Lung transplants, which have been seen as a treatment of
last resort for cystic fibrosis, seem to be rarely beneficial to children with
that condition or even worse, they can be harmful, according to a study
released on Thursday.
CF is a hereditary disease that affects mainly the lungs and
digestive system. The disease leads to progressive disability and in some
cases, early death. The disease affects 1 in 3,900 children in the U.S.
Europeans and Ashkenazi Jews seem to have higher rates, while 1 in 20 people of
The study lead by the
There were 514 patients under 18 participating at the study, according to the report. A number of 248 children, suffering from CF, who were on the waiting list during the 10-year-period, underwent lung transplantation.
"Children undergoing lung transplant did much worse than
expected," Dr. Theodore G. Liou of the
Only five children had a significant estimated benefit following the lung transplantation. More than a half – 315 children or 61 percent – encountered risk factors from lung transplant, while 194 children did not have either harmful or beneficial results after the surgery.
"We fully expected, at the start of the study, that we would find a group that did well and others that did not do so well. Unfortunately, we found that the group that most likely did well with transplant was very small, while the group that most likely did poorly (worse than if not transplanted) was quite large," Liou said.
“Although transplantation may improve quality of life, it may not improve
survival,” says Dr. Julian Allen of Children’s
Until 2005, children underwent lung transplant in the order they were placed
on the awaiting list and as soon as the receiving organs were available. Now,
the rules have changed. Doctors look mostly at the patient’s condition and
sicker children are getting the surgery sooner, Allen said.
"In some cases, children who were put on intensive therapy were deferred, because they got better. There was something about the children in that group who didn't get transplanted that made them get better," Allen said.
This study was intended to deliver new information for
patients and their families in order to make better decisions about the timing
of lung transplants as Preston Campbell, executive vice president for medical
affairs of the Cystic Fibrosis Foundation, which helped fund the study, said.
“Ultimately, I think we will reach a point that children
won't require lung transplants" because of advances in care of C.F.
patients, he added.