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Overview of the management and prognosis of sickle cell disease

Joshua J Field, MD
Elliott P Vichinsky, MD
Michael R DeBaun, MD, MPH
Section Editors
Stanley L Schrier, MD
Donald H Mahoney, Jr, MD
Deputy Editor
Jennifer S Tirnauer, MD


Vasoocclusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease (SCD). Vasoocclusion results in recurrent painful episodes (previously called sickle cell crisis) and a variety of serious organ system complications that can lead to life-long disabilities and even death. Hemolysis of red blood cells (RBC) causes chronic anemia and pigment gallstones. (See "Overview of the clinical manifestations of sickle cell disease".)

There are multiple components to the management of SCD, including the prevention and treatment of the complications of SCD, as well as the potential cure for this illness:

Prevention of complications – Primary prevention of the acute complications of SCD includes routine health management with a hematologist or a health care provider with expertise in SCD. Initial prevention of complications includes the use of penicillin prophylaxis started in the newborn period, appropriate immunizations, and blood transfusions for those at risk for stroke. (See 'Infection prevention' below.)

The only US Food and Drug Administration approved therapy to prevent pain episodes in SCD is the use of hydroxyurea to reduce sickle hemoglobin polymerization process by increasing the production of fetal hemoglobin [1]. This important component of the management of SCD is discussed separately. (See "Hydroxyurea and other disease-modifying therapies in sickle cell disease", section on 'Use of hydroxyurea'.)

Treatment of complications – Several treatments are available for the complications of SCD, such as pain medications for vasoocclusive events and antibiotics for infection.

Cure – A life-long cure for SCD is available only through hematopoietic cell transplantation. However, this treatment is primarily limited to individuals under 16 years of age due to the risk of severe toxicities and death among individuals >16 years of age with an HLA-matched sibling transplant. This issue is discussed in detail separately. (See "Hematopoietic cell transplantation in sickle cell disease".)


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Literature review current through: Sep 2016. | This topic last updated: Sep 30, 2016.
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