Improved survival in homozygous sickle cell disease: lessons from a cohort studyBMJ 1995; 311 doi: https://doi.org/10.1136/bmj.311.7020.1600 (Published 16 December 1995) Cite this as: BMJ 1995;311:1600
- Allison Lee, medical studenta,
- Peter Thomas, statisticiana,
- Lena Cupidore, nurse practitionera,
- Beryl Serjeant, chief technologista,
- Graham Serjeant, directora
- Correspondence to: Professor G Serjeant.
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.
Conflict of interest None.