But many will reach a point where they require a lung transplant to prolong their life. Usually someone would be referred for transplant assessment when their lung function falls to about 30%.
Register as an organ donor today. You could give the gift of life to six people.
Risks and opportunities
The success rate of lung transplants for people with CF is encouraging, and recipients will enjoy a much greater quality of life, but the procedure does carry considerable risks, including rejection or infection. It is also important to remember that a transplant is not always the most appropriate form of treatment for someone who is severely ill with cystic fibrosis.
The transplant process is a lengthy one, and patients and their families can often face a range of physical and emotional issues. To help you deal with some of these problems, it is routine practice to offer assessment and care at every stage. This is particularly important during the pre-transplant phases.
There is a shortage of suitable donor organs, and approximately a third of all those on the waiting list for a lung transplant will die before they receive a donor lung. We are committed to increasing access to successful lung transplants – find out more about our Hope for More campaign.
Read about research we are funding into how to make more donor lungs viable for transplant.
If you are undergoing assessment for a transplant, we may be able to help with an emergency grant.
Although most people with cystic fibrosis will have no severe CF-related liver problems, it has been reported that up to 40% of adults with CF have some liver abnormalities.
Most people with CF-related liver problems show little or no deterioration over time and often a clinic review about once a year with a scan and blood tests is all that is required. Complications of CF-related liver disease can be managed with monitoring and treatment, however a small minority do deteriorate and then need to be considered for liver transplantation.
Liver transplantation in people with CF has proved to be a success, despite early concerns about the outcome of the procedure and the effects of immunosuppression on respiratory infections.
Organ Donor Line 0300 123 2323
Right now there are more than 8,000 people in the UK who need an organ transplant that could save their life or improve their quality of life, including around 70 people with cystic fibrosis waiting for a heart, lung, or both.
If you want to help someone live after your death, sign up to the register now.
Thank You for Life
Published by the Royal College of Physicians for NHS Blood and Transplant, 'Thank You for Life' brings together letters to organ donor families from patients who have received donated organs. The aim of the book is to show the positive side to organ donation, and to encourage people to sign up to the organ register. Proceeds from sales go to promote organ donor registration.
Find out more about this book