Pediatric pediatric pulmonary expert comments on study of lung transplants for cystic fibrosis

Commenting on a study of lung transplantation for children with cystic fibrosis, a leading pediatric pulmonologist calls the study "startling" in an editorial in this week's New England Journal of Medicine that accompanies the study.

Julian Allen, M.D., who holds the Robert Gerard Morse Endowed Chair in Pediatric Pulmonary Medicine at The Children's Hospital of Philadelphia, analyzed the study by a University of Utah research team, which found that cystic fibrosis (CF) patients younger than age 18 did not survive longer after a lung transplant than CF patients who did not receive a lung transplant.

In contrast to a previous study by the same researchers, led by Theodore Liou, M.D., which found a survival advantage for adult transplant recipients when the predicted five-year survival rate without transplantation was 30 percent or worse, the new study found no lower threshold at which transplantation provided better survival in children—a finding that Allen called surprising.

“The study's results make it clear that physicians must be more judicious than ever in referring children with cystic fibrosis for lung transplantation, with careful consideration of the severity of the child's illness,” said Allen. He said that cystic fibrosis centers must provide appropriate social and psychological support to help families make the best choice for their children.

Allen added that it is possible that a lung transplant may improve a patient's quality of life, as the current study had only limited information on that question. He urged that future studies of lung transplantation in CF patients ought to carefully measure quality of life as well as measuring how closely patients adhere to post-transplant medical treatments.

One fact that may change future results, said Allen, is that the new organ allocation system, initiated in 2005, takes into account the severity of a patient's illness, in addition to other factors such as the length of time a patient remains on the waiting list for a donated organ. The system in place when the study was performed allocated lungs based only on the duration of the patient's wait.