume 78, Number 223 (Tuesday, November 19, 2013)]
[Proposed Rules]
[Pages 69324-69336]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 2013-27514]
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SOCIAL SECURITY ADMINISTRATION
20 CFR Parts 404
[Docket No. SSA-2010-0055]
RIN 0960-AF88
Revised Medical Criteria for Evaluating Hematological Disorders
AGENCY: Social Security Administration.
ACTION: Notice of proposed rulemaking.
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SUMMARY: We propose to revise the criteria in the Listing of
Impairments (listings) that we use to evaluate cases involving
hematological disorders in adults and children under titles II and XVI
of the Social Security Act (Act). The proposed revisions reflect
advances in medical knowledge, our adjudicative experience, and
information we received from medical experts and the public.
DATES: To ensure that your comments are considered, we must receive
them no later than January 21, 2014.
ADDRESSES: You may submit comments by one of three methods--Internet,
fax, or mail. Do not submit the same comments multiple times or by more
than one method. Regardless of which method you choose, please state
that your comments refer to Docket No. SSA-2010-0055 so that we may
associate your comments with the correct regulation.
Caution: You should be careful to include in your comments only
information that you wish to make publicly available. We strongly urge
you not to include in your comments any personal information, such as
your Social Security number or medical information.
1. Internet: We strongly recommend that you submit your comments
via the Internet. Please visit the Federal eRulemaking portal at http://www.regulations.gov. Use the Search function to find docket number
SSA-2010-0055. The system will issue a tracking number to confirm your
submission. You will not be able to view your comment immediately
because we must post each comment manually. It may take up to a week
for your comment to be viewable.
2. Fax: Fax comments to (410) 966-2830.
3. Mail: Address your comments to the Office of Regulations, Social
Security Administration, 107 Altmeyer Building, 6401 Security
Boulevard, Baltimore, Maryland 21235-6401.
Comments are available for public viewing on the Federal
eRulemaking portal at http://www.regulations.gov, or in person, during
regular business hours, by arranging with the contact person identified
below.
FOR FURTHER INFORMATION CONTACT: Cheryl A. Williams, Office of Medical
Listings Improvement, Social Security Administration, 6401 Security
Boulevard, Baltimore, Maryland 21235-6401, (410) 965-1020. For
information on eligibility or filing for benefits, call our national
toll-free number, 1-800-772-1213 or TTY 1-800-325-0778, or visit our
Internet site, Social Security Online, at http://www.socialsecurity.gov.
SUPPLEMENTARY INFORMATION:
What revisions are we proposing?
We propose to:
Revise and expand the introductory text to the
hematological disorders body system for both adults (section 7.00) and
children (section 107.00);
Revise and reorganize the listings in this body system to
update them and to make the adult and childhood rules more consistent;
and
Add criteria to the adult rules for establishing
disability under the listings
[[Page 69325]]
based on functional limitations associated with hematological
disorders.
Why are we proposing to make these changes?
We last issued final rules making comprehensive revisions to the
hematological disorders listings on December 6, 1985.\1\ Since then, we
have generally only extended the effective date of the rules.\2\ In the
preamble to the 1985 rules, we stated that we would carefully monitor
these listings to ensure that they continue to meet program purposes,
and that we would revise them if warranted. We are now proposing to
update the medical criteria in the current listings and provide more
information about how we evaluate hematological disorders. For example:
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\1\ (50 FR 50068)
\2\ We published some revisions to the hematological body system
on April 24, 2002, and November 15, 2004. See 67 FR 20018 and 69 FR
67017 (corrected at 70 FR 15227). These revisions were not
comprehensive; they addressed only specific listings. The current
listings will no longer be effective as of July 2, 2012, unless we
extend them or revise and issue them again. See 75 FR 33166.
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We propose to update current listing 7.08, which provides
transfusion criteria for spontaneous hemorrhage (bleeding) in
hemophilia. It does not reflect the current standard of care, because
physicians now use other treatments for this type of bleeding.
We propose to update current listing 7.17, which addresses
bone marrow and stem cell transplantation only for aplastic anemias.
Other hematological disorders, such as sickle cell disease, may now be
treated with bone marrow or stem cell transplantation.
We are also proposing changes to the current listings to reflect
the considerable adjudicative experience we have gained since we issued
the 1985 rules. Some of these proposals also reflect information we
received at outreach conferences from people who have hematological
disorders, their family members, physicians who treat hematological
disorders, and advocates who represent people who have these disorders.
These proposals also take into consideration recommendations we
received in public comments in response to a previous notice of
proposed rulemaking (NPRM), which we explain in more detail below.
How did we develop these proposed rules?
On November 27, 2001, we published an NPRM proposing revisions to
both the listings for hematological disorders and the listings for
malignant neoplastic diseases.\3\ We received public comments raising
significant issues about the proposed listings for some of the
hematological disorders. To obtain more information, on April 18, 2002,
we published a notice providing an additional public comment period.\4\
We also held meetings on April 8, 2002, April 24, 2002, and August 26,
2002, with medical professionals and representatives of advocacy and
legal-services groups. During these meetings, we asked the participants
for information about the issues.\5\
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\3\ 66 FR 59306.
\4\ 67 FR 19138.
\5\ You can read the notes from these meetings at http://www.regulations.gov/#!docketDetail;dct=FR%252BPR%252BN%252BO%252BSR;rpp=10;po=0;D=SSA-
2006-0113.
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Based on the information we received from these activities, we
published a notice on November 15, 2004, withdrawing the 2001 proposed
rules for hematological disorders.\6\ We later hosted a policy
conference on sickle cell disease and hemophilia in Boston, MA, on
November 18, 2004.\7\ At this conference, we heard comments and
suggestions for updating and revising the current rules for sickle cell
disease and hemophilia from people who have these disorders, their
family members, and physicians, advocates, and other professionals. In
developing this NPRM, we considered the information we obtained at this
conference, our earlier meetings, and the comments we received on the
2001 NPRM.\8\
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\6\ 69 FR 67039.
\7\ You can read the transcript of the November 18, 2004, policy
conference at http://www.regulations.gov/#!docketDetail;dct=FR%252BPR%252BN%252BO%252BSR;rpp=10;po=0;D=SSA-
2006-0113.
\8\ You can view the comments we received on the 2001 NPRM by
going to http://www.regulations.gov/#!docketDetail;dct=FR%252BPR%252BN%252BO%252BSR;rpp=10;po=0;D=SSA-
2006-0113.
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What general changes are we proposing?
We propose to use only broad categories of hematological disorders
in the listings instead of the mixture of specific hematological
disorders and broad categories of hematological disorders that are in
the current listings. We believe that it would be better to use only
broad categories throughout this body system so that we can include
more types of hematological disorders. We also propose to remove some
of the current listings and revise the criteria of others.
The following chart shows the headings of the current listings for
evaluating hematological disorders in adults and the name of the
proposed listing, or the proposed listing under which we would evaluate
the disorder that is currently listed:
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Current listings * Proposed listings
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7.02 Chronic anemia (hematocrit Evaluate under the appropriate
persisting at 30 percent or less due listing for the underlying
to any cause). hematological disorder or
under 7.18.
7.05 Sickle cell disease, or one of its 7.05 Hemolytic anemias.
variants.
7.06 Chronic thrombocytopenia (due to Evaluate under 7.08.
any cause).
7.07 Hereditary telangiectasia......... Evaluate under the body system
where the bleeding occurs.
7.08 Coagulation defects (hemophilia or 7.08 Disorders of hemostasis.
a similar disorder).
7.09 Polycythemia vera (with Removed.
erythrocytosis, splenomegaly, and
leukocytosis or thrombocytosis).
7.10 Myelofibrosis (myeloproliferative 7.10 Disorders of bone marrow
syndrome). failure.
7.15 Chronic granulocytopenia (due to Evaluate under 7.10.
any cause).
7.17 Aplastic anemias with bone marrow 7.17 Hematological disorders
or stem cell transplantation. treated by bone marrow or stem
cell transplantation.
7.18 Repeated complications of
hematological disorders.
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* The listings in this body system are not numbered consecutively. This
chart contains the only listings in this body system.
We also propose to replace the current introductory text with
updated and expanded guidance that reflects the proposed listings. The
following chart shows the headings of the current and proposed sections
of the introductory text:
[[Page 69326]]
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Current introductory text Proposed introductory text
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7.00A Impairment caused by anemia.. 7.00A What hematological disorders
do we evaluate under these
listings?
7.00B Chronicity is indicated by... 7.00B What evidence do we need to
document that you have a
hematological disorder?
7.00C Sickle cell disease.......... 7.00C What are hemolytic anemias,
and how do we evaluate them under
7.05?
7.00D Coagulation defects.......... 7.00D What are disorders of
hemostasis, and how do we evaluate
them under 7.08?
7.00E What are disorders of bone
marrow failure, and how do we
evaluate them under 7.10?
7.00F How do we evaluate bone
marrow or stem cell
transplantation under 7.17?
7.00G How do we use the functional
criteria in 7.18?
7.00H How do we consider your
symptoms, including your pain,
severe fatigue, and malaise?
7.00I How do we evaluate episodic
events in hematological disorders?
7.00J. How do we evaluate
hematological disorders that do
not meet one of these listings?
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What specific changes are we proposing to make in the introductory text
to the listings for evaluating hematological disorders in adults?
The following is a detailed explanation of the proposed changes to
the introductory text:
Proposed section 7.00A--What hematological disorders do we evaluate
under these listings?
In this new section, we explain which hematological disorders we
evaluate under these listings and which we evaluate under the listings
in other body systems.
Proposed section 7.00B--What evidence do we need to document that you
have a hematological disorder?
In this new section, we explain the evidence we need to establish
the existence of a hematological disorder. In proposed sections 7.00B1
and B2, we provide two methods for establishing the existence of the
disorder when we have a copy of definitive laboratory test results. In
proposed section 7.00B3, we provide an additional method for
establishing the existence of the disorder when we do not have a copy
of definitive laboratory test results.
In proposed section 7.00B1, we explain that a laboratory report of
a definitive test that establishes a hematological disorder, signed by
a physician, is sufficient to document that you have a hematological
disorder. As an alternative, we also explain in proposed section 7.00B2
that, if we have a copy of the laboratory report of a definitive test
that establishes a hematological disorder, but a physician has not
signed it, we also require a report from a physician confirming that
the person has the hematological disorder. We need this statement
because our rules require evidence from an ``acceptable medical
source'' to establish the existence of a medically determinable
impairment, and a physician is the only such source we can accept for
hematological disorders.\9\ We are proposing these changes only to
clarify our current rules and are not proposing that the physician
needs to provide any more information to establish the existence of the
disorder than we require under our current rules.
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\9\ We define the terms ``medically determinable impairment''
and ``acceptable medical source'' in Sec. Sec. 404.1508, 404.1513,
416.908, and 416.913 of our regulations.
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In proposed section 7.00B3, we explain how we can establish the
existence of a hematological disorder when we do not have a copy of the
laboratory report of a definitive test. Under section 7.00B3, we need a
persuasive report from a physician that a positive diagnosis of the
person's hematological disorder was confirmed by appropriate laboratory
analysis or other diagnostic method(s). We also explain that to be
persuasive, the report must state that the person has had the
appropriate definitive laboratory test or tests for diagnosing the
disorder and provide the results, or explain how the diagnosis was
established by other diagnostic techniques consistent with the
prevailing state of medical knowledge and clinical practice.
We propose to remove the information in current section 7.00B
because it primarily discusses medically acceptable imaging techniques.
These techniques would apply to the proposed listings primarily to
establish the presence of certain complications of hematological
disorders, such as blood clots. There are many other types of
laboratory tests and clinical findings we may need to establish a
hematological disorder and the nature of any complications. We do not
believe it would be practical or necessary to include them all in the
introductory text of the proposed listings. We propose to remove,
rather than expand, the limited guidance in current section 7.00B.
Current section 7.00B also includes two sentences that explain how
we establish ``chronicity.'' We would no longer need this rule because
we do not use the term ``chronicity'' in any of the proposed listings.
Instead, we provide specific criteria in each proposed listing for
which we need evidence of chronicity. For example, in some of the
proposed listings we require a certain number of events (such as
hospitalizations) directly associated with the person's hematological
disorder occurring at least 30 days apart and within a 12-month period.
In proposed section 7.00B4, we explain that we will make every
reasonable effort to obtain the results of appropriate laboratory
testing. We also explain that we will not purchase tests of clotting
factors, bone marrow aspirations, or bone marrow biopsies. We will not
purchase these tests because obtaining, handling, or evaluating the
blood or tissue samples may be too complex, invasive, or costly.
Proposed section 7.00C--What are hemolytic anemias, and how do we
evaluate them under 7.05?
In this new section, we describe hemolytic anemias and provide
examples of these disorders. We propose to evaluate all hemolytic
anemias under listing 7.05 instead of listing only sickle cell disease
or its variants.
In proposed section 7.00C2, we address a concern raised at our
meetings on sickle cell disease: That some hospitalizations are for
complications of sickle cell disease, and that our adjudicators should
recognize and consider such hospitalizations when determining whether a
person's impairment meets current listing 7.05B. Since we also have
requirements for hospitalizations in the proposed listings, we propose
to address this concern by providing examples of common complications
of hemolytic anemias (including sickle cell disease) that could result
in hospitalization. These examples include some of the complications
that we term ``major visceral episodes'' in current section 7.00C. We
also specify that the hospitalizations do not all have to be for the
same complication, such as a painful (vaso-occlusive) crisis. The three
hospitalizations we require in proposed
[[Page 69327]]
listing 7.05B may be for three different complications of a hemolytic
anemia.
In proposed section 7.00C3, we explain that the hemoglobin
measurements required in proposed listing 7.05C do not have to occur
when the person is free of complications of his or her hemolytic
anemia. The frequency of very low hemoglobin measurements required in
the proposed listing provides a way for finding disability without
considering the person's complications because it would establish a
hemoglobin level associated with serious chronic anemia.
We propose a new listing 7.05D for transfusion-dependent beta
thalassemia major. In proposed section 7.00C4, we define the term
``transfusion-dependent'' as it is widely used in the medical community
to emphasize that transfusion dependency is necessary to sustain life.
We exclude prophylactic red blood cell (RBC) transfusion for sickle
cell disease because we do not consider this therapy to be of equal
medical significance to transfusion-dependent thalassemia.
Proposed section 7.00D--What are disorders of hemostasis, and how do we
evaluate them under 7.08?
In this new section, we propose to use a more inclusive term,
``disorders of hemostasis,'' to reflect the criteria in proposed
listing 7.08. We provide examples of these disorders, which include
coagulation defects.
We propose to remove the guidance in current section 7.00D about
prophylactic therapy because this guidance would no longer be
applicable in light of proposed listing 7.08. Prophylactic therapy for
coagulation defects is usually self-administered and does not reflect
the requirement in proposed listing 7.08 that the disorder result in
hospitalization.
In proposed section 7.00D2, we provide examples of common
complications of disorders of hemostasis that may result in
hospitalization or contribute to functional limitations. We explain
that surgery is a complication in disorders of hemostasis if it
requires treatment with factor infusions or anticoagulant medication to
control bleeding or coagulation in connection with the surgery.
Proposed section 7.00E--What are disorders of bone marrow failure, and
how do we evaluate them under 7.10?
Proposed listing 7.10, Disorders of bone marrow failure, includes
several hematological conditions that we now list separately:
Myelofibrosis (current listing 7.10), granulocytopenia (current listing
7.15), and aplastic anemia (current listing 7.17). We name these
conditions as examples of disorders of bone marrow failure to emphasize
that we still include them in the proposed hematological disorders
listings. In proposed section 7.00E2, we provide examples of common
complications of disorders of bone marrow failure that may result in
hospitalization or contribute to functional limitations. As we do for
other hematological disorders that require hospitalizations, we specify
in 7.00E2 that the hospitalizations in proposed listing 7.10A do not
all have to be for the same complication. We also provide that we will
consider other types of systemic infections that may result in
hospitalizations. As we explain below in our summary of proposed
listing 7.10A, we would include viral and fungal infections because
they can have the same impact as bacterial infections required in
current listing 7.10B.
Proposed section 7.00F--How do we evaluate stem cell or bone marrow
transplantation under 7.17?
In this section, we explain that under proposed listing 7.17, we
will consider a person to be disabled for 12 months from the date of
bone marrow or stem cell transplantation, or we may consider a person
to be disabled for a longer period if he or she has any serious post-
transplantation complications, such as graft-versus-host (GVH) disease.
The proposed rule is consistent with how we evaluate bone marrow and
stem cell transplantation in other body systems.\10\
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\10\ See, for example, section 13.00L4 in the malignant
neoplastic diseases body system.
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Proposed section 7.00G--How do we use the functional criteria in 7.18?
We are proposing new listing 7.18 to evaluate repeated
complications of hematological disorders, including those complications
listed in 7.05, 7.08, and 7.10 that do not have the requisite findings
for those listings, or other complications. Under listing 7.18, the
complications listed in 7.05, 7.08, and 7.10 that do not have the
requisite findings for those listings, or the other complications the
person has that are not contained in those specific listings, must
result in ``significant, documented symptoms or signs.'' The person
must also have a marked limitation in at least one of three broad areas
of functioning. We explain each part of this listing in detail in
proposed section 7.00G. We modeled listing 7.18 after a number of
listings in the immune disorders body system (14.00), and we based the
rules in proposed section 7.00G on the rules in section 14.00I of the
introductory text of the immune disorders body system.
Proposed listing 7.18 requires a marked limitation of activities of
daily living; a marked limitation in maintaining social functioning; or
a marked limitation in completing tasks in a timely manner due to
deficiencies in concentration, persistence, or pace. In proposed
section 7.00G4, we use essentially the same definition of ``marked'' as
we use in section 14.00I5, but we are not including the description of
``marked'' as ``more than moderate but less than extreme.'' Instead, we
would use an explanation based on the language describing the rating
scale for mental disorders in current Sec. Sec. 404.1520a(c)(4) and
416.920a(c)(4). This rating scale describes ``marked'' as the fourth
point on a five-point rating scale. We explain that we would not
require our adjudicators to use such a scale, but that ``marked'' would
be the fourth point on a scale of ``no limitation, mild limitation,
moderate limitation, marked limitation, and extreme limitation.'' With
this guideline, it would be unnecessary to state that ``marked'' falls
between ``moderate'' and ``extreme.'' In proposed sections 7.00G5,
7.00G6, and 7.00G7, we explain what we mean by ``activities of daily
living,'' ``social functioning,'' and ``completing tasks in a timely
manner.'' We based these proposed sections on current sections 14.00I6,
14.00I7, and 14.00I8 in our immune system listings.
Proposed section 7.00H--How do we consider your symptoms, including
your pain severe fatigue, and malaise?
In this section, we explain how we consider the effects of the
symptoms of hematological disorders on a person's ability to function.
Except for a reference to section 7.00 instead of section 14.00, this
paragraph would be identical to section 14.00H in our immune system
disorders body system.
Proposed section 7.00I--How do we evaluate episodic events in
hematological disorders?
Several of our current hematological listings include a requirement
for events (pain crises, transfusions, or infections) within the 5
months or 12 months before we adjudicate a claim. We propose similar
requirements in several of the proposed hematological listings, but
also propose several changes. In proposed section 7.00I, we would
explain that under listings 7.05, 7.08, and 7.10A, we require a
specific number of events within a consecutive 12-month period and that
when we use such criteria, the 12-month period must occur within the
period we are considering in
[[Page 69328]]
connection with your application or continuing disability review. Our
current rules require that the events must take place in a period
immediately before we adjudicate a case. This proposed change would be
consistent with how we evaluate episodic events in other body
systems.\11\ We believe this change also is both more logical and fair,
and that it would address many adjudicator questions we have received
over the years. In some cases, for example, we must determine whether a
person was disabled in a period that ended before we adjudicated the
claim.
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\11\ See, for example, section 4.00A3e in the cardiovascular
system.
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How are we proposing to revise the criteria in the listings for
evaluating hematological disorders in adults?
We propose to remove several current hematological listings:
Current listing 7.02, for chronic anemia. We would
evaluate anemia that results from an underlying hematological disorder
under the appropriate proposed listing for the disorder or under the
functional criteria in proposed listing 7.18. We would also remove the
guidance in current section 7.00A for evaluating impairments caused by
anemia ``according to the ability of the person to adjust to the
reduced oxygen[-]carrying capacity of the blood.'' This guidance does
not consider that a person who can adjust to his or her anemia may have
other serious complications that could be disabling. We provide
examples of these other complications in proposed sections 7.00C,
7.00D, and 7.00E, the sections of the proposed introductory text that
describe the major categories of hematological disorders in the
proposed listings. As we have already mentioned, some proposed listings
establish the presence of chronic anemia that meets the requirement of
three hospitalizations within 12 months spaced 30 days apart,
essentially replacing the ``chronicity'' requirement in current section
7.00B.
Current listings 7.05D for sickle cell disease, 7.09 for
polycythemia vera, and 7.10A for myelofibrosis with chronic anemia.
These listings are reference listings. Reference listings are redundant
because they are met by satisfying the criteria of other listings, and
we are removing them from our listings as we update the body
systems.\12\
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\12\ Current listing 7.10A also cross-refers to current listing
7.02, which we are proposing to remove.
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Current listing 7.06, for chronic thrombocytopenia. We
would include thrombocytopenia under proposed new listing 7.08,
``Disorders of hemostasis.''
Current listing 7.07 for hereditary telangiectasia.
Hereditary telangiectasia is a disorder that may result in bleeding
from defects in the blood vessels in various organs. We believe it is
more appropriate to evaluate hereditary telangiectasia under the body
system where this bleeding occurs, such as the digestive body system
(for example, listing 5.02) or the neurological body system (for
example, listing 11.04).
Current listing 7.10C for myelofibrosis with intractable
bone pain. We believe it is more appropriate to evaluate this
impairment under the criteria for the affected body system.
Current listing 7.15, for chronic granulocytopenia. We
would include granulocytopenia under proposed new listing 7.10,
``Disorders of bone marrow failure.''
While incorporating the disorders from several of the foregoing
listings into other proposed listings, we also propose either to revise
the criteria in the current listing or replace it with new criteria.
Two changes would be common to several listings that include criteria
for episodic events (for example, painful crises or hospitalizations):
We would require at least 30 days between these events to ensure that
we are evaluating separate events, and we would require that these
events occur within a relevant 12-month period, consistent with our
rules in other body systems.
The following is a detailed explanation of the changes we are
proposing to the hematological disorder listings for evaluating
hematological disorders in adults that need further explanation.
Proposed Listing 7.05--Hemolytic Anemias
In addition to expanding the scope of current listing 7.05A, we
propose to make the following changes:
We would add a requirement for the treatment of documented painful
crises with parenteral (intravenous or intramuscular) narcotic
medication. Physicians usually provide this treatment (in outpatient or
inpatient settings) only for crises they cannot alleviate with initial
treatment, such as oral narcotics or non-narcotic medications. We
believe that the proposed requirement for parenteral narcotic
medication will confirm the severity of the crisis and provide a more
objective measure than the requirement in the current listing.
We would also require at least 6 painful crises treated with
parenteral narcotic medication in a 12-month period, instead of the
three in the 5-month period prior to adjudication in the current
listing. We believe the need for parenteral narcotic medication on such
a frequent basis is indicative of recurring severe pain that prevents a
person from working for the required 12-month duration. We based the
change in frequency of painful crises on our adjudicative experience
and the prevailing state of medical knowledge and clinical practice.
Although people who have painful crises less frequently than 6 times in
a 12-month period may be limited in functioning, we believe they are
not precluded from engaging in any gainful activity.
We would consider a person with hemolytic anemia who has less
severe painful episodes or other complications that result in
functional limitations under proposed listing 7.18, which we describe
in detail below.
In addition, people who have severe painful episodes may have
impairments that meet proposed listing 7.05B. Proposed listing 7.05B
corresponds to current listing 7.05B in that it would include people
who have three hospitalizations in a 12-month period because of their
hemolytic anemia. We would revise the current listing as follows:
We explain that the hospitalization can be for any complication of
hemolytic anemia, which, as we explain in proposed section 7.00C2,
would include painful crises. We believe that three hospitalizations in
a 12-month period establish hemolytic anemia of listing-level severity
because complications of hemolytic anemia that require hospitalization
are generally more serious and involve longer recovery periods than
those treated solely in outpatient settings. We also specify in the
introductory text that the three hospitalizations do not have to be for
the same complication.
We would include criteria for hospitalizations similar to current
listing 7.05B but specify that each hospitalization must last at least
48 hours. We believe a hospitalization period of at least 48 hours is
indicative of a severe complication of hemolytic anemia, and would more
clearly define our intent in the current rule for an ``extended
hospitalization.'' We would include the hours the person spends in the
emergency department immediately before hospital admission as part of
his or her hospitalization. We would include these hours in the
emergency department because the person is likely to be receiving the
same intensity of care as he or she will receive in the hospital.
In proposed listing 7.05C, we would require hemoglobin measurements
[[Page 69329]]
instead of the current requirement for hematocrit values. Hemoglobin is
measured directly. Hematocrit values are calculated, and therefore they
are less precise. We would accept the hemoglobin measurements required
in proposed listing 7.05C regardless of whether the person was
experiencing complications of his or her hemolytic anemia at the time
of the measurements.
Current listing 7.05C requires a persistence of a hematocrit of 26
percent or less, which is comparable to a hemoglobin measurement of
approximately 8.5 grams per deciliter (g/dL) or less. We believe that
hematocrit or hemoglobin at these levels does not necessarily correlate
with an inability to do any gainful activity. Instead, the proposed
listing would require a hemoglobin measurement of 7.0 g/dL or less. We
believe a hemoglobin measurement at this level provides a better
description of a listing-level impairment because many people who have
this finding will have related problems, such as an abnormal heartbeat,
shortness of breath with mild exertion, and significant fatigue. We
also believe that the frequency of the hemoglobin measurements in the
proposed listing provides a way for finding a person to be disabled
without having to consider the person's specific complications since it
establishes a hemoglobin level associated with serious chronic anemia.
Even though we are proposing a specific laboratory finding for
evaluating anemia in proposed listing 7.05C, we would also consider
anemia under proposed new listing 7.18. Proposed listing 7.18 will
allow us to make an individualized determination about disability for
people whose impairments do not meet proposed listing 7.05.
Proposed Listing 7.08--Disorders of Hemostasis
This proposed listing corresponds to current listing 7.06,
``Chronic thrombocytopenia (due to any cause),'' and current listing
7.08, ``Coagulation defects (hemophilia or similar disorder).'' We
would evaluate thrombocytopenia and coagulation defects under this
proposed listing because they are both disorders of hemostasis. The
proposed listing would also cover any other hypo- or hypercoagulation
disorder.
We believe that the criterion in proposed 7.08 for complications
requiring at least three hospitalizations within a 12-month period and
occurring at least 30 days apart is a more accurate medical description
of listing-level thrombocytopenia than the current requirements for
platelet counts and spontaneous bleeding. Some people who have
thrombocytopenia that satisfies the criteria in the current listing for
platelet counts repeatedly below 40,000/mm\3\ and one episode of
spontaneous bleeding (current listing 7.06A) will have serious
limitations in their functioning. Others, however, will not have
limitations that prevent them from doing any gainful activity for at
least 12 continuous months, the duration requirement in our definition
of disability. Some people who have thrombocytopenia with the requisite
platelet counts and who experience one episode of intracranial bleeding
(current listing 7.06B) also do not have impairments that meet the 12-
month duration requirement. Likewise, we believe that the episodes of
bleeding we include in the other current listings for disorders of
hemostasis, including bleeding episodes resulting from hemophilia, do
not necessarily preclude a person from doing any gainful activity for
at least 12 months.
The requirement for transfusions in current listing 7.08 is out of
date. Instead of blood transfusions, physicians now use blood-clotting
factor VIII, factor IX, or other factor components to treat
uncontrolled bleeding in hemophilia. A person usually receives
intensive treatment with factor in a hospital if he or she cannot
control a bleed with factor through outpatient treatment or self-care.
We believe that the requirement for hospitalization will confirm the
severity of the bleeding episode and provide an objective measure.
Similarly, the requirement for hospitalization would be an objective
measure for other complications of disorders of hemostasis, such as
thromboses (blood clots) that result from a hypercoagulation disorder.
We would use the criteria in proposed listing 7.18 to evaluate
hemostasis disorders that do not meet the criteria of proposed listing
7.08 but that cause complications that affect a person's functioning.
For example, proposed listing 7.18 would include some people who have
joint deformity (arthropathy) from repeated bleeding into a joint. We
may also use the criteria in the musculoskeletal listings to evaluate
the effects of joint deformity.\13\
---------------------------------------------------------------------------
\13\ See proposed section 7.00J1.
---------------------------------------------------------------------------
Proposed Listing 7.10--Disorders of Bone Marrow Failure
This proposed listing corresponds to current listings 7.10,
``Myelofibrosis (myeloproliferative syndrome),'' 7.15, ``Chronic
granulocytopenia (due to any cause),'' and 7.17, ``Aplastic anemias.''
We would evaluate myelofibrosis, granulocytopenia, and aplastic
anemias, as well as any other disorder of bone marrow failure, under
the proposed listing. We would also evaluate aplastic anemias and other
disorders of bone marrow failure treated with bone marrow or stem cell
transplantation under proposed listing 7.17.
In proposed listing 7.10A, we would require three hospitalizations
within a 12-month period (and occurring at least 30 days apart) for
complications of a disorder of bone marrow failure (such as systemic
infections). As we noted earlier in our explanation of proposed section
7.00E, in proposed 7.10A we would broaden the criterion in current
listing 7.10B to include systemic viral and fungal infections. Systemic
viral and fungal infections that must be treated in the hospital are as
serious as systemic bacterial infections. People who have episodes of
systemic infections that do not meet the requirement in proposed
listing 7.10A may qualify under proposed listing 7.18.
We propose to remove current listing 7.10C because intractable bone
pain is rare in myelofibrosis. When a person has this symptom, we would
be able to evaluate his or her impairment under proposed listing 7.18.
We can also use an appropriate listing in the musculoskeletal body
system, as we make clear in proposed section 7.00J1.
Proposed Listing 7.17--Hematological Disorders Treated by Bone Marrow
or Stem Cell Transplantation
Current listing 7.17 is for aplastic anemias treated with bone
marrow or stem cell transplantation. We would broaden this listing to
include all hematological disorders treated with these transplantation
procedures. We would consider the person disabled until ``at least'' 12
months from the date of transplantation. The phrase ``at least'' would
provide our adjudicators with the flexibility to consider the person
disabled for a period longer than 12 months from the date of
transplantation if the evidence justifies it. After that period, we
would evaluate any residual impairment(s) under the criteria for the
affected body system.
Proposed Listing 7.18--Repeated Complications of Hematological
Disorders
As we have already noted, we propose a new listing based on
repeated complications of any hematological disorder together with
functional limitations that result from the disorder. We modeled this
proposed listing after several listings in our immune disorders
[[Page 69330]]
body system.\14\ The proposed listing reflects symptoms, signs, and
complications of hematological disorders. Like immune disorders,
hematological disorders can be characterized by episodes of
complications and symptoms that can significantly affect functioning.
For this reason, we believe it is appropriate to have a listing that
includes functional limitations for hematological disorders like the
listings in the immune disorders body system. We believe these
functional criteria would help us more quickly and easily adjudicate
some claims.
---------------------------------------------------------------------------
\14\ See listings 14.02B, 14.03B, 14.04D, 14.05E, 14.06B,
14.07C, 14.08K, 14.09D, and 14.10B.
---------------------------------------------------------------------------
How are we proposing to change the introductory text and listings for
evaluating hematological disorders in children?
With one exception, the proposed childhood introductory text and
listings are the same as the proposed adult rules, apart from minor
differences such as referring to children instead of adults. The
reasons we gave earlier for changing or removing current criteria for
adults also apply to the childhood criteria.
We are not proposing a listing for children like proposed listing
7.18 for adults. Instead, we would use our current childhood rules for
evaluating functional equivalence to the listings.\15\ These rules
accomplish the same objective for children as proposed listing 7.18
would for adults.
---------------------------------------------------------------------------
\15\ See Sec. 416.926a.
---------------------------------------------------------------------------
What is our authority to make rules and set procedures for determining
whether a person is disabled under the statutory definition?
Under the Act, we have full power and authority to make rules and
regulations and to establish necessary and appropriate procedures to
carry out such provisions.\16\
---------------------------------------------------------------------------
\16\ Sections 205(a), 702(a)(5), and 1631(d)(1).
---------------------------------------------------------------------------
How long would these proposed rules be effective?
If we publish these proposed rules as final rules, they will remain
in effect for five years after the date they become effective, unless
we extend them or revise and reissue them.
Clarity of These Proposed Rules
Executive Order 12866, as supplemented by Executive Order 13563,
requires each agency to write all rules in plain language. In addition
to your substantive comments on this NPRM, we invite your comments on
how to make them easier to understand.
For example:
Would more, but shorter, sections be better?
Are the requirements in the rules clearly stated?
Have we organized the material to suit your needs?
Could we improve clarity by adding tables, lists, or
diagrams?
What else could we do to make the rules easier to
understand?
Do the rules contain technical language or jargon that is
not clear?
Would a different format make the rules easier to
understand, (for example, grouping and order of sections, use of
headings, paragraphing)?
When will we start to use these rules?
We will not use these rules until we evaluate public comments and
publish final rules in the Federal Register. All final rules we issue
include an effective date. We will continue to use our current rules
until that date. If we publish final rules, we will include a summary
of relevant comments we received, our responses to them, and an
explanation of how we will apply the new rules.
Regulatory Procedures
Executive Order 12866, as Supplemented by Executive Order 13563
We consulted with the Office of Management and Budget (OMB) and
determined that these proposed rules meet the requirements for a
significant regulatory action under Executive Order 12866, as
supplemented by Executive Order 13563. Thus, OMB reviewed them.
Regulatory Flexibility Act
We certify that these proposed rules would not have a significant
economic impact on a substantial number of small entities because they
affect only individuals. Therefore, the Regulatory Flexibility Act, as
amended, does not require us to prepare a regulatory flexibility
analysis.
Paperwork Reduction Act
These proposed rules do not impose new or affect any existing
reporting or recordkeeping requirements and are not subject to OMB
clearance.
References
We consulted the following references when we developed these
proposed rules:
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Brousseau, DC, et al., Acute care utilization and rehospitalizations
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[[Page 69331]]
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Gevirtz, C., Pain management in patients with sickle cell disease,
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Harvard Medical School, Information Center for Sickle Cell and
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Josephson, C.D., et al., Transfusion in the patient with sickle cell
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Kilcoyne, R.F., et al., Evolution of imaging tests in hemophilia
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Kohne, T., et al., Newly diagnosed idiopathic thrombocytopenic
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Kruskall, M.S., The perils of platelet transfusions, New England
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at: http://asheducationbook.hematologylibrary.org/cgi/reprint/2005/1/416.pdf.
Matthew, H.M., et al., Allogeneic hematopoietic stem-cell
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McCarty, J.M., Transplant strategies for idiopathic myelofibrosis,
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McClish, D.K., et al., Gender differences in pain and healthcare
utilization for adult sickle cell patients: The PiSCES project,
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[[Page 69332]]
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We included these references in the rulemaking record for these
proposed rules and will make them available for inspection by
interested persons who make arrangements with the contact person
identified above.
(Catalog of Federal Domestic Assistance Program Nos. 96.001, Social
Security--Disability Insurance; 96.002, Social Security--Retirement
Insurance; 96.004, Social Security--Survivors Insurance; and 96.006,
Supplemental Security Income)
List of Subjects in 20 CFR Part 404
Administrative practice and procedure, Blind, Disability benefits,
Old-Age, Survivors, and Disability Insurance, Reporting and
recordkeeping requirements, Social Security.
Dated: November 8, 2013.
Carolyn W. Colvin,
Acting Commissioner of Social Security.
For the reasons set out in the preamble, we propose to amend 20 CFR
chapter III, part 404, subpart P as set forth below:
PART 404--FEDERAL OLD-AGE, SURVIVORS AND DISABILITY INSURANCE
(1950- )
0
1. The authority citation for subpart P of part 404 is revised to read
as follows:
Authority: Secs. 202, 205(a)-(b), and (d)-(h), 216(i), 221(a),
(i), and (j), 222(c), 223, 225, and 702(a)(5) of the Social Security
Act (42 U.S.C. 402, 405(a)-(b) and (d)-h), 416(i), 421(a), (i), and
(j), 422(c), 423, 425, and 902(a)(5)); sec. 211(b), Pub. L. 104-193,
110 Stat. 2105, 2189; sec. 202, Pub. L. 108-203, 118 Stat. 509 (42
U.S.C. 902 note).
Appendix 1 to Subpart P of Part 404--[Amended]
0
2. Amend appendix 1 to subpart P of part 404 by revising:
0
a. Item 8 of the introductory text before part A;
0
b. Section 7.00 of part A;
0
c. Section 13.00K2c(ii) of part A;
0
d. Second sentence of section 13.00K3 of part A; and
0
e. Section 107.00 of part B.
The revisions read as follows:
APPENDIX 1 TO SUBPART P OF PART 404--LISTING OF IMPAIRMENTS
* * * * *
8. Hematological Disorders (7.00 and 107.00): (Date 5 years from
the effective date of the final rules).
* * * * *
Part A
* * * * *
7.00 HEMATOLOGICAL DISORDERS
A. What hematological disorders do we evaluate under these
listings?
1. We evaluate non-malignant (non-cancerous) hematological
disorders, such as hemolytic anemias (7.05), disorders of hemostasis
(7.08), and disorders of bone marrow failure (7.10), which disrupt
the normal development and function of white blood cells, red blood
cells, platelets, and blood-clotting factors.
2. We evaluate malignant (cancerous) hematological disorders,
such as lymphoma, leukemia, and multiple myeloma, under the
appropriate listings in 13.00, except for lymphoma associated with
human immunodeficiency virus (HIV) infection, which we evaluate
under 14.08E.
B. What evidence do we need to document that you have a
hematological disorder? We need the following evidence to document
that you have a hematological disorder:
1. A laboratory report of a definitive test that establishes a
hematological disorder, signed by a physician; or
2. A laboratory report of a definitive test that establishes a
hematological disorder that is not signed by a physician and a
report from a physician that states you have the disorder; or
3. When we do not have a laboratory report of a definitive test,
a persuasive report from a physician that a positive diagnosis of
your hematological disorder was confirmed by appropriate laboratory
analysis or other diagnostic method(s). To be persuasive, this
report must state that you had the appropriate definitive laboratory
test or tests for diagnosing your disorder and provide the results,
or explain how your diagnosis was established by other diagnostic
method(s) consistent with the prevailing state of medical knowledge
and clinical practice.
4. We will make every reasonable effort to obtain the results of
appropriate laboratory testing you have had. We will not purchase
complex, costly, or invasive tests, such as tests of clotting
factors, bone marrow aspirations, or bone marrow biopsies.
C. What are hemolytic anemias, and how do we evaluate them under
7.05?
[[Page 69333]]
1. Hemolytic anemias include an array of disorders that result
in premature destruction of red blood cells (RBCs). The diagnosis of
hemolytic anemia is based on hemoglobin electrophoresis or analysis
of the contents of the RBC (hemoglobin, enzymes) and the envelope
(membrane) of the RBC. Sickle cell disease, thalassemia, and their
variants are some examples of hemolytic anemias.
2. The hospitalizations in 7.05B do not all have to be for the
same complication of the hemolytic anemia. They may be for three
different complications of the disorder. Examples of complications
of hemolytic anemia that may result in hospitalization include
osteomyelitis, painful (vaso-occlusive) crisis, pulmonary infections
or infarctions, acute chest syndrome, pulmonary hypertension,
chronic heart failure, gallbladder disease, hepatic (liver) failure,
renal (kidney) failure, nephrotic syndrome, aplastic crisis, and
cerebrovascular accident (stroke).
3. For 7.05C, we do not require hemoglobin to be measured during
a period in which you are free of pain or other symptoms of your
disorder. We will accept hemoglobin measurements made while you are
experiencing complications of your hemolytic anemia.
4. Transfusion-dependent in 7.05D refers to the most serious
type of beta thalassemia major, in which the bone marrow cannot
produce sufficient numbers of RBCs to maintain life. Transfusion
dependency requires life-long chronic treatment with RBC
transfusions at least once every 6 weeks. We exclude prophylactic
RBC transfusions for sickle cell disease (for example, to prevent
stroke) because we do not consider them to be of equal medical
significance to transfusion-dependent thalassemia.
D. What are disorders of hemostasis, and how do we evaluate them
under 7.08?
1. Disorders of hemostasis are characterized by abnormalities in
blood clotting and include both hypocoagulation (inadequate blood
clotting) and hypercoagulation (excessive blood clotting). The
diagnosis of a disorder of hemostasis is based on evaluation of
plasma clotting factors or platelets. Hemophilia, von Willebrand
disease, and thrombocytopenia are some examples of hypocoagulation
disorders. Protein C or protein S deficiency and Factor V Leiden are
examples of hypercoagulation disorders.
2. The hospitalizations in 7.08 do not all have to be for the
same complication of a disorder of hemostasis. They may be for three
different complications of the disorder. Examples of complications
that may result in hospitalization include uncontrolled bleeding
requiring multiple factor concentrate infusions or platelet
transfusions, anemia, thromboses, and embolisms. We will also
consider any surgery that you have to be a complication of your
disorder of hemostasis if you require treatment with factor
infusions or anticoagulant medication to control bleeding or
coagulation in connection with your surgery.
E. What are disorders of bone marrow failure, and how do we
evaluate them under 7.10?
1. Disorders of bone marrow failure are characterized by bone
marrow that does not make enough healthy RBCs, granulocytes
(specialized types of white blood cells), platelets, or a
combination of these cell types. The diagnosis is based on bone
marrow aspirations or bone marrow biopsies. Myelodysplastic
syndromes, aplastic anemia, granulocytopenia, and myelofibrosis are
some examples of disorders of bone marrow failure.
2. The hospitalizations in 7.10A do not all have to be for the
same complication of bone marrow failure. They may be for three
different complications of the disorder. Examples of complications
that may result in hospitalization include uncontrolled bleeding,
anemia, and systemic bacterial, viral, or fungal infections.
3. For 7.10B, transfusion-dependent for myelodysplastic
syndromes or aplastic anemias has the same meaning as it does for
beta thalassemia major. (See 7.00C4.)
F. How do we evaluate bone marrow or stem cell transplantation
under 7.17? We will consider you to be disabled for 12 months from
the date of bone marrow or stem cell transplantation, or we may
consider you to be disabled for a longer period if you are
experiencing any serious post-transplantation complications, such as
graft-versus-host (GVH) disease, frequent infections after
immunosuppressive therapy, or significant deterioration of organ
systems. We do not restrict our determination of the onset of
disability to the date of the transplantation in 7.17. We may
establish an earlier onset date of disability due to your
transplantation if evidence in your case record supports such a
finding.
G. How do we use the functional criteria in 7.18?
1. When we use the functional criteria in 7.18, we consider all
relevant information in your case record to determine the impact of
your hematological disorder on your ability to function
independently, appropriately, effectively, and on a sustained basis
in a work setting. Factors we will consider when we evaluate your
functioning under 7.18 include, but are not limited to: Your
symptoms, the frequency and duration of complications of your
hematological disorder, periods of exacerbation and remission, and
the functional impact of your treatment, including the side effects
of your medication.
2. Repeated complications means that the complications occur on
an average of three times a year, or once every 4 months, each
lasting 2 weeks or more; or the complications do not last for 2
weeks but occur substantially more frequently than three times in a
year or once every 4 months; or they occur less frequently than an
average of three times a year or once every 4 months but last
substantially longer than 2 weeks. Your impairment will satisfy this
criterion regardless of whether you have the same kind of
complication repeatedly, all different complications, or any other
combination of complications; for example, two of the same kind of
complication and a different one. You must have the required number
of complications with the frequency and duration required in this
section. Additionally, the complications must occur within the
period we are considering in connection with your application or
continuing disability review.
3. To satisfy the functional criteria in 7.18, your
hematological disorder must result in a ``marked'' level of
limitation in one of three general areas of functioning: Activities
of daily living, social functioning, or difficulties in completing
tasks due to deficiencies in concentration, persistence, or pace.
Functional limitation may result from the impact of the disease
process itself on your mental functioning, physical functioning, or
both your mental and physical functioning. This limitation could
result from persistent or intermittent symptoms, such as pain,
severe fatigue, or malaise, resulting in a limitation of your
ability to do a task, to concentrate, to persevere at a task, or to
perform the task at an acceptable rate of speed. (Severe fatigue
means a frequent sense of exhaustion that results in significant
reduced physical activity or mental function. Malaise means frequent
feelings of illness, bodily discomfort, or lack of well-being that
result in significantly reduced physical activity or mental
function.) You may also have limitations because of your treatment
and its side effects.
4. Marked limitation means that the symptoms and signs of your
hematological disorder interfere seriously with your ability to
function. Although we do not require the use of such a scale,
``marked'' would be the fourth point on a five-point scale
consisting of no limitation, mild limitation, moderate limitation,
marked limitation, and extreme limitation. We do not define
``marked'' by a specific number of different activities of daily
living or different behaviors in which your social functioning is
impaired, or a specific number of tasks that you are able to
complete, but by the nature and overall degree of interference with
your functioning. You may have a marked limitation when several
activities or functions are impaired, or even when only one is
impaired. Additionally, you need not be totally precluded from
performing an activity to have a marked limitation, as long as the
degree of limitation interferes seriously with your ability to
function independently, appropriately, and effectively. The term
``marked'' does not imply that you must be confined to bed,
hospitalized, or in a nursing home.
5. Activities of daily living include, but are not limited to,
such activities as doing household chores, grooming and hygiene,
using a post office, taking public transportation, or paying bills.
We will find that you have a ``marked'' limitation in activities of
daily living if you have a serious limitation in your ability to
maintain a household or take public transportation because of
symptoms such as pain, severe fatigue, anxiety, or difficulty
concentrating, caused by your hematological disorder (including
complications of the disorder) or its treatment, even if you are
able to perform some self-care activities.
6. Social functioning includes the capacity to interact with
others independently, appropriately, effectively, and on a sustained
basis. It includes the ability to communicate effectively with
others. We will find that you have a ``marked'' limitation in
maintaining
[[Page 69334]]
social functioning if you have a serious limitation in social
interaction on a sustained basis because of symptoms such as pain,
severe fatigue, anxiety, or difficulty concentrating, or a pattern
of exacerbation and remission, caused by your hematological disorder
(including complications of the disorder) or its treatment, even if
you are able to communicate with close friends or relatives.
7. Completing tasks in a timely manner involves the ability to
sustain concentration, persistence, or pace to permit timely
completion of tasks commonly found in work settings. We will find
that you have a ``marked'' limitation in completing tasks if you
have a serious limitation in your ability to sustain concentration
or pace adequate to complete work-related tasks because of symptoms,
such as pain, severe fatigue, anxiety, or difficulty concentrating
caused by your hematological disorder (including complications of
the disorder) or its treatment, even if you are able to do some
routine activities of daily living.
H. How do we consider your symptoms, including your pain, severe
fatigue, and malaise? Your symptoms, including pain, severe fatigue,
and malaise, may be important factors in our determination whether
your hematological disorder(s) meets or medically equals a listing,
or in our determination whether you are otherwise able to work. We
cannot consider your symptoms unless you have medical signs or
laboratory findings showing the existence of a medically
determinable impairment(s) that could reasonably be expected to
produce the symptoms. If you have such an impairment(s), we will
evaluate the intensity, persistence, and functional effects of your
symptoms using the rules throughout 7.00 and in our other
regulations. (See Sec. Sec. 404.1528, 404.1529, 416.928, and
416.929 of this chapter.) Additionally, when we assess the
credibility of your complaints about your symptoms and their
functional effects, we will not draw any inferences from the fact
that you do not receive treatment or that you are not following
treatment without considering all of the relevant evidence in your
case record, including any explanations you provide that may explain
why you are not receiving or following treatment.
I. How do we evaluate episodic events in hematological
disorders? Some of the listings in this body system require a
specific number of events within a consecutive 12-month period. (See
7.05, 7.08, and 7.10A.) When we use such criteria, the 12-month
period must occur within the period we are considering in connection
with your application or continuing disability review.
J. How do we evaluate hematological disorders that do not meet
one of these listings?
1. These listings are only examples of common hematological
disorders that we consider severe enough to prevent a person from
doing any gainful activity. If your disorder does not meet the
criteria of any of these listings, we must consider whether you have
a disorder that satisfies the criteria of a listing in another body
system. For example, we will evaluate hemophilic joint deformity or
bone or joint pain from myelofibrosis under 1.00; polycythemia vera
under 3.00, 4.00, or 11.00; chronic iron overload resulting from
repeated RBC transfusion (transfusion hemosiderosis) under 3.00,
4.00, or 5.00; and the effects of intracranial bleeding under 11.00
or 12.00.
2. If you have a severe medically determinable impairment(s)
that does not meet a listing, we will determine whether your
impairment(s) medically equals a listing. (See Sec. Sec. 404.1526
and 416.926 of this chapter.) Hematological disorders may be
associated with disorders in other body systems, and we consider the
combined effects of multiple impairments when we determine whether
they medically equal a listing. If your impairment(s) does not
medically equal a listing, you may or may not have the residual
functional capacity to engage in substantial gainful activity. We
proceed to the fourth, and, if necessary, the fifth steps of the
sequential evaluation process in Sec. Sec. 404.1520 and 416.920. We
use the rules in Sec. Sec. 404.1594, 416.994, and 416.994a of this
chapter, as appropriate, when we decide whether you continue to be
disabled.
7.01 Category of Impairments, Hematological Disorders
7.05 Hemolytic anemias (including sickle cell disease,
thalassemia, and their variants) (see 7.00C), with:
A. Documented painful (vaso-occlusive) crises requiring
parenteral (intravenous or intramuscular) narcotic medication,
occurring at least six times within a 12-month period with at least
30 days between crises.
OR
B. Complications of hemolytic anemia requiring at least three
hospitalizations within a 12-month period and occurring at least 30
days apart. Each hospitalization must last at least 48 hours, which
can include hours in a hospital emergency department immediately
before the hospitalization. (See 7.00C2).
OR
C. Hemoglobin measurements of 7.0 grams per deciliter (g/dL) or
less, occurring at least three times within a 12-month period with
at least 30 days between measurements.
OR
D. Transfusion-dependent beta thalassemia major (see 7.00C4).
7.08 Disorders of hemostasis (including hemophilia and
thrombocytopenia) (see 7.00D), with complications requiring at least
three hospitalizations within a 12-month period and occurring at
least 30 days apart. Each hospitalization must last at least 48
hours, which can include hours in a hospital emergency department
immediately before the hospitalization. (See 7.00D2.)
7.10 Disorders of bone marrow failure (including
myeloproliferative syndrome, aplastic anemia, and granulocytopenia)
(see 7.00E), with:
A. Complications of bone marrow failure requiring at least three
hospitalizations within a 12-month period and occurring at least 30
days apart. Each hospitalization must last at least 48 hours, which
can include hours in a hospital emergency department immediately
before the hospitalization. (See 7.00E2.)
OR
B. Transfusion-dependent myelodysplastic syndromes or aplastic
anemias (see 7.00C4).
7.17 Hematological disorders treated by bone marrow or stem cell
transplantation (see 7.00F). Consider under a disability for at
least 12 months from the date of transplantation. After that,
evaluate any residual impairment(s) under the criteria for the
affected body system.
7.18 Repeated complications of hematological disorders (see
7.00G2), including those complications listed in 7.05, 7.08, and
7.10 but without the requisite findings for those listings, or other
complications (for example, anemia, osteonecrosis, retinopathy, skin
ulcers, silent central nervous system infarction, cognitive or other
mental limitation, or limitation of joint movement), resulting in
significant, documented symptoms or signs (for example, pain, severe
fatigue, malaise, fever, night sweats, headaches, joint or muscle
swelling, or shortness of breath), and one of the following at the
marked level (see 7.00G4):
A. Limitation of activities of daily living (see 7.00G5).
B. Limitation in maintaining social functioning (see 7.00G6).
C. Limitation in completing tasks in a timely manner due to
deficiencies in concentration, persistence, or pace (see 7.00G7).
* * * * *
13.00 Malignant Neoplastic Diseases
* * * * *
K. How do we evaluate specific malignant neoplastic diseases?
* * * * *
2. Leukemia.
* * * * *
c. Chronic lymphocytic leukemia.
* * * * *
ii. We evaluate the complications and residual impairment(s)
from chronic lymphocytic leukemia (CLL) under the appropriate
listings, such as 13.05A2 or an appropriate listing in 7.00.
* * * * *
3. Macroglobulinemia or heavy chain disease. * * * We evaluate
the resulting impairment(s) under the criteria of 7.00 or any other
affected body system.
* * * * *
Part B
* * * * *
107.00 HEMATOLOGICAL DISORDERS
A. What hematological disorders do we evaluate under these
listings?
1. We evaluate non-malignant (non-cancerous) hematological
disorders, such as hemolytic anemias (107.05), disorders of
hemostasis (107.08), and disorders of bone marrow failure (107.10),
which disrupt the normal development and function of white blood
cells, red blood cells, platelets, and blood-clotting factors.
2. We evaluate malignant (cancerous) hematological disorders,
such as lymphoma, leukemia, and multiple myeloma under the
appropriate listings in 113.00, except for lymphoma associated with
human
[[Page 69335]]
immunodeficiency virus (HIV) infection, which we evaluate under
114.08E.
B. What evidence do we need to document that you have a
hematological disorder? We need the following evidence to document
that you have a hematological disorder:
1. A laboratory report of a definitive test that establishes a
hematological disorder, signed by a physician; or
2. A laboratory report of a definitive test that establishes a
hematological disorder that is not signed by a physician and a
report from a physician that states you have the disorder; or
3. When we do not have a laboratory report of a definitive test,
a persuasive report from a physician that a positive diagnosis of
your hematological disorder was confirmed by appropriate laboratory
analysis or other diagnostic method(s). To be persuasive, this
report must state that you had the appropriate definitive laboratory
test or tests for diagnosing your disorder and provide the results,
or explain how your diagnosis was established by other diagnostic
method(s) consistent with the prevailing state of medical knowledge
and clinical practice.
4. We will make every reasonable effort to obtain the results of
appropriate laboratory testing you have had. We will not purchase
complex, costly, or invasive tests, such as tests of clotting
factors, bone marrow aspirations, or bone marrow biopsies.
C. What are hemolytic anemias, and how do we evaluate them under
107.05?
1. Hemolytic anemias include an array of disorders that result
in premature destruction of red blood cells (RBCs). The diagnosis of
hemolytic anemia is based on hemoglobin electrophoresis or analysis
of the contents of the RBC (hemoglobin, enzymes) and the envelope
(membrane) of the RBC. Sickle cell disease, thalassemia, and their
variants are some examples of hemolytic anemias.
2. The hospitalizations in 107.05B do not all have to be for the
same complication of the hemolytic anemia. They may be for three
different complications of the disorder. Examples of complications
of hemolytic anemia that may result in hospitalization include
dactylitis, osteomyelitis, painful (vaso-occlusive) crisis,
pulmonary infections or infarctions, acute chest syndrome, pulmonary
hypertension, chronic heart failure, gallbladder disease, hepatic
(liver) failure, renal (kidney) failure, nephrotic syndrome,
aplastic crisis, and cerebrovascular accident (stroke).
3. For 107.05C, we do not require hemoglobin to be measured
during a period in which you are free of pain or other symptoms of
your disorder. We will accept hemoglobin measurements made while you
are experiencing complications of your hemolytic anemia.
4. Transfusion-dependent in 107.05D refers to the most serious
type of beta thalassemia major, in which the bone marrow cannot
produce sufficient numbers of RBCs to maintain life. Transfusion
dependency requires life-long chronic treatment with RBC
transfusions at least once every 6 weeks. We exclude prophylactic
RBC transfusions for sickle cell disease (for example, to prevent
stroke) because we do not consider them to be of equal medical
significance to transfusion-dependent thalassemia.
D. What are disorders of hemostasis, and how do we evaluate them
under 107.08?
1. Disorders of hemostasis are characterized by abnormalities in
blood clotting and include both hypocoagulation (inadequate blood
clotting) and hypercoagulation (excessive blood clotting). The
diagnosis of a disorder of hemostasis is based on evaluation of
plasma clotting factors or platelets. Hemophilia, von Willebrand
disease, and thrombocytopenia are some examples of hypocoagulation
disorders. Protein C or protein S deficiency and Factor V Leiden are
examples of hypercoagulation disorders.
2. The hospitalizations in 107.08 do not all have to be for the
same complication of a disorder of hemostasis. They may be for three
different complications of the disorder. Examples of complications
that may result in hospitalization include uncontrolled bleeding
requiring multiple factor concentrate infusions or platelet
transfusions, anemia, thromboses, and embolisms. We will also
consider any surgery that you have to be a complication of your
disorder of hemostasis if you require treatment with factor
infusions or anticoagulant medication to control bleeding or
coagulation in connection with your surgery.
E. What are disorders of bone marrow failure, and how do we
evaluate them under 107.10?
1. Disorders of bone marrow failure are characterized by bone
marrow that does not make enough healthy RBCs, granulocytes
(specialized types of white blood cells), platelets, or a
combination of these cell types. The diagnosis is based on bone
marrow aspirations or bone marrow biopsies. Myelodysplastic
syndromes, aplastic anemia, granulocytopenia, and myelofibrosis are
some examples of disorders of bone marrow failure.
2. The hospitalizations in 107.10A do not all have to be for the
same complication of bone marrow failure. They may be for three
different complications of the disorder. Examples of complications
that may result in hospitalization include uncontrolled bleeding,
anemia, and systemic bacterial, viral, or fungal infections.
3. For 107.10B, transfusion-dependent for myelodysplastic
syndromes or aplastic anemias has the same meaning as it does for
beta thalassemia major. (See 107.00C4.)
F. How do we evaluate bone marrow or stem cell transplantation
under 107.17? We will consider you to be disabled for 12 months from
the date of bone marrow or stem cell transplantation, or we may
consider you to be disabled for a longer period if you are
experiencing any serious post-transplantation complications, such as
graft-versus-host (GVH) disease, frequent infections after
immunosuppressive therapy, or significant deterioration of organ
systems. We do not restrict our determination of the onset of
disability to the date of the transplantation in 107.17. We may
establish an earlier onset of disability due to your transplantation
if evidence in your case record supports such a finding.
G. How do we consider your symptoms, including your pain, severe
fatigue, and malaise? Your symptoms, including pain, severe fatigue,
and malaise, may be important factors in our determination whether
your hematological disorder meets or medically equals a listing, or
in our determination whether you otherwise have marked and severe
functional limitations. We cannot consider your symptoms unless you
have medical signs or laboratory findings showing the existence of a
medically determinable impairment(s) that could reasonably be
expected to produce the symptoms. If you have such an impairment(s),
we will evaluate the intensity, persistence, and functional effects
of your symptoms using the rules throughout 107.00 and in our other
regulations. (See Sec. Sec. 416.928 and 416.929 of this chapter.)
Additionally, when we assess the credibility of your complaints
about your symptoms and their functional effects, we will not draw
any inferences from the fact that you do not receive treatment or
that you are not following treatment without considering all of the
relevant evidence in your case record, including any explanations
you provide that may explain why you are not receiving or following
treatment.
H. How do we evaluate episodic events in hematological
disorders? Some of the listings in this body system require a
specific number of events within a consecutive 12-month period. (See
107.05, 107.08, and 107.10A.) When we use such criteria, the 12-
month period must occur within the period we are considering in
connection with your application or continuing disability review.
I. How do we evaluate hematological disorders that do not meet
one of these listings?
1. These listings are only examples of common hematological
disorders that we consider severe enough to result in marked and
severe functional limitations. If your disorder does not meet the
criteria of any of these listings, we must consider whether you have
a disorder that satisfies the criteria of a listing in another body
system. For example, we will evaluate hemophilic joint deformity
under 101.00; polycythemia vera under 103.00, 104.00, or 111.00;
chronic iron overload resulting from repeated RBC transfusion
(transfusion hemosiderosis) under 103.00, 104.00, or 105.00; and the
effects of intracranial bleeding under 111.00 or 112.00.
2. If you have a severe medically determinable impairment(s)
that does not meet a listing, we will determine whether your
impairment(s) medically equals a listing. (See Sec. 416.926 of this
chapter.) Hematological disorders may be associated with disorders
in other body systems, and we consider the combined effects of
multiple impairments when we determine whether they medically equal
a listing. If your impairment(s) does not medically equal a listing,
we will also consider whether it functionally equals the listings.
(See Sec. 416.926a of this chapter.) We use the rules in Sec.
416.994a of this chapter when we decide whether you continue to be
disabled.
107.01 Category of Impairments, Hematological Disorders
107.05 Hemolytic anemias (including sickle cell disease,
thalassemia, and their variants) (see 107.00C), with:
A. Documented painful (vaso-occlusive) crises requiring
parenteral (intravenous or
[[Page 69336]]
intramuscular) narcotic medication, occurring at least six times
within a 12-month period with at least 30 days between crises.
OR
B. Complications of hemolytic anemia requiring at least three
hospitalizations within a 12-month period and occurring at least 30
days apart. Each hospitalization must last at least 48 hours, which
can include hours in a hospital emergency department immediately
before the hospitalization. (See 107.00C2.)
OR
C. Hemoglobin measurements of 7.0 grams per deciliter (g/dL) or
less, occurring at least three times within a 12-month period with
at least 30 days between measurements.
OR
D. Transfusion-dependent beta thalassemia major (see 107.00C4).
107.08 Disorders of hemostasis (including hemophilia and
thrombocytopenia) (see 107.00D), with complications requiring at
least three hospitalizations within a 12-month period and occurring
at least 30 days apart. Each hospitalization must last at least 48
hours, which can include hours in a hospital emergency department
immediately before the hospitalization. (See 107.00D2.)
107.10 Disorders of bone marrow failure (including
myeloproliferative syndrome, aplastic anemia, and granulocytopenia)
(see 107.00E), with:
A. Complications of bone marrow failure requiring at least three
hospitalizations within a 12-month period and occurring at least 30
days apart. Each hospitalization must last at least 48 hours, which
can include hours in a hospital emergency department immediately
before the hospitalization. (See 107.00E2.)
OR
B. Transfusion-dependent myelodysplastic syndromes or aplastic
anemias (see 107.00C4).
107.17 Hematological disorders treated by bone marrow or stem
cell transplantation (see 107.00F). Consider under a disability for
at least 12 months from the date of transplantation. After that,
evaluate any residual impairment(s) under the criteria for the
affected body system.
* * * * *
`[FR Doc. 2013-27514 Filed 11-18-13; 8:45 am]
BILLING CODE 4191-02-P