Open Splenectomy in Jamaican Children with Sickle Cell Disease

Jamaican patients with homozygous sickle cell (SS) disease are at greatest risk of death from ASS between the ages of 6 and 12 months (4, 5). Open splenectomy has been performed with low morbidity in Jamaican children with the SS genotype (1, 6, 7–9), which occurs once in every 300 Jamaican births (10) Sickle cell disease includes not only genotypic homo-zygotes (SS) but also the double heterozygotes (SC, S β0 and S β+ thalassaemias) (10, 11). These heterozygotes to varying degrees also develop splenic complications. Haemoglobin SC disease, which occurs once in every 500 Jamaican births, causes splenic complications only rarely (10). The genotypes S β0 thalassaemia and S β+ thalassaemia are rare in the Jamaican population, occurring once in every 7000 and once in every 3000 births respectively, but their propensity for splenic complications is high (10). This paper reviews the recent experience at the Uni-versity Hospital of the West Indies (UHWI) with open splenectomy, in children with homozygous and heterozygous sickle cell disease