Sickle cell haemoglobinopathies in England.

Abstract

Ninety-six Birmingham children with sickle cell disease were studied prospectively between 1969 and 1979. Thirty-five were homozygotes for HbS (SS), 12 had sickle thalassaemia (S thal), and 23 were double heterozygotes for HbS and C (SC). Twenty-six whose family studies were incomplete were classified as SS or S thal although most were thought to be SS. The average length of follow-up was 5.1 years. Four SS children and 1 SC child died, the annual mortality rates being 1.3% for SS and presumed SS, 0% for S thal, and 0.9% for SC children. The incidence of pulmonary illnesses and anaemic crises was greater than reported from Jamaica, while leg ulceration described there and in New York was not observed in Birmingham. Severe infections were less common than in the series reported from New York and no case of salmonella osteomyelitis was observed in Birmingham. In general the S thal and SC children had milder illnesses than the SS, and the SS children often showed impairment of growth and sexual maturation.