The prognosis of cystic fibrosis in the Western Cape region of South Africa

Objective : To study the prognosis of cystic fibrosis (CF) in South Africa.
Methodology : Retrospective chart review of 102 children with CF over 20 years.
Results : Survival at 18 years was 54% (95% confidence intervals 30–79). Prognosis was not influenced by gender or genotype but survival was noted to be worse for Cape Coloured children than for European children. This appeared mainly to be due to the death of eight Cape Coloured infants, four of whom presented with oedema and anaemia, mimicking kwashiorkor. Infection with Pseudomonas aeruginosa occurred earlier in Cape Coloured children than European children (median, 1 vs 4 years) and they had a worse 5-year survival (56 vs 89%, P < 0.05) after infection. Of the 40 children born in the second decade studied, six Coloured and no European children died.
Conclusions : Ethnic differences in prognosis exist for children with CF in South Africa and are probably related to underrecognition of CF and the socio-economic status of some patients.