Gaby Harder
September 22, 2020

Sickle cell disease (SCD), an inherited blood disorder, is prevalent throughout sub-Saharan Africa. Approximately 240,000 children are born with SCD each year across the continent of Africa, and up to 70% of those infants born with sickle cell disease die before the age of five. 

There have been significant improvements in mortality rates for children with SCD in developed countries with high access to resources and robust healthcare systems. By implementing newborn screening programs, regular blood transfusions, and hydroxyurea therapy, 99% of the children born with SCD in countries like the United States have promising prognoses. While these interventions would result in similar benefits if implemented in Africa, most countries are resource-limited, making these treatments unfeasible and mortality rates much higher. 

The NIH and NINDS funded Stroke Prevention in Nigeria (SPIN) trial sought to address the treatment gap in children with SCD in Africa, as limited research existed before this study on inexpensive alternatives to blood transfusion therapy for primary stroke prevention. Dr. Aliyu, Dr. DeBaun, and their colleagues in Nigeria concluded that a moderate fixed dose of hydroxyurea was effective in preventing strokes in Nigerian children with sickle cell disease. Minimizing the costs and maximizing the convenience of hydroxyurea was also an essential focus of the study. For the SPIN trials, the drug was produced locally in Nigeria for USD 4.00 per 30 tablets, ensuring that the treatment was optimized for accessibility. 

These results are encouraging as the usage of hydroxyurea is a feasible treatment plan that can be executed in countries with limited resources, while also being successful in preventing strokes, which can be fatal. This research will transform the treatment trajectories for the thousands of children with SCD in sub-Saharan Africa. 

Source: American Journal of Hematology