Lung Transplantation

If a person with CF develops serious lung complications, their physician may refer them for evaluation at a lung transplantation center. An evaluation involves measurement of lung, heart and renal functions, infectious disease studies and psychological examination. Acceptance as a transplant candidate is specific for each center. The patient may change her/her chosen center, but some new evaluation is usually necessary.

The transplant lungs are CF free, but CF remains in the patient's sinuses, pancreas, intestines, sweat glands and reproductive tract after the transplant. To prevent the body from "rejecting" the new lungs, physicians prescribe immunosuppressive drugs to reduce the immune response and protect the organs from rejection. The patient continues taking immunosuppressives for the rest of their lives. These drugs may increase one's susceptibility to some infections and cause side effects such as diabetes, decreased kidney function and osteoporosis. The doses of these medicines can be adjusted to maintain adequate immunosuppression and minimize side effects.

Lung transplantation can involve a brain-dead organ donor or living donors. If a brain-dead donor is used, a full double-lung transplant can be performed, one lung at a time. With living donors, one donates a right lower lobe, and one donates a left lower lobe. Using living donors is complex, because three people must have major surgery. The donors must be significantly larger than the recipient so that the two lower lobes are large enough to fill the recipient's chest cavity. The success rate is comparable between the two techniques. Health insurance companies and government insurance programs like Medicare generally consider transplantation to be a covered procedure. Donor lungs are allocated on the basis of blood type, size and accrued time on the transplant list. Transplant centers generally require patients to live within a short distance when they are near the top of the waiting list.

If a patient is infected with pseudomonas or B. cepacia prior to a transplant, the infection remains in the upper airways and sinuses. The transplanted lungs are at risk of recurrent infection, and the risks seem to be highest shortly after the transplant operation, when the doses of immunosuppressive drugs are highest. There are also risks of viral infections and chronic rejection. Not all lung transplant centers accept B. cepacia -colonized patients for transplant.