Lung Transplantation

What is a lung transplant?

A lung transplant is a surgical procedure performed to remove one or both diseased lungs from a patient and replace it with a healthy one from another person. The majority of lungs that are transplanted come from deceased organ donors. This type of transplant is called a cadaveric transplant. Healthy, nonsmoking adults who make a good match may be able to donate a part (a lobe) of one of their lungs. This type of transplant is called a living transplant. Individuals who donate a part of a lung can live healthy lives with the remaining lung tissue.

Various types of lung transplant procedures include single lung (transplantation of one lung); double lung, bilateral sequential, or bilateral single (transplantation of two lungs); and heart-lung transplants. The type of procedure performed depends on the condition of the recipient.

Why is a lung transplant recommended?

Lung transplantation is a treatment option for children and young adults with severe cystic fibrosis (CF), with end-stage lung disease, and other chronic lung diseases. CF is the most common underlying disease that may require a lung transplant among children, adolescents, and young adults. Some of the other diseases that may require a lung transplant among this population include the following:

• Bronchopulmonary dysplasia or chronic lung disease, CLD (This is a general term for long-term respiratory problems in premature babies resulting from lung injury to babies who must use a mechanical ventilator and oxygen for breathing.)
• Pulmonary hypertension (increased pressure in the arteries of the lungs)
• Heart disease or heart defects affecting the lungs (may require a heart-lung transplant)
• Pulmonary fibrosis (scarring of the lung)
• Alpha-1-antitrypsin deficiency

Lung transplants can now be performed in all ages--from newborn to adult. Your child's doctor will discuss the lung transplant selection criteria with you.

Where do transplanted organs come from?

The majority of lungs that are transplanted come from deceased organ donors. Organ donors are adults or children who have become critically ill and will not live as a result of their illness. If the donor is an adult, he or she 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 United States. This type of transplant is called a cadaveric transplant.

How are transplanted organs allocated?

The Organ Procurement and Transplant Network (OPTN) is responsible for transplant organ distribution in the United States. OPTN oversees the allocation of many different types of transplants, including liver, kidney, pancreas, heart, lung, and cornea.

OPTN receives data from hospitals and medical centers throughout the country regarding adults and children who need organ transplants. The medical team that currently follows your child is responsible for sending the data to OPTN and updating them as your child's condition changes.

Criteria have been developed to ensure that all people on the waiting list are judged fairly as to 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 and given a "status" code. The people in most urgent need of a transplant are placed highest on the status list and 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 and sets aside those who are not good matches for the available lung. A new list is made from the remaining candidates. The person at the top of the specialized list is considered for the transplant. If he or she is not a good candidate, for whatever reason, the next person is considered, and so forth. Some reasons that people lower on the list might be considered before a person at the top include the size of the donor organ and the geographic distance between the donor and the recipient.

How is my child placed on the waiting list for a new lung?

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

• Blood tests
• Diagnostic tests
• Psychological and social evaluation of the child (if old enough) and the family

Tests are done to gather information that will help you ensure your child receives a donor organ that is a good match. These tests include those to analyze the general health of the body, including the child's heart, lung, and kidney function, the child's nutritional status, and the presence of infection. Blood tests will help improve the chances that the donor organ will not be rejected. These tests may include:

• Your child's blood type. Each person has a specific blood type: type A+, A-, B+, B-, AB+, AB-, O+, or O-. When receiving a transfusion, the blood received must be a compatible type with your child's own, or an allergic reaction will occur. The same allergic reaction will occur if the blood contained within a donor organ enters your child's body during a transplant. Allergic reactions can be avoided 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 that may increase the likelihood of rejecting the donor organ, such as cytomegalovirus (CMV).

The diagnostic tests that are performed are extensive, but necessary to understand the complete medical status of your child. The following are some of the other tests that may be performed, although many of the tests are decided on an individual basis:

• Blood tests
• Urine tests
• Echocardiogram. A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
• Electrocardiogram (ECG or EKG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.
• Cardiac catheterization. A diagnostic procedure in which a tiny, hollow tube (catheter) is guided through a vein or artery into the heart in order to image the heart and blood vessels. A colorless, liquid dye is given through the catheter and moving X-ray pictures are made as the dye travels through the heart.
• Magnetic resonance imaging (MRI). A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
• Lung biopsy.  A procedure in which tissue samples are removed (with a needle or during surgery) from a lung for examination 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 performed on the material that is coughed up from the lungs and into the mouth. A sputum culture is often performed to determine if an infection is present.
• Pulmonary function tests. Diagnostic tests into which help to measure the lungs' ability to move air into and out of the lungs effectively. The tests are usually performed with special machines into which a child must breathe.
• Tuberculosis (TB) test
• Chest computed tomography scan of the lungs (also called a CT or CAT scan). A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays.

The transplant team will consider all information from interviews, your child's medical history, physical examination, and diagnostic tests in determining whether your child can be a candidate for lung transplantation. After your child has been accepted to have a lung transplant, your child will be placed on the Organ Procurement and Transplant Network (OPTN) list..

The lung transplant team

The group of specialists involved in the care of children who are undergoing a transplant procedure is often referred to as the transplant team. Each individual works together to provide the best chance for a successful transplant. The lung transplant team consists of:

• Transplant surgeons (thoracic).  Doctors who specialize in transplantation and who will be performing 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 the function and disease of the lungs. 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 and help your family deal with many issues that may arise including lodging and transportation, finances, and legal issues. They can also help coordinate alternative means for school, so that your child does not get behind.
• Nutritionists and dietitians. Professionals who will help your child meet his or her 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

How long will it take to get a new lung?

There is no definite answer to this question. It may take one to two years on the waiting list before a suitable donor lung is available. During this time, your child will have close follow-up with his or her doctors and the transplant team. Various support groups are also available to assist you during this waiting time.

How are we notified when a lung is available?

Each transplant team has their own specific guidelines regarding waiting on the transplant list and being notified when a donor organ is available. In most instances, you will be notified by phone or pager that an organ is available. You will be told to come to the hospital immediately so your child can be prepared for the transplant.

What is involved in lung transplant surgery?

Once an organ becomes available to your child, you and your child will be immediately called to the hospital. This call can occur at any time, so you should always be prepared to go to the hospital if needed. Once at the hospital, the 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 require from six to 12 hours, but can vary greatly depending on the type of surgery and on each individual case. Single lung transplantation is performed with the child under general anesthesia. It is not always necessary to have cardiopulmonary bypass (rerouting of the blood through a heart-lung bypass machine) with a single lung transplant.

However, if two separate lungs are transplanted, cardiopulmonary bypass is often necessary. Heart-lung transplants always require the use of cardiopulmonary bypass.

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

Postoperative care for lung transplant

After the surgery, your child will go to an intensive care unit (ICU) to be monitored 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, he or she will be sent to the special unit in the hospital that cares for lung transplant patients. Your child will continue to be monitored closely. You will be educated on all aspects of caring for your child during this time. This will include information about medications, activity, follow-up, diet, and any other specific instructions from your transplant team.

What is rejection?

Rejection is a normal reaction of the body to a foreign object. When a new lung is placed in a 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. To allow the organ to successfully live in a new body, medications must be given to trick the immune system into accepting the transplant and not thinking it is a foreign object.

What are the symptoms of rejection?

The following are the most common signs and symptoms of rejection. However, each child may experience symptoms differently. Symptoms may include:

• Fever
• Chills
• Flu-like aches
• Decreased urine output or fewer wet diapers than usual
• Shortness of breath
• Pain over the transplanted lung

Your child's transplant team will instruct you on who to call immediately if any of these symptoms occur.

What is done to prevent rejection?

Medications must be given for the rest of the child's life to fight rejection. Each child is unique, and each transplant team has preferences for different medications.

Because anti-rejection medications affect the immune system, children who receive a transplant will be at higher risk for infections. A balance must be maintained between preventing rejection and making your child very susceptible to infection. Blood tests are performed periodically to measure the amount of medication in the body to ensure your child does not get too much or too little of the medication. White blood cells are also an important indicator of how much medication your child needs.

What about infection?

The risk of infection is especially great in the first few months because higher doses of anti-rejection medicines are given during this time. Your child will most likely need to take medications to prevent other infections from occurring. Some of the infections your child will be especially susceptible to include oral yeast infection (thrush), herpes, and respiratory viruses.

Lung transplant follow-up

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

• Complete physical examination
• Chest X-ray
• Blood work
• Lung function tests
• Continued education for you and your child
• Medication changes

Children who have received a lung transplant will need lifelong follow-up with doctors who  specialize in transplant medicine. Keeping appointments with your child's transplant doctor, as well as maintaining contact with the transplant team when signs and symptoms of rejection occur is vital. Parents (and the recipient, when old enough) are the first line of defense; they must understand and recognize the signs and symptoms of rejection and report them as soon as possible to the transplant team.

Long-term outlook for a child after a lung transplant

Living with a transplant is a lifelong process. Medications must be given that trick the immune system so it will not attack the transplanted organ. Other medications must be given to prevent side effects of the anti-rejection medications, such as infection. Frequent visits to and contact with the transplant team are essential. Knowing the symptoms of organ rejection (and watching for them on a daily basis) is critical. When the child becomes old enough, he or she will need to learn about anti-rejection medications (what they do and the signs of rejection), so he or she can eventually care for himself or herself independently.

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

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