SwansonSCDAJPM2011 (1)

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Disability Among Individuals with
Sickle Cell Disease
Literature Review from a
Public Health Perspective
Mark E. Swanson, MD, MPH, Scott D. Grosse, PhD, Roshni Kulkarni, MD
Context: Young people with blood disorders face challenges in maintaining their physical health as
they age. Sickle cell disease has well-documented complications in various organ systems. Increas-
ingly, professionals, consumers, and advocates involved in blood disorders are concerned about the
cumulative and ongoing effect of organ-specifıc complications on function and participation.
Evidence acquisition: Publications were identifıed that looked at the relationship between sickle
cell disease and associated impairments and restrictions in participation as defıned by the Interna-
tional Classifıcation of Function, Disability, and Health (ICF).
Evidence synthesis: This article organizes a literature review in PubMed using ICF terms that
defıne functional limitations and participation restrictions in sickle cell disease.
Conclusions: Individuals with sickle cell disease experience complications in multiple organ sys-
tems that affect related functions and, consequently, participation in community living. The effects
begin early in childhood and accumulate across the life course into adulthood. Intervention research
is needed to understand how contextual factors can promote optimal function and participation in
the face of mounting impairments.
(Am J Prev Med 2011;41(6S4):S390–S397) © 2011 Published by Elsevier Inc. on behalf of American Journal of
Preventive Medicine
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Background
Disability refers to restrictions in the types of ac-
tivities and social participation that an individual
can perform as a result of physical or mental
mpairments or conditions of longstanding duration
ithin an environment that does not provide suffıcient
ccommodation to facilitate participation. This inte-
rates three distinct traditions in disability research.1 The
edicalmodel of disability focuses on the individual who
xperiences sequelae of medical conditions; it tends to
quate impairment with disability. The functional model
f disability also operates on the individual level and
escribes functioning and activity without regard to eti-
From the Division of Human Development and Disability (Swanson), the
Division of BloodDisorders (Grosse),National Center onBirthDefects and
Developmental Disabilities, CDC, Atlanta, Georgia; and theDepartment of
Pediatrics and Human Development (Kulkarni), College of Human Medi-
cine, Michigan State University, East Lansing, Michigan
Address correspondence to: Mark E. Swanson, MD, MPH, Division of
HumanDevelopment andDisability, National Center on Birth Defects and
Developmental Disabilities, CDC, 1600 Clifton RoadNE,MS E-87, Atlanta
GA 30333. E-mail: meswanson@cdc.gov.
t
0749-3797/$36.00
doi: 10.1016/j.amepre.2011.09.006
S390 Am J PrevMed 2011;41(6S4):S390–S397 ©2011Publishe
logy. Finally, the socialmodel of disability focuses on the
ocial context in which disablement occurs and identifıes
isability as the product of social disadvantage and exclu-
ion. The purpose of this narrative review article is to
ighlight the multifactorial nature of disability among
eople with sickle cell disease, which includes physical,
sychological, and social dimensions.
The International Classifıcation of Function, Disabil-
ty, andHealth (ICF), adopted by theWHO in 2001, is the
nternationally recognized framework of concepts and
easures of disability, and embodies the integrated ap-
roach to disability.2 The ICF distinguishes functional or
tructural impairments from limitations in personal ac-
ivities and restrictions on social participation, observing
hat these limits occur within the context of environmen-
al factors, not strictly because of the impairment. It de-
ınes participation as including education, work or em-
loyment, community living, and healthy relationships
ith family and peers. Degrees of impairment, activity
imitation, and restrictions in social participation may be
elated to the affected person’s quality of health care and
ocial and physical environment. Therefore, disability is
he summed experience of impairments interacting with
dbyElsevier Inc. on behalf ofAmerican Journal of PreventiveMedicine
mailto:meswanson@cdc.gov
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Swanson et al / Am J Prev Med 2011;41(6S4):S390–S397 S391
contextual factors, resulting in variation in participation.
It changes over time and therefore should be measured
longitudinally. The International Classifıcation of Func-
tion, Disability and Health for Children and Youth
(ICF-CY) likewise defınes participation for children
(Table 1).3
Chronic conditions may lead to disability as the
result of the primary impairment, as well as secondary
conditions or complications. Heightened physician,
patient, and family awareness and monitoring for early
signs can detect complications in a timely manner so
that clinical, social, and educational interventions can
be used to prevent, minimize, or delay the emergence
of impairments and disability. The prevention of com-
plications can prevent or reduce disability and pro-
mote the goal of a full and active life. Control or
management of disease symptoms should be accompa-
nied by the promotion of healthy behaviors and social
Table 1. Domains in the ICF2,3
ICF-CY (children)
Impairments
Sensory Vision
Hearing
Pain
Learning Academic skills
Concepts
Communication Understanding others
Speaking
Mobility Using hand, fingers
Walking
Participation
Behavior Self-care
Independence
Avoid harm to self
Social interaction Formal relationships
Informal relationships
Family relationships
Major life activities Playing
School
Using money
Sources: Krahn et al.2 and WHO3
ICF-CY, International Classification of Function, Disability and Health
policies that lead to education, meaningful life experi-
December 2011
ences (work, volunteer-
ing), active community
life (recreation, civic),
and successful relation-
ships with family and
friends.
Evidence
Acquisition
The literature review was ac-
complished through PubMed
search, using sickle cell disease
in combination with each of
the following terms: pain, vi-
sion, hearing, education, cog-
nition, school performance,
language, employment, depres-
sion, mobility, emotional prob-
lems, or behavioral problems.
No restriction was placed on
year of publication, but the
oldest article included was
published in 1986.
Sickle Cell Disease and
Disability
Sickle cell disease (SCD) de-
scribes a group of hemoglo-
bin disorders inherited in an
autosomal recessive manner
that are characterized by the
presence of one hemoglobin
S (Hb S) allele on the �-glo-
bin gene and one other vari-
ant allele, either another
structural hemoglobin defect
(e.g., Hb S, Hb C, Hb DPunjab,
r Hb OArab) or an allele associated with �-thalassemia that
auses reduced production of normal hemoglobin (Hb A).
here are multiple mutations causing �-thalassemia, but people
ho have both Hb S and the �-thalassemia allele are classifıed
nto two types, Hb S/��-thalassemia or Hb S/�0-thalassemia,
depending on the level of Hb A produced. Individuals homozy-
gous for the sickle cell allele, or Hb SS, are said to have sickle cell
anemia and typically have relatively severe disease symptoms.
People with Hb S/�0 thalassemia have similarly severe disease to
Hb SS homozygotes, whereas those with Hb S/��-thalassemia
or Hb SC typically have mild to moderate symptoms. An anal-
ysis of data from the Cooperative Study of Sickle Cell Disease
(CSSCD) conducted during the 1980s projectedmedian survival
for SS disease to be 48 years for women and 42 years for men,
and for SC disease to be 68 years for women and 60 years for
men.4 Over the years, child survival in SCD has improved, but it
ontinues to be much better in Hb SC than in Hb SS. A recent
ohort study of children receiving care at a comprehensive SCD
enter reported that about 94% of children with Hb SS or HbS�0
ICF (adults)
Vision
Hearing
Pain
Basic learning
Applying knowledge
Receiving
Producing
Handling, moving objects
Walking and moving
Using transportation
Caring after body parts
Looking after one’s health
Interpersonal behaviors
(general and particular)
Work/employment
Education
Economic life
hildren and Youth
and 98% with Hb SC or Hb S�� survive to adulthood.5
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S392 Swanson et al / Am J Prev Med 2011;41(6S4):S390–S397
Unless otherwise indicated, the term SCD is used to refer to
mixed samples representative of individuals with Hb SS, Hb SC,
and Hb S/�-thalassemia or just Hb SS and Hb SC; and individ-
als with Hb SS and/or Hb S/�0-thalassemia are said to have
sickle cell anemia.
Children and adults with SCD, particularly those with sickle cell
anemia, are at risk of the impairments described in following
sections. Having impairment may lead to activity limitations and
restricted social participation.2 Most commonly, impairments are
ddressed in a clinical setting using a medical model of disability,
hich recognizes the cumulative injury to multiple organ systems
rom red blood cell sickling and inflammation, and the occurrence
f unpredictable and debilitating pain episodes and chronic ane-
ia. Themedicalmodel uses a physiologic approach to disease and
ocuses on the individual in isolation from the social context.
onsequently, it has been critiqued as incomplete by social scien-
ists employing a social model of disability, which looks at func-
ional outcomes and interactions with the social and physical
nvironments.6–8
The impact of sickle cell disease on disability can bemediated by
a number of potential etiologic mechanisms. These include, vaso-
occlusive events (also called pain crises) and organ dysfunction,
depression, sensory impairments of vision or hearing, anemia,
bone disease, pulmonary complications, skin ulceration, and de-
pression or behavioral disorders. Moderate to severe anemia is a
common feature of sickle cell anemia, By age 5 years, mean hemo-
globin levels are 8.1 g/dL in children with Hb SS or Hb S/�0, and
10.7 g/dL in children with Hb SC or Hb S/��.9
The following sections discuss the evidence for impairment and
participation restriction among people with SCD using the catego-
ries of the ICF and ICF-CY.
Evidence Synthesis
Functional Impairments in Sensation: Pain,
Vision, Hearing
Pain is a common experience of people with SCD. In a
prospective cohort study of 232 adults with SCD, roughly
one third reported experiencing pain crises due to vaso-
occlusive events daily and another 50% reporting pain
crises more than half of days in a daily diary record.10
Another U.S. study of a clinic-based sample of 41 adults
with SCD reported by diary entry that pain crises oc-
curred on one third of all days.11
Pain episodes especially limit participation in school,
activities of daily living, and social events for children. A
United Kingdom (U.K.) study reported that pain epi-
sodes occurred twice a month and caused a 7-fold in-
crease in absence from school and disrupted other forms
of social participation as well.12 Baseline social, emo-
tional, and school functioning are signifıcantly lower in
children with SCD, and these values decrease further
during a vaso-occlusive event.13
An excellent review of pain studies in adults with SCD
has summarized key fındings about pain and function.14
In the Pain in Sickle Cell Epidemiology Study (PiSCES),
researchers confırmed the association between pain and
activity limitation in a study of 308 adults (mean age 33
years). After controlling for demographic variables, the
mean amount of pain was highly predictive of physical
function, social function, emotional roles, and physical
roles assessed on the basis of the Short Form-36 instru-
ment.15 The 27% of PiSCES patients with depression had
signifıcantly more days of pain, a fınding similar to other
chronic conditions where depression and pain are
interrelated.16
The Multicenter Study of Hydroxyurea in Sickle Cell
Anemia (MSH) enrolled 299 U.S. and Canadian adults
with sickle cell anemia who had frequent (three or more
per year) painful crises. Baseline daily pain was a signifı-
cant predictor of quality of life (QoL) outcomes, includ-
ing physical and social functioning, at all time points.17
Painmade a small independent contribution to decreased
physical and social functioning in 96 adults in London.18
The most recent study from the Comprehensive Sickle
Cell Centers (CSCC) looked at QoL in1046 adults (me-
dian age: 28 years, 73% with SS or S/�0) and found that
acute and chronic pain impaired QoL more than any
other disease-related complication.19
Pain is not simply the result of a biological process but
is influenced by the social context. Psychosocial stress
and negative mood have been shown to influence and
predict the onset of pain among adults and adolescents
with SCD.11 Conversely, positivemood and active coping
trategies have been shown to weaken the negative effects
f pain on QoL and functioning.18,20
Individuals with SCD often develop sensory impair-
ments in vision or hearing. Vision loss in SCD can be
secondary to stroke or result from proliferative sickle cell
retinopathy.21 Although children with SCD are more
ikely to be reported to have visual impairment,22,23 there
ere no studies found that assessed the impacts of visual
mpairments in SCD on function or participation. Senso-
ineural hearing loss is also reported to be common
mong children with SCD,22–25 but information on func-
ion and participation is lacking. Finally, it should be
oted that chelation therapy used for treating transfu-
ional iron overload in transfusion-dependent patients
an in some cases lead to hearing and vision disturbances,
nd regularmonitoring is required with dose reduction if
isturbances are noted.26
Functional Impairments in Cognition and
Language
The biggest risk of permanent impairment and disabil-
ity for individuals with SCD is cognitive and psy-
chomotor impairment secondary to a stroke. About
11% of children with sickle cell anemia experience a
clinical stroke prior to age 18 years, 250 times higher
than the risk in the general pediatric population.27
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Swanson et al / Am J Prev Med 2011;41(6S4):S390–S397 S393
Nine children aged 6 to 12 years with sickle cell anemia
enrolled in the CSSCD who had experienced an overt
stroke were reported to have an average IQ score al-
most 20 points lower than 105 other children with
sickle cell anemia who had normal magnetic resonance
imaging (MRI) scans.28 Of the 22 children with SCD in
he Atlanta study who had intellectual disability (men-
al retardation) or cerebral palsy, 13 (59%) had had a
ocumented stroke, mostly before the age of 5 years.22
In particular, a signifıcant elevation in risk was re-
stricted to those with IQ scores less than 50. Among
individuals without a stroke, there was less likelihood
of having an IQ score less than 70.22
Silent cerebral infarcts, as documented by abnormali-
ties onMRI scans in the absence of a reported stroke, can
cause cognitive impairment to a lesser extent. Children
with sickle cell anemia in the CSSCD who had MRI evi-
dence of an infarct had average IQ scores that were 5 to 9
points lower than children who had normalMRI scans.28
A more recent study that used MRI to detect evidence of
a previous stroke, found that 27% of 65 children with
SCD had had a stroke, with an additional 13% identifıed
with a silent infarct.29 That study reported similar decre-
ents in mean IQ scores to the CSSCD, 19 points for
hosewith stroke and 7points for thosewith silent infarct,
lthough a large percentage of subjects in the newer study
ere classifıed as having experienced stroke.
Silent infarcts can cause other, more specifıc cognitive
efıcits, notably in attention and executive function,
hich are critical for successful academic performance.30
Aneuropsychological study found that 53% of 19 chil-
drenwith sickle cell anemia and silent infarcts had abnor-
mally low performance on attention and executive skills,
compared with 13% of 45 children with sickle cell anemia
seen at the same institutions without infarcts and none of
18 siblings.31 No signifıcant effect of silent infarct on
emory, language, or visual–spatial performance was
etected. A Dutch study, however, found no signifıcant
eurocognitive differences between nine children with
ickle cell anemia and silent infarcts and 12 other children
ith sickle cell anemia.32
Longitudinal data indicate decreased cognitive
functioning among children with SCD that is a func-
tion of cumulative neurologic impairment even among
those without either overt or silent cerebrovascular
infarct.33 For example, an analysis of CSSCD data for
22 children followed over a period of 9 years reported
n average decrease in IQ scores of 2.3 points among
hildren who retained normal MRI status, compared
ith an average loss of 6.4 points for those who had a
ilent infarct.34
The best established mechanism for neurologic im-
pairment in the absence of infarct is anemia; several stud-
December 2011
ies involving children with sickle cell anemia have found
that low hemoglobin concentration is predictive of neu-
rocognitive impairment.32,33,35,36 One of these studies
ound thrombocytosis as well as anemia to be predictive
f intellectual disability (IQ�75).35Another study impli-
ated nutritional defıciencies resulting in low height-for-
ge in neurocognitive impairment among children with
ickle cell anemia.37
A meta-analysis of data from 17 published cognitive
studies that compared a total of 631 children with SCD
with 446 demographically matched peers or siblings,
without any underlying illness that might impair cogni-
tion, reported a mean difference of 4.3 points in IQ
scores.34 The difference inmean IQ scoreswas not altered
y the exclusion of children with evidence of stroke or
ilent infarct based on MRI.38 It was noted that most
hildren with SCD have multiple environmental risk fac-
ors for disadvantage, and there may be an interaction
etween the biology and the environment with negative
actors from each reinforcing each other.
Language defıcits, unrelated to cerebrovascular events,
ften occur among children with SCD. In one study, 20%
o 23% of school-aged children with sickle cell anemia
ad poor language performance, regardless of the results
f MRI scans, compared with 6% of their siblings.39 At
school age, children with sickle cell anemia are reported
to have higher levels of language processing defıcits in
three important areas of language development (seman-
tics, syntax, and phonologic processing).40 This could
lead to communication problems in school and the
workplace.
Cognitive decline continues into adulthood as demon-
strated byVichinsky et al.,41 even in the absence of stroke.
Their study found a decrement of 5 IQ points in adults
with asymptomatic sickle cell anemia, compared to con-
trols. The difference was associated with both increasing
age and severity of anemia.
Functional Impairments in Mobility
Mobility impairment can occur among children with
SCD as a result of cerebral palsy22 (which can result from
troke), stroke, and other etiologies.13 Recurrent skeletal
disease due to repeated bone infarction, avascular necro-
sis of the femoral head,42 decreased bone density with
ertebral disease leading to chronic back pain,43 and nu-
ritional defıciencies44 are some of the complications of
SCD that can affect mobility. Even in the absence of
stroke, there can be a cumulative toll on the musculosk-
eletal system, resulting in decreased mobility. Published
information on the prevalence of mobility impairment in
SCD was not identifıed.
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S394 Swanson et al / Am J Prev Med 2011;41(6S4):S390–S397
Participation Restrictions: Emotional and
Behavioral
People with SCD, like those with other chronic health
conditions, may be at risk for psychosocial or mental
health problems, including depression, and self-concept
and peer socialization diffıculties.45 It should be empha-
ized that behavioral or mental health problems do not
esult from the condition itself but from the interaction of
he individual with their environment. One study re-
orted that in children with SCD and overt stroke, those
ith a silent infarct and children with a normal MRI all
cored in the normal range on a behavior checklist.28
Another study reported that children with SCD were no
more likely to have behavioral problems than their sib-
lings.46 In particular, behavior problems that are some-
times reported for children with SCD appear to be asso-
ciated primarily with family conflict and have little or no
association with SCD or its complications.34 Parenting
tress from unpredictable disease complications can be
ggravated by environmental stressors such as poverty
nd community violence; hence, family-centered inter-
entions that aim to strengthen the family’s ability to
anage stressors may be warranted.47
Most of the existing evidence supports an increased
prevalence of anxiety and depression among adults, ado-
lescents, and children with SCD.48 In the PiSCES project,
the prevalence of depression was much higher than what
has been elsewhere reported in African-American adults.
In turn, these symptoms predicted more daily pain and
poorer physical and mental QOL in adults with SCD.15
Participation Restrictions: General Aspects
Moderate to severe anemia is a common feature of SCA
and may lead to fatigue and weakness, resulting in poor
performance and reduced physical as well as cognitive
function. Anemia in the elderly is a well-established pre-
dictor of functional decline and disability, which could
result from reduced oxygen delivery to muscles and the
brain.49 To date, there have been no published studies of
he specifıc impact of anemia on functional or participa-
ion restriction in adults and childrenwith SCD.OneU.S.
tudy of children and young adults with SCD reported
hat cardiopulmonary complications such as acute chest
yndrome, pulmonary hypertension, and asthma lead to
ecreased exercise capacity.50 Whether reduced capacity
lso affects participation in sports or gym class and phys-
cal activities of daily living needs to be studied.
Participation Restrictions: School and Work
One of the areas of greatest limitation to full participation
for children with SCD is education. Due to the challenges
inmanaging frequent vaso-occlusive events and frequent
hospitalizations, children with SCD often miss multiple d
days of school at a time.8,51,52 One U.S. study, with a
sample of 50 school-aged children with SCD recruited
from pediatric hematology clinics, found that a mean of
18.2 days of school were missed due to illness, equivalent
to 10% of the school year.53 In a large U.K. study that
recruited 569 young people with SCD, the mean number
of days missed for SCD-related illness was 16.3, or 8.4%,
of the school year; and 12.3% of the sample reportedly
missed at least 32 days.8 Most respondents reported that
they were unable to make up much of the school work
they missed. The U.S. study reported that the number of
days of school missed due to SCD contributed to grade
retention but not to poorer achievement test scores.53
It should be emphasized that the number of school
days missed due to sickle cell pain is not a fıxed charac-
teristic of the disease. Rather, it depends on the ability of
the child and their family to manage pain and the under-
standing and support shown by school staff and peers.8
This was demonstrated in a randomized trial of a school-
based education intervention for teachers and peers of
children with SCD that succeeded in reducing the num-
ber of days missed.54
The combination of general cognitive defıcits plus spe-
cifıc attention, executive functioning, and language pro-
cessing defıcits put children with SCD at a biological
disadvantage for schoolsuccess. Therefore, it is not sur-
prising that children with SCD frequently experience de-
creased school achievement and attainment relative to
other children of the same social and ethnic backgrounds.
For example, Schatz reported that 30% of 50 children
with SCD had repeated a grade, compared with 8% of 36
matched community controls.53 Another study that used
RI scans reported that 57% of 19 children with sickle
ell anemia and silent infarcts had repeated a grade or
equired special education, compared with 27% of an-
ther sample of 45 childrenwith SCDwhohad not had an
nfarct and 6%of 18 siblings without SCD.31 Clinic-based
studies of adults with SCD suggest that 15%–20% of
adults with SCD do not complete high school.14
Adults with SCD have high levels of unemployment
and nonparticipation in the labor force.55,56 Two stud-
ies of small clinic samples in North Carolina reported
that less than half (38%–50%) of adult SCD patients
were employed.57,58 Another study reported that
mong those who were employed, workers missed
ork an average of 17% of days on which they had
ainful crises.11 A qualitative study of U.K. adults with
CD derived from 32 focus group discussions reported
hat employment was diffıcult to maintain because of
requent absences associated with painful episodes and
nsupportive attitudes of employers.59 In addition,
epressive symptoms, low vitality, and emotional
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Swanson et al / Am J Prev Med 2011;41(6S4):S390–S397 S395
problems in adults with SCD may cause role limita-
tions independent of pain episoides.18
In MSH, 81% had graduated from high school, and
49% had post-secondary education. Just 16% were in
full-time employment, and 59%were unemployed or dis-
abled.20 Subjectsmoved into and out of employment over
the course of the study, but on average were employed
30% of the time. Although hydroxyurea was associated
with a large reduction in the frequency of painful crises, it
had a small, nonsignifıcant impact on employment rates.
Because the MSH study was representative only of the
very small number of adults with sickle cell anemia who
have three or more painful crises in a year, one cannot
generalize to adults with SCD.
Discussion
As most children born with SCD now survive to adult-
hood, they have a right to expect to live productive,
independent lives, with full participation in work,
community, and relationships. Health providers and
parents need to track function in academic, cognitive,
social, and emotional domains to identify children
who need help. Individual data on school, emotional,
and social progress need to be available to examine the
interaction between health status, age, and these do-
mains. Enhanced individual health records and linkage
to other systems that include valuable follow-up data
(i.e., education, social services, mental health) and
must be promoted to help us better understand the
critical factors that determine quality of life and par-
ticipation. The ICF provides principles and an organi-
zational framework to guide these efforts for better
identifıcation of relevant variables (biological, per-
sonal, environmental), more systematic data collection
and heightened surveillance for secondary conditions
that influence the eventual goal of full participation.
Implications for Future Research
This paper identifıed limited numbers of studies that
assessed disability and functioning in people with SCD.
Few large-scale SCD research studies have assessed mea-
sures of participation and function. A notable exception
is the MSH study, which prospectively gathered data on
employment for study subjects.20 Most studies that did
ssess measures of participation were of small samples of
onvenience, typically recruited from clinics. Most stud-
es had short duration in follow-up, usually less than 1
ear, so the progression of impairments and resulting
mpact on function and participation could not be as-
essed. Population-based, longitudinal data across the life
ourse of SCD would help correlate functional outcomes
ith interventions and environmental factors. These data
December 2011
ould be gathered through awell-designed and -maintained
atient registry. In particular, the progression of cognitive
efıcits in adultswithnohistoryof strokeandnoevidenceof
ilent infarct needs to be documented.
There needs to be a better understanding of the factors
eading to restrictions in participation in people with
CD (progression of neurologic and brain effects, educa-
ional opportunities lost from disease flare-ups in child-
ood, family stress due to unpredictable acute crises,
orkplace and school response to frequent absences, im-
act of pain and analgesic use on function). It is impor-
ant to identify existing and new interventions that hold
romise to improve outcomes: (active coping strategies,
onpharmacologic management of pain, use of social
etworks to promote social and emotional development,
ole of exercise and nutrition, individualized school and
ork accommodations). Since pain is such a hallmark
omponent of SCD, better studies are needed to defıne
hronic pain and including only those with chronic
ain.14 Interventions to address skills for living and cop-
ing with pain need special attention. Developing positive
life skills, such as acceptance of condition, focus on per-
sonal strengths, self-worth, and independence could be
an important strategy for adults to avoid developing
chronic illness behavior, a series of maladaptive re-
sponses that lock an individual into a sick role.60 At the
ndividual, family, and community levels, there are op-
ortunities to construct flexible policies and practices
hat empower the individual, promote functional out-
omes, and increase quality of life.
Publication of this article was supported by the Centers for
Disease Control and Prevention through a Cooperative Agree-
ment with the Association for Prevention Teaching and Re-
search award # 09-NCBDDD-01.
The fındings and conclusions in this report are those of the
authors and do not necessarily represent the offıcial position of
the CDC.
No fınancial disclosures were reported by the authors of this
paper.
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	Disability Among Individuals withSickle Cell Disease Literature Review from a Public Health Perspective
	Background
	Evidence Acquisition
	Sickle Cell Disease and Disability
	Evidence Synthesis
	Functional Impairments in Sensation: Pain, Vision, Hearing
	Functional Impairments in Cognition and Language
	Functional Impairments in Mobility
	Participation Restrictions: Emotional and Behavioral
	Participation Restrictions: General Aspects
	Participation Restrictions: School and Work
	Discussion
	Implications for Future Research
	References