Update: newborn screening for sickle cell disease--California, Illinois, and New York, 1998.

Sickle cell disease (SCD) is a common single-gene disorder that affects three of every 1000 black newborns and approximately 50,000 persons in the United States. Children affected with SCD are at increased risk for severe morbidity (e.g., severe hemolytic anemia, splenic dysfunction, pain crises, and bacterial infections) and mortality, especially during the first 3 years of life. In 1993, California, Illinois, and New York collectively accounted for approximately 20% of all births to blacks. All three states offer universal newborn screening for hemoglobinopathies. To assess the effectiveness of newborn screening programs for SCD and for receipt of and compliance with early medical interventions (e.g., penicillin prophylaxis and pneumococcal vaccination and other vaccination patterns), a 3-year collaborative follow-up study was conducted from 1995 through 1998 in California, Illinois, and New York. This report summarizes the results of this study, which demonstrate the difficulty in retrospectively finding children who were screened at birth so that data for evaluating program effectiveness can be assessed.