Lung Transplantation for Cystic Fibrosis

Cystic fibrosis is a genetic disease that affects the body's ability to produce mucus, leading to recurrent infections and inflammation in the lungs. Over time, these infections damage the lungs.

As cystic fibrosis progresses, it becomes more difficult to breathe and to engage in activities you enjoy. When your lung damage becomes severe, you may consider a lung transplant to improve your quality of life and help manage symptoms of the disease.

Lung transplants are increasingly used to treat severe cystic fibrosis. In 2017, for example, 250 people with cystic fibrosis in the U.S. received lung transplants.

This article discusses the benefits and risks of lung transplant for cystic fibrosis, and everything you need to know about the procedure before seeking out this type of treatment.

Risks and Benefits of a Lung Transplant

A lung transplant can give you a new, healthier set of lungs, which are not affected by cystic fibrosis. A new set of lungs can allow you to breathe more easily, get off of certain treatments such as nebulizers and supplemental oxygen, and engage in more activities.

A lung transplant can also extend your lifespan. A recent study found that over half of adults with cystic fibrosis who received a lung transplant live for 10 or more years following their surgery.

While receiving a new set of lungs may help some people with severe CF, this treatment option carries its own set of risks:

• Rejection. Your immune system can recognize your new set of lungs as foreign and attack them. This is known as rejection. Rejection may not cause any symptoms. If caught early, rejection is often treatable. To prevent rejection, you will need to take anti-rejection drugs that suppress your immune system for the rest of your life.
• Infections. Anti-rejection (immunosuppressive) drugs are necessary to prevent your immune system from attacking your new lungs. Because these drugs suppress your immune system, they increase your vulnerability to infections.
• Cancer. Your immune system helps keep abnormal cells from growing out of control and becoming cancerous. When your immune system is suppressed, you have an elevated risk of developing cancer.
• Other immunosuppressive drug side effects. Immunosuppressive drugs raise your risk of other health complications, such as kidney disease and low white blood cell counts.
• Restricted blood flow to the lungs. During transplantation, the airways of your new lungs are surgically attached to your airways. Sometimes blood flow to these areas of attachment becomes restricted. The lack of blood flow may heal itself, or you may need treatment to reopen your airways.
• Chronic lung allograft dysfunction (CLAD). People who have received a lung transplant can experience a significant loss in lung function. The exact reasons for this loss are unclear, but risk factors include infections and episodes of acute rejection.
• Childbearing-related complications. Immunosuppressive drugs can cause birth defects if taken during pregnancy. Young children also frequently develop and spread respiratory infections, which can threaten the health of a parent with a lung transplant.
  

Assessment and Eligibility

Before you can be listed for a lung transplant, you will need to undergo a thorough evaluation at one or more transplant centers to determine your eligibility.

When Should You Start Thinking About a Lung Transplant?

Receiving a lung transplant is a lengthy, challenging process, so it's a good idea to start thinking about whether you want to be listed to receive a new set of lungs well in advance—ideally before your symptoms progress and cause severe illness.

Starting early gives you time to discuss the possibility of a lung transplant with your cystic fibrosis care team, meet the transplant team, undergo an evaluation, and tackle certain barriers that might prevent you from being listed.

The Cystic Fibrosis Foundation's guidelines recommend discussing a lung transplant with your care team when your forced expiratory volume in one second (FEV1) drops below 50% predicted without an exacerbation.

Referral to a Transplant Center

If you and your cystic fibrosis care team decide you want to be referred for a lung transplant, your care team will contact a transplant care center and send them your medical records. You will then make an appointment at the care center.

Being listed to receive a lung transplant requires an extensive evaluation. After the evaluation your transplant team might decide:

• You are eligible to be listed at their center.
• You are not eligible to be listed right now, but could become eligible.
• You are not eligible for listing at their center.
  

The Evaluation Process

The transplant evaluation process is a comprehensive effort to ensure you will benefit from, and be able to care for, a new set of lungs. It involves:

• A medical examination. The transplant team at the care center will conduct a thorough physical exam, including tests to see how well your organs such as your lungs, heart, and kidneys are functioning, They will also perform tests to see what germs you carry in your lungs. The results of these tests may necessitate further testing, or rule you out as a candidate at certain transplant centers.
• A psychological evaluation. You will undergo a thorough psychological evaluation to determine your ability to cope with the challenges involved in receiving and caring for a lung transplant. A social worker or psychologist will gather information about you, your friends and family, and your social support system. The transplant team will also assess your ability to take your medications as prescribed and to abstain from smoking and illegal drug use.
• A financial evaluation. A financial coordinator at the center will meet with you to review your finances. They will help you determine what your insurance company will cover and to develop a plan for paying your out-of-pocket costs, such as co-pays and the cost of relocation.

These evaluations may identify barriers to you being listed at a particular transplant center. Some of these barriers may be surmountable; others may not. The Cystic Fibrosis Foundation recommends evaluation at least two centers before deciding you are ineligible for a lung transplant.

If you are deemed eligible, you will decide when to be listed on the United Network for Organ Sharing (UNOS) national waiting list. Once you are listed, you could receive a call to receive a transplant at any time.

Your position on the wait list will be determined by your lung allocation score (LAS). The LAS ranges from 1 to 100 and depends on your age, BMI, and how urgently you need new lungs.

Your blood and tissue types and your chest size will help determine whether you are a match for a particular donor.

Preparing for a Lung Transplant

Once you are listed, you could receive the call to head to the hospital at any time. It might take weeks or years.

During this time, be sure to keep up with your appointments and medications to stay as healthy as possible.

To prepare, you will need to have a bag packed with supplies for a three-week hospital stay. Once you receive the call, do not drink or eat anything so you will be ready for surgery.

While on the wait list, you may experience one or more dry runs—heading to the hospital only to learn that the donor lungs you were to receive are not healthy enough to be transplanted.

Lung Transplant Surgery

You will receive general anesthesia for your double lung transplant surgery, which will keep you sedated during the procedure.

Your surgeon will make an incision below your breasts, remove your lungs, and replace them with lungs from a deceased donor. The new lungs will be connected to your airways and blood vessels, and your incision will be closed.

Your surgical team will insert temporary drains in your chest to help remove fluid. They will also insert a temporary breathing tube. You will be catheterized until you are able to urinate on your own.

After surgery, you will be moved to the intensive care unit (ICU). You will spend another seven to 14 days in the hospital for monitoring and recovery. You will receive medications through an IV line. As you recover, you will slowly regain your ability to breathe and eat on your own.

Recovery and Prognosis

When you first head home from the hospital, you will not be able to drive or lift heavy objects. You will need help fulfilling your many responsibilities, including:

• Caring for your incision
• Attending follow-up appointments at your transplant center
• Receiving physical or occupational therapy
• Maintaining treatments for cystic fibrosis in the rest of your body

You will find yourself slowly getting stronger and more capable as you recover from your surgery and adjust to life with your new lungs.

Most lung transplant patients experience one or more hospitalizations after receiving their new lungs. Remember, being hospitalized is not necessarily a sign of transplant failure or that you did anything wrong.

You will need to maintain your old precautions against cross-infection, avoiding others with cystic fibrosis. Because your immune system is suppressed, you will also need to take precautions to avoid infections, such as washing your hands, getting a yearly flu shot, and avoiding others who are sick.

A Word From Verywell

A lung transplant can extend and improve your life, allowing you to resume many activities you once enjoyed and to get off of the leash of supplemental oxygen.

However, getting evaluated, being listed, and receiving a lung transplant can be a lengthy and arduous process, with many frustrations and setbacks along the way.

Be sure to start discussions with your care team early, so you can identify potential barriers to transplant, line up social support, and prepare yourself for the challenges ahead. You may find online support groups, such as CF Connect, useful during your journey.