Cystic fibrosis and lung transplants

Cystic fibrosis is a genetic disease that causes mucus to build up in your lungs. Over time, repeated bouts of inflammation and infection can cause permanent lung damage. As your condition progresses, it will get harder to breathe and participate in the activities you enjoy.

Lung transplants are increasingly used to treat cystic fibrosis. In 2014, 202 patients with cystic fibrosis in the United States received a lung transplant, according to the Cystic Fibrosis Foundation (CFF).

A successful lung transplant can make a substantial difference in how you feel on a day-to-day basis. While it’s not a cure for cystic fibrosis, it can provide you with a healthier set of lungs. This can allow you to do more activities and potentially lengthen your life.

There are many things to consider before having a lung transplant. Continue reading to learn more about the potential benefits and risks of lung transplant surgery.

What are the potential benefits of a lung transplant?

If you have cystic fibrosis and your lungs are functioning poorly, you might be eligible for a lung transplant. You’re probably having trouble breathing and sitting out activities you once enjoyed.

A successful lung transplant may improve your quality of life in tangible ways.

A new set of healthier lungs will make it easier to breathe. This can help you participate in more of your favorite pastimes.

What are the potential risks of a lung transplant?

A lung transplant is a complex procedure. Some of the primary risks are:

  • Organ rejection: Your immune system will treat your donor lungs as foreign and try to destroy them, unless you take antirejection drugs. While organ rejection is most likely to happen within the first six months after your surgery, you’ll have to take antirejection drugs to suppress your immune system for the rest of your life.
  • Infection: Antirejection medications dampen your immune system, increasing your chances of developing infections.
  • Other diseases: Because antirejection medications suppress your immune system, you’ll also be at increased risk of cancer, kidney disease, and other conditions.
  • Problems with your airways: Sometimes, blood flow from your airways to your donor lungs may be restricted. This potential complication may heal on its own, but if not, it can be treated.

In men, antirejection medications can cause birth defects in their children. Women who have had a lung transplant may be at risk of serious complications during pregnancy.

Who is eligible for a lung transplant?

Not everyone is eligible for a lung transplant. Your doctor will need to assess the chances that you’ll benefit from it and be able to stick with your treatment plan. It can take weeks to evaluate your case and determine if you’re an eligible candidate.

This process may involve:

  • Physical evaluations, including tests to assess your lung, heart, and kidney functions. This can help your doctor assess your need for a lung transplant, as well as your risk of potential complications.
  • Psychological evaluations, including consultations with a social worker or therapist. Your doctor, social worker, or therapist may also want to meet some of your friends and family members to make sure that you have a good support system and the ability to manage your post-op care.
  • Financial evaluations to assess your medical coverage and help you determine how you’ll pay for out-of-pocket expenses, both in the short and long term.

If your doctor determines that you’re a good candidate, you’ll be added to the lung transplant list. You’ll be instructed in how to prepare for your surgery. You could receive a call that donor lungs are available at any time.

Donor lungs come from people who are recently deceased. They’re only used when they’re found to be healthy.

What’s involved in a lung transplant?

To perform a double lung transplant, your surgical team will likely make a horizontal incision below your breasts. They will remove your damaged lungs and replace them with donor lungs. They will connect blood vessels and airways between your body and your donor lungs. In some cases, they may use a heart-lung bypass machine to keep oxygen flowing through your body during this procedure.

Your surgical team will close your chest using stitches or staples. They will dress your incision wound, leaving a few tubes in to allow fluids to drain. These tubes are temporary. You’ll also have a breathing tube inserted until you can breathe without it.

Immediately following your surgery, you’ll be monitored for breathing, heart rhythms, blood pressure, and oxygen levels. When everything is functioning in a satisfactory way, you’ll be moved out of intensive care. You’ll continue to be closely watched as you recover. You will undergo periodic blood tests to learn how well your lungs, kidneys, and liver are working.

Your hospital stay will likely last a week or two, depending on how well you’re doing. Before you’re discharged, your surgical team should give you directions on how to care for your incision and promote your recovery at home.

What’s the recovery like?

A lung transplant is a major surgery. It can take months to fully recover from it.

Your surgical team should provide full instructions for your home care. For example, they should teach you how to keep your incision clean and dry until your stitches or staples are removed. They should also teach you how to recognize signs of infection.

You’ll be at increased risk of infection due to the antirejection drugs that you need to take following a lung transplant. If you have any of the following symptoms, call your doctor right away:

  • fever of 100.4°F or higher
  • fluids leaking from your incision
  • worsening pain at your incision site
  • shortness of breath or trouble breathing

You may have to make more frequent doctor visits in the year following your lung transplant surgery. Your doctor may order tests to monitor your recovery, such as:

  • blood tests
  • lung function tests
  • chest X-ray
  • bronchoscopy, an examination of your airways using a long thin tube

If your lung transplant is successful, you will have a new set of lungs that work better than your old lungs, but you will still have cystic fibrosis. That means you’ll need to continue your cystic fibrosis treatment plan and visit your doctor regularly.

What’s the outlook?

Your individual outlook will depend on your age and how well your body adjusts to your lung transplant.

In the United States, more than 80 percent of people with cystic fibrosis who have a lung transplant are alive after a year following their procedure, reports the CFF. Over half survive more than five years.

A Canadian study published in 2015 in the Journal of Heart and Lung Transplantation found the five-year survival rate for cystic fibrosis patients following a lung transplant was 67 percent. Fifty percent live 10 years or more.

A successful lung transplant can potentially change your life by alleviating your symptoms and allowing you to be more active.

Tips for talking to your doctor

When considering a lung transplant, ask your doctor if all other options have been explored first. Ask them to help you understand the potential benefits and risks of a transplant. Ask what you can expect if you don’t opt for the transplant.

Once you feel comfortable with the idea of a lung transplant, it’s time to learn more about what lies ahead. Once you’re on the transplant list, you’ll need to be prepared to get the call that your donor lungs have arrived, no matter when it comes.

Here are a few questions to get the conversation with your doctor started:

  • What do I need to know and do while I’m on the waiting list?
  • What preparations should I make for when the lungs become available?
  • Who will make up the lung transplant team and what is their experience?
  • How long should I expect to stay in the hospital after the surgery?
  • What medications will I need to take following the surgery?
  • After surgery, what symptoms mean I need to see a doctor?
  • How often will I need to follow up and what testing will be involved?
  • What will recovery be like and what’s my long-term outlook?

Let your doctor’s answers guide you toward more in-depth questions.