Abstract

Sickle cell disease is a genetic blood disorder affecting Red-blood cells. Is an umbrella term for a group of life-long debilitating autosomal recessive disorders that are caused by a single-point mutation (Glu→Va) that results in polymerization of hemoglobin (Hb) and reversible sickle-shape deformation of erythrocytes. The most common form of SCD is caused by homozygosity for the -globin S gene mutation (SS disease). The United Nations has recognized SCD as a global public health concern, and the World Health Organization (WHO) recommends that 50% of member states will have established SCD control programs by 2020 (World Health Organization, 2006) [1] is the central pathophysiology of this disease, although the importance of chronic anemia hemolysis and vasculopathy has been establish. One of the main problems of sickle-cell disease in children is the development of cerebrovascular disease and cognitive impairment, and the role of blood transfusion and hydroxycarbamide for prevention of these complications is starting to be understood. Recurrent episodes of vasoocclusion and inflammation result in progressive damage to most organs, including the brain, kidneys, lungs, bones, and cardiovascular system, which becomes apparent with increasing age [2]. Clinical manifestations of SCD occur early in life, are variable and are modified by several genetic and environmental factors. Nearly 500 children with SCD continue to die prematurely every day, due to delayed diagnosis and /or lack of access to comprehensive care in sub-Saharan Africa, a trend that needs to be urgently reversed [3]. A simple representative and affordable approach to estimate SCD child mortality is to test blood specimens already collected through large population surveys targeting conditions such as HIV, malaria, malnutrition, and covering children of varying ages. Thus, although there is enough evidence to justify investments in screening, prophylaxis, and treatment for African children with SCD, better data are needed to estimate the numbers of children death preventable by such interventions, and their cost effectiveness [4].