Diagnosis at Birth Improves Survival of Children With Sickle Cell Anemia

Abstract

The increased survival of children who have sickle cell disease is primarily due to state-of-the-art improvements in general pediatric medical care with particular emphasis on the management of the infectious complications that occur. Studies reported from New York City, Los Angeles, New Haven, and Jamaica clearly demonstrate the calendar era change in survival that has occurred during the 1970s and 1980s, and the greatest improvement is found among those children who have sickle cell anemia. The accurate identification of the specific hemoglobinopathy at or near birth provided the foundation for these studies documenting infant and young child mortality. In Africa, Molineaux et al and Fleming et al reported an epidemiologic investigation subsequent to a cord blood diagnosis program initiated in Garke, Nigeria, in 1976. A total of 534 infants were screened for major hemoglobinopathies, and 11 babies with SS and 125 babies with AS were identified. Minimal medical care was available for follow-up of the children. On entry to the school program at 5 years of age, the same population from the same small rural town was restudied. Only one child of 439 was found to have sickle cell anemia but 133 were AS. The inescapable conclusion was that the African babies with SS had died during early childhood, contributing disproportionate numbers to the high infant and childhood mortality in Nigeria. Not until Dr Fleming and the investigators of the British Medical Research Council had performed a cord blood hemoglobinopathy surveillance program was the incidence of sickle cell anemia and its effect on childhood mortality in Nigeria documented.