Treatment of

Sickle Cell
Disease
Treatment
There is no available cure for Sickle Cell Disease so
the treatments are aimed at relieving the symptoms
and preventing complications.
Early diagnosis and prevention of complications is
critical in sickle cell disease treatment.
Management
Management of chronic pain syndromes
Management of chronic hemolytic anemia
Prevention and treatment of infections
Management of the complications and the various
organ damage syndromes associated with the
disease
Prevention of stroke
Detection and treatment of pulmonary
hypertension
Medications
Antibiotics (Penicillin) helps prevent infections, such
as pneumonia, which can be life-threatening to an
infant or child with sickle cell anemia.
Pain-relieving medications
Hydroxyurea for frequent and severe pain
Supplemental Oxygen - Dypsnea
Chronic Pain
long-acting oral morphine preparations
acetaminophen
NSAIDs - particularly effective in reducing deep bone
pain.
oral opiates - codeine and hydrocodone
Antidepressants
Nonpharmacological approaches - physical therapy and
acupuncture
Treatment of Acute Chest
Syndrome
Covers conditions characterized by chest pain, cough,
fever, hypoxia and lung infiltrates.
Treatment consists of oxygen, antibiotics, incentive
spirometry, simple transfusion, and bronchodilators.
Treatment measures include oxygen therapy with close
monitoring for hypoxemia with continuous pulse
oximetry-
antibiotics against S pneumoniae
Chronic Anemia
Because of the high RBC turnover, folate stores are often
depleted. Folic acid supplementation may raise the Hb level and
support a healthy reticulocyte response.
folic acid therapy:
For patients younger than 6 months, the usual dose is 0.1 mg per
day
For infants aged 6 months to 1 year, 0.25 mg per day
For children aged 1-2 years, 0.5 mg per day
For patients older than 2 years, 1 mg per day
Hydroxyurea
the only drug currently approved
by the US Food and Drug
Administration (FDA) for the
treatment of SCD
In babies, it works by stimulating
production of fetal hemoglobin
a type of hemoglobin found in
newborns that helps prevent the
formation of sickle cells.
Hydroxyurea increases total
hemoglobin.
Helps to prevent the cells from
sickling and improves anemia.
Blood Transfusion
A sudden worsening of anemia
Donated red blood cells are given intravenously to a
person with sickle cell anemia.
Blood transfusions increase the number of normal red
blood cells in circulation, helping to relieve anemia
Also help to prevent strokes.
Stem Cell Transplant
Stem Cell Transplant (bone marrow transplant) involves
replacing bone marrow affected by sickle cell anemia with healthy
bone marrow from a donor.
Recommended only for people who have significant symptoms
and problems from sickle cell anemia.
The diseased bone marrow is first depleted with radiation or
chemotherapy.
The healthy stem cells are injected intravenously into the
bloodstream of the person with sickle cell anemia, where they
migrate to the bone marrow cavities and begin generating new
blood cells.
The procedure requires a lengthy hospital stay.
References
1. Jeffrey L Arnold, M. F. (2011, july 11). Sickle Cell
Anemia. Retrieved november 1, 2011, from
Medscape:
http://emedicine.medscape.com/article/205926-
treatment
2. NIH . (2002, June). Retrieved 11 2011, from The
management of Sickle Cell Disease:
http://www.nhlbi.nih.gov/health/prof/blood/sickle/
sc_mngt.pdf
3. Lonergan, G. (2001-07). Sickle cell
anemia. Radiographics, 21(4), 971-94.
http://radiographics.rsna.org/content/21/4/971.full