Sickle cell disease in Britain

Abstract

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts for 80% of all acute admissions; 12% of admissions are for acute chest syndrome. Comparison of clinical features in Brent and in Jamaica shows that the Brent patients with homozygous sickle cell anaemia are admitted with painful crises more frequently than Jamaican patients. However, the frequency of admissions for chest syndrome and priapism, and the incidence of splenomegaly are similar. Leg ulcers are uncommon in Brent. Patients with sickle cell haemoglobin C disease appeared more severely affected in Jamaica than in Brent. During the past two years 3165 newborn babies have been screened for sickle cell disease at the Central Middlesex Hospital: five babies with homozygous sickle cell anaemia and three babies with sickle cell haemoglobin C disease were detected. The overall incidence of sickle cell trait was 3.2% and of haemoglobin C trait 0.8%. A significant number of babies with sickle cell disease are born in London every year. It is essential that such babies are detected at birth and offered prophylaxis against pneumococcal infection, which is one of the major causes of infant mortality. Sickle cell disease is becoming an important blood disease in Britain and firm guidelines for the management of acute and chronic complications are required.