Improved survival in homozygous sickle cell disease: lessons from a cohort study.

Abstract

To examine whether simple interventions in a sickle cell clinic improve survival in sickle cell disease. Survival curve analysis and hazard ratios in a cohort study followed from birth. MRC Laboratories (Jamaica) at the University of the West Indies, and Victoria Jubilee Hospital, Kingston, Jamaica. 315 patients with homozygous sickle cell disease detected during the screening of 100,000 consecutive non-operative deliveries between June 1973 and December 1981 at the main government maternity hospital, Kingston, Jamaica. Prophylactic penicillin to prevent pneumococcal septicaemia, parental education in early diagnosis of acute splenic sequestration, close monitoring in sickle cell clinic. Survival. Survival appeared to improve, the log rank test for trend comparing the first, second, and last third of the study reaching borderline significance (P = 0.05). Combined deaths from acute splenic sequestration and pneumococcal septicaemia-meningitis declined significantly (test for trend, P = 0.02). Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.