Lung transplants good in Cystic Fibrosis

Lung transplants generally lead to good long-term results in patients with cystic fibrosis.However, the number of patients who die waiting for organs is around 40 percent to 50 percent, dependent on country to country. To determine how recipients fared in the long term, researchers from England reviewed data from 176 cystic fibrosis patients who underwent lung transplants. It was found that pulmonary function improved from an average of 21 percent pre-transplantation to 78 percent after 1 year. Survival was 82 percent after 1 year, 62 percent after 5 years and 51 percent after 10 years. Thirty-five of 69 deaths (51 percent) were caused by bronchiolitis obliterans, a manifestation of organ rejection that develops in about 50 percent of patients who receive a lung transplant from an unrelated donor. The inflammatory response results in fibrosis and narrowing of the airway. This can cause airway obstruction and patient death.Infection caused another 18 deaths (26 percent); bacterial infection with Burkholderia cepacia complex caused 7 of these deaths. Although B. cepacia do not usually infect healthy people, patients with cystic fibrosis or who are immunodepressed are susceptible to infection. Survival free of bronchiolitis obliterans was 74 percent at 5 years and 38 percent at 10 years.The above results demonstrate that cystic fibrosis patients undergoing lung transplantation have excellent long-term survival results after the operation. However, work is still required to increase donor numbers, and also to reduce the long-term side effects of anti-rejection drugs.

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