While life has improved for many with cystic fibrosis, lung transplants remain a reality for some.
Erin Tallarico, B.S.N., RN
Clinical Sr. Director, Advanced Lung Disease, Cystic Fibrosis Foundation
Cystic fibrosis (CF) is a rare, progressive, genetic disease that damages vital organs, including the lungs. It is caused by genetic mutations in the cystic fibrosis conductance regulator (CFTR) gene, producing the mutated protein that can cause CF. Roughly 40,000 people in the United States have the disease. It impacts all races and ethnicities, and there is no cure.
While breakthrough treatments, such as modulators that correct the mutated protein, and advances in care over the past few decades have prolonged and improved life for many, an unmet need remains. Not everyone with CF responds to modulators, which only correct certain types of mutations. Some have been living with CF long before these transformative treatments became available and well after the disease caused harm, making lung transplant a necessary yet daunting step in their healthcare journey.
Life with a lung transplant
CF is a full-body disease, affecting not only the respiratory system, but also the digestive and reproductive systems. Lung transplants are not a cure and will not eliminate other manifestations of the disease. Undergoing a lung transplant can extend and improve quality of life, but it isn’t an easy choice.
On top of managing an already burdensome disease, an individual with CF who chooses to undergo a lung transplant may face additional tradeoffs and challenges. Those who decide to undergo the treatment face the reality of living with a suppressed immune system and side effects from anti-rejection medications. They must also schedule frequent follow-ups at their transplant center. This typically involves undergoing blood tests, lung function tests, and a biopsy to examine lung tissue. These visits are added to appointments for ongoing CF care, which involve a team of interdisciplinary healthcare specialists.
While the number of people with CF receiving transplants each year has decreased, according to 2024 Cystic Fibrosis Foundation Patient Registry data, there were still more than 1,400 people who were reported as living post-transplant. The Cystic Fibrosis Foundation continues to work with urgency toward improving lung transplant outcomes for people with CF and furthering research on transplant complications, such as transplant rejection. To learn more about cystic fibrosis and how the Foundation is working to prolong and improve life for everyone with CF, go to www.cff.org.
