AN INVESTIGATION INTO CAUSES OF SICKLE CELL ANEMIA IN CHILDREN OF 0-5 YEARS IN OFFA LOCAL GOVERNMENT AREA OF KWARA STATE, NIGERIA.

Sickle cell disease (SCD) IS an umbrella term for a group of life long debilitating autosomal recessive disorders that are caused by a single point mutation (GLU-Val) that result in poly merization of hemoglobin (Hb) and reversible sickle -shap? deformation of crythrocytes. This lead to increase hermolysis of erythrocytes and micro vascular occlusion, inschemia - reperfusion injury, and tissue infarction, ultimately causing multi-system end organs complication. Sickle celi aneamia (Hbss) is the most common and severe genotype of SCD, followed by Hbsc, Hbs B° thalassemia and rare and being genotype clinical manifestation of SCD occur early in life, are variable, and modified by several genetic and environment factors. Nearly (500) children with SCD continue to die prematurely every day, due to comprehensive care in sub -Saharan African (SSA), A trend that need to be urgently reversed. Despite proven efficacy in developed countries newborn screening program are not universal in SSA. This call for a consolidation effort to make this possible through the use of rapid, accurate, and cheap point of care text kit which require minimal training. For almost two decades, hydroxyurea (hydroxyl cerbamide) a century - old during. was the only disease and modifying therapy approved by the U.S food and drug administration. Recenty, the list expended to L-glutamine, crizanlizumah and voxelotor, with several promising novel therapist in the pipeline. Despite its several limitations, hematapoietic stem cell transplant (HBCT) remains the only curative intervention for SCD. Meanwhile recent advances in gene therapy trials offer a glimpse of hope for the nearest future, although its use maybe limited