[Federal Register Volume 82, Number 178 (Friday, September 15, 2017)]
[Pages 43442-43445]
From the Federal Register Online via the Government Publishing Office [www.gpo.gov]
[FR Doc No: 2017-19551]

[[Page 43442]]



[Docket No. SSA-2017-0007]

Social Security Ruling, SSR 17-3p; Titles II and XVI: Evaluating 
Cases Involving Sickle Cell Disease (SCD)

AGENCY: Social Security Administration.

ACTION: Notice of Social Security Ruling (SSR).


SUMMARY: We are providing notice of SSR 17-3p. This SSR provides 
guidance on SCD and how we evaluate SCD in disability claims under 
titles II and XVI of the Social Security Act.

DATES: This SSR is applicable on September 15, 2017.

Disability Policy, 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: Although 5 U.S.C. 552(a)(1) and (a)(2) do 
not require us to publish this SSR, we are doing so in accordance with 
20 CFR 402.35(b)(1).
    Through SSRs, we make available to the public precedential 
decisions relating to the Federal old-age, survivors, disability, 
supplemental security income, and special veterans' benefits programs. 
We may base SSRs on determinations or decisions made at all levels of 
administrative adjudication, Federal court decisions, Commissioner's 
decisions, opinions of the Office of the General Counsel, or other 
interpretations of the law and regulations.
    Although SSRs do not have the same force and effect as statutes or 
regulations, they are binding on all components of the Social Security 
Administration. 20 CFR 402.35(b)(1).
    This SSR will remain in effect until we publish a notice in the 
Federal Register that rescinds it, or until we publish a new SSR that 
replaces or modifies it.

(Catalog of Federal Domestic Assistance, Programs Nos. 96.001, 
Social Security--Disability Insurance; 96.002, Social Security--
Retirement Insurance; 96.004, Social Security--Survivors Insurance; 
96.006--Supplemental Security Income.)

Nancy A. Berryhill,
Acting Commissioner of Social Security.

Policy Interpretation Ruling

Titles II and XVI: Evaluating Cases Involving Sickle Cell Disease (SCD)

    Purpose: This Social Security Ruling (SSR) provides background 
information on SCD and how we evaluate SCD during our adjudication 
process. We provide this guidance to help adjudicators consistently 
apply our policies in disability claims involving SCD.
    Citations: Sections 216(i), 223(d), 223(f), 1614(a)(3) and 
1614(a)(4) of the Social Security Act, as amended; Regulations No. 4, 
subpart P, sections 404.1502, 404.1505, 404.1509, 404.1512, 404.1513, 
404.1520, 404.1520a, 404.1520b, 404.1521, 404.1522, 404.1523, 404.1525, 
404.1526, 404.1529, 404.1545, 404.1560-404.1569a, 404.1593, 404.1594, 
appendices 1 and 2; and Regulations No. 16, subpart I, sections 
416.902, 416.905, 416.906, 416.909, 416.911, 416.912, 416.913, 416.920, 
416.920a, 416.920b, 416.921, 416.922, 416.923, 416.924, 416.924a, 
416.925, 416.926, 416.926a, 416.929, 416.945, 416.960-416.969a, 
416.987, 416.993, 416.994, and 416.994a.


    SCD is the most common inherited blood disease in the United 
States, affecting an estimated 100,000 Americans.\1\ SCD is not always 
easy to evaluate due to its varying nature and complications. In this 
SSR, we provide basic information about SCD and its variants and 
clarify that sickle cell trait is not a variant of SCD. We also provide 
guidance for assessing SCD under the hematological disorder listings 
and determining how this impairment may affect the residual functional 
capacity finding for adults and the functional equivalence finding for 

    \1\ See Centers for Disease Control and Prevention, ``Sickle 
Cell Disease.'' (https://www.cdc.gov/ncbddd/sicklecell/data.html.)

Policy Interpretation

    We consider all medical evidence when we evaluate a claim for 
disability benefits. The following information is in a question and 
answer format that provides guidance about SCD and how to consider 
evidence regarding this impairment. Questions 1 and 2 provide basic 
background information about SCD and its variants. Question 3 clarifies 
that sickle cell trait is not a variant of SCD. Question 4 discusses 
the complications and symptoms of SCD. Questions 5 through 7 explain 
how adjudicators should evaluate SCD at various points of the 
adjudication process, including the adult and child hematological 
listings we consider.

List of Questions

    1. What is SCD?
    2. What are the different variants of SCD?
    3. Is sickle cell trait a variant of SCD?
    4. What are the common complications and symptoms of SCD?
    5. How do we evaluate the complications of SCD under the 
hematological disorder listings?
    6. How do we evaluate the complications of SCD when assessing 
residual functional capacity (RFC) for adults?
    7. How do we evaluate the complications of SCD under functional 
equivalence for children?


1. What is SCD?
    SCD is a type of hemolytic anemia and an inherited hematological 
disorder that affects the hemoglobin within a person's red blood cells 
(RBC). Hemoglobin is the protein within RBC that carries oxygen. The 
abnormal hemoglobin makes the RBC more prone to distortion 
(``sickling''), which results in blocked blood vessels and a shortened 
RBC lifespan. Hemolytic anemia results when the abnormal RBC are 
destroyed faster than the body can produce them.
    When hemoglobin is normal, a person's RBC are round and easily 
travel through blood vessels, bringing oxygen to the body's organs and 
tissues. SCD causes sickle-shaped RBC that are not flexible and can 
stick to vessel walls, causing blockages (vaso-occlusion) that slow or 
stop the flow of blood and oxygen. This blockage may in turn cause 
pain. Persons with SCD are predisposed to pain, infection, and other 
complications. Because people inherit SCD, the disease is present at 
birth, but the age when children display symptoms varies.\2\

    \2\ See National Heart, Lung, and Blood Institute, ``What Are 
the Signs and Symptoms of Sickle Cell Disease?'' (https://www.nhlbi.nih.gov/health/health-topics/topics/sca). Health problems 
usually do not appear until an infant is around 5 to 6 months of 

2. What are the different variants of SCD?
    The different variants of SCD may indicate the severity of 
complications and the resulting functional limitations caused by SCD. 
Laboratory blood tests such as hemoglobin electrophoresis establish the 
existence and the variants

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of SCD. The following are the most common variants of SCD: \3\

    \3\ See Centers for Disease Control and Prevention, ``Sickle 
Cell Disease.'' (https://www.cdc.gov/ncbddd/sicklecell/facts.html).

     Hemoglobin (Hb) SS (HbSS)--a person with this form of SCD 
inherits one sickle cell gene from each parent. HbSS is the most common 
and usually most severe form of SCD.
     HbSC--a person inherits one sickle cell gene from one 
parent, and another gene for an abnormal hemoglobin called ``C'' from 
the other parent. HbSC is usually a milder type of SCD.
     Hb S-beta (S[beta]) thalassemia-- a person inherits one 
sickle cell gene from one parent, and a gene for beta thalassemia from 
the other parent. There are two forms of beta thalassemia, sickle beta 
zero thalassemia (Hb S[beta]\0\ thalassemia) and sickle beta plus 
thalassemia (Hb S[beta]\+\ thalassemia). Sickle beta zero thalassemia 
is usually a more severe form of SCD. People with sickle beta plus 
thalassemia tend to have a milder form of SCD.
     HbSD, HbSE, and HbSO--people with these variants of SCD 
have one sickle cell gene plus another abnormal hemoglobin gene, ``D,'' 
``E,'' or ``O.'' These are rarer types of SCD with varying severity.
3. Is sickle cell trait a variant of SCD?
    No. Sickle cell trait is not a variant of SCD. Sickle cell trait 
occurs when a person inherits one sickle hemoglobin gene from one 
parent and a normal gene from the other parent. People with sickle cell 
trait rarely have signs and symptoms associated with SCD and usually do 
not need treatment. However, in rare cases and under extreme conditions 
such as intense exercise, people with sickle cell trait have a higher 
risk of severe breakdown of muscle tissue (exertional rhabdomyolysis) 
that can lead to serious complications.\4\ In spite of this higher 
risk, recent evidence indicates that sickle cell trait is not 
associated with an increased probability of death.\5\

    \4\ Other conditions that could be harmful for people with 
sickle cell trait include high altitudes, dehydration, low oxygen 
levels in the air, and increased pressure in the atmosphere. We 
evaluate impairments that result from sickle cell trait under the 
affected body system.
    \5\ See Nelson D.A., et al. Sickle Cell Trait, Rhabdomyolysis, 
and Mortality among U.S. Army Soldiers. New England Journal of 
Medicine, Aug; 375(17), 1695-6 (2016).

    Sickle cell trait alone is not an impairment. As defined by the 
Social Security Act, an impairment must result from anatomical, 
physiological, or psychological abnormalities that can be shown by 
medically acceptable clinical and laboratory diagnostic techniques. To 
establish an impairment in this context, we require objective medical 
evidence (medical signs and laboratory findings) from an acceptable 
medical source of complications from sickle cell trait. In addition, a 
person's complications from sickle cell trait must meet the statutory 
duration requirement, i.e., be expected to result in death or last or 
be expected to last for a continuous period of not less than 12 months. 
Therefore, we cannot find a person disabled due to sickle cell trait if 
there are no medical signs or laboratory findings of complications from 
sickle cell trait and the complications from sickle cell trait do not 
meet the duration requirement.
4. What are the common complications and symptoms of SCD?
    Complications of SCD may include, but are not limited to pain 
crises, anemia, osteomyelitis, leg ulcers, pulmonary infections or 
infarctions, acute chest syndrome, pulmonary hypertension, chronic 
heart failure, gallbladder disease, liver failure, kidney failure, 
nephritic syndrome, aplastic crisis, stroke, and mental impairments 
such as depression. Examples of symptoms that may stem from these 
complications include pain, fatigue, malaise, shortness of breath, and 
difficulty feeding in infants. The symptoms of SCD vary from person to 
person and can change over time.
    A. Pain (vaso-occlusive) crisis is a common complication of SCD. 
Pain crises are either acute or chronic. Acute pain crises occur 
suddenly when sickled RBC stop blood flow and reduce oxygen delivery. 
This pain can be intense, stabbing, or throbbing. Pain can strike 
almost anywhere in the body and in more than one spot at a time. The 
pain often occurs in the lower back, legs, arms, abdomen, and chest.\6\ 
Chronic pain in SCD is more than a continuation of acute pain crisis. 
It usually occurs when lack of oxygen to the bone due to vaso-occlusion 
results in the death of bone tissue (avascular necrosis) at various 
joints such as the hips, shoulders and ankles.\7\

    \6\ See National Heart, Lung, and Blood Institute, ``What Are 
the Signs and Symptoms of Sickle Cell Disease?'' (http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs).
    \7\ See Okpala I, Tawil A. Management of Pain in Sickle-Cell 
Disease. Journal of the Royal Society of Medicine, Sep; 95(9), 456-
458, 2002 (available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1279994/).

    B. Anemia is another complication of SCD. It occurs when sickled 
RBC die prematurely, which reduces the amount of oxygen-carrying 
hemoglobin in the blood. Symptoms from anemia can include fatigue, 
weakness, shortness of breath, and dizziness. Chronic deprivation of 
oxygen-rich blood can damage nerves and organs in the body, including 
the spleen, brain, eyes, joints, bones, lungs, liver, heart, kidneys, 
and other organs.
    C. Pulmonary complications such as acute chest syndrome (ACS) and 
pulmonary hypertension are the leading cause of death for SCD 
patients.\8\ ACS is a vaso-occlusion of the pulmonary vessels. Symptoms 
of ACS include but are not limited to chest pain, fever, tachypnea 
(abnormally rapid breathing), wheezing, or coughing. Pulmonary 
hypertension can occur when sickled RBC cause pulmonary arteries to 
become narrow and blocked. The result of this damage to the pulmonary 
arteries is high blood pressure in the lungs. Symptoms of pulmonary 
hypertension include shortness of breath, fatigue, and chest pain.\9\

    \8\ See Gladwin MT, Miller, A. Pulmonary Complications of Sickle 
Cell Disease. American Journal of Respiratory and Critical Care 
Medicine, Jun; 185(11), 1154-1165, 2012 (available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3373067/).
    \9\ See National Institutes of Health. MedlinePlus. ``Pulmonary 
Hypertension.'' (https://medlineplus.gov/pulmonaryhypertension.html).

    D. Strokes and silent strokes affect people with SCD at a higher 
rate because sickled RBC clump along the walls of larger arteries going 
to the brain. Strokes can result in full or partial paralysis on one 
side of the body, problems with balance, or difficulty speaking or 
understanding. Silent strokes can occur without outward symptoms and 
are only detectable by brain imaging. However, silent strokes can 
impair intellectual ability, attention, visual-spatial skills, 
language, and long-term memory.\10\

    \10\ See, The Internet Stroke Center, ``Stroke as a Complication 
of Sickle Cell Disease.'' (http://www.strokecenter.org/patients/about-stroke/pediatric-stroke/stroke-as-a-complication-of-sickle-cell-disease/).

    E. Bacterial infections are often severe complications in people 
with SCD. Anemia from SCD and vaso-occlusions can damage the spleen, 
which ultimately increases risk of infection and damages other organs. 
Infection frequently leads to hospitalization and is the primary cause 
of death in young children with SCD.\11\

    \11\ See Booth, C., et al. Infection in Sickle Cell Disease: A 
Review. International Journal of Infectious Diseases, Jan; 14(1), 
e2-e12, 2010 (available at: http://www.sciencedirect.com/science/article/pii/S1201971209001453).

    F. Mental disorders in people with SCD are often secondary to the 
impact of treatment, pain, and other symptoms. For example, depression 
from reoccurring pain is especially common

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in people with SCD.\12\ Other mental disorders that may occur include, 
but are not limited to, anxiety and cognitive disorders from 

    \12\ See Jonassaint CR, Jones VL, Leong S, Frierson GM. A 
Systematic Review of the Association between Depression and Health 
Care Utilization in Children and Adults with Sickle Cell Disease. 
British Journal of Hematology, Jul; 174(1), 136-47, 2016.
    \13\ Becker M, Axelrod DJ. Hematologic Problems in Psychosomatic 
Medicine. Psychiatric Clinics of North America, Dec; 30(4), 739-759, 
2007 (available at: http://www.sciencedirect.com/science/article/pii/S0193953X07000767).

5. How do we evaluate the complications of SCD under the hematological 
disorder listings?
    We may evaluate SCD under the following hematological disorder 
     Listing 7.05 and 107.05, Hemolytic anemias; or
     Listing 7.17 and 107.17, Hematological disorders treated 
by bone marrow or stem cell transplantation; or
     Listing 7.18, Repeated complications of hematological 
    Under listing 7.05 and 107.05, we assess hemolytic anemias, 
including sickle cell disease, thalassemia, and their variants. We 
evaluate pain crises caused by SCD under listings 7.05A and 107.05A. We 
assess complications of SCD requiring hospitalizations under listings 
7.05B and 107.05B. Listings 7.05C and 107.05C describes the criteria we 
use to evaluate SCD that results in anemia with low hemoglobin levels.
    Under listings 7.17 and 107.17, we consider people who receive bone 
marrow or stem cell transplantation to treat their SCD, to be disabled 
for at least 12 months after the date of transplant.
    We evaluate adults who have repeated complications from SCD, but do 
not have the requisite findings for listing 7.05 or 7.17, under listing 
7.18.\14\ To meet listing 7.18, SCD must cause repeated complications, 
resulting in significant, documented symptoms or signs and a ``marked'' 
level of limitation in one of the general areas of functioning: 
Activities of daily living, social functioning, or completing tasks 
because of deficiencies in concentration, persistence, or pace. We use 
listing 7.18 to evaluate only hematological disorders.\15\

    \14\ We evaluate a child's functioning under the rules for 
functional equivalence. See 20 CFR 416.926a.
    \15\ We use listing 7.18 to evaluate hematological disorders and 
complications caused by hematological disorders. We can only 
evaluate anemia under 7.18 if it results from an underlying 
hematological disorder. If the person's anemia results from a 
condition that is not a hematological disorder, we would evaluate 
the anemia under the listing for that impairment.

    If a person's SCD does not meet a hematological listing, we will 
compare the specific findings in each case to any appropriate 
hematological listings to determine whether medical equivalence may 
exist. We may also find medical equivalence if the person has multiple 
impairments, including SCD, none of which meet or medically equal the 
requirements of a listing alone, but the combination of impairments is 
medically equivalent in severity to a listed impairment.
    If the person's SCD does not meet or equal the criteria in a 
listing, we will consider whether he or she has an impairment that 
satisfies the criteria in a listing in another body system. For 
example, we may evaluate the effects of intracranial bleeding or stroke 
under 11.00 or 12.00.
6. How do we evaluate the complications of SCD when assessing residual 
functional capacity (RFC) for adults?
    For adults, we assess RFC when the effects of a person's SCD, 
either alone or in combination with another impairment(s), do not meet 
or medically equal a listing. We base the RFC assessment on all the 
relevant evidence in the record, including the effects of 
treatment.\16\ In assessing RFC, we must consider all of a person's 
work-related limitations, whether due to SCD, other impairment(s), or a 
combination of impairments. For example, adults with SCD may have pain, 
fatigue, and shortness of breath that may affect their ability to stand 
and walk. In addition, a person experiencing repeated acute pain crises 
may have difficulty maintaining concentration to complete tasks and 
have frequent absences from work.

    \16\ See 20 CFR 404.1545 and 416.945, and SSR 96-8p.

7. How do we evaluate the complications of SCD under functional 
equivalence? \17\

    \17\ Functional equivalence applies only to claims for children 
under title XVI. All claims for title II, even if the claimant is 
under age 18, are decided under the adult rules.

    Children with SCD that does not meet or medically equal a listing 
may nevertheless have an impairment(s) that functionally equals the 
listings under our rules for evaluating disability in children.\18\ 
When we determine whether a child's impairment(s) functionally equal 
the listings, we use the six domains of functioning.

    \18\ See 20 CFR 416.926a, SSR 09-1p, 74 FR 7527 (2009) also 
available at https://www.ssa.gov/OP_Home/rulings/ssi/02/SSR2009-01-ssi-02.html, and SSR 09-2p, 74 FR 7525 (2009) also available at 
For the complete titles of all SSRs cited in this footnote and those 
following, see the CROSS-REFERENCES section at the end of this SSR.

    When we evaluate a child's functioning in these six domains, we 
consider how the child functions compared to children the same age who 
do not have impairments. We must explain any limitation in a child's 
ability to function appropriately for his or her age based on a 
medically determinable impairment(s).\19\ It is important to remember 
that the cumulative physical effects of SCD and its treatment can vary 
in kind and intensity, affecting each child differently. The six 
domains of functioning are:

    \19\ See 20 CFR 416.924a(b) and 416.926a.

    Acquiring and using information. Some children with SCD may have 
limitations in acquiring and using information due to stroke, including 
silent stroke.\20\ A stroke can cause brain injury that impairs a 
child's ability to learn, concentrate, speak, and remember.

    \20\ See 20 CFR 416.926a(g) and SSR 09-3p.

    Attending and completing tasks. Frequent pain crises can result in 
limitations in attending and completing tasks at school and at 
home.\21\ If a child does not feel well due to pain, it may be 
difficult for him or her to stay focused on activities long enough to 
complete them in an age-appropriate manner. A child with SCD who is 
experiencing pain may also have difficulty paying attention to details 
and may make mistakes on schoolwork due to an inability to concentrate.

    \21\ See 20 CFR 416.926a(h) and SSR 09-4p.

    Interacting and relating with others. SCD can also cause 
limitations interacting and relating with others.\22\ The unpredictable 
nature of pain in SCD may cause anxiety and difficulty maintaining 
relationships. Children suffering from complications of SCD may become 
withdrawn, uncooperative, or unresponsive.

    \22\ See 20 CFR 416.926a(i) and SSR 09-5p.

    Moving about and manipulating objects. If SCD limits a child's 
ability to move and manipulate objects, we evaluate those effects in 
the domain of ``Moving about and manipulating objects.'' \23\ For 
example, sickling in the hip bones, knees, and ankles due to SCD may 
cause joint pain and problems with walking, running, and climbing up 
and down stairs.

    \23\ See 20 CFR 416.926a(j) and SSR 09-6p.

    Caring for yourself. Caring for yourself involves a child's basic 
understanding of his or her body's normal functioning

[[Page 43445]]

and the adequate emotional health for carrying out self-care tasks.\24\ 
A child with SCD may avoid taking medication or ignore complications of 
the disease out of frustration with the limitations of SCD.

    \24\ See 20 CFR 416.926a(k) and SSR 09-7p.

    Health and physical well-being. The ongoing effects of SCD and its 
treatment may affect a child's health and physical well-being.\25\ In 
this domain, we evaluate the effects of periodic exacerbations of pain 
crises due to sickle cell anemia. We consider the frequency and 
duration of the exacerbations as well as the extent to which they 
affect a child's ability to function physically.

    \25\ See 20 CFR 416.926a(l) and SSR 09-8p.

    This SSR is applicable on September 15, 2017. \26\

    \26\ We will use this SSR beginning on its applicable date. We 
will apply this SSR to new applications filed on or after the 
applicable date of the SSR and to claims that are pending on and 
after the applicable date. This means that we will use this ruling 
on and after its applicable date in any case in which we make a 
determination or decision. We expect that Federal courts will review 
our final decisions using the rules that were in effect at the time 
we issued the decisions. If a court reverses our final decision and 
remands a case for further administrative proceedings after the 
applicable date of this SSR, we will apply this SSR to the entire 
period at issue in the decision we make after the court's remand.

    Cross References: SSR 86-8: Titles II and XVI: The Sequential 
Evaluation Process; SSR 96-3p: Titles II and XVI: Considering 
Allegations of Pain and Other Symptoms in Determining Whether a 
Medically Determinable Impairment is Severe; SSR 96-8p: Titles II and 
XVI: Assessing Residual Functional Capacity in Initial Claims; SSR 09-
1p: Title XVI: Determining Childhood Disability Under the Functional 
Equivalence Rule--The ``Whole Child'' Approach; SSR 09-2p: Title XVI: 
Determining Childhood Disability--Documenting a Child's Impairment-
Related Limitations; SSR 09-3p: Title XVI: Determining Childhood 
Disability--The Functional Equivalence Domain of ``Acquiring and Using 
Information''; SSR 09-4p: Title XVI: Determining Childhood Disability--
The Functional Equivalence Domain of ``Attending and Completing 
Tasks''; SSR 09-5p: Title XVI: Determining Childhood Disability--The 
Functional Equivalence Domain of ``Interacting and Relating with 
Others''; SSR 09-6p: Title XVI: Determining Childhood Disability--The 
Functional Equivalence Domain of ``Moving About and Manipulating 
Objects''; SSR 09-7p: Title XVI: Determining Childhood Disability--The 
Functional Equivalence Domain of ``Caring for Yourself''; SSR 09-8p: 
Title XVI: Determining Childhood Disability--The Functional Equivalence 
Domain of ``Health and Physical Well-Being''; SSR 16-3p: Titles II and 
XVI: Evaluation of Symptoms in Disability Claims; and Program 
Operations Manual System (POMS) DI 22001.001, DI 22505.001, DI 
22505.003, DI 24501.021, DI 24510.005, DI 25201.005, DI 25220.010, DI 
25505.025, and DI 25505.030.

[FR Doc. 2017-19551 Filed 9-14-17; 8:45 am]