Lung Transplantation May Extend Lives in Children With Cystic Fibrosis

From the WebMD Archives

Nov. 10, 1999 (Atlanta) -- A new study from London may offer some hope to terminally ill children with respiratory illnesses such as cystic fibrosis (CF).

"We have confirmed that even with the current poor survival after lung transplantation, the procedure still provides an increase in life expectancy for the majority of children," British physician Paul Aurora tells WebMD.

Aurora, a specialist in pediatric respiratory medicine at the Royal Brompton Hospital in London, says that the findings in this new study are consistent with three earlier studies which focused on adults receiving lung transplants.

When compared to other organs, transplanting lungs has been much less successful. Commonly accepted figures for survival rates worldwide for lung transplantation are about 70% one year after the transplant and 45% after four years. Most of these deaths are due to infections and rejection of the organs.

In the study, reported in the November issue of The Lancet, Aurora and colleagues examined the effect of lung or heart-lung transplantation on the survival of 124 children with cystic fibrosis and severe lung disease. Children were accepted for transplantation if they had a life expectancy of two years or less, a poor quality of life, and no factors that would rule out transplantation.

Of the total, nearly 50 children received transplants, nearly 70 died while they awaited organs, and nine remained on the active waiting list. Of the children receiving transplants, 90% received heart-lung transplants, four received a double lung transplant, and one received a single lung transplant. The average waiting time for organs was just over six months. After one year, almost 75% of those receiving transplants were still alive. After five years, approximately one-third of the patients were still alive.

According to the Cystic Fibrosis Foundation, CF is a genetic disease affecting approximately 30,000 children and adults in the United States. It causes the body to produce an abnormally thick, sticky mucus due to the faulty transport of sodium and chloride within cells lining organs, such as the lungs and pancreas, to their outer surfaces. The thick CF mucus not only causes an excessive amount of thick secretions in the airway, but it also blocks the release of pancreatic enzymes which aid in the process of food breakdown and digestion in the intestines. Many organs of the body are adversely affected by cystic fibrosis.

"We are encouraged by our findings," Aurora says, explaining that the key is to select candidates for lung transplant very carefully. He says that the transplant should extend the child's life longer than the predicted length of time without the transplant. "If others follow our criteria for accepting patients for transplantation, they could expect a survival benefit for their patients in line with our results."

Aurora adds that unless survival without transplantation improves dramatically, priority should be given for cystic fibrosis patients with terminal lung disease. He notes that fewer than half of the children accepted for transplantation in the U.K. during the study period received organs.

This is the same problem facing cystic fibrosis patients in the U.S. According to Suzanne R. Pattee, vice president of public policy and patient affairs at the Cystic Fibrosis Foundation in Bethesda, Md., people with CF have trouble obtaining lung transplants in a timely fashion, and more people are dying while on transplant waiting lists. "We want to promote the unique needs of people with CF in any new national policies on organ transplantation," Pattee said in a prepared statement. "We want to make sure that people with CF have timely access to transplants."

Lung transplantation has been available for patients suffering from terminal lung disease since the early 1980s, but because of the low survival rate there is a great deal of controversy concerning the cost and benefits.