Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease

Sickle cell anemia can only occur when two people who carry sickle cell trait have a child together. Genetic counseling is recommended for all carriers of sickle cell trait. About 1 in 12 African Americans has sickle cell trait.

It is possible to diagnose sickle cell anemia during pregnancy.

If you have sickle cell anermia, you can prevent the change in red blood cell shape by:

• Getting enough fluids
• Getting enough oxygen
• Quickly treating infections

Have a physical exam every 3 - 6 months to make sure that you are getting enough nutrition and activity, and that you are receiving the proper vaccinations. Regular eye exams are recommended.

PREVENTING CRISES

It is important to maintain good oxygen levels and to prevent dehydration. The following steps can help prevent a sickle cell crisis:

• Avoid strenous activities, stress, smoking, high-altitudes, nonpressurized flights, and other events that reduce your oxygen level
• Always have plenty of fluids with you
• Avoid too much sun exposure

Consider having the child with sickle cell anemia wear a Medic Alert bracelet. Share the above information with teachers and other caretakers, when necessary.

PREVENTING INFECTIONS

• People with sickle cell anemia need to keep their immunizations up to date to prevent illness.
• Some patients may receive antibiotics to prevent infections.

Lee MT, Piomelli S, Granger S, et al. Stroke prevention trial in sickle cell anemia (STOP): extended follow-up and final results. Blood. 2006;108:847-852.

Brawley OW, Cornelius LJ, Edwards LR, Gamble VN, Green BL, Inturrisi C, et al. National Institutes of Health consensus development conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med. 2008;148:932-938.

Geller AK, O'Connor MK. The sickle cell crisis: a dilemma in pain relief. Mayo Clin Proc. 2008;83:320-323.

Hebbel RP. Pathobiology of sickle cell disease. In: Hoffman R, Benz EJ, Shattil SS, et al., eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone;2008:chap 42.

Saunthararajah Y, Vichinsky EP, Embury SH. Sickle cell disease. Clinical features and management. In: Hoffman R, Benz Jr. EJ, Shattil SS, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingston; 2008:chap 43.

U.S. Preventive Services Task Force. Screening for Sickle Cell Disease in Newborns: U.S. Preventive Services Task Force Recommendation Statement. Agency for Healthcare Research and Quality, Rockville, MD. Sep 2007:AHRQ Publication No. 07-05104-EF-2.

Images

Red blood cells, normal

Formed elements of blood

Red blood cells, sickle cell

Red blood cells, multiple sickle cells

Blood cells

Red blood cells, sickle and pappenheimer

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Sickle cell anemia Treatment

Review Date: 02/28/2011
Reviewed By: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)