Improved survival in homozygous sickle cell disease: lessons from a cohort study.
OBJECTIVE
To examine whether simple interventions in a sickle cell clinic improve survival in sickle cell disease.


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


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


METHODS
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.


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


METHODS
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).


CONCLUSIONS
Early diagnosis and simple prophylactic measures significantly reduce deaths associated with homozygous sickle cell disease.
Lee
A
A
MRC Laboratories, (Jamaica), University of the West Indies, Kingston, Jamaica.
Thomas
P
P
Cupidore
L
L
Serjeant
B
B
Serjeant
G
G
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D006720:Homozygote; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007563:Jamaica / Q000453:epidemiology; D008581:Meningitis / Q000401:mortality / Q000517:prevention & control; D010290:Parents / Q000193:education; D010406:Penicillins / Q000627:therapeutic use; D011008:Pneumococcal Infections / Q000401:mortality / Q000517:prevention & control; D016016:Proportional Hazards Models; D018805:Sepsis / Q000401:mortality / Q000517:prevention & control; D013158:Splenic Diseases / Q000175:diagnosis / Q000401:mortality; D015996:Survival Rate
D010406:Penicillins