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

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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 100000 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.