Personal practice Practical management of sickle cell disease

Sickle cell disease is a common problem in several inner city populations in this country, but the overall incidence is low. It has been estimated that 5000 people in the United Kingdom have the disease, at least 2000 of these living in London alone. ' Sickle haemoglobin results from a single amino acid substitution in the 0 globin chain. The disease is inherited and the important sickling syndromes are homozygous sickle cell disease (Hb SS) and the double heterozygotes, haemoglobin SC disease (Hb SC) and sickle i thalassaemia. Hb SC disease and sickle thalassaemia tend to be milder than Hb SS. There is tremendous variability in the severity of sickle cell disease within all these groups. Some patients are plagued by constant hospital admission