International leaders in transplantation and cystic fibrosis (CF) met in June 2008 in Lansdowne, Virginia in a roundtable conference to consider the state of the art in lung transplantation for CF. The meeting brought together different members of the pediatric and adult lung transplant community, experts in biostatistics, clinical research techniques, quality of life, and advocates of the CF community.
Lung transplantation for CF was first performed using a combined heart–lung transplant in 1983, with the first bilateral lung transplants for CF performed a few years later. Short-term success with lung transplantation prompted growth in the use of the procedure for CF and fostered hope in children and adults whose CF lung disease had progressed to end stage. According to the Organ Procurement and Transplantation Network, approximately 200 patients with CF, including approximately 25 children, undergo lung transplantation in the United States each year.
As with all transplantation, there are more candidates than available donors. Another issue with CF patients is the fact that lung transplant is not a cure for CF lung disease. Adult CF patients have a the median survival after lung transplantation of only 6.4 years. This makes choosing the time to list a patient with CF for a lung transplant crucial.
Reports intended to provide guidance to physicians and surgeons regarding the timing of listing began to appear in the early 1980s. One of the first and most widely referenced reports is from the Toronto Lung Transplant Program. Subsequent studies have attempted to refine those criteria.
The authors of the Toronto paper found that in their experience, patients with a forced expiratory volume in one second (FEV1) of 20% of predicted had a 70% chance of death within 2 years, but the chance of death halved for every 10 percentage-point increase in FEV1. Using that information, the authors concluded that CF patients should be considered candidates for lung transplantation when the FEV1 falls below 30% of the predicted value. The conference agreed that better clinical predictors of short-term mortality among patients with CF are needed as “prediction of the appropriate time to offer transplant for CF remains largely subjective.”
Lung transplantation is an option for selected pediatric and adult patients with advanced or severe lung disease that has failed to respond to standard therapy. Despite the steady increase in the number of candidates listed for lung transplantation, there have been few population-based studies on the benefits (including survival) of lung transplantation for adults and children with end-stage lung disease. The June 2008 Lansdowne Conference participants agreed that:
Conference participants agreed that:
Optimal care for CF children and adults, whether before or after lung transplantation, should remain a primary goal.
Lung transplantation should remain an option for selected patients with CF and advanced lung disease.
Multiple factors influence the outcome of lung transplant recipients including:
- physiological variables
- patient-specific variables
- lung transplant and CF Center-specific practices
- infectious agents
There is a need for evidence-based practice guidelines for lung transplant centers
A prospective, multicenter study on patients with CF and lung transplant is necessary to answer important questions on criteria for candidate selection and transplant practice guidelines