Lung Transplant Surgery for Cystic Fibrosis

Reviewed by: HU Medical Review Board | Last reviewed: May 2022

When someone with cystic fibrosis (CF) develops severe lung disease, a lung transplant may be an option. People with CF receiving lung transplants remain a small percent of the total CF population and most who get a lung transplant are 30 or older. According to the 2017 Patient Registry Annual Data Report, 264 people with CF had received a transplant in 2017 while 146 had been accepted and remained on the waiting list.1

How common are lung transplants among cystic fibrosis patients?

The CF Foundation reports that up to half of the people with CF who have had lung transplants were alive 9.5 years after their transplant.2 A Canadian study found that patients who received a lung transplant for CF actually lived longer than people who received lung transplants for other reasons.3

A double-lung transplant is most common among people with CF, but some receive a double lung and liver transplant, double-lung and heart transplant, or double-lung and kidney transplant.

Qualifying for a transplant

In order to receive a lung transplant, the person must be sick enough to need the organs yet still healthy enough to survive what will be a complicated surgery and difficult recovery. The person and their doctor should discuss lung transplant surgery early on. This gives the person time to know the process and get to know the transplant team before they need the surgery. If the person is interested, they are referred to an organ transplant center for evaluation. In general, people cannot qualify for a lung transplant if they smoke, have HIV, or hepatitis.

Tests to evaluate lung transplant candidates

Tests to see if the person qualifies for transplant include function tests for the lungs, heart, and kidneys, and cultures to see what types of bacteria are in the lungs. A psychological evaluation will assess their mental well-being and support network of family, friends, and professional help. Each transplant recipient must be committed to living the lifestyle required to keep their new lungs healthy.

All transplant centers have slightly different standards for acceptance into their program. Someone can be rejected as a candidate at one center and accepted by another. Once a person qualifies for a transplant, they then must register for the national waiting list. There are always more people on the waiting list than available organs.

Lung transplant surgery

As you can imagine, waiting for organs to become available and then the lung transplant surgery itself is complicated and grueling. The operation itself takes hours, but in general, most people go home from the hospital in 2 weeks.

After a transplant, the recipient must take anti-rejection (immunosuppressive) medication daily to prevent their body from reacting against – or rejecting – the new organs. Immunosuppressive drugs can have many side effects, including diabetes, kidney problems, cancer, and osteoporosis.

Life after a lung transplant

Many people who receive a double-lung transplant feel significantly stronger and more energetic after their surgery. Their new lungs do not have CF, so breathing is easier, and coughing is dramatically reduced. However, the recipient still has CF in the rest of their body and the complications that come with that. The immunosuppressants required to keep the body from rejecting the new organs may also decrease the body’s overall ability to fight germs that cause infections.

Still, in the U.S., post-transplant complications are the second leading cause of death among people with CF after end-stage lung disease.1

The choice to pursue a lung transplant

Choosing to get a lung transplant is a very personal and difficult decision. Financing a transplant can be very expensive, even with insurance. Depending on your coverage, insurance probably won’t pay non-medical costs, such as living expenses, relocating close to the transplant center, and transportation to and from the transplant center. Insurance may only pay a portion of tests, medications, and post-surgery rehabilitation and those costs can add up quickly. Your CF care team and transplant team can help you find ways to pay for the costs.4

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