PURPOSE: To determine the effect of lung transplantation on survival of pediatric patients with cystic fibrosis (CF).
METHODS: We identified patients less than 18 years of age that were listed for lung transplantation from 1991 through 2002. Using Cox proportional hazards with time-dependent covariates, we examined the effect of multiple covariates and lung transplantation on survival. The data reported here were supplied by the CF Foundation Patient Registry and the United Network for Organ Sharing as the contractor for the Organ Procurement and Transplantation Network (OPTN). The interpretation and reporting of these data are the responsibility of the authors and in no way should be seen as an offical policy or interpretation by OPTN or the US government.
RESULTS: We identified 558 patients less than 18 years of age that were listed for lung transplantation from 1991 through 2002, of whom 265 were transplanted. The final model identifies five factors that increase the hazard of lung transplantation: Burkholderia cepacia infection, high FEV1%, and diabetes on the waiting list, and age and Staphylococcus aureus infection after transplantation. For example, the model predicts a hazard of transplantation of 6.75 (p = 0.02) for 16 year olds with an FEV1% of 20%, no diabetes and no S aureus or B cepacia infections. Regardless of covariate values, we found no proportional hazard that was less than 1 with a significant p-value for any pediatric patient group.
CONCLUSION: Lung transplantation does not increase survival for any group of pediatric patients with CF relative to their survival on the waiting list.
CLINICAL IMPLICATIONS: Although there are anecdotal successes, this analysis shows that the overall effect of lung transplantation is harmful for pediatric patients with CF. Transplant will remain harmful unless we can further improve patient selection. Under current practice, pediatric patients with CF should not be offered lung transplantation solely for the sake of extending life. Those seeking improved quality of life despite its uncertainty should realize that transplantation probably reduces survival.
DISCLOSURE: Theodore Liou, None.