Mortality among children with sickle cell disease identified by newborn screening during 1990-1994--California, Illinois, and New York.

Sickle cell disease (SCD) is an autosomal recessive disorder characterized by production of abnormal (sickle) hemoglobin, resulting in anemia, susceptibility to pneumococcal and other infections, pain, stroke, and multiple organ dysfunctions. The most common types include hemoglobin SS (homozygous) disease, sickle cell-hemoglobin C disease, and the sickle beta-thalassemia syndromes. A randomized controlled trial published in 1986 indicated that daily oral penicillin prophylaxis reduced the incidence of serious infection in young children with SCD and led to widespread adoption of newborn screening programs for SCD. To study the effectiveness and utilization of prevention programs among large populations of infants with SCD, several newborn screening programs in the United States are now attempting to determine rates of complications and actual use of early medical interventions (e.g., penicillin prophylaxis and pneumococcal vaccination). This report focuses on recent mortality in California, Illinois, and New York. In California and Illinois, mortality from all causes among black children born during 1990-1994 with SCD was slightly less than overall mortality for all black children born in the same time period.