ABSTRACT
Sickle cell disease (SCD) is the most important genetic disorder in Sub-Saharan Africa (SSA), where over 300,000 affected children are born every year. Although children with SCD represent 1–2% of all births within the region they account for between 10% and 25% of all blood transfusions administered to children <5 years old. While recent improvements in the diagnosis and management of SCD have resulted in survival improvements in Africa, this has led to increasing levels of hospital admission for the management of crises, which often involve transfusions. Better management and diagnosis of SCD has also resulted in improved survival of individuals affected by the condition.
Blood transfusions are a critical component of the management of SCD for the resolution of anemia and/or SCD-related complications. The aim of the transfusion is to replace sickle shaped red blood cells with normal red blood cells that are more deformable.
Despite acute blood shortages experience on the continent, about 30–90% of SCD admissions in SSA receive a transfusion (Diop & Pirenne, 2021). Unlike SCD patients in developed countries those in SSA present with infections like malaria and/or acute hemolysis. Frequent transfusions can lead to iron overload as well as erythrocyte alloimmunization, delayed hemolytic transfusion reactions, and hyper-hemolysis.
