sickle cell disease
There is no cure for the disease, but advancements in treatment have improved median survival to 42 years for men and 48 years for women. Cerebral hemorrhage or shock is the usual cause of mortality in children. Recent studies have indicated that regular blood transfusions can prevent strokes in children. Anemia is treated with folic acid. Sickle cell crises may be treated with intravenous hydration, pain medication, antibiotics, oxygen, and transfusions. Hydroxyurea, formerly used as a cancer treatment, has been helpful to many adults with the disease, lessening the frequency and severity of crises. New drugs for reducing the severity of crises are being tested as well. One acts as a lubricant, allowing sickled cells to flow more easily through tiny vessels. The other helps to prevent tissue deprived of blood from dying during a crisis.
The Columbia Electronic Encyclopedia, 6th ed. Copyright © 2012, Columbia University Press. All rights reserved.