Lung Transplants May Not Help Most Cystic Fibrosis Children

Lung Transplants May Not Help Most Cystic Fibrosis Children
WEDNESDAY, Nov. 21 (HealthDay News) -- Hardly any of the childrenwho receive lung transplants because of severe damage caused by cysticfibrosis benefit from the risky operation, a study concludes.
It is a controversial conclusion, made more controversial because thetransplant rules have been changed since the study was done, said Dr.Theodore G. Liou, associate professor of internal medicine at theUniversity of Utah, and lead author of a report in the Nov. 22 NewEngland Journal of Medicine.
CF is a hereditary disease in which the lungs and digestive tractbecome clogged with mucus. People with CF die at a relatively young age.Previous studies have shown some survival benefit for adult cysticfibrosis sufferers who received lung transplants as a last resort, Liousaid.
The new report is one of very few looking at such transplants inchildren, he said. "In 2005, we published one [study] showing nodifference in outcome between patients who were transplanted and those whowere not, although they were equally sick as far as we could tell," Liousaid. "That got us into a lot of trouble, because people didn't like theresults."
The new study looked at 514 children with cystic fibrosis who were onthe waiting list for lung transplants from 1992 to 2002. A total of 248 ofthe children did undergo the procedure during the study period.
The researchers found a significant estimated benefit for only five ofthose who had transplants, with "a significant risk of harm" associatedwith lung transplantation for 315 of the young patients, meaning thatother treatment would have benefited them more.
The process has changed since the study was conducted, partly in waysthat work against transplantation, Liou noted. Children selected for lungtransplants now are first put on an intensive course of treatment intendedto strengthen them for the surgery, he said, and "conventional treatmentshave 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 atthe cystic fibrosis center at the Children's Hospital of Philadelphia. Heis also co-author of an accompanying editorial in the journal.
Until 2005, all children awaiting a lung transplant were placed on asingle list, receiving organs as they became available. Now, the decisionto transplant includes consideration of the patient's condition, withsicker children getting the operation sooner, Allen said.
"In some cases, children who were put on intensive therapy weredeferred, because they got better," he said. "There was something aboutthe children in that group who didn't get transplanted that made them getbetter."
The bottom line, Liou said, is that a decision that has always beencomplex has gotten even more so. "Maybe people will pay attention and tryto be more careful about selecting patients for lung transplants," hesaid.
"You need to be careful about who you refer," Allen said. "You need tosee 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 futurestudies that evaluate the quality of life."
One complicating factor with younger recipients of lung transplants isthe need to be sure that they adhere to the strict regimen needed toprevent rejection of the organ, Allen added. The better success rate witholder cystic fibrosis recipients is due partly to their better ability tofollow instructions, he said.
More information
There's more on cystic fibrosis at the Cystic FibrosisFoundation.