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Improved survival in homozygous sickle cell disease: lessons from a cohort study
1MRC Laboratories, (Jamaica), University of the West Indies, Kingston, Jamaica.
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.