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

Abstract

Objective: To examine whether simple interventions in a sickle cell clinic improve survival in sickle cell disease.

Design: Survival curve analysis and hazard ratios in a cohort study followed from birth.

Setting: MRC Laboratories (Jamaica) at the University of the West Indies, and Victoria Jubilee Hospital, Kingston, Jamaica.

Subjects: 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.

Interventions: Prophylactic penicillin to prevent pneumococcal septicaemia, parental education in early diagnosis of acute splenic sequestration, close monitoring in sickle cell clinic.

Main outcome measures: Survival.

Results: 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).

Conclusion: Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.