An Examination of Differences in Intra-Personal Resources, Self-Care Management, and Health Outcomes in Older and Younger Adults with Sickle Cell Disease

Southern Online Journal of Nursing Research
www.snrs.org

Issue 3, Vol. 7
October 2006



An Examination of Differences in Intra-Personal Resources,
Self-Care Management, and Health Outcomes in Older and
Younger Adults with Sickle Cell Disease


Coretta M. Jenerette, PhD, RN, AOCN1; Richard C. S. Phillips, BS, BSN2

1Post-Doctoral Fellow, Yale University School of Nursing, New Haven, CT; 2Howard University Division
of Nursing

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SOJNR, issue 3, vol. 7, p.2
ABSTRACT

Background: Sickle cell disease (SCD) and its manifestations often result in a lifetime
of pain and hospitalizations. Although modern medicine has made advances in the
diagnosis and treatment of SCD, these patients often receive inadequate health care and
may lack the skills needed to improve self-care management, health status, and quality
of life (QOL).
Purpose: To examine differences in intra-personal resources, self-care management,
and health outcomes in older and younger adults with SCD.
Conceptual Framework: Theory of Self-Care Management for Vulnerable
Populations
Design: Comparative descriptive
Sample and Setting: Sample of 57 older adults and 103 younger adults recruited
from two southeastern United States SCD clinics
Measures: Simplified Rathus Assertiveness Schedule, Family Coping Project Coping
Scale, Sickle Cell Self-Efficacy Scale, Medical Outcomes Study Social Support Survey,
Jenerette Self-Care Assessment Tool, a brief Health Status Questionnaire, and the
Chronic Illness QOL Scale.
Results: There were significant differences in assertiveness, spiritual activities and
focusing on others' coping behaviors, and tangible and positive social interaction social
support.
Conclusions: The data suggests that older and younger adults with SCD use different
intra-personal resources to deal with SCD. Further studies need to be done to develop
appropriate interventions for younger and older adults with SCD to enhance self-care
management and improve health outcomes.

Key Words: sickle cell disease, self-care management

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An Examination of Differences in Intra-Personal Resources, Self-Care
Management, and Health Outcomes in Older and Younger Adults with
Sickle Cell Disease

Introduction
caused by a genetic mutation that
Sickle cell disease (SCD) is an
produces defective hemoglobin.8,9 This
inherited, autosomal, recessive genetic
mutation leads to the possibility of
disorder that is expressed as sickle cell
damage to every system in the body.10
anemia, sickle cell thalassemia disease,
Advancements in the detection and
or sickle hemoglobin C disease.1,2 SCD
treatment of SCD have led to increased
is a chronic blood disorder that, in the
life expectancy.11,12 Thirty years ago, the
United States, most often affects African
median length of survival for SCD
Americans (Figure 1). In African
patients was 14 years.13 Because of
Americans in the US, SCD is one of the
advances in medical management, those
most prevalent genetic disorders with a
affected with SCD are living much
birth rates of 2.7 per 1000 for sickle cell
longer.14,15 In the US, the average
anemia or Hb SS (homozygous disease),
lifespan of African Americans with sickle
1.2 per 1000 for Hb SC (heterozygous
cell anemia, the most severe form of
disease), 0.6 per 1000 for Hb S (beta-
SCD, is 42 to 48 years,16 compared to
thalasemia) and 83.3 per 1000 for Hb
healthy African Americans, who have an
AS (sickle cell trait).3 Globally, SCD is
average lifespan of 67 to 75 years.
most common among people originating
Advances in the care of people affected
from sub-Saharan Africa, but it also
with SCD include genetic counseling,
affects people of Mediterranean,
neonatal screening, early initiation of
Caribbean, Middle Eastern, and Asian
prophylactic penicillin therapy, closer
origin.4 SCD affects an estimated 1–2%
medical monitoring, newer medications
(120,000) of newborns in Africa
such as hydroxyurea (the only treatment
annually.5 Approximately 178 babies
currently available that can reduce the
(2.8 per 10,000 conceptions) are
severity and frequency of painful
affected by sickle cell disease in England
episodes), and early intervention to
annually,6 and 10, 000 in the United
relieve painful symptoms.11,17 However,
Kingdom suffer from the disease.7 It is
the only cure for SCD is bone marrow

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SOJNR, issue 3, vol. 7, p.4
transplantation, which is not an option
inherent risks posed by the drug
for most people with SCD due to either a
regimen required before
shortage of compatible donors or the
transplantation.11,17,18

Figure 1
Prevalence of Sickle Cell Disease in the U.S., 19933


SCD is a lifelong disorder
and frequent hospitalizations.20 Pain is
characterized by recurrent and
the most frequent reason that adults
unpredictable episodes of pain,
with SCD seek health care. The intensity
hemolytic anemia, anemic crises, stroke,
of an acute sickle cell crisis has been
infections, renal and pulmonary
qualitatively compared to terminal bone
problems, and numerous complications
cancer pain, but because pain is
related to organ dysfunction of varying
subjective and immeasurable, the
severity.1,4,19 People with SCD may have
patient, rather than a somatic origin, is
high levels of emergency department use
often thought to be the problem.21

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Because people with SCD are living
of Self-Care Management for Vulnerable
longer, it imperative for health care
Populations (Figure 2) proposes an
providers to make sure that those
approach to identifying variables that
affected with SCD get the best possible
influence self-care management, health
comprehensive care.22
status, and quality of life among
In order to develop comprehensive
populations who experience health
models of care, it is important to know
disparities. Concepts include contextual
the needs of adults with SCD so that
factors (community resources,
their self-care management, health
community values), vulnerability factors
status, and quality of life can be
(age, complications, employment,
enhanced. Moreover, it is important to
father’s education), intra-personal
understand differences in intra-personal
resources (assertiveness, coping
resources, self-care management, and
behaviors, self-efficacy, social support),
health outcomes of older and younger
self-care management, health status,
adults with SCD. The purpose of this
and quality of life. Vulnerability factors
research was to examine differences in
influence intra-personal resources,
intra-personal resources (coping
which in turn influence self-care
behaviors, self-efficacy, and social
management. Self-care management is
support), self-care management, and
theorized to directly predict both health
health outcomes (health status and
status and quality of life. All
quality of life) in older and younger
hypothesized relationships in the model
adults with SCD.
have been supported in prior published
Theoretical Perspective. The Theory
research.23

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Figure 2
Theory of Self-Care Management for Vulnerable Populations


Research Questions. This study
younger adults with SCD?
addresses three research questions:
Review of Literature. The literature
1. What are the differences in intra-
review, guided by the conceptual
personal resources (assertiveness,
framework, focuses on intra-personal
coping behaviors, self-efficacy,
resources (assertiveness, coping
social support) between older and
behaviors, self-efficacy, and social
younger adults with SCD?
support), self-care management, and
2. What are the differences in self-
health outcomes (health status and
care management between older
quality of life) in adults with SCD and
and younger adults with SCD?
other chronic illnesses. Because life
3. What are the differences in health
expectancy for individuals with the most
outcomes (health status and
severe type of SCD was less than 30
quality of life) between older and
years and SCD was described as a

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SOJNR, issue 3, vol. 7, p.7
disease of childhood, there is a limited
taking charge of the situation, may be
amount of research about intra-personal
useful when patients with SCD are faced
resources, self-care management, and
with inappropriate care by poorly
health outcomes in adults with SCD.15,24
informed nurses or medical staff.29
Moreover, the research most often does
In older adults with breast cancer,
not differentiate between older and
black women of lower socio-economic
younger adults with SCD.
status were more likely to have full
Assertiveness is defined as behavior
staging of their tumors when they made
that enables one to “stand up” for one’s
an assertive request. In fact,
rights without infringing on the rights of
assertiveness led to more careful
others.25 This behavior may be useful to
diagnostic testing for patients who were
adults such as those with SCD who are
disadvantaged.30 Similarly, research
facing a life-long struggle with a chronic
with a group of African American elders
illness. Few studies have described the
found that the individuals felt a need to
role of assertiveness as an influential
advocate or stand up for themselves
factor affecting an individual’s self-care
either with healthcare providers or
management. In individuals with
significant others in order to have their
serious illnesses, assertiveness training
needs met.31
is considered useful to increase an
Coping behaviors are cognitive and
individual’s assertiveness, internal locus
behavioral actions used to master
of control beliefs, and positive ways of
conditions of harm, threat, or challenge
coping with a serious illness.26
when a normal or routine response is
Individuals who manage their pain at
not available.32 Chronic illnesses such as
home express a strong sense of self-
SCD frequently leave the sufferer with
responsibility for their pain
feelings of powerlessness, and decreased
management and advocate self-
self-esteem which may lead to the
education, assertiveness, and resistance
inability to use normal coping resources
to strategies toward hospital
such as physical strength, psychological
services.27,28 However, adults with SCD
stamina, and positive self-esteem.33
may have to enter the health care system
Brief training in cognitive coping skills
when a SCD pain crisis cannot be
has been shown to increase coping
managed at home. Assertiveness, or
attempts, decreased negative thinking,

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SOJNR, issue 3, vol. 7, p.8
and lower tendency to report pain
illness.42 In one study, self-efficacy
during a laboratory-induced noxious
among African American adults with
stimulation.34 Coping strategies in
SCD was inversely related to disease
adults with SCD have been shown to be
symptomatology and these relationships
stable over time.35 Negative thinking,
persisted over time.43 In research that
passive coping, and somatic awareness
used case management to improve the
have been shown to be related to several
transition of adolescents with SCD to
measures of poor health in SCD.
adult care, patients who maintained
However, some relationships are better
follow-up appointments were more self-
accounted for by general measures of
efficacious.14 Additionally, patients who
negative affectivity.36 In other studies,
reported higher scores on the Feeling
adults with SCD indicated that on pain
Concerned and Worried subscale of the
days when they practiced coping
Chronic Illness Assessment Interview
strategies such as distraction, relaxation,
for SCD, an instrument based on a
or provided coping audio tapes, they
model of self-care for adult patients with
sought less health care than on days that
chronic medical conditions, also
they did not use these strategies.37,38 In
reported increased visits to the
another study, cognitive behavioral
emergency department, increased pain
therapy was immediately effective for
severity, and decreased levels of self-
management of SCD pain in terms of
esteem, self-efficacy, and mastery.15
reducing the pain of psychological
In elderly adults, self-efficacy may be
distress, as well as improving coping.39
protective against a decline in functional
Self-efficacy refers to one’s belief
status by buffering diminished self-care
about the ability to achieve a desired
capabilities related to diminished
health outcome.40,41 More specifically,
physical capacity.44 A study found that
self-efficacy focuses on individuals’
individuals who have suffered
convictions that they can exercise
cerebrovascular accidents (strokes)
control over motivations, behaviors, and
benefit from incorporating self-efficacy
social environment.42 Although there
techniques in order to succeed in their
has been little research related to self-
self-care management after stroke.45
efficacy in SCD, self-efficacy may be a
Self-efficacy was the only variable found
determinant of adjustment to chronic
to be correlated with adherence in a

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SOJNR, issue 3, vol. 7, p.9
study of medication compliance in
partnership of care in which nurses and
patients suffering from rheumatoid
other health care professionals function
arthritis.46
in a supportive and educational role
Social support is defined as one’s
enhanced the older adult’s lifelong self-
internal perception that may influence
care management and ability to stay in
interpersonal behaviors including
control of multiple chronic health
expression of positive affect, affirmation
conditions.50 In studies pertaining to
of another’s behaviors or views, or
adherence to prescribed treatments,
giving symbolic or material aid.47 Self-
enlisting social support from family and
esteem, social assertiveness, and use of
friends improved adherence.50-52
social support were significant
Self-care management is defined as
predicators of social functioning, mental
engaging in therapeutic behaviors and
health, health perceptions, total
taking action to access resources to
functioning score, and adherence in
maintain and/or improve health status
adolescents with SCD.48 Research about
and quality of life.23 For the adult with
the role social support plays in the
SCD priority self-care measures are
health of adults with SCD is limited.
adequate hydration, refraining from
Nash presented a review of studies
over-exercise, keeping medical
related to the psychosocial aspects of
appointments, adhering to health care
SCD.49 The major theme that permeates
provider’s instructions, and making sure
the review is the continuous need for
medical questions are answered.15 It was
social support. Support is needed from
also found that individuals with SCD
family and health care providers.
may employ strategies to take control of
Support from family, noted in studies of
their lives in order to maintain a
other chronic illnesses, may increase
“normal” life. These self-care
compliance and decrease depression,
management strategies include coping
while support from health care
behaviors which involves negotiation
providers may increase satisfaction with
and engagement in both personal and
the healthcare delivery system and
structural factors.29 Furthermore, it was
decrease the discrimination felt by some
found that one’s ability to engage in self-
patients with SCD. In a study of older
care management is influenced by social
adults, it was also found that building a

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SOJNR, issue 3, vol. 7, p.10
and material resources, life transitions,
individuals with other chronic
and the responses of society.29
conditions, adults with SCD report lower
Another self-care management
general health and health-related quality
behavior for adults with SCD is
of life.55,56 Haque and Telfair found that
religiosity. It was found that religious
self-reported medical problems were
involvement played an important role in
higher for older adults with SCD.57
assisting some adults with SCD to
Quality of life (QOL) is defined as a
manage their disease. In fact, attending
subjective sense of well-being with
church at least once per week is
physical, psychological, and social
associated with fewer negative
dimensions of one’s life.58 SCD is a
experiences with pain and lower levels
condition that has profound
of psychiatric disturbance for patients
consequences for the QOL of individuals
with SCD.53 In a sample of African
with SCD and their family members.59
American elders living with chronic
Individuals with SCD experience health-
health conditions, reliance on God was
related QOL worse than the general
found to an important strategy.31
population.55 This reduced QOL may be
Health status can be defined as one’s
due to the chronicity of the illness
subjective or self-assessment of physical,
combined with frequent hospitalizations
social, and mental well-being.54 The
for pain or other complications, which
health status of adults with SCD may be
can contribute to impaired psychosocial
influenced by a clinical course that often
functioning.60 Although increasing life
worsens. Chronic organ damage may
expectancy is an important
become evident, acute painful crises
accomplishment, QOL is also
become harder to tolerate, and adults
important.61 Interventions in SCD
may face losses of productivity and
should consider improvements in
psychological distress.17 Additionally,
health-related QOL as an important
adults with SCD may experience organ
outcome.55,56
damage that may result in stroke,
pulmonary failure or hypertension, renal
Methods
failure, congestive heart failure, leg
As a part of a larger cross-sectional
ulcers, and osteonecrosis of the femoral
descriptive study, a convenience sample
or humeral head.16 Compared to
of 232 adults with a diagnosis of SCD

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