Lung Transplantation in Children

A lung transplant is surgery to remove 1 or both diseased lungs and replace them with healthy lungs from a suitable organ donor. In most cases, lungs that are transplanted come from people who have died and donated their organs. This is called a deceased donor transplant. But healthy, nonsmoking adults who make a good match may also be able to donate part of 1 of their lungs. This type of transplant is called a living donor transplant. People who donate part of a lung can live healthy lives with the remaining lung.

Types of lung transplant procedures include:

• Single lung. One lung is transplanted.

• Double lung. Both lungs are transplanted.

• Bilateral sequential. Both lungs are transplanted, one at a time.

• Heart-lung transplants. Both lungs and the heart are taken from a single donor.

The type of procedure done will depend on your child’s condition.

A lung transplant is an option for children and young adults with long-term (chronic) lung diseases, when other treatments no longer work. A lung transplant may be needed if your child has any of these diseases:

• Severe cystic fibrosis (CF). This is a common inherited and underlying disease that may require a lung transplant among children, teens, and young adults.

• Chronic lung disease, bronchopulmonary dysplasia, or interstitial lung disease. This is a general term for long-term respiratory problems in premature babies. It is caused by a lung injury to babies who must use a machine (ventilator) and oxygen for breathing.

• Bronchiolitis obliterans. Damage to the lungs due to severe lung infections or injuries.

• Pulmonary hypertension. This is increased or high pressure in the arteries of the lungs.

• Heart disease. Heart disease or heart defects affecting the lungs may need a heart-lung transplant.

• Pulmonary fibrosis. This is scarring of the lungs.

• Other lung diseases. These include end-stage lung disease, other chronic lung diseases, or inherited disorders that may cause lung disease.

Lung transplants can now be done on people of all ages, from newborn to adult. Your child's healthcare provider will discuss the lung transplant selection criteria with you.

In most cases, lungs that are transplanted come from organ donors who have died. This is a deceased donor transplant. These organ donors are adults or children who have become critically ill and won't live as a result of their illness. If the donor is an adult, they may have agreed to be an organ donor before becoming ill. Parents or spouses can also agree to donate a relative's organs. Donors can come from any part of the U.S.

Healthy, nonsmoking adults who make a good match may also be able to donate part of 1 of their lungs. This is a living donor transplant.

The Organ Procurement and Transplant Network (OPTN) is responsible for allocating transplant organs in the U.S. OPTN receives data from hospitals and medical centers all over the country about adults and children who need organ transplants. The healthcare team that currently follows your child will send the data to OPTN. They will update OPTN as your child's condition changes.

Criteria have been developed to be sure that all people on the waiting list are judged fairly in terms of the severity of their illness and the urgency of receiving a transplant. Once OPTN receives the data from local hospitals, people waiting for a lung transplant are placed on a waiting list. The people in most urgent need of a transplant are placed highest on the list. They are given first priority when a donor lung becomes available.

When a donor lung becomes available, a computer searches all the people on the waiting list for a lung. People who are not good matches for the available lung are set aside. A new list is made from the remaining people. The person at the top of this list is considered for the transplant. If they are not a good candidate, for whatever reason, the next person is considered, and so forth. In some cases people lower on the list may be considered before a person at the top. Reasons for this can include the size of the donor organ and how far away the donor is located from the recipient.

Your child's name may also be given to the United Network for Organ Sharing (UNOS) and placed on the waiting list.

An extensive evaluation must be done before your child can be placed on the transplant list. Testing includes:

• Blood tests

• Pulmonary function tests
• Tests to measure your child's ability to exercise and the amount of oxygen they need

• Imaging exams and other diagnostic tests may be needed

• Psychological and social evaluation of your child (if old enough) and your family

Tests are done to gather information to help make sure your child receives a donor organ that is a good match. These tests will analyze your child’s general health (including heart, lung, and kidney function), nutritional status, and the presence of infection. Blood tests will help improve the chances that the donor organ won't be rejected. These tests may include:

• Your child's blood type. Each person has a certain blood type. When getting a transfusion, the blood received must be compatible with your child's blood type. If it isn't, your child's body will make antibodies against the blood. The same type of reaction will occur if the blood in a donor organ enters your child's body during a transplant. Reactions can be prevented by matching the blood types of your child and the donor.

• Kidney, liver, and other vital organ function tests

• Tissue typing

• Viral studies. These tests determine if your child has antibodies to viruses such as cytomegalovirus (CMV) that may increase the likelihood of rejecting the donor organ.

The diagnostic tests that are done are extensive. But they’re needed to understand your child’s full health status. Other tests that may be done include:

• Blood tests

• Urine tests

• Echocardiogram. This test evaluates the structure and function of the heart. It uses sound waves recorded on an electronic sensor that make a moving picture of the heart and heart valves.

• Electrocardiogram (ECG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and finds heart muscle damage.

• Cardiac catheterization.  This diagnostic procedure uses a tiny, hollow tube (catheter). It is guided through a vein or artery into the heart. This is done to image the heart and blood vessels. A colorless, liquid dye is given through the catheter. Moving X-ray pictures are made as the dye travels through the heart.

• MRI. A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to make detailed images of organs and structures within the body.

• Lung biopsy. This procedure removes tissue samples from a lung (with a needle or during surgery). The samples are checked under a microscope.

• MUGA heart imaging. A nuclear scan to see how the heart walls move and how much blood is expelled with each heartbeat.

• Sputum culture. A diagnostic test done on the material that is coughed up from the lungs and into the mouth. A sputum culture is often done to determine if an infection is present.

• Pulmonary function tests.  These tests help to measure the lungs' ability to move air into and out of the lungs effectively. The tests are usually done with special machines into which a child must breathe.

• Tuberculosis (TB) test

• CT scan. A diagnostic imaging test that uses a combination of X-rays and computer technology to make horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body. This includes the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays.

• Bronchoscopy. This test uses a video scope to check the lungs and to do a biopsy and culture for possible infection.

The transplant team will look at all the information from interviews, your child's health history, physical exam, and diagnostic tests to determine if your child can be a candidate for a lung transplant. After your child has been accepted to have a lung transplant, they will be placed on the OPTN organ transplant list. 

The group of specialists involved in the care of children who are undergoing a transplant procedure is often called the transplant team. The lung transplant team consists of:

• Transplant surgeons (cardiothoracic or thoracic). These are doctors who specialize in transplantation and who will be doing the surgery. The transplant surgeons coordinate all team members. They follow your child before the transplant and continue to follow your child after the transplant and after discharge from the hospital.

• Pulmonologists. Doctors who specialize in lung function and disease. Pulmonologists will help manage your child before and after the surgery.

• Transplant nurse coordinator. A nurse who organizes all aspects of care provided to your child before and after the transplant. The nurse coordinator will provide patient education and coordinate the diagnostic testing and follow-up care.

• Social workers. Professionals who will provide support to your family. They can help your family deal with many issues that may come up. This includes lodging and transportation, finances, and legal issues. They can also help coordinate alternative means for school, so that your child does not get behind. Licensed clinical social workers (LCSWs) can also provide solo and family counseling.

• Nutritionists and dietitians. Professionals who will help your child meet their nutritional needs before and after the transplant. They will work closely with you and your family.

• Physical therapists. Professionals who will help your child become strong and independent with movement and endurance after the transplantation.

• Pastoral care. Chaplains who provide spiritual care and support.

• Other team members. Several other team members will evaluate your child before transplantation and provide follow-up care, as needed. These include, but are not limited to, the following:

  • Pharmacists

  • Anesthesiologists

  • Respiratory therapists

  • Cardiologists

  • Hematologists

  • Urologists

  • Nephrologists

  • Infectious disease specialists

  • Lab technicians

  • Psychologists

  • Child life specialists

There is no definite answer to this question. It may take 1 to 2 years on the waiting list before a suitable donor lung is available. During this time, your child will have close follow-up with their healthcare providers and the transplant team. Many support groups are also available to help you during this waiting time.

Each transplant team has its own guidelines about waiting on the transplant list and being told when a donor organ is available. In most cases, you will be notified by phone that a donor organ is available. You will be told to come to the hospital right away so your child can be ready for the transplant.

Once an organ becomes available to your child, you and your child will be immediately called to the hospital. This call can happen at any time. So you should always be ready to go to the hospital if needed. Have your bags packed with items suggested by the transplant team and copies of all necessary paperwork. Once at the hospital, your child will have some more final blood work and tests to confirm the match of the organ.

The child will then go to the operating room. The transplant surgery may take from 6 to 12 hours. But this can vary greatly depending on the type of surgery and on each individual case. Single lung transplantation is done with the child under general anesthesia. For this type of transplant it is not always necessary to reroute the blood through a heart-lung bypass machine (cardiopulmonary bypass).

But if 2 separate lungs are transplanted, cardiopulmonary bypass is often needed. Heart-lung transplants always require the use of cardiopulmonary bypass.

During the surgery, a transplant team member will keep you informed on the progress of the transplant.

After the surgery, your child will go to an intensive care unit (ICU) to be watched closely. The length of time your child will spend in the ICU will vary based on your child's unique condition. After your child is stable, they will be sent to the special unit in the hospital that cares for people who have had lung transplants. Your child will continue to be watched closely. You will be educated on all aspects of caring for your child during this time. This will include information about medicines, activity, follow-up, diet, surgical site care, and any other specific instructions from your transplant team.

Rejection is the body’s normal reaction to a foreign object. When a new lung is placed in your child's body, the body sees the transplanted organ as a threat and tries to attack it. The immune system makes antibodies to try to destroy the new organ, not realizing that the transplanted lung is beneficial.

Each child may have different symptoms of rejection. Some common symptoms of rejection may include:

• Fever

• Chills

• Flu-like aches

• Decreased urine output or fewer wet diapers than normal

• Shortness of breath

• Pain over the transplanted lung

Your child's transplant team will tell you who to call right away if any of these symptoms occur.

Your child must take medicine for the rest of their life to fight rejection. Each child is unique, and each transplant team prefers different medicines.

Anti-rejection medicines (immunosuppressants) affect the immune system. They reduce the immune system's strength. This helps protect the new lungs against rejection. So children who have a transplant will be at higher risk for infections. A balance must be kept between preventing rejection and making your child more likely to have an infection. Your child will have blood tests at different times to measure the amount of medicine in their body. This makes sure your child doesn't get too much or too little of the medicine. White blood cells are also an important indicator of how much medicine your child needs.

The risk of infection is especially great in the first few months. That’s because higher doses of anti-rejection medicines are given during this time. Your child will most likely need to take medicines to prevent other infections from occurring. Some of the infections your child will be more at risk for include oral yeast infection (thrush), herpes, and respiratory viruses. Your child's transplant team can advise about certain vaccines your child needs. Family members are advised to get the flu vaccine each year. Talk with your transplant team about recommendations for COVID vaccines.

A lung transplant is a major life event. It can cause mood changes and relational stress. It may also cause your child to have concerns about body image and school. Depression or anxiety can happen before, during, or after a transplant. Discuss your concerns with your child's healthcare team. There may be a social worker, counselor, psychologist, or case manager who can help. Support groups are also available in person or online for children and their families. Ask your healthcare team about local support groups.

Your child will have very close follow-up with the transplant team after leaving the hospital. This will allow for close watching of your child and the function of the transplanted lung. Follow-up visits may include:

• Complete physical exam

• Chest X-ray

• Blood work

• Lung function tests

• Bronchoscopy

• Continued education for you and your child

• Medicine changes

Children who have had a lung transplant will need lifelong follow-up with healthcare providers who specialize in transplant medicine. It’s vital to keep appointments with your child's transplant care provider. You must also keep in contact with the transplant team when symptoms of rejection occur. You (and your child, when old enough) are the first line of defense. You must know and recognize the signs and symptoms of rejection. Report them as soon as possible to the transplant team.

Living with a transplant is a lifelong process. Medicines must be given that trick the immune system so it will not attack the transplanted organ. Other medicines must be given to prevent side effects of the anti-rejection medicines, such as infection. Frequent visits to and contact with the transplant team are key. Knowing the symptoms of organ rejection (and watching for them on a daily basis) is critical. When your child is old enough, they will need to learn about anti-rejection medicines (what they do and the symptoms of rejection). Over time your child must be able to be independent.

Children who have had lung transplants should never smoke. They should also stay away from secondhand smoke.

Every child is unique and every transplant is different. Results keep improving as doctors and scientists learn more about how the body deals with transplanted organs and search for ways to improve transplantation.