Primary Prevention of Stroke in Children With Sickle Cell Anaemia in Nigeria in the Community

The overall goal of this feasibility study is to establish a standard of care stroke prevention program for children with sickle cell anemia in a community hospital by task shifting stroke detection and transcranial Doppler ultrasound screening to nurses. In Nigeria, approximately 150,000 children with sickle cell anemia (SCA) are born annually, accounting for more than half of the total births with SCA worldwide. In comparison, only 1,700 children with SCA are born in the United States annually. An estimated 11% of unscreened and untreated children at increase of strokes with SCA will have at least one stroke by 17 years of age. In high-income countries, evidence-based practices (EBP) for primary stroke prevention in children with SCA involves screening for abnormal transcranial Doppler ultrasound (TCD) velocity (\>200cm/s) coupled with regular blood transfusion therapy for at least one year followed by treatment with hydroxyurea is considered standard care. This strategy decreases the risk of stroke by 92%. Due to safety and availability, regular blood transfusion is not a viable option for primary stroke prevention in most low-income settings, including Nigeria, where \~50% of the 300,000 children with SCA are born. Among each birth cohort, 15,000 children will have stroke annually in Nigeria. The American Society of Hematology (ASH) Central Nervous System Guidelines recommends moderate dose hydroxyurea (20mg/kg) to children with SCA with abnormal TCD measurements, living in resource-constrained settings where regular blood transfusions are not readily available. Our team has demonstrated in a previous trial the feasibility of primary stroke prevention with hydroxyurea in Kano, Nigeria. In 2016, as part of capacity building objective of Stroke Prevention Trial in Nigeria (1R01NS094041-SPRING) at Barau Dikko Teaching Hospital in Kaduna, TCD screening was adopted as standard of care. Before the trial, no TCD screening was done at our trial site in Kaduna. Now, as s