Deciding When to Get Listed

There are many things that you can do while waiting for donor lungs to become available. Preparing for a lung transplant includes maintaining your health, performing your routine cystic fibrosis care, and being ready to respond when donor lungs are available.

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Summary
  • Deciding if you want to be listed for a lung transplant can be overwhelming.
  • Knowing the risks and benefits can help you decide.
  • Your transplant team can help you decide.

Deciding when to get listed for a lung transplant can be overwhelming. You have to be physically and emotionally ready for the commitment before you decide to list. There are several factors that determine when you get new lungs. First, of course, is how urgently you need lungs, which is represented by your lung allocation score (LAS). Next, you have to be physically compatible with the donor lungs, which includes matching your blood and tissue types. In addition, the donor lungs need to be the right size to fit into your chest. Once you're listed, there is no way to predict when the transplant call will come. It could be as soon as the next day or you could wait years.

In addition, many people with cystic fibrosis adapt to their physical decline. It becomes a “new normal.” Thus, they feel that they aren't ready to be listed because they “don't feel that bad.”

Your CF care team can guide you to a transplant center with expertise in cases like yours so that you can be fully informed prior to making your decision.

It is critical to discuss your life goals with your care team and learn about what you can expect throughout the lung transplantation process. Doing so will enable the team to recommend the best plan for you. The more they know about what you want, the better they'll be able to help you weigh the benefits and risks that matter most to you.

Jeremy Weyandt, an adult with cystic fibrosis, describes his journey to getting a transplant.

What Are the Risks?

Lung transplantation can extend and improve your life, but it is not without risks. It is possible that none of these complications will affect you; it is also possible that you will experience more than one complication.

  • Organ rejection: Your immune system's job is to recognize things in your body that aren't supposed to be there and get rid of them. These things could be viruses, bacteria, or even cancer cells. When you have a lung transplant, your immune system might recognize your new lungs as a foreign object. If this happens, your immune system will try to get rid of them or “reject” them. Acute rejection is most common in the first 12 months after a transplant, but it can occur at any time. It can occur suddenly or gradually and it can occur without any detectable symptoms. Fortunately, rejection can be treated, especially if it is caught early. However, you may need to return to the hospital to receive treatment for rejection.
  • Chronic Lung Allograft Dysfunction (CLAD): CLAD refers to a significant loss in lung function that can occur in people who have received a lung transplant. Although little is known about the mechanisms in the body that lead to CLAD, associated risk factors include episodes of acute rejection and infection. About 50 percent of people develop CLAD within five years -- for some people, CLAD can be associated with rapid decline and lung failure.
  • Infections: After your transplant, you will take medications that help to reduce the risk of rejection. These medicines are called immunosuppressants or immunosuppressive drugs. Immunosuppressants work by weakening, or suppressing, your immune system. When your immune system is weaker, it cannot fight against bacteria and viruses. As a result, transplant recipients are at an increased risk of getting infections. After transplant, you will be on medications to prevent infections. Infections can be very serious and may require further hospitalizations.
  • Airway problems: Problems can arise when there isn't enough blood flow to the areas where your airways were surgically connected to the airways of the donor lungs. This may heal by itself, but sometimes additional treatment is required. Some individuals require many procedures to open their narrowed airways.
  • Cancer: A healthy immune system works constantly to destroy abnormal cells in your body. When you take immunosuppressive drugs and your immune system cannot do its job very well, abnormal cells can multiply and turn into cancer. After your transplant, you will have to take immunosuppressive drugs for the rest of your life. This puts you at an increased risk for developing cancers, such as post-transplant lymphoproliferative disease (PTLD), skin cancer, or colon cancer. Individuals with CF have a higher risk of developing PTLD because the virus that causes PTLD (Epstein-Barr virus, EBV) is more likely to be a new exposure in the younger CF population. Most transplant programs screen for EBV in the blood routinely. Colon cancer screening is recommended more frequently and earlier for post-transplant individuals with CF.
  • Complications of CF: You still will have CF in your other organs, except for in your lungs. You will need to continue your routine care for these organs because you will still be at risk for CF-related complications in these areas, including diabetes, osteoporosis, and vitamin deficiencies.
  • Other complications: You will have to take immunosuppressive drugs for your entire life. In addition to an increased risk for developing cancer, you also may be at an increased risk for developing other diseases, such as chronic kidney disease, or low white blood cell counts. 
  • Other considerations: Commonly prescribed antirejection medications have been known to cause birth defects. Women with CF who become pregnant after transplant may experience health risks. Young children carry respiratory viruses frequently, which can be seen as a threat to transplant recipients of either gender. It is important to discuss family planning with your CF team, transplant team, and an obstetrician with experience in high-risk cases prior to becoming pregnant.


Finding a Match

It is essential to the success of your transplant that the lungs you receive are closely matched to your body. Finding lungs that are a good match for you is not as simple as matching your blood type. The tissue that your donor's lungs are made of must also be compatible with the antibodies you have circulating in your blood. Additionally, the donor's lungs must be the right size to fit into your chest cavity.

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Blood Type

There are four blood types: A, B, AB, and O. Every person has one type of blood. Not all blood types are compatible with another. If you get donor lungs from someone whose blood type is not compatible with yours, your immune system will recognize the lungs as foreign and try to destroy them.

  • Type A: Can receive lungs from blood types A and O.
  • Type B: Can receive lungs from blood types B and O.
  • Type AB: Can receive lungs from blood types A, B, AB, and O (People with type AB blood are known as universal recipients and are compatible with any other blood type.).
  • Type O: Can receive lungs from blood type O (People with type O blood are known as universal donors, because all blood types are compatible with type O, but they can only receive lungs or blood from others with type O.),

Antibodies

Your body uses human leukocyte antigens (HLAs) as one way of identifying foreign proteins. HLAs are proteins that are located on the surfaces of your white blood cells and other tissues in your body. People who have been exposed to someone's HLA (for example, through pregnancy, blood transfusion, or prior transplant) can develop antibodies against those HLAs. Blood tests are done during your evaluation to determine your HLA type and whether you have HLA antibodies. If you have HLA antibodies, your donor's HLA will have to be screened before lungs can be accepted for you.

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What Are the Benefits?

A lung transplant is not without risks, but it offers you a chance you may not otherwise have to improve your health and extend your life. If the transplant is successful, you will have a healthy set of lungs that are not affected by cystic fibrosis. It is important to remember, however, that a lung transplant is not a cure and you still will have CF in other parts of your body. You still will need to continue your CF care routine for the affected areas.

Doctors and researchers are always working to improve surgical techniques, immunosuppressive drugs, post-transplant care procedures, and the methods used to preserve donor organs until they can be transplanted. Advances in these areas have helped to increase the number of successful transplants done every year, as well as the quality of life of transplant recipients.

In this clip, from ‪18:12-18:30, Joseph Pilewski, MD, discusses that among adults with CF, more than 80 percent of lung transplant recipients are still alive after one year, and more than 50 percent are alive after nine years.


“So you see this drop in survival over the first 5-10 years and a little bit of a flattening out of the curve as you go further out. But it clearly demonstrates that the survival isn't perfect, and we want to be careful not to have patients get transplanted too soon because this clearly has some significant risk of death in the first several years after transplant.” -- Joseph Pilewski, MD
Many people who have had successful transplants report being able to breathe more easily and resume doing things they enjoyed before their lung function declined. Some people have been able to participate in challenging physical activities after transplant.

“The past year has been rough, but exciting and full of huge changes. I used to be on an oxygen leash 24/7, nebulized breathing treatments every four hours, and IV antibiotics every three weeks. Now, I am IV-less, rarely cough, and no longer use supplemental oxygen.” -- Tiffany Rich, adult with CF, from the CF Community Blog

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Lung Transplantation
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