A Qualitative Analysis of Best Self-management Practices: Sickle Cell Disease

The Best Self-management Practices workshop aimed to identify specific strategies used by adult clients with SCD to achieve optimal physical health. The emergent themes were self-awareness, emotional support, career selection and success factors, nutrition, advocacy, knowledge, physical, and complementary and alternative medicine.

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010 1033
o r i g i n a l c o m m u n i c a t i o n
A Qualitative Analysis of Best Self-management
Practices: Sickle Cell Disease
Paula Tanabe, PhD, MPH; Jerlym Porter, PhD; Melissa Creary, MPH; Eric Kirkwood, BS; Shirley Miller, BBA;
Efa Ahmed-Williams, MA; Kathryn Hassell, MD
IntroductIon
S
ickle cell disease (SCD) is associated with serious
comorbidities resulting in a shortened lifespan and a
lower quality of life when compared to most patients
with renal failure.1-3 Many clients with SCD also suffer from
frequent pain episodes, often on a daily basis.4 Many health
care providers perceive patients with SCD to be addicted
to opioids, and negative attitudes toward patients are com-
mon. 5,6 However, many patients with SCD successfully
manage their disease in the outpatient setting without the
need for frequent health care utilization and do not require
frequent hospitalizations and/or emergency department vis-
its. This group of patients is very productive; however, little
is known about specific strategies used to maintain good
health. Much could be learned from these individuals with
SCD who manage this complex disease within the context
of school, employment, and family life, and maintain good
health. The purpose of this project was to describe specific
strategies used by adult clients with SCD to achieve optimal
physical health and quality of life.
Method
design
Grounded theory using a constant comparative
method was used to conduct the study. In grounded the-
ory, theories are generated based on the data collected.
This research method is very useful when little is known
about a topic. 7 Institutional review board approval was
obtained, and subjects (panel members) signed a written
consent prior to study participation.
Participants/Setting
Participants were identified using purposeful sam-
pling. The Sickle Cell Adult Provider Network (SCAPN)
Funding/Support: This research was supported by the Sickle
Cell Adult Provider Network. Dr Tanabe was supported by
grants from the National Institutes of Health (K23NR010940) and
the Mayday Fund. Dr Porter was supported by a grant from the
Agency for Healthcare Quality and Research (T32 HS00078).
Background: Sickle cell disease (SCD) is associated with
serious comorbidities resulting in a shortened lifespan, and
many clients suffer from frequent pain episodes. However,
others successfully manage their disease in the outpatient
setting without the need for frequent health care utilization.
The purpose of this project was to describe specific strate-
gies used by adult clients with sickle cell disease to achieve
optimal physical health.
Method: A Best Self-management Practices workshop was
held in conjunction with the Sickle Cell Disease Association
of America meeting. A panel discussion was organized;
adult clients were recruited for participation. The workshop
was divided into 3 topics: complementary and alternative
medicine, psychosocial issues, and work/education/train-
ing. Panel discussions were audiotaped, transcribed, and
analyzed using the constant comparative method.
Results: Seven adult patients with sickle cell disease and 1
social worker participated. The following themes emerged:
self-awareness, emotional support, career selection and
success factors, nutrition, advocacy, knowledge, physical,
and complementary and alternative medicine. Self-aware-
ness was the most reported strategy with emphasis on jour-
naling and body awareness. Emotional support included
spiritual support, friends, family, professional counseling, and
spiritual support. A variety of suggestions were discussed
related to the other themes. All participants used many
strategies daily to maintain optimal health.
Keyword: sickle cell anemia
J Natl Med Assoc. 2010;102:1033-1041
Author Affiliations: Department of Emergency Medicine (Dr Tanabe) and the
Institute for Healthcare Studies (Drs Tanabe and Porter), Feinberg School of
Medicine, Northwestern University, Chicago, Illinois; Division of Blood Disorders,
National Center on Birth Defects and Developmental Disabilities, Centers for
Disease Control and Prevention, Atlanta, Georgia (Ms Creary); Uriel Owens
Chapter, Sickle Cell Disease Association of America, Kansas City, Kansas
(Mr Kirkwood); Southwestern Comprehensive Sickle Cell Center, Children’s
Medical Center, Dallas, Texas (Ms Miller); The Sickle Cell Organization,
Baltimore, Maryland (Ms Ahmed-Williams); Division of Hematology and
Colorado Sickle Cell Treatment and Research Center, University of Colorado
Denver School of Medicine, Aurora, Colorado (Dr Hassell).
correspondence: Paula Tanabe, PhD, MPH, Institute for Healthcare Studies,
750 N. Lake Shore Drive, 10th Floor, Chicago, IL 60611 ([e-mail]).

1034 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010
SICKLE CELL DISEASE SELF-MANAGEMENT
group hosted a preconference workshop in conjunction
with the annual meeting of the Sickle Cell Disease
Association of America, held in New Orleans, Louisiana,
in September 2008. The SCAPN network is a group of
adult providers (physicians, nurses, and other disci-
plines) that provide care to adults with SCD. Adult cli-
ents with SCD or parents with children with SCD are
also group members. A list serve is used throughout the
year to post questions regarding disease management
and facilitate discussion of challenging issues. SCAPN
also hosts approximately 2 face-to-face meetings yearly
held in conjunction with national SCD meetings. Each
participant was provided with a stipend by SCAPN to
attend the annual meeting, and all subjects received
additional monies through their work or through addi-
tional fundraising.
Workshop description
A small committee of SCAPN members (providers
and clients) was formed to plan the workshop. The topic
for the workshop was best self-management practices.
In an attempt to minimize researcher bias, the client
committee members, not the investigators, chose the
speakers and topics for the conference. The committee
identified adult clients who would be able to attend the
meeting in New Orleans and were known to use multiple
strategies to maintain good health. A total of 7 different
individuals were invited to present, including 1 social
worker identified by the committee as having valuable
information to contribute. The committee members also
identified the specific topics that they felt would be
meaningful for discussion during the workshop. Three
topics for discussion were selected to organize the work-
shop: complementary and alternative medicine, psycho-
social issues, and work/education/training topics. The
committee matched topics with speaker expertise.
Notably, pain was not identified as a discussion topic by
the members; however, the discussion of the selected
topics touched on ways to improve pain management.
The workshop was divided into 3 segments accord-
ing to the topics listed above. Prior to the beginning of
the workshop, the 7 client participants completed a brief
survey, which included demographic characteristics
(age, gender), sickle cell genotype, frequency of pain
episodes, emergency department utilization, complica-
tions of SCD, relationship with a physician, and
table 1. Sample Characteristics
characteristics results (n = 7)
Genotype
SS
SC
Number
5
2
Within the last year, approximately how often did you have pain
episodes that you were able to manage at home?
Median: 3
Range: 0-20
Data: no pain, 0, 3, 3, 6, 9, ≥20
Within the last year, approximately how often did you have pain
episodes that required you to come to the emergency department?
Mean: 0.8
Range: 0-3
Data: 0, 0, 0, 0, 1, 2, 3
Within the last year, approximately how many times did you have to be
admitted to the hospital to manage your pain?
Mean: 0.4
Range: 0-1
Data: 0, 0, 0, 1, 1, 1,
Compared to the last year, rate the frequency of pain episodes
compared to the last 5 years.
More episodes
About the same
Fewer episodes
1
2
4
Within the last year, approximately how many times did you have to be
admitted to the hospital for complications from sickle cell OTHER than pain?
N=1
List all complications you have ever had related to sickle cell disease.
Stroke
Acute chest syndrome
Iron overload
Aseptic necrosis of the joints
Priapism (4 males)
Leg ulcers
Liver disease
Gall bladder disease
Kidney disease
Frequency
0
1
1
4
2
3
0
4
1
How many times per year do you see your physician in an outpatient
setting?
Median: 4
Range: 1-24

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010 1035
SICKLE CELL DISEASE SELF-MANAGEMENT
frequency of visits with physician. While genotypes
were not verified via medical record review, all subjects
were very educated about their disease and were very
knowledgeable of their genotype as well as all other
complications associated with the disease throughout
their life. Many of the participants frequently speak on
patient panels in a public forum. Two to 3 speakers were
invited to present an overview of their comments on the
topic for each segment. Each speaker presented for 30 to
45 minutes, followed by discussion among the panel
members. This portion of the workshop was audiotaped
using digital recorders. During the actual workshop, the
speakers were not interrupted by the investigator at any
time. Upon completion of the panel member discussion,
the tape recorders were turned off and a discussion with
the audience was held. Audience discussion was not
audio-taped because it would not be possible to obtain
informed consent from audience members who came in
and out of the room during the 4-hour workshop.
data Analysis
Digital files were transcribed into 3 different Word
documents (Microsoft, Redmond, Washington) and ana-
lyzed using constant comparative methods, which is a
process used to evaluate interrater reliability. More spe-
cifically, the principal investigator and a health services
research fellow not involved with the project first inde-
pendently reviewed the majority of the 3 transcripts and
identified common themes. Analysis of latent (meaning
of the words) vs manifest (actual words) content analy-
sis was used. After the initial independent review, the
principal investigator and fellow met and discussed the
themes. A new coding structure was identified. The
reviewers then independently reviewed all the transcripts
again using the new coding structure. Following the sec-
ond independent review using the revised coding struc-
ture, the reviewers met again to review the themes. The
original agreement was 59%, and after meeting the sec-
ond time and discussing the themes, agreement was
100%. In order to assess validity of the themes identi-
fied, member checking was conducted. One panel mem-
ber from each of the 3 topics for discussion was asked to
review their corresponding transcript, the final coding
scheme, and results to evaluate the validity of the final
coding scheme. No changes in the coding structure were
required after panel member review.
reSultS
Eight adults participated in the workshop (50% male;
median age, 48 years; range, 36-55 years; 3 participants
did not report an age). Participants resided in the follow-
ing states: Florida, Kansas, Maryland, Tennessee, and
Texas (n = 2) (missing data [n = 2]). All participants
reported having a physician, and no participant primar-
ily used the emergency department to manage their dis-
ease. Results from the survey are presented in Table 1.
Table 2 summarizes the emergent themes and sub-
themes as well as selected quotes associated with each
theme. Themes are listed in order of frequency within
the table, and subthemes are also listed in order of fre-
quency within each major theme. The following 8
themes emerged as the final parsimonious coding struc-
ture and are listed in order of frequency of occurrence,
followed by the number of mentions within the tran-
scripts: self-awareness (52), emotional support (30),
career selection and success factors (29), nutrition (25),
advocacy (23), knowledge (15), physical (15), and com-
plementary and alternative medicine (11).
Self-awareness
A general sense of self-awareness emerged as the
most important strategy used by clients to manage their
symptoms. Self-awareness was conveyed as being aware
of certain behaviors that promote or diminish health.
Clients discussed the importance of an overall positive
outlook as being critically important. The topic of jour-
naling was also discussed at length, both by the panel
members and later during the audience discussion sec-
tion. Many clients used journaling to begin to under-
stand what type of diet or activities triggered episodes of
illness or pain. Journaling was often a key turning point
in several participants’ life. Through journaling they
began to take control of their disease. Journaling and a
general sense of “body awareness” were often linked.
Clients described the importance of staying in tune with
their body and recognizing when they needed more rest,
were doing too much, or beginning to feel ill. Finally, the
importance of a physician relationship was also identi-
fied as an important subtheme. Clients were very aware
of how important this relationship was to maintaining
good health.
emotional support
Emotional support emerged as the second most com-
mon theme. Emotional support can help mitigate the
impact of mental health conditions, and emotional stress
and improve psychosocial functioning. The panel partici-
pants highlighted specific means of emotional support,
including spiritual support, friends and family, and profes-
sional counseling. All participants identified spiritual sup-
port as the most important source of emotional support.
career Selection and Success Factors
One of the 3 panel topics specifically discussed strate-
gies for successful career selection. A major focus of this
panel discussion surrounded mechanisms to be able to
afford and obtain training or college education. Tuition
reimbursement and grants were discussed as examples.
Once employed, the health benefits, as well as other
401(k) and retirement benefits were also discussed.
Assuring that the health benefits allowed seeing one’s
own physician was also an important point of discussion.

1036 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010
SICKLE CELL DISEASE SELF-MANAGEMENT
Participants discussed a wide variety of factors when
selecting a career and included selecting a job that was
not physically demanding, considering the weather con-
ditions (inside vs outside in extreme temperatures), and
the general weather of the state (eg, Alaska or very cold
climates may not be an ideal choice). Selecting a career
that allowed some flexibility was also discussed.
Examples included a real estate agent and positions asso-
ciated with teaching or an academic calendar.
nutrition
Participants also identified nutrition as a major theme
to achieve optimal health. A major subtheme was fol-
lowing a generally healthy diet consisting of fruits, veg-
etables, and whole grain fiber; and limiting meat with
high iron content, fast foods, and refined foods. Many
participants endorsed the use of a variety of vitamins
(multivitamin, B vitamins, C, and folic acid), and herbal
supplements (dandelion, burdock, ginger, garlic, milk
thistle). All participants also acknowledged the need to
table 2. Best Self-management Practices: Themes and Selected Quotes
emergent themes
and Subthemes Selected Quotes Illustrating emergent themes
Self-awareness
Positive attitude
Body awareness
Journaling
Setting realistic
goals
Importance
of physician
relationship
Lifestyle changes
“ You need to be proactive and have a positive mental attitude.”
“ Don’t be afraid to live, don’t be afraid because you have sickle cell, you can
manage it if you want to, you can do anything you want to do.”
“So I don’t believe in excuses, I don’t believe in being an enabler.”
“ I started to understand what things, what I was doing to contribute to my being
sick. And I felt like there was some way to control it and it was my need to
control this disease and not let it control me.”
“ I started journaling because I started trying to research why I would get sick, and
what happened to make me get sick because I got tired of getting sick. I got
tired of not being able to do things. So I said, ‘well maybe I should kind of figure
out what I did the day before.’”
“ It is about planning. And somebody said it earlier about planning your work and
working your plan.”
“ I had to learn how to say ‘no’ and listen to my body and just take off and to say
well today I am staying at the house.”
“ It is crucial for us to be compliant in maintaining a relationship with our health
care provider and one way to do that is to go to your appointments and the
second thing is to make sure you take your medications.”
“ ..these kids were born eating wrong. It is hard now to change. You cannot
change your taste buds overnight.”
Emotional support
Spiritual
Counseling
Social support
“ There is a text in the Bible that deals with being joyful, and they call it the fruit of the
spirit, joy and peace, and goodness and etc, and that is what I read, that is where I
get my energy so that I can go on.”
“ Every night it does not fail, to get down on my knees when I can and simply thank
God for another day and I trust you. Thank you and I trust you. That is my simple
prayer I say every night.”
“ So don’t be afraid to seek professional help. I have talked to a psychologist
before, and you know it helped me. It is good to have someone who is trained
that can give you strategies for managing your mental health…There’s kind of a
stigma…you must be crazy going to see a shrink…but depression is a real disease
and it is treatable…”
“ Seek the support of your family and friends, talk to people in your family, have
people pray for you.”
“ I am extremely blessed to have some men friends, and we know how we are
as men and there is a rumor that men can’t talk to each other and share their
feelings.”

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010 1037
SICKLE CELL DISEASE SELF-MANAGEMENT
evaluate the use of vitamins and minerals on an individ-
ual basis.
Advocacy
All of the panel members were formally engaged in
many activities to help inform and educate others about
sickle cell anemia. Panel members were very engaged in
teaching clients and health care practitioners. They also
viewed these activities as helping achieve an optimal
state of health and quality of life. Self-advocacy was
important to ensure positive interactions with members
of the health care team.
Knowledge
Knowledge of federal employment rights and regula-
tions was identified as being important to protect against
potential illegal job loss. Panel members also acknowl-
edged the importance of understanding SCD and being
aware of individual baseline lab values (hemoglobin,
hematocrit). Participants also discussed the importance
of being able to articulate an accurate medical history,
including all complications.
Physical
All panel members identified the importance of par-
ticipating in some form of exercise as well as the impor-
tance of obtaining adequate sleep and rest. Clients iden-
tified days when they needed to stay in bed an entire day.
Everyone also identified times when they did not obtain
adequate rest which resulted in pain crises or other ill-
nesses. Finally, constipation prevention was also an
important theme. Some clients take opioids on a daily
basis, and even when not the case, this was identified as
an important strategy to avoid illness.
complementary and
Alternative Medicine
A wide variety of complementary and alternative
medicine strategies, such as acupuncture and massage,
were used by panel members.
table 2. Best Self-management Practices: Themes and Selected Quotes (cont)
emergent themes
and Subthemes Selected Quotes Illustrating emergent themes
Career selection and
success factors
Finances
Weather
Training
Flexibility
Proximity to
physician
Interest
Staying healthy
“ Resources for paying and staying in college, you want to consider federal and
state grants, those are things you don’t have to pay back…school-related funds
that you receive such as scholarships, grants, assistantships, work study…..”
“ A thing that you should consider when you are looking for programs is the school
conducive to your health. So if you have sickle cell disease, do you really want to
go to the University of Alaska?”
“ …online courses cut down on travel to campus, walking, being out in bad
weather conditions.”
“ So the main goal about education is that it comes in many different forms, not
just going to college. So it could also be apprenticeships or community college
or technical or vocational school as well.”
“ You may want to consider online courses.”
“ You need things that give you flexibility in the workplace, with your schedule…I
myself chose real estate. I work my own hours, I work at my own pace. I make
good money when I close…and I add it to that these are certificates that you
can go get and you can get them quickly to get started.”
“ How far is it from home because you can have a particular doctor that you are
attached to.”
“ You want to consider the areas of interests that you want to study….You want to
evaluate what your purpose is for returning to college. Is it something you want
to enhance the career that you already have? Do you want to gain new skills or
get a better job?”
“ When you seek employment the number 1 thing you have to do is get your
health in shape. You have to figure out how to manage your health so that you
can be a productive employee because there are no laws, you are subject to
the same rules everybody else is subject to when it comes to a job.”

1038 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010
SICKLE CELL DISEASE SELF-MANAGEMENT
dIScuSSIon
Initial reaction to these findings may lead the reader
to believe the panel participants were an aberrant cohort
of adults with SCD who were functioning at a higher
level than normal and must therefore not have a severe
form of SCD. As reported in Table 1, with the exception
of 1 participant, individuals had very few hospitaliza-
tions or ED visits. The question was asked with respect
only to the last year. Participants also reported a median
number of pain episodes of 3, which has been found to
be associated with more serious health outcomes.
Subjects also reported having multiple complications
throughout their lifetime. Most persons with SCD have
many years where their illness is more under control and
other years where their complications and pain episodes
are much more severe. This cohort was functioning at a
high level, however: (1) there are no comparison data
available to understand how the majority of persons with
SCD function or reason to suspect their disease was any
better or worse than most persons with SCD; and (2)
subjects only reported their pain episodes and emer-
gency department and hospital use within the past year.
Discussion with the subjects revealed that each person
had been very ill in the past, experienced many years of
very severe illness, and finally was able to learn how to
manage the disease—which are the reasons they were
selected to participate in the workshop.
The panel discussion revealed that effective self-
management of SCD involves several aspects, including
self-awareness, knowledge, advocacy, and a positive
attitude. The results indicate the importance of utilizing
a more holistic (mind, body, and spirit) approach to care
table 2. Best Self-management Practices: Themes and Selected Quotes (cont)
emergent themes
and Subthemes Selected Quotes Illustrating emergent themes
Nutrition
Diet
Herbal supplements
Vitamins
Asian practices
“ Food is one of the best medicines, eating wholesome balanced meals that
include fresh fruits, vegetables, whole grain nuts and beans is the first line of
defense…It is amazing now the additives and things that are in foods. So, if you
can eat a wholesome balanced meal on a regular basis….”
“ I have used dandelion and burdock as tonic over the years and it seems to be
a blood purifier and helps your liver….Goldenseal is good for colds and your
immune system, and ginger, and garlic is good for everything.”
“ I take vitamins C and B on a regular basis. B vitamins help with your energy levels,
and also folic acid…but I supplement a daily multiple vitamin, zinc, and a B
complex….Zinc really helps if you have a leg ulcer.”
“ …read about balancing your body, the yin and yang and being mindful of your
environment and how to balance yourself with the whole ecosystem…kind of
gave me insight into how to live in harmony with your environment and learn
about how the body works with the different cycles of life.”
Advocacy
Informing and
educating others
Self
Documenting illness
Filing employment
complaints
“ We have to get this information out to the medical staff because sometimes the
medical staff, are, you know, in nursing school—they don’t teach you anything
about sickle cell, like 2 paragraphs in a book.”
“ We have to do whatever it takes. Sometimes we have to be our own advocates.
If someone is not doing it we have to do it ourselves. Not everybody is going
to be caring about you or for you...you have to find your little niche in life and
make it work for you and live, live. You are living with sickle cell, you are not
dying of sickle cell.”
“ If you become sick within that period of time, then always be sure to have your
doctors notes, turn those in on time, and because of the ADA companies are not
supposed to discriminate against you.”
“ We have situations where there are people that have been fired because
they found out later on that they had sickle cell and they said well you didn’t
originally disclose that, so we are going to have to let you go. You do not have
to disclose in an interview and once you do, if you are fired for that, that is a
legitimate cause of action for you to go to the EEOC…”

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010 1039
SICKLE CELL DISEASE SELF-MANAGEMENT
for patients with SCD. Other important areas high-
lighted: the impact of SCD in career selection and suc-
cessful job performance and the importance of emo-
tional support through family, friends, and spiritual
outlets, following a healthy diet and obtaining adequate
rest and exercise.
Many of the self-awareness and emotional support
strategies identified by the panelist could be useful in
helping to support both the physical and emotional
health of patients with SCD. In particular, depression is
common among patients with SCD,8,9 and depression
and anxiety have been associated with lower physical
and mental health quality of life and a worse patient pain
experience. 10 Results from the current study suggest that
it may be beneficial for providers to encourage patients
to use journaling and other self-awareness activities to
improve their quality of life and gain a sense of per-
ceived ability to control their disease. An evaluation of
emotional and psychosocial functioning and availability
of information about resources for professional therapy
or counseling and support groups/mentorship programs
may also be helpful for some patients.
table 2. Best Self-management Practices: Themes and Selected Quotes (cont)
emergent themes
and Subthemes Selected Quotes Illustrating emergent themes
Knowledge
Federal
employment rights
and regulations
Sickle cell disease
Employer-specific
policies
“ Know your rights…..The Americans with Disabilities Act protects discrimination
in all employment practices…The Equal Employment Opportunity Commission
enforces the piece where all employers including state and local government
with 15 or more employees that EEOH enforces with them. And the Department
of Justice enforces when it comes to discrimination in state and local
governments.”
“ You need to know as much as you can about sickle cell…..know your levels,
your hemoglobin and all those different things, know about how the body works,
anatomy…know how the different organ systems are impacted so that you know
to have your gallbladder checked out or to have your eyes checked, or you
know, see a cardiologist….”
“ You want to do just basic things that all employees would do, go to work on
time, be proficient, don’t take long lunches, don’t show acts of insubordination,
just basic things.”
Physical
Heat
Exercise
Constipation
prevention
Sleep
“ …she still wants to put me into the hot tub.”
“ ..when we go to camp the kids come out of the pool we make them take a hot
shower to keep the temperature up.”
“ …try to do some type of exercise every day. Just get up and move around, um
walk if you can. Do yoga, stretching, some type of stretching, weighting training
if you can do it, join a gym. Use the sauna and the steam room to seat out
toxins and a Jacuzzi and whirlpool really help to relieve stress and ease muscle
tension.”
“ You take those pain medicines they will give you stomach problems. An
accumulation of waste in your colon has a bad effect on your overall health. It
is essential you have regular bowel movements…. To combat constipation take
plenty of water, eat high fiber foods, take like psyllium husk or flax for bulk or use
herbal laxatives.”
“ When you are tired you need to rest and don’t go beyond your endurance.
Sometimes I don’t feel well; I stay in bed all day. If I am tired I take a day off, I
have to recoup. If not, I am going to be in the hospital.”
“ If you snore go have a sleep test done and check for sleep apnea, because
that is something that I found was really important for me that has made one of
the biggest impacts on how I feel…I would wake up in the middle of the night
with sickle cell crisis because I wasn’t getting enough oxygen. So now, I use a
CPAP machine to sleep with.”

1040 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010
SICKLE CELL DISEASE SELF-MANAGEMENT
Academic and vocational issues are important for
patients with SCD because physical complications may
lead to academic failure, limited career options, and dis-
ability status.11 Further, there is a lack of appropriate
resources for patients with SCD, and academic and
career personnel may not be aware of the particular aca-
demic issues faced by these patients. 11 The panel discus-
sion on career-related concerns points to a need for pro-
viders to inquire about any issues related to career choice
and employment to help maximize patients’ ability to
seek meaningful work or pursue academics.
Nutrition and dietary intake were also identified as an
important issue. While the use of the vitamin/comple-
mentary medicine modalities was discussed at length,
it is important to understand that these therapies have
not been well studied in persons with SCD.Some
patients with SCD may reduce their caloric and macro-
nutrient intake during pain episodes, resulting in acute
malnutrition, decreased metabolism, and inability to
maintain a healthy weight. 12 Chronic anemia leading to
underweight and adequate nutrition are often challenges
for many patients. The importance of nutrition among
the panel members was evident. Referral to a nutritionist
may also be helpful for individual patients who have dif-
ficulty maintaining weight or who have poor dietary hab-
its. Physical activity was also identified as being an
important strategy for maintaining good health. However,
similar to patients with chronic pain, some patients with
SCD have a fear of movement because they fear physical
activity may lead to pain episodes. 13 Exploration of this
topic may be beneficial for some patients and referral to
physical therapy may also be helpful in certain cases,
especially to help treat chronic pain.
Finally, the role of advocacy and education was identi-
fied as being very important. Recently, the Sickle Cell
Summit group published a position paper emphasizing the
need for increased advocacy and education about SCD
and the unique role of the patient as an agent for change.14
The results from this study are consistent with stud-
ies involving another chronic disease, diabetes. While
there is no stigma associated with diabetes, SCD and
diabetes are both chronic diseases associated with many
serious comorbidities that benefit from patient self-man-
agement. Data have been demonstrated that effective
control of diabetes requires that patients be actively
engaged in the self-management of their health, and
patients’ feelings of self-efficacy and perceived control
over their illness both impact their ability to effectively
manage their chronic illness. 15-17 Sarkar et al found that
diabetic patients with higher levels of self-efficacy (the
level of confidence in one’s ability to perform health
behaviors) reported better diet, exercise, self-monitoring
of blood glucose, and foot care practices. 17 Jerant et al
explored the barriers to self-management of chronic dis-
eases and found that factors such as depression, poor
physician communication, low family support, and lack
of awareness were barriers to accessing and engaging in
self-management support resources. 15 Some of these
concepts were evident in the current study, including the
importance of compliance, a good working relationship
table 2. Best Self-management Practices: Themes and Selected Quotes (cont)
emergent themes and
Subthemes Selected Quotes Illustrating emergent themes
Complementary and
alternative medicine
Acupuncture
Massage
Reiki
Jinshinjitsu
Chiropractic
Visualization
“ I know a person that the way she controls the sickle cell she has acupuncture like
I guess every 2 weeks, and that is her therapy.”
“ Massage is a good therapy for sickle cell. It also gets rid of the toxins from the
body.”
“ Reiki is another healing system deals with… healing touch….it is just like laying
hands on people and I have seen it help to relax and actually remove pain.”
“ ..is sort of like reflexology….it is like a relaxation technique that deals with the
different pulse points of the body…the patients that tried this out found that they
were able to calm themselves down and feel more relaxed.”
“ I have used chiropractors before for back strains or whatever…sometimes they
are covered by insurance.”
“ I got a picture of red blood cells and put them up in my room. I put them in
different places where I could see them every day so that I would see a red
blood cell, what a healthy red blood cell looked like, and I visualized that is what
was going through my body.”

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 2010 1041
SICKLE CELL DISEASE SELF-MANAGEMENT
with health care providers, and professional and social
support. In addition, the panelists discussed at length
about using knowledge and awareness to gain control
over their illness in both their personal and professional
lives. Panelists specifically discussed the important role
of physician providers in the self-management of SCD.
Diabetes research has also demonstrated this impor-
tance. In a systematic review of interventions to support
diabetes self-management, the authors conclude that
physician self-management support can have a positive
impact on medication adherence, monitoring levels,
management of comorbid psychological conditions, and
a patient’s self-efficacy.16 Specifically, the quality of
patient-provider communication has been found to be
related to adherence and outcomes.18 Further, patient-
centered care, in which the provider supports patient
autonomy, has been found to be related to improved gly-
cemic control, 19 and provider participatory decision
making has been associated with higher self-manage-
ment assessments overall with chronic diseases. 20 These
findings, in addition to the current results, emphasize the
importance of the provider in creating an environment in
which patients feel understood, validated, and actively
engaged in their health self-management.
A strength of the study was that, to the best of our
knowledge, this was the first attempt to identify specific
strategies used by adult clients with sickle cell anemia
who successfully self-manage their disease. This was a
carefully selected panel of very high-functioning adults
with SCD. However, all clients reported being very ill
and having frequent pain episodes earlier in their life.
None of the participants reported ever being entirely
healthy and described a journey of self-awareness and
the development of a positive attitude that resulted in
taking control of the disease.
A limitation of the study was that it was not possible
to audiotape audience participation discussion, which
contributed to the overall richness of the workshop. In
general, the themes discussed in this manuscript were
acknowledged by audience members and the discussion
was similar. No additional major themes were remem-
bered by the investigator.
Future research is needed to assess motivation, self-
efficacy, and perceived control over illness in addition to
exploring the barriers to self-care management for those
patients who are not as successful. Also, designing inter-
ventions to teach effective self-management strategies
and improve utilization of these strategies may increase
self-management skills and improve overall quality of
life for patients with SCD.
AcKnoWledgMentS
We would like to thank Christian Billington and
Sickle Cell Disease Association of America for work-
shop coordination. We would also like to thank all pro-
viders and clients who participated in the workshop.
Finally, we would like to thank the following members of
the planning committee: Nicole Artz, David Bessman,
Letisha Collins, Pat Corley, Delbert DeFlora, Ronlanda
Foley, Melanie Gilmore, Elizabeth Gunier, Carlton
Haywood, J. Hoxi Jones, Terri Knowles, Liza LeGrier,
Alison Maynard-Thompson, Sola Odesina, Carolyn
Rowley, Craigie Sanders, and Campsey Thomas.
reFerenceS
1. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life
expectancy and risk factors for early death. N Engl J Med. 1994;330:1639-
1644.
2. Houston-Yu P, Rana SR, Beyer B, Castro O. Frequent and prolonged hos-
pitalizations: a risk factor for early mortality in sickle cell disease patients.
Am J Hematol. 2003;72:201-203.
3. McClish DK, Penberthy LT, Bovbjerg VE, et al. Health related quality of
life in sickle cell patients: the PiSCES project. Health Qual Life Outcomes.
2005;3:50.
4. Smith WR, Penberthy LT, Bovbjerg VE, et al. Daily assessment of pain in
adults with sickle cell disease. Ann Intern Med. 2008;148:94-101.
5. Pack-Mabien A, Labbe E, Herbert D, Haynes J, Jr. Nurses’ attitudes and
practices in sickle cell pain management. Appl Nurs Res. 2001;14:187-192.
6. Waldrop RD, Mandry C. Health professional perceptions of opioid
dependence among patients with pain. Am J Emerg Med. 1995;13:529-
531.
7. Fraenkel JR, Wallen NE. How to design and evaluate research in edu-
cation: McGraw-Hill Higher Education; 2009: www.mhhe.com/fraenkel7e.
Accessed June 17, 2009.
8. Alao AO, Cooley E. Depression and sickle cell disease. Harv Rev Psychia-
try. 2001;9:169-177.
9. Alao AO, Dewan MJ, Jindal S, Effron M. Psychopathology in sickle cell
disease. West Afr J Med. 2003;22:334-337.
10. Levenson JL, McClish DK, Dahman BA, et al. Depression and anxiety
in adults with sickle cell disease: the PiSCES project. Psychosom Med.
2008;70:192-196.
11. Day S, Chismark E. The cognitive and academic impact of sickle cell
disease. J Sch Nurs. 2006;22:330-335.
12. Pells JJ, Presnell KE, Edwards CL, et al. Moderate chronic pain, weight
and dietary intake in African-American adult patients with sickle cell dis-
ease. J Natl Med Assoc. 2005;97:1622-1629.
13. Pells J, Edwards CL, McDougald CS, et al. Fear of movement (kinesio-
phobia), pain, and psychopathology in patients with sickle cell disease.
Clin J Pain. 2007;23:707-713.
14. Hassell K, Pace B, Wang W, et al. Sickle cell disease summit: from clinical
and research disparity to action. Am J Hematol. 2009;84:39-45.
15. Jerant AF, von Friederichs-Fitzwater MM, Moore M. Patients’ perceived
barriers to active self-management of chronic conditions. Patient Educ
Couns. 2005;57:300-307.
16. Nuovo J, Balsbaugh T, Barton S, et al. Interventions to support diabetes
self-management: the key role of the patient in diabetes care. Curr Diabe-
tes Rev. 2007;3:226-228.
17. Sarkar U, Fisher L, Schillinger D. Is self-efficacy associated with diabe-
tes self-management across race/ethnicity and health literacy? Diabetes
Care. 2006;29:823-829.
18. Ciechanowski PS, Katon WJ, Russo JE, Walker EA. The patient-provider
relationship: attachment theory and adherence to treatment in diabetes.
Am J Psychiatry. 2001;158:29-35.
19. Williams GC, Freedman ZR, Deci EL. Supporting autonomy to motivate
patients with diabetes for glucose control. Diabetes Care. 1998;21:1644-
1651.
20. Heisler M, Bouknight RR, Hayward RA, Smith DM, Kerr EA. The relative
importance of physician communication, participatory decision making,
and patient understanding in diabetes self-management. J Gen Intern
Med. 2002;17:243-252.n