Public health implications of sickle cell disease

Sickle cell disease (SCD), a genetic blood disorder caused by abnormal hemoglobin that damages and deforms red blood cells, affects millions of people throughout the world. Approximately 80,000 Americans are estimated to have SCD and approximately 2 million are estimated to carry sickle cell trait (SCT), making sickle cell the most common hereditary blood disorder in the US. The abnormal red cell break down that occurs in SCD can lead to anemia, recurrent episodes of severe pain and multiorgan ischemic damage. Although individuals with SCT are generally healthy, growing evidence suggests that there is increased risk of certain morbidities such as thrombosis and stroke among individuals with SCT. In SCD, clinical severity varies and although symptomatic treatments exist, there is no cure for SCD. There has been extensive clinical and basic science research in SCD, however many public health issues such as blood safety surveillance, stroke prevention, pregnancy complications, preconception health, quality of life, and thrombosis in people with sickle cell trait remain unaddressed. Currently, efforts are under way to strengthen SCD-related activities within the Centers for Disease Control and Prevention (CDC). To date, several activities are being or have been conducted by centers within CDC, including quality assurance of newborn screening tests for SCD, morbidity and mortality studies, genetic studies, and studies focusing on the protective effects of SCT for malaria. This presentation will discuss the public health implications of SCD and the future directions that can be taken to begin to address some of these issues.