Pediatric Pulmonary Expert Comments on Study of Lung Transplants for Cystic Fibrosis

Newswise — 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.

Allen's co-author on the editorial is Gary Visner, D.O., a transplant surgeon formerly at The Children's Hospital of Philadelphia, and now at Children's Hospital, Boston.

The Cystic Fibrosis Center at The Children's Hospital of Philadelphia provides comprehensive multidisciplinary care for children with CF, and has been recognized as a model program by the Cystic Fibrosis Foundation. One of the largest programs for cystic fibrosis in the U.S., it also offers lung transplantation among its treatments. The Center staff works in conjunction with the University of Pennsylvania Health System, which provides a program for adult CF patients.

About The Children's Hospital of Philadelphia: The Children's Hospital of Philadelphia was founded in 1855 as the nation's first pediatric hospital. Through its long-standing commitment to providing exceptional patient care, training new generations of pediatric healthcare professionals and pioneering major research initiatives, Children's Hospital has fostered many discoveries that have benefited children worldwide. Its pediatric research program is among the largest in the country, ranking third in National Institutes of Health funding. In addition, its unique family-centered care and public service programs have brought the 430-bed hospital recognition as a leading advocate forchildren and adolescents. For more information, visit http://www.chop.edu.