Lung transplants no help for cystic fibrosis: study

Lung transplants no help for cystic fibrosis: study
An empty operating theatre is seen at the University hospital in western German city of Bonn March 16, 2006. Children with severe cystic fibrosis are seldom helped by a lung transplant, researchers said on Wednesday in findings they said stunned them. (Ina Fassbender/Reuters)BOSTON (Reuters) - Children with severe cystic fibrosis areseldom helped by a lung transplant, researchers said onWednesday in findings they said stunned them.
Only five of the 514 U.S. children on the waiting list fora transplant from 1992 through 2002 lived longer as a clearresult of the expensive and complicated operation, Dr. TheodoreLiou of the University of Utah in Salt Lake City and colleaguesfound.
"We were stunned, literally," Liou said in a telephoneinterview.
"We went into this thinking there would be a number ofpatients who would benefit and a number who would not, and itwould balance out. This study showed it wasn't close to beingequal," said Liou, whose findings were reported in the NewEngland Journal of Medicine.
Transplantation is an accepted treatment for severe casesof the fatal genetic disease, which affects about 70,000 peopleworldwide and creates an abnormally thick mucus that clogs thelungs and digestive system.
But few studies have assessed the effectiveness of lungtransplants for helping treat the incurable disease.
Although the Cystic Fibrosis Foundation says that 50percent of recipients are alive five years after surgery,complications from transplants are responsible for 12 percentof all deaths among people with CF.
"A basic tenet regarding lung transplantation is that itextends life," Dr. Julian Allen of Children's Hospital ofPhiladelphia and Dr. Gary Visner of Children's Hospital inBoston wrote in a commentary.
"Most workers in transplant centers have encounteredpatients in whom the results of transplantation seem nothingshort of miraculous," they wrote.
However there have been hints that the operation may not bebest for sufferers under 18. The five-year survival rate of 33percent for children is less than for adults or people who havereceived a transplant for another reason, Allen and Visnersaid.
But the duo also cautioned that because there has been achange in the rules that determine who gets a new set of lungs,waiting times for the sickest patients have shortened. Thus theLiou findings may no longer apply.
Liou disagreed. The new system of ranking patients will nothelp people who are unlikely to benefit from a transplant inthe first place, he said.
"I think it's going to make people think twice about lungtransplants," he said.
Because of improvements in treatment, children who mighthave died in their mid-teens in the 1970s now survive untiltheir mid-30s.
"The results underscore that sustained, multidisciplinarycare rather than lung transplantation is central to longevityin children with cystic fibrosis," the Liou team concluded.
(Reporting by Gene Emery; Editing by Maggie Fox and VickiAllen)