Sickle Cell Anemia

Brett Bonetti,
Justin Christman,
Brandon Roberson,
Nicole Sellers
ETIOLOGY AND PREVALENCE

Sickle cell anemia (SCA): body produces sickle-
shaped (crescent) red blood cells

Normal red blood cells contain an iron-rich
protein, hemoglobin that causes the blood cells
to be disc-shaped

Sickle cells: abnormal hemoglobin (sickle
hemoglobin or hemoglobin S)
ETIOLOGY AND PREVALENCE
The most common theory on how sickle cell anemia began
involves people who lived in countries where malaria was a huge
cause of death, killing about 1.2 million people a year (The
Guardian, 2012)
Life-changing mutations to form sickle-cell shaped blood cells
Preventing them from hosting parasites, making them virtually
immune to malaria

ETIOLOGY AND PREVALENCE
Most commonly inherited blood disorder in the United States impacting up to
80,000 citizens, not including an additional 3 million people carrying the sickle
cell trait (Sickle Cell Disease, 2012)
ETIOLOGY AND PREVALENCE

1 out of every 500 African-
Americans (sickle cell trait affects 1
out of every 12)

1 out of every 1,000 to 1,400
Hispanic Americans (Sickle Cell
Disease, 2012).

During the course of 1999-2002
mortality rates dropped 68 percent
for children 0-3 years old, 39
percent for children 4-9 years old,
and 24 percent for children 10-14
years old (Data & Statistics, 2012)
DESCRIPTION OF CLIENT
8 year old girl with Sickle Cell Anemia
(African-American/Caucasian)
Family Dynamics: Mother- Caucasian (sickle cell trait) and
Father- African-American (sickle cell trait),
2 brothers and 1 sister
Home: 2 Bedroom Apartment
Location: South Side Chicago
Income: Father- works full time/ over time
Mother- works part time= (Low Income family)
Family Goals:
- Education
- Church
- Helping one another (SCA)

Average Medical Cost for
Sickle Cell Anemia Patients

- Total costs per patient-month were highest in adults, peaking at
$2,853 per patient-month for patients aged 30 to 39, and lowest in
children from birth through age 9, at $892 per patient-month
(Alicia).
- On an annualized basis, the total cost of care ranged from
$10,704 for children aged 0 to 9 years to $34,266 for the age 30 to
39.
- "For an average patient with sickle cell disease reaching age 45,
total undiscounted health care costs were estimated to reach
$953,640," the researchers report in the American Journal of
Hematology.
SOURCE: American Journal of Hematology, June 2009.

CLIENT (ALICIAS) DAILY ROUTINE
- Sleep
- Nutritious Well-Balanced Meal
- Medications
- Moderate Physical Activity
- Hydration throughout the day
Have a good relationship with your doctor and find out
ways to adjust your daily routine if needed

WHAT IS DEVELOPMENTALLY APPROPRIATE FOR AN 8-YEAR
OLD GIRL?
2nd grade
Games with rules, organized sports
Peer play, have consistent friends
Good dexterity for crafts/construction
Bilateral coordination, precision and motor planning
Run, hop, jump, skip, catch and throw ball with accuracy and precision
Abstract reasoning, complex problem solving
Cooperative, less egocentric
Behavior regulation
Competitive relationships
WHAT IS FUNCTIONALLY DESIRED FOR ALICIA?
Play organized sports, soccer
Play with friends, riding bikes, joking, etc.
Draw and build projects
Active playground time
Prolonged periods for reading
COMMON SYMPTOMS AFFECTING PERFORMANCE
Acute chest syndrome
Pain episodes
Stroke
Seizure
Organ damage or failure
Susceptibility to bacterial infections

AFFECTING PERFORMANCE
Acute chest syndrome:
Lung condition
Causes pain spells
May need blood transfusion
Leading cause of death for those with SCA
Limits aerobic activity, exercise and play
AFFECTING PERFORMANCE
Pain episodes:
Most common acute problem for SCA
Reversible pain in extremities, back, chest, or
abdomen
Mild pain for 5 minutes to excruciating pain for days or
weeks
Caused by inflammatory response to bone or bone
marrow necrosis, ischemic muscle or bowel from
obstruction and sludging of blood flow
Increased risk during physical or psychological stress
Need rest, hydration, pain medicine
AFFECTING PERFORMANCE
Stroke and Seizure:
Can occur if sickle cells block blood flow to an area of the brain
Seizure, weakness or numbness of arms and legs, sudden speech difficulties,
and loss of consciousness are signs of stroke
Immediate medical attention is needed, can be fatal
Can affect physical, cognitive and/or emotional health
AFFECTING PERFORMANCE
Organ damage or failure:
Sickle cells block blood flow through vessels depriving organ of blood and
oxygen
Chronic deprivation of O2 rich blood can damage nerves and organs including
kidneys, liver and spleen
Can be fatal
Long bouts of hospitalization, organ transplants


AFFECTING PERFORMANCE
Bacterial infections:
Lung infections extremely common, pneumonia causes hospitalization
Brain and spinal fluid infections, meningitis
Bone infections, Salmonella bacteria
Early detection, antibiotics
Germ-free, healthy environments

ASSESSMENT TOOLS
Child Occupational Self-
Assessment (COSA)
Series of statements relating to
everyday occupational
participation (school, home, and
community)
Self-rating system available in
two forms, card sort and
checklist
Permit the patient to record
their understanding of
occupational competence
and values using familiar
visual symbols and simple
language
ASSESSMENT TOOLS
Short Child Occupational
Profile (SCOPE)
Designed to record
progress towards
occupational therapy
intervention goals as well
as to screen for
occupational therapy
services.
Can be utilized for
children from birth
through 21 years of age
Includes a wide variety of
diagnoses and abilities
Very versatile
Scoring:
F (Facilitates): facilitates
participation in occupation
A (Allows): allows participation
in occupation
I (Inhibits): inhibits participation
in occupation
R (Restricts): restricts
participation in occupation
PRACTICE MODEL
Model of Human Occupation (MOHO)
Volition (process of how Alicia decides which
activities she wishes to engage in)
Habituation (process of how Alicia organizes
things she does into routines)
Performance capacity (addresses Alicias mental
and physical abilities and how she uses those
abilities to complete meaningful occupations)
Environment
INTERVENTION APPROACHES
Prevention of pain

Environmental

Hydration Levels

Colds and Infections

INTERVENTION APPROACHES
Maintenance of physical activity

Medications

Physical activity


ACTIVITIES TO PROMOTE HIGHER FUNCTION
Daily Physical Activity

Medication regimen

Proper nutrition

Daily planner
INTERVENTION CONSIDERATIONS
Heat
Water breaks
Monitoring of ambient temperatures
Massage
Relaxation exercises
Distractions
Deep breathing techniques
Pain Diary
FUN FACTS
Famous people with SCA
Tionne T-Boz Watkins (TLC)
Tiki Barber
Miles Davis
MULTIPLE CHOICE QUESTIONS
1. Sickle cell anemia mainly affects which people:
a. African-Americans
b. Hispanic-Americans
c. Girls
d. Boys
e. Both A and B
f. All of the above

MULTIPLE CHOICE QUESTIONS

2. In order to prevent a pain crisis a person with sickle cell
anemia can:

a. Keep hydrated
b. Maintain a moderate body temperature
c. Increase daily physical activity
d. Both A and B
e. None of the above

MULTIPLE CHOICE QUESTIONS

3. Persons with sickle cell anemia can suffer from:
a. Rapid bone growth
b. Internal organ failure
c. Impaired vision
d. Both A and C
e. Both B and C

REFERENCES
AJOT. (n.d.). AJOT. Retrieved December 1, 2013, from
http://ajot.aotapress.net/content/64/4/621
Scope. (n.d.). Scope Summary Form. Retrieved December 1, 2013, from
http://www.uic.edu/depts/moho/images/assessments/SCOPE%20Summary%20Rating
%20Form.pdf
American Occupational Therapy Association. (2008). Occupational therapy practice
framework:
Domain and process (2nd ed.). American Journal of Occupational Therapy, 62, 625-
683.
Boyse, K., Kelly, B. P., (May 2009). Pain in Sickle Cell Disease (Sickle Cell Anemia).
Med.umich.edu. Retrieved November 21, 2013, from
http://www.med.umich.edu/yourchild/topics/sicklecell.htm
Data & Statistics. (n.d.). Centers for Disease Control and Prevention. Retrieved from
http://www.cdc.gov/ncbddd/sicklecell/data

REFERENCES CONTD
Malaria kills twice as many people as previously thought, research finds. (2012, February 2).
The
Guardian. Retrieved November 24, 2013, from http://www.nhlbi.nih.gov/health/health-
topics/topics/sca/http://www.theguardian.com/society/212/feb/03/malaria-deaths-
researchcauses.html
Menaa, F. (2013). Stroke in sickle cell anemia patients: A need for multidisciplinary
approaches.
Atherosclerosis, 229, 496-503. Retrieved from
http://dx.doi.org/10.1016/j.atherosclerosis.2013.05.006
Rees, D. C., Williams, T. N., & Gladwin, M. T. (2010). Sickle-cell disease. The Lancet,
376(9757), 2018-2031. Retrieved from
http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(10)61029-X/fulltext
Sickle cell disease. (n.d.). Genetics Home Reference. Retrieved November 24, 2013, from
http://ghr.nlm.nih.gov/condition/sickle-cell-disease
Sickle Cell Disease Association of America, Inc.. (n.d.). Sickle Cell Disease Association of
America, Inc.. Retrieved from http://www.sicklecelldisease.org/
What Causes Sickle Cell Anemia?. (2012). NHLBI, NIH. Retrieved November 24, 2013, from
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/causes.html