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Hydroxyurea therapy improves survival in most severely affected sickle cell patients

the treatment of sickle cell anemia. "Presently only a minority of eligible patients are taking hydroxyurea. Our study strongly suggests that adults with sickle cell anemia and clinically significant complications of their disease, like frequent painful episodes and the common pneumonia-like events, should take hydroxyurea under the supervision of a knowledgeable physician," Steinberg said.

"Most people who take this medication will feel better; we now know they will live longer," Steinberg added. Patients in the study will be followed for an additional 5 years, noted Bonds, who also added that hydroxyurea studies in children are planned.

Sickle cell anemia is an inherited disease that is most common among people whose ancestors come from Africa, the Middle East, the Mediterranean basin, and India. In the U.S., it affects primarily African Americans, about 72,000 of whom have the disease. One in 12 African Americans carries the sickle cell trait.

In patients with the disease, molecules of sickle hemoglobin stick to one another, forming long rods inside red blood cells and causing these cells to take on a sickle shape and become rigid. The sickled red cells are unable to squeeze through tiny blood vessels, depriving tissue of an adequate blood supply and causing painful episodes.


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Contact: NHLBI Communications Office
301-496-4236
NIH/National Heart, Lung, and Blood Institute
1-Apr-2003


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