Lung transplantation and life extension in children with cystic fibrosis

Background Lung transplantation has been available as therapy for end-stage lung disease since the early 1980s, but survival after transplantation rem ains poor, with continued controversy as to the survival benefit from the p rocedure. We examined the effect of lung or heart-lung transplantation on t he survival of a cohort of children with cystic fibrosis and severe lung di sease. Methods Between May, 1988, and May, 1998, 124 children with cystic fibrosis were accepted for lung transplantation. 47 received transplants, 68 died w hile they awaited organs, and nine remained on the active waiting list. We constructed a proportional-hazards model that used variables of prognostic significance in this population. By including transplant status as a time-d ependent covariate, we were able to calculate a hazard ratio for transplant ation. Date of entry into the study was the dale when children were added t o the list for transplantation, and measurements were taken at this time. C hildren were accepted for transplantation if they had a life expectancy of 2 years or less, a poor quality of life, and no contraindications to transp lantation. Findings After 1 year, 35 (74%) children were still alive; after 5 years 12 (33%) children were alive. The univariate hazard ratio for transplantation was 0.41 (95% CI 0.23-0.74; p=0.003). Transplantation remained significant ly associated with survival after correction for differences in age, sex, h eight-corrected forced expiratory volume in 1 s, minimum oxygen saturation during a 12 min walk, haemoglobin concentration, albumin concentration, and age-corrected resting heart rate (hazard ratio 0.31 [0.13-0.72]; p=0.007). Interpretation If centres follow our criteria for accepting patients for tr ansplantation, and achieve similar survival after transplantation, they cou ld expect a survival benefit for their patients in line with our results.