Management of sickle cell disease
There are many effective treatments for the complications of sickle cell anemia, but many children in developing countries do not have access to these treatments. Sickle cell anemia is caused by a defect in hemoglobin, which produces a type of hemoglobin called hemoglobin S. This abnormal hemoglobin causes red blood cells to form a sickle shape, which in turn causes them to get caught in small blood vessels. This causes most of the complications of sickle cell anemia. Analgesics, blood transfusions, hydroxyurea, and bone marrow transplantation are common treatments. Experimental treatments are under development.
Publication Name: The New England Journal of Medicine
Pathophysiological-based approaches to treatment of sickle cell disease
This review discusses the treatment methodology for sickle cell disease based on its pathological and physiological manifestations. Such treatment options include inhibiting sickle hemoglobin, preventing or repairing erythrocyte dehydration, and replacing sickle hemoglobin-producing erythroid progenitors with stem cells. Gene therapy, endothelium protection, and inhibition of oxidative dmage are also envisaged.
Publication Name: Annual Review of Medicine
Hydroxyurea and sickle cell disease: a chance for every patient
All adult patients with sickle cell anemia who have at least three painful crises a year should take the drug hydroxyurea. This drug increases blood levels of fetal hemoglobin, which compensates for the defective hemoglobin that causes this disease. A study published in 2003 found a 40% reduction in mortality rates among sickle cell patients who took the drug. The drug appears to have few serious side effects.
Publication Name: JAMA, The Journal of the American Medical Association
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