Lung Transplantation for Cystic Fibrosis

By Dr. Catherine Shaffer, Ph.D.Reviewed by Dr. Liji Thomas, MD

Cystic fibrosis is a life-limiting disease caused by a defect in a gene responsible for transporting salts in and out of cells. It causes symptoms and complications throughout the body. The most serious problems caused by the disease affect the lungs. A thick, sticky mucus clogs the lungs, obstructing airways and promoting frequent lung infections. Lung transplantation is one option for increasing survival in people with cystic fibrosis.

Survival Rates

A recent study showed that the five-year survival rate for Canadians with cystic fibrosis who have received a lung transplant was 67 percent, and half of the transplant recipients survived longer than 10 years. The study, using data from the Canadian Cystic Fibrosis Registry, included 580 patients who received their transplants between 1988 and 2012.

Lung transplantation for cystic fibrosis was first performed in 1983, with a combined heart and lung transplant. The first bilateral lung transplant was attempted a few years later. It is generally considered as an option during the end stages of cystic fibrosis disease of the lungs, when the chances of survival with the transplant become more favorable than the chances of survival without the transplant.

The decision to get on the list for a lung transplant is a balance between the severity of disease necessitating a transplant, and being well enough to survive the wait time for the organs.

Guidelines for listing a patient with cystic fibrosis for lung transplant according to the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation include:

1. ICU stay due to exacerbation of pulmonary disease
2. Reduction in forced expiratory volume in 1 second (FEV1) by more than 30 percent
3. Increased frequency of exacerbations requiring antibiotics
4. Refractory or recurrent pneumothorax
5. Recurrent hemoptysis that cannot be controlled by embolization

Additional events that could lead to being considered for a lung transplant include oxygen-dependent respiratory failure, hypercapnia (retention of CO2), and pulmonary hypertension.

Complications

Complications of a lung transplant include allograft failure, infections, and complications of immune-suppressing medications.

Sinus infections with multidrug resistant organisms are a common, serious complication following lung transplant for patients with cystic fibrosis. Colonization of the upper airway can predispose such patients to pulmonary infections. Pseudomonas aeruginosa is the most common organism that colonizes airways and sinuses in cystic fibrosis patients. Other bacteria that can cause complications post-transplant include Burkholderia cepacia species.

Fungal infections can also complicate lung transplantation, mainly organisms from the genus Aspergillus, as well as mycobacteria like M. tuberculosis, and viral infections including cytomegalovirus and herpes viruses.

Noninfectious complications that can occur include endocrine problems, gastrointestinal complications, and cancer.

Contraindications for Lung Transplant

Some patients are not recommended for transplant due to certain contraindications.

Those include:

1. History of malignancy within the previous two years
2. Untreatable advanced disease of another organ system
3. HIV infection
4. Hepatitis B with positive surface antigen
5. Hepatitis C with liver disease
6. Inability to comply with aftercare instructions
7. Substance addiction within the previous 6 months

Life After Transplant

Lung transplant does not cure cystic fibrosis. The disease no longer exists in the lungs after transplant. However, other organs of the body will continue to be affected by the disease. Patients continue to need treatment to manage cystic fibrosis after transplantation.

When caring for the new lungs, it is important to avoid potential sources of infection because the immune system will be weakened by immunosuppressive drugs that prevent rejection. The transplant recipient should practice strict hygiene and avoid people who are sick or have recently received live vaccines.

Immunosuppressive drugs are required for life to prevent rejection of the organ. These drugs can increase the risk of cancer, diabetes, cataracts, and tooth decay.

Retransplantation

Some transplant recipients with cystic fibrosis require a second transplant due to failure of the transplanted lungs or rejection by the immune system. This requires a second evaluation, listing, and waiting process.

References

1. Remarkable long-term survival post-lung transplantation among Canadian patients with cystic fibrosis, http://www.jhltonline.org/article/S1053-2498(15)01281-4/abstract
2. Lancet, Heart-lung transplantation for cystic fibrosis, https://www.ncbi.nlm.nih.gov/pubmed/2899664/
3. Ann Chir., Double lung transplantation. Report of the 1st French case and comments on the 5 subsequent cases, https://www.ncbi.nlm.nih.gov/pubmed/2589793/
4. J Heart Lung Transplant, International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation, https://www.ncbi.nlm.nih.gov/pubmed/16818116
5. Am J Respir Crit Care Med, Infection complications of lung transplantation. Impact of cystic fibrosis, https://www.ncbi.nlm.nih.gov/pubmed/7516251
6. Cystic Fibrosis Foundation, Caring for Your New Lungs, https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Lung-Transplantation/Surgery-Recovery-and-Life-Post-Transplant/Caring-for-Your-New-Lungs/

Further Reading

• All Cystic Fibrosis Content
• Cystic Fibrosis (CF)
• Cystic Fibrosis Causes
• Cystic Fibrosis Symptoms
• Cystic Fibrosis Diagnosis