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

BMJ 1995; 311 doi: https://doi.org/10.1136/bmj.311.7020.1600 (Published 16 December 1995) Cite this as: BMJ 1995;311:1600
  1. Allison Lee, medical studenta,
  2. Peter Thomas, statisticiana,
  3. Lena Cupidore, nurse practitionera,
  4. Beryl Serjeant, chief technologista,
  5. Graham Serjeant, directora
  1. a MRC Laboratories (Jamaica), University of the West Indies, Kingston, Jamaica
  1. Correspondence to: Professor G Serjeant.

    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.

    Footnotes

    • Funding None.

    • Conflict of interest None.

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