Partnerships and strategies to help define the burden of sickle cell disease in Uganda: The Uganda Sickle Surveillance Study (US3)

Over 300,000 babies are born with sickle cell disease (SCD) each year in sub-Saharan Africa, and most affected children die before age 5 years. The UN and WHO have declared SCD a public health priority in Africa, and recommend high-burden countries develop and implement SCD programs as part of national health strategies. In Uganda, the number of annual SCD births and distribution of sickle cell trait (SCT) and disease across the country are unknown, and there is lack of knowledge about sickle cell among healthcare providers and the public. Cincinnati Children’s Hospital Medical Center (CCHMC), in partnership with the Uganda Central Public Health Laboratories (CPHL), Ministry of Health (MOH), and Makerere University College of Health Sciences, devised a strategy to help define the burden of SCD by testing dried blood spots collected in the Early Infant Diagnosis Program from HIV-exposed children from all 112 districts of Uganda. In February 2014, a team from CCHMC trained CPHL personnel on isoelectric focusing (IEF) procedures to build local sickle cell laboratory capacity. Preliminary data document a high prevalence but substantial variation across the country, with SCT ranging from 2-25% and SCD ranging from 0-1.5%. The partnership is providing education to healthcare providers in high-burden districts to support pilot sickle cell newborn screening programs. Final prevalence estimates and geospatial distribution maps will inform the MOH about the current SCD burden in Uganda, and provide a framework for the development of a comprehensive national sickle cell strategic plan that includes diagnosis, care, and treatment programs.