Sickle cell disease in africa and the Arabian Peninsula: Current management and challenges

Abstract

Africa and the Arabian Peninsula are two regions of the world that are of particular interest in sickle cell disease. While the former has the highest burden of the disease in the world, the latter has the highest variety in terms of the genotypes, haplotypes and phenotypes that are encountered. The disease is usually severe in Africa because of complex interactions between genetic and environmental factors, but the Arabian Peninsula has a relatively mild expression because of the prevalence of the high-HbF phenotype, although the presentation is still quite heterogeneous, with some patients having a severe clinical course and developing complications. One major difference in the two regions is that the vast majority of African patients are homozygous, SS, while among Arabs, there is a high prevalence of other compound heterozygotes especially Sβ0 -thal. This chapter presents the contrasting pictures in terms of the epidemiology, clinical presentation, management practices and the prevailing challenges. It looks at the peculiar issues of resource limitation in Africa and outlines strategies that could surmount some of the challenges. While most of the countries in the Arabian Peninsula are endowed with the necessary resources, the wide variation in the phenotypic patterns poses challenges in adopting uniform control strategies.