Lung Transplants and CF 

For those with cystic fibrosis (CF) who continue to experience lung progression with treatment, a lung transplant may be an additional treatment option to prevent respiratory failure.

From the period of 2000 to 2017, 1000 children with CF received a lung transplant.¹ This is in comparison to 7000 adults who received a transplant from 1995 to 2017.²

Who is a candidate for lung transplant?

The current recommendation issued by the CF Foundation is to avoid delay in transplant referrals.³ The recommendation states that those who have a forced expiratory volume in one second (FEV1) less than 50% should have a discussion about the possibility of a transplant. At the same time, the clinician should help prepare the individual for a possible transplant. This preparation could include setting up referrals pre-transplant for nutritionists to manage nutritional status and CF-related diabetes, and to psychologists to assess ability to cope. Some other considerations for referral to a center that specializes in lung transplants include:

• A 6-minute walk test of less than 400 meters
  • This test measures exercise capability; it is the distance someone can walk on a flat surface over a total of 6 minutes
• Liver cirrhosis, or severe scarring of the liver
• Chronic kidney disease
• Number of exacerbations that require the use of intravenous antibiotics
• History of pneumothorax, or a collapsed lung
• Low levels of oxygen in the blood

Note that the presence of any of the above alone does not indicate that a lung transplant for CF is necessarily warranted; it is only upon the doctor’s discretion to recommend a transplant referral after conducting various other tests.

How long is the wait list?

Waiting for a transplant can be exceptionally stressful. In the United States, there is a priority list for transplants based on a scoring system. The score is referred to as the Lung Allocation Score (LAS), and is based on several factors including:⁴

• Type of lung diagnosis
• Supplemental oxygen use and need for assisted ventilation
• Age
• Body mass index (BMI)
• Presence of diabetes
• Six-minute walk distance
• Pulmonary artery systolic pressure
• Pulmonary capillary wedge pressure
• Serum creatinine (measure of kidney function)
• Functional status

Who is not a good candidate for a transplant?

There are some people who are generally not recommended to undergo a lung transplant. This may include people who are infected with various organisms in the lungs, such as Burkholderia cenocepacia (B. cepacia).⁵. While it is possible to get a lung transplant with B. cepacia, this is an educated decision made by the CF care team. In addition, some people who are already intubated may not be good candidates.

Life with CF after a lung transplant

Lung transplants often improve survival rates, but the journey to recovery can be long. Getting a lung transplant does not fully resolve all problems that existed prior to transplant. For example, lung transplants do not reverse cirrhosis, diabetes, or osteoporosis. Furthermore, some of the post-transplant medications such as cyclosporine and tacrolimus are difficult to tolerate and may cause further adverse events. It is important to connect with a support system during the transplant process, to find emotional support in others who understand.

If you or a loved one with CF have received a lung transplant, how long did it take you or them to receive the procedure? Share your experiences below!