Lung transplants may be an option for people with end stage cystic fibrosis. However, lung transplants are complex operations that carry a risk of significant complications.

Doctors may only consider this option if a person has already tried all other treatment methods and their cystic fibrosis has led to life threatening lung problems.

This article details important facts about lung transplants for cystic fibrosis.

A group of surgeons and assistants performing a lung transplant for cystic fibrosis. -1Share on Pinterest

In people with cystic fibrosis, a thick and sticky mucus builds up in the lungs. This mucus buildup can cause breathing difficulties, lung inflammation, and lung infections.

As a 2022 study explains, cystic fibrosis can also lead to life threatening forms of lung disease.

People are born with cystic fibrosis, and it gradually worsens over time. As the disease reaches its late stages, doctors may consider a lung transplant. This will not cure cystic fibrosis, but it may alleviate some symptoms and increase life expectancy.

According to the Cystic Fibrosis Foundation (CFF), a doctor may refer someone as a candidate for a lung transplant if they meet specific criteria. The criteria include some of the following:

  • rapidly declining lung function
  • inability to walk 400 meters in 6 minutes due to low lung function
  • hypoxemia, or a low level of oxygen in the blood
  • hypercapnia, or a high level of carbon dioxide in the blood
  • frequent worsening of symptoms that require medical attention

The type of lung transplant suitable for people with cystic fibrosis is called a double lung transplant. This is where a surgeon removes both lungs and replaces them with lungs from a donor.

According to the CFF, a person can expect the following from a lung transplant:

  1. The lung transplant occurs under general anesthesia, which a person will receive as gas through a mask or as liquid through an injection. They will become unconscious and will not feel any pain.
  2. A surgeon will begin the procedure by attaching a tube to the person’s lungs. The tube connects to a ventilator, which induces artificial breathing. The ventilator is necessary because surgeons cannot operate on the lungs when they are breathing naturally.
  3. During the operation, the surgeon will make incisions in the person’s chest. They will then remove one lung before replacing it with a donor lung. This involves attaching airways and blood vessels to the donor lung. They then repeat this procedure for the other lung.
  4. Once the operation is complete, the surgeon closes the incision with staples or stitches. They will attach drainage tubes to the chest. This will help the body to clear postoperative buildups of air, fluid, and blood.

If a doctor suggests a lung transplant, healthcare professionals will need to run tests to check whether the person is well enough to have the surgery. They will check the functioning of all major organs to ensure they will still work after surgery.

Once a doctor approves a person for a lung transplant, they will put them on a waiting list for a donor. The person may need to wait for up to 3 years for a donor to become available.

A person may wish to use this time to work with a doctor to make lifestyle changes that will prepare them for surgery. These may include reaching and maintaining a moderate weight, eating a balanced diet, and regularly exercising.

A doctor may ask a person not to eat or drink the night before the procedure.

When a person arrives at the hospital for the operation, healthcare professionals will monitor them using blood tests, chest X-rays, and electrocardiograms.

As the CFF also explains, recovery from a lung transplant begins in an intensive care unit (ICU).

Medical staff will attach machines to the person to monitor vital signs. Nurses and doctors will use these to check the person’s condition regularly.

Many people experience pain after surgery. For this reason, people will receive pain-relieving medication through an IV drip.

A person will not be able to breathe on their own for a few hours after surgery. They will need to rely on a ventilator.

Once a healthcare professional removes the ventilator, a person can start drinking liquids. Doctors will encourage them to gradually eat solid foods.

Healthcare professionals will provide special physical therapy and breathing exercises.

After some time, the person will be well enough to leave the ICU but will remain in the hospital. During this time, they will gradually work toward increasing their physical activity level.

It may be several weeks before an individual can return home. Doctors must be confident that serious complications are unlikely before they release someone.

People must also receive immunosuppressive drugs after surgery. These reduce the risk of the body rejecting the donor lungs.

Lung transplants cannot cure cystic fibrosis. However, research shows that lung transplants tend to increase the life expectancy of people with cystic fibrosis.

Lung transplants can also cause a significant reduction in symptoms of this condition.

A lung transplant is a complex operation that carries a lot of risks. It is best to discuss these risks with a doctor.

According to a 2022 study, it is possible for people to die during the operation. Additionally, after respiratory failure, transplant-related complications are the second most common cause of death among people with cystic fibrosis.

Lung transplants can also lead to life threatening complications after the surgery. These include organ rejection, infections, and blood clots.

A healthcare team will monitor a person in the hospital for the weeks following the operation, and the person will receive medications to help reduce these risks.

According to a 2022 review, no treatments for late stage cystic fibrosis are as effective as lung transplants.

For people who cannot have surgery, pharmaceutical treatment is the only option. However, this option is not very effective at this stage of the disease.

Experts emphasize the importance of additional measures in people with cystic fibrosis. These include regular exercise and diets that are high in calories, fats, and supplemental fat-soluble vitamins.

This section answers some frequently asked questions about lung transplants and cystic fibrosis.

What is the life expectancy after a lung transplant for cystic fibrosis?

The CFF states that between 1992–2017, around half of people who received lung transplants were alive 9.9 years after the surgery.

What percentage of people with cystic fibrosis get lung transplants?

The CFF states that every year, only 6–8% of people with end stage cystic fibrosis receive a lung transplant.

What disqualifies a person from getting a lung transplant?

Doctors may have to disqualify someone from a lung transplant for various reasons. Some reasons include a recent history of cancer, a significant problem with another major organ, or an unmanageable bleeding disorder.

A person also has to be in otherwise good health to receive a lung transplant, as it is a major operation.

Doctors may be less likely to recommend lung transplants for people over age 65 years old and not in good overall health, as well as people who have obesity.

For people with cystic fibrosis, lung transplants are the most effective means of extending life expectancy and reducing symptoms. The operation typically involves replacing the lungs, one at a time, with new donor lungs.

Despite the benefits of lung transplants, this procedure comes with the risk of serious complications.