Educational Series: Hydroxyurea Use in Children with Sickle Cell Disease, Congestive Cardiac Failure in Children, Management of Coma in Children and Oesophageal Atresia

Abstract

Sickle Cell Disease (SCD) is a global health problem, although about 90% of the burden occurs in Low-and Middle-Income Countries (LMICs). Globally, about 300,000 babies are born with the disease annually, with about 150,000 of them in Nigeria alone. The Nigeria 2018 Demographic Health Survey estimated the average birth prevalence of homozygous sickle cell disease (HbSS) as 1·21% and 0·24% for the heterozygous state HbSC.^1,2  SCD is a single gene mutation characterized by GAG to GTG transversion resulting in the replacement of soluble glutamic acid by insoluble valine at the sixth position of the β-globin chain (β6 Glu →Val, GAG>GTG).³ Clinically, it typically manifests as episodic ischaemia and hypoxia, resulting in recurrent pains (the disease hallmark), chronic haemolysis and increased predisposition to infections.