Lung transplantation increases the survival of patients with cystic fibrosis (CF) by an average of almost four-and-a-half years, according to research led by Washington University School of Medicine in St. Louis.
The study will be presented at 8:15 a.m. ET on May 24 at the American Thoracic Society International Conference in Orlando. It also will be featured at a press briefing at the conference at 2 p.m. ET.
“Initially the risks from lung transplantation for cystic fibrosis patients are quite high, but they drop over time, and transplantation becomes worth the risk in the long run,” says Roger D. Yusen, M.D., M.P.H., assistant professor of medicine.
Yusen led the study; medical resident Stephen C. Ryan, M.D., is first author. Other key contributors include G. Alexander Patterson, M.D., the Joseph C. Bancroft Professor of Surgery, and Elbert P. Trulock III, M.D., the Rosemary and I.J. Flance Professor in Pulmonary Medicine.
According to the Cystic Fibrosis Foundation, CF afflicts about 30,000 people in the United States, and individuals with CF live an average of about 33 years. Although it can affect people of all ethnicities, CF is most prevalent in Caucasians and is the most common fatal genetic disease in the Caucasian population.
Individuals with CF have an abnormal gene that triggers production of thick, sticky mucus. The mucus can cause a variety of problems in different organs, but the most common and inevitable effect is that it clogs the lungs and leads to severe lung disease. CF patients who receive lung transplants to treat the disease must therefore have both lungs replaced in order for the surgery to be effective.
Lung transplants, however, are hard to come by. Approximately 1,000 are performed each year in the United States, and only 150 of them, or 15 percent, are performed on CF patients. At the moment, lungs are allocated based on the amount of time a patient has been on the waiting list, but, according to Yusen, that process is currently being revised to incorporate medical urgency and chance of survival.
To determine the impact of lung transplantation on survival in this population, Yusen and colleagues studied all 247 patients with CF who were on the waiting list for lung transplantation at the School of
Medicine’s clinical affiliate, Barnes-Jewish Hospital in St. Louis, since the program began in 1988. Patients were followed until 2002.
The patient population in this study reflected the CF population worldwide: most were Caucasian and their average age at the time of placement on the waiting list was 28. All patients had significantly impaired lung function and other severe symptoms.
Transplantation had a significant death risk in the first few months after surgery compared with continued waiting. But Yusen’s team found that, over time, the risks of transplantation decreased and the risks of waiting increased.
Patients who received a lung transplant had an 84 percent chance of survival one year after surgery— similar to the survival rate of patients who had been on the waiting list for one year. Two years after transplant, the chance of survival was 76 percent, while only 67 percent of individuals who were on the waiting list for two years were still alive. After five years, 55 percent of transplant recipients were still alive; no such data are available for the non-transplant group because very few CF patients remain on the waiting list that long.
Using statistical models, the researchers determined that lung transplantation improved the life expectancy of patients with CF by almost four-and-a-half years.
“Next, we want to determine if transplanted patients are not only living longer, but enjoying a better quality of life than those who remain on the waiting list,” Yusen says.
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Ryan SC, Morrow LE, Meyers BF, Thompson P, Hachem RR, Chakinala MM, Patterson GA, Trulock EP, Yusen RD. The impact of lung transplantation on the survival of patients with cystic fibrosis. American Thoracic Society’s 100th International Conference, May 24, 2004.
Funding from the Mentors in Medicine Program at Washington University School of Medicine in St. Louis supported this research.
The full-time and volunteer faculty of Washington University School of Medicine are the physicians and surgeons of Barnes-Jewish and St. Louis Children’s hospitals. The School of Medicine is one of the leading medical research, teaching and patient care institutions in the nation, currently ranked second in the nation by U.S. News & World Report. Through its affiliations with Barnes-Jewish and St. Louis Children’s hospitals, the School of Medicine is linked to BJC HealthCare.