Talking With Your Transplant Team and Care Team
Deciding when to get listed for a lung transplant can be overwhelming. You must be physically and emotionally ready for the commitment before you decide to list. Your CF care team can guide you to a transplant center with expertise in cases like yours so that you can be fully informed before making your decision.
It is critical to discuss your life goals and learn about what you can expect throughout the lung transplant process. Doing so will help you decide on the best plan for you. The more your care team and transplant team know about what you want, the better they'll be able to help you weigh the benefits and risks that matter most to you.
Note: 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.
Another consideration: 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.”
What Are the Risks?
A lung transplant 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.
- Cancer: Immunosuppressants also increase your risk of certain types of cancer. These cancers can include, but are not limited to, post-transplant lymphoproliferative disease (PTLD), skin cancer, and colon cancer. People 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. The HPV (human papillomavirus) vaccine is also recommended to reduce the risk of several types of cancer.
- Other immune system complications: You will have to take immunosuppressants for your entire life. On top of an increased risk for developing infections and cancer, you may also be at an increased risk for developing other diseases, such as chronic kidney disease or low white blood cell counts.
- Airway problems: Problems can arise when there isn't enough blood flow to the donor lungs. This may resolve itself, but sometimes treatment is needed, such as procedures to open narrowed airways.
- 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. You can review this Post-Lung Transplant Care Checklist for more information about the care you may need after a transplant.
- Family planning 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 (called a maternal-fetal medicine specialist) before getting pregnant.
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 will still need to continue your CF care routine for the affected areas.
Doctors and researchers are always working to improve surgical techniques, immunosuppressants, 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.
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. Still, it is important to note that recovery takes time, and everyone's healing process looks different.
“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
Factors That Inform When You Get New Lungs
There are several factors that determine when you get new lungs, starting with how urgently you need lungs. The factors are represented by your Composite Allocation Score (CAS). These scores are based on factors that are specific to each person and their circumstances. The scores range from 0 to 100. The higher your score, the sooner you may be offered donor lungs. Each person’s score is calculated based on the factors below.
Factor considered | Maximum points |
Candidate medical urgency: How urgent a candidate’s need for transplant is. | 25 |
Likely survival: How likely a candidate is to survive five or more years after transplant. | 25 |
Blood type match: If the donor and candidate have matching blood types. | 5 |
Immune system matching (CPRA): If a candidate has pre-formed immune antibodies that may cause rejection of lungs from certain donors (panel reactive antibodies) and limit who they can receive lungs from. | 5 |
Height match: If the candidate is a similar height as the donor. | 5 |
Listed younger than 18: If the candidate is younger than 18 years old. | 20 |
Prior living donor: If the candidate has donated an organ in the past. | 5 |
Travel efficiency: How efficient it is to transport the donor organ to the candidate. | 5 |
Proximity efficiency: How near a candidate is to the donor organ. | 5 |
Total Possible Score | 100 |
You can calculate your CAS on the Organ Procurement and Transplantation Network web site.
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. Also, the donor's lungs must be the right size to fit into your chest cavity.
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 attack 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 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.