ABSTRACT

DIAGNOSIS The diagnosis is made by hemoglobin electrophoresis, according to the definition above. In all 50 U.S. states, newborns are screened for sickle cell disease at birth.

EPIDEMIOLOGY/INCIDENCE Sickle cell disease occurs in about 1 and 600 AfricanAmericans. Sickle cell trait occurs in 1 and 12 African-Americans, resulting in the birth of approximately 1100 infants with sickle cell disease annually in the United States. HbSS accounts for 60% to 70% of sickle cell disease in the United States. The prevalence of sickle cell disease and patients with sickle cell trait is highest in West Africa (25% of the population have one mutation), Mediterranean, Saudi Arabia, India, South and Central America, and Southeast Asia (1,6).