Lung Transplants May Not Help Most Cystic Fibrosis Children
Study found little improvement, but procedures have also changed since analysis was made
WEDNESDAY, Nov. 21 (HealthDay News) -- Hardly any of the children who receive lung transplants because of severe damage caused by cystic fibrosis benefit from the risky operation, a study concludes.
It is a controversial conclusion, made more controversial because the transplant rules have been changed since the study was done, said Dr. Theodore G. Liou, associate professor of internal medicine at the University of Utah, and lead author of a report in the Nov. 22 New England Journal of Medicine.
CF is a hereditary disease in which the lungs and digestive tract become clogged with mucus. People with CF die at a relatively young age. Previous studies have shown some survival benefit for adult cystic fibrosis sufferers who received lung transplants as a last resort, Liou said.
The new report is one of very few looking at such transplants in children, he said. "In 2005, we published one [study] showing no difference in outcome between patients who were transplanted and those who were not, although they were equally sick as far as we could tell," Liou said. "That got us into a lot of trouble, because people didn't like the results."
The new study looked at 514 children with cystic fibrosis who were on the waiting list for lung transplants from 1992 to 2002. A total of 248 of the children did undergo the procedure during the study period.
The researchers found a significant estimated benefit for only five of those who had transplants, with "a significant risk of harm" associated with lung transplantation for 315 of the young patients, meaning that other treatment would have benefited them more.
The process has changed since the study was conducted, partly in ways that work against transplantation, Liou noted. Children selected for lung transplants now are first put on an intensive course of treatment intended to strengthen them for the surgery, he said, and "conventional treatments have gotten to be very good," he added.
The rules for actual performance of a transplant have also changed, said Dr. Julian L. Allen, chief of the division of pulmonary medicine at the cystic fibrosis center at the Children's Hospital of Philadelphia. He is also co-author of an accompanying editorial in the journal.
Until 2005, all children awaiting a lung transplant were placed on a single list, receiving organs as they became available. Now, the decision to transplant includes consideration of the patient's condition, with sicker children getting the operation sooner, Allen said.
"In some cases, children who were put on intensive therapy were deferred, because they got better," he said. "There was something about the children in that group who didn't get transplanted that made them get better."
The bottom line, Liou said, is that a decision that has always been complex has gotten even more so. "Maybe people will pay attention and try to be more careful about selecting patients for lung transplants," he said.
"You need to be careful about who you refer," Allen said. "You need to see if the results in this study hold true under the current rules. Also, the quality of life after a transplant has to be looked at. We need future studies that evaluate the quality of life."
One complicating factor with younger recipients of lung transplants is the need to be sure that they adhere to the strict regimen needed to prevent rejection of the organ, Allen added. The better success rate with older cystic fibrosis recipients is due partly to their better ability to follow instructions, he said.
There's more on cystic fibrosis at the Cystic Fibrosis Foundation.
Copyright © 2007 ScoutNews, LLC. All rights reserved.