Management of Sickle Cell Disease

One of every 600 black people in the United States has sickle cell anemia. In addition, sickle cell–hemoglobin C disease and sickle cell–β-thalassemia, which are other common genotypes of sickle cell disease, together are as common as sickle cell anemia. Sickle hemoglobin (hemoglobin S, α2 β2S) accounts for over half the hemoglobin in patients with these disorders. Eight percent of black Americans are heterozygous carriers of the sickle cell trait; about 40 percent of their hemoglobin is hemoglobin S. They do not have anemia and need neither treatment nor occupational restrictions. About 5 percent have hematuria at . . .