The prospect of needing a lung transplant can be emotionally difficult. Some with cystic fibrosis may view needing a transplant as a “failure” to manage their disease. Others may see it as the “beginning of the end” or merely “trading one disease for another.” Given recent improvements in outcomes, lung transplantation can extend and improve the quality of life for many.
Although lung transplantation can be a difficult process, discussing the possibility of transplant early on — even before you may actually need it — will give you and your family more time to consider it as a treatment option in the future.
"It was drastic and seemed to come out of nowhere; my forced expiratory volume (FEV1) fell from 45 percent to 26 percent seemingly overnight. I found myself so out of breath just taking a shower that I'd have to sit down and recover for a few minutes before being able to get dressed." — Jen Bleecher, adult with CF, from the CF Community Blog
Cystic Fibrosis Foundation guidelines recommend that your CF care team start discussing lung transplant with you as a future treatment option long before the need for transplant arises. The guidelines define this as when your forced expiratory volume in one second (FEV1) is less than 50 percent of predicted. This does not necessarily mean that you need a transplant, only that transplant might be considered as an option at some point in the future.
Download this graphic to help you start a conversation about the lung transplant process with your family and care team.
Why Start Early?
It is important that all people with CF who need a lung transplant, or may need one in the future, understand what the transplant process entails and are given the information to make an informed decision. By having a conversation with your CF care team about your goals and what the transplant process is like, you can work through the emotions or feelings you may have about lung transplant.
Discussing transplant with your CF care team before you need one gives you time to learn about the type of support you'll need from your family and friends, and how best to partner with your CF care team to map out what the journey might look like for you.
An early referral to a transplant center gives you the chance to meet the multidisciplinary transplant team before a formal transplant evaluation. In addition, getting to know your transplant team prior to the need for listing may help identify and address any potential obstacles to you being listed for transplant in the future. You will work with the transplant team closely before and after the lung transplant surgery.
"Psychological services were available at my transplant center, and each patient is assigned a psychologist. I utilized my psychologist prior to transplant to cope with some anxiety surrounding intense pain after surgery. Additionally, while on the waitlist, [the transplant center] offered group psychoeducation sessions that focused on coping skills, anxiety reduction, and mindfulness." — Jenavese Armstrong, adult with CF, from the CF Community Blog
Lung transplantation is a process in which you and the family and friends who make up your social support team will need to make thoughtful decisions throughout many phases. These decisions will affect you and your social support team medically, logistically, financially, socially, and emotionally. Starting the conversation early will give you more time to plan ahead by:
- Getting the help you need to work on anything that could prevent you from being a candidate for transplant.
- Making it easier for you and your family to make informed decisions.
- Securing the necessary financial resources while you are still well enough to do so.
Anyone living with CF knows it can be unpredictable. Although some may experience a slow decline in lung function, that decline can also happen very quickly, making it necessary to be referred for a lung transplant with little time to prepare.
The timing of the transplant procedure itself is a balancing act. People need to be sick enough to need a transplant, but also well enough to recover from the transplant surgery. This is known as the transplant window. By starting the referral process before you are in this window, you'll have more time to prepare.
Many people with CF want to delay a lung transplant as long as possible, but there is risk associated with this. Each year, approximately 10 percent of people with advanced cystic fibrosis die without a transplant, while only 6-8 percent undergo transplant as a life-sustaining treatment option.
The median survival for adults with CF who had lung transplant between 1999 and 2016 is 9.5 years. That means that half of the people with CF who have had lung transplants were alive 9.5 years after transplant.
"About 10 percent of the patients who did not get a transplant die each year. So, the risk in the first year, second year, it's about 10 percent for individuals with FEV1 of about 30 percent of predicted. That gives us some idea of what the population 'at risk' looks like. Based on these considerations, we've come to a consensus that we want to recommend early referral." — Joseph Pilewski, MD