Cystic fibrosis (CF) is a serious and debilitating genetic disease. In years past, a cystic fibrosis diagnosis meant a short life expectancy. But new medical advances have given people with CF a better prognosis and additional treatment options, such as lung transplantation.
“Cystic fibrosis is a genetic disease that is inherited and can cause a lot of different problems,” Dr. Joseph Pilewski, MD, UPMC Transplant Services, Cardiothoracic, tells WPXI. “But the primary problems are in the lung, where patients develop cough, chest congestion, and gradually progressive lung disease that, until we had new treatments, was oftentimes fatal early in life.”
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Is Lung Transplant an Option?
Cystic fibrosis can affect many areas of the body, including the pancreas and liver, but generally, CF most severely affects the lungs, according to the Cystic Fibrosis Foundation. CF can cause your body to secrete a thick, glue-like substance in your lungs, pancreas, and liver.
Having damaged lungs hinders your ability to breathe, your quality of life, and your life expectancy. A person with cystic fibrosis may have frequent and recurring lung infections, such as bronchitis or pneumonia. Each infection causes additional damage to the lungs.
What Is a Lung Transplant?
Only people with severe lung disease or damage are considered for lung transplantation.
After completing a thorough evaluation, they are placed on the lung transplant waiting list to wait for a pair of donor lungs to become available. During a lung transplant, a surgeon replaces the damaged lungs from a person with CF with the healthy lungs of a recently deceased donor.
Recovery after surgery may take some time, as this procedure is invasive.
You may be housed in the intensive care unit for some time and placed on a ventilator, which mechanically breathes for you. Antibiotics and immunosuppressant drugs are typically administered immediately. These immunosuppressant drugs are required for the rest of your life to keep your transplanted lungs healthy.
Once you are discharged from the hospital, you may have to return to the transplant center every day for follow-up appointments and additional testing, including x-rays, lung biopsies, and blood tests.
Risks of Lung Transplant
Like any type of surgery, lung transplantation has risks.
The body can reject the new lungs, either rapidly after surgery or progressively over months with no obvious symptoms. Some symptoms include fever, tiredness, and trouble breathing.
Some people may experience serious loss of lung function, or chronic lung allograft dysfunction, even after transplantation with healthy lungs. Some will experience chronic infections, blockage of blood flow to the airways, or even cancer, diabetes, or kidney disease as a result.
The Cystic Fibrosis Foundation recommends weighing the risks and benefits of a lung transplant for cystic fibrosis before making a decision to undergo surgery because it is a complex process. Lung transplantation can offer some people improved quality of life, while others may experience continued complications.
Can You Cure Cystic Fibrosis with a Lung Transplant?
Lung transplantation will not cure a person who has cystic fibrosis. After going through a lung transplant, you will have a healthy set of working lungs, provided your body doesn’t reject the transplant.
However, you will still have CF in other parts of your body. Those parts will still be affected by CF, and you should still perform routine care for those organs.
About 87 percent of people who have lung transplants for CF will be alive one year later, according to the Cystic Fibrosis Foundation. By the nine-year mark, approximately 50 percent of people who had a lung transplant are still living.
When a lung transplant is successful, the recipient can breathe more easily and begin doing activities they enjoyed before their lungs were damaged.
For more information about transplant and treatment options for cystic fibrosis, visit the UPMC Lung Transplant program website
Editor's Note: This article was originally published on , and was last reviewed on .
About Transplant Services
For more than four decades, UPMC Transplant Services has been a leader in organ transplantation. Our clinicians have performed more than 20,000 organ transplant procedures, making UPMC one of the foremost organ transplant centers in the world. We are home to some of the world’s foremost transplant experts and take on some of the most challenging cases. Through research, we have developed new therapies that provide our patients better outcomes — so organ recipients can enjoy better health with fewer restrictions. Above all, we are committed to providing compassionate, complete care that can change – and save – our patients’ lives. Visit our website to find a provider near you.