The Role of Preventive Medicine in Sickle Cell Disease: The Watson Smith Lecture

Homozygous sickle cell disease is one of the most common genetic abnormalities in the world; it affects approximately 100,000 births annually in Nigeria alone. With this scale of public health problem, the newer high technology approaches to prevention, such as antenatal diagnosis, or to treatment, such as bone marrow transplantation, are unlikely to be affordable or to have a discernible population impact. Experience in Jamaica suggests that many low technology affordable procedures can markedly reduce the morbidity and mortality of the disease. Furthermore, certain aspects of current management in the United Kingdom, such as frequent hospital admission and transfusion, not only consume scarce resources but may increase morbidity. Prophylactic penicillin to prevent pneumoccocal complications, education of parents so as to reduce the mortality from acute splenic sequestration, awareness of the features and epidemic pattern of aplastic crises, and exchange transfusion for the acute chest syndrome represent cost-effective measures which may markedly influence outcome. Reducing dependency on inpatient facilities might actually reduce morbidity and save resources that could be used to improve day care and outpatient management.