Cystic fibrosis, Burkholderia cenocepacia and lung transplantation

During last Wednesday's Satellite Symposium 3: The Challenges of Lung Transplantation in Cystic Fibrosis (CF) at the International Society of Heart and Lung Transplantation (ISHLT) Annual Meeting & Scientific Sessions, clinicians and researchers discussed some of the unique challenges in achieving excellent lung transplant outcomes in patients with CF.

A highlight of the session was the concluding debate: Burkholderia Cenocepacia is an Absolute Contradiction to Transplantation between Duane Davis, MD, Duke University Medical Center, Durham, NC and Redha R. Souilemas, MD, European Georges Pompidou Hospital, Paris.

Cystic Fibrosis, Burkholderia Cenocepacia and Lung Transplantation

Patients with life threatening lung disease due to CF have a lot to gain by lung transplantation. Compared to other possible indications for transplant, patients with CF have, in general, the best outcomes, and a successful transplant can mean a significant increase in lifespan and a huge improvement in quality of life.

A hallmark of advanced CF lung disease is repeated severe chest infections. In most cases these infections are responsive to treatment with powerful antibiotics and their presence does not influence the outcomes after transplant in CF patients.

However in 5 percent of patients with CF, bacteria called Burkholderia cenocepacia infects the lungs before transplant. This infection is very resistant to antibiotics and if it returns after the transplant surgery it is extremely difficult to control and can be life threatening.

It is estimated that less than only 30 percent of patients with Burkholderia cenocepacia who receive lung transplants survive beyond five years with many dying very early after transplant due to recurrence of the severe infection with this bacteria. In comparison, CF patients without this infection can expect 5-year post-transplant survival of up to 70 percent on average. The debate within the transplant community is whether or not to allocate donor lungs to patients with Burkholderia cenocepacia knowing that the success rate is much worse than in CF patients without this infection.

According to Dr. Davis, lung transplants should not be performed on CF patients with Burkholderia cenocepacia until new research indicates a way to achieve better early results by better control of the infection. He went on to say new ways of treating patients with Burkholderia cenocepacia should be examined in clinical trials with a hypothesis, careful data collection and safety monitoring. Only after such research approaches have generated a clinical advance in treatment of this infection should lung transplants in CF patients with Burkholderia cenocepacia be performed in the same manner as those who do not have the disease.

Representing the counter argument, Dr. Souilemas said he believes it is very difficult to choose between two patients who need the same procedure. “Medicine is science and art,” said Dr. Souilemas in the debate. During his argument, Dr. Souilemas also presented information on reversible factors other than the infection itself, which may contribute to the patients' poor health before receiving a lung transplant, and thus contribute to poor outcomes. Dr. Souilemas finished his debate by proposing that each patient be viewed as a potential lung transplant candidate and encouraged clinicians to attempt to transplant each as successfully as possible.

In the end, both physicians agreed that more careful research into transplants in CF patients with Burkholderia cenocepacia would improve success rates for these patients.

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