Sickle cell disorders are very common in Africa. In West Africa, 15-20% of the coastal and forest populations have sickle cell trait and 1% of all babies have a form of sickle cell disease (SCD). In most African countries SCD is not considered a major public health problem because the impact of SCD on childhood mortality has not been carefully studied. In some parts of West Africa it is estimated that 98% of children with SCD do not live past 5 years of age. In Africa, the leading cause of death in children with SCD is malaria, followed by pneumococcal infection. The effect of malaria makes the clinical course of SCD in Africa quite different from that in the U.S. The reasons underlying this increased risk of death due to malaria and the extent of the problem have not been carefully studied. Young children with SCD in Africa, like their counterparts in the U.S., face increased mortality from infection. In the U.S. this problem is being addressed through newborn screening and penicillin prophylaxis therapy. In fact, most children born with SCD in Africa are not diagnosed prior to serious illness and death. This is because there are no programs organized for the screening of newborns for SCD, and there are no longitudinal studies in which children with SCD have been followed closely from birth to collect accurate data on the clinical course of SCD from infancy. It is only through such programs that the true morbidity and mortality of SCD in the African environment can be determined. The main objective of this project is to design and implement a pilot newborn screening program for SCD in Kumasi, the second largest city in Ghana as a demonstration project for West Africa. Secondary objectives are to &fin the staff who will work in the screening project, and to design a longitudinal study of the clinical course of SCD into which babies found to have SCD in early childhood will be enrolled for long-term close monitoring. The project is planned as a collaborative one between this Sickle Cell Center and the Noguchi Memorial Institute for Medical Research in Ghana, with participation by the Ministry of Health of Ghana and the School of Medical Sciences in Kumasi. Laboratory support for the project will be based at Noguchi and the clinical services will be based at Komfo Anokye Teaching Hospital in Kumasi. Technical assistance will be provided by this Center and the Core Laboratory (Project 9001) will serve as a reference laboratory for the laboratory at Noguchi. In conjunction with this project, other research studies to study the effects of malaria on children with SCD, and the efficacy of antimalarial and penicillin prophylactic therapy are planned.
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