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Lung Transplantation

  • "Transplant tolerance means that your body accepts the lung with minimal to no immunosuppression. That's the holy grail of transplant research."

    --Gary Visner, MD, medical director, Pediatric Lung Transplant Program

    If your child has severe end-stage lung disease that no longer responds to treatment, a lung transplant might be an option to give her a longer and healthier life.

    A lung transplant is an operation in which diseased lungs are replaced with a healthy replacement pair from another person. Sometimes, only one lung will be transplanted, but in most cases, the transplant surgeon will replace both.

    • According to 2010 statistics from United Network for Organ Sharing (UNOS), about 55 children receive lung transplants each year.
       
    • According to the National Heart, Lung and Blood Institute (NHLBI), part of the National Institutes of Health (NIH), short-term survival from lung transplant has improved.
       
    • If your child has both heart disease and pulmonary disease, she could be a candidate for a heart-lung transplantation.

    How Boston Children's Hospital approaches a lung transplant

    At Boston Children's, your child’s team understands that a lung transplant is a time of hope but also concern for both your child and your family. Our program has been developed to address these concerns through:

    • strong integration with a number of highly-specialized initiatives at Children's, such as the Cystic Fibrosis Center, Pulmonary HypertensionPulmonary Vein Stenosis, Infant Lung Disease and End-Stage Lung Disease programs
       
    • experts from additional sub-specialties throughout Children's, who will join your child’s care team as needed
       
    • Child Life specialists, psychologists, social workers and resource specialists who will be there to support your child and your family before, during and after transplant

    We are committed to working with you every step of the way.

    Lung transplant: Reviewed by Gary Visner, DO, 2010
    © Boston Children's Boston, 2010


  • We understand that you may have a lot of questions when your child may need a lung transplant.

    • How does it work?
    • How long will my child need to be hospitalized?
    • How will it affect my child long term?
    • What do we do next?

    We’ve tried to provide some answers to those questions in the following pages, and when you meet with our experts, we can explain your child’s condition fully.

    Who gets lung transplants?

    Diseases and conditions that end in a lung transplant often differ between children and adults. Among adults, many conditions that lead to lung transplants include chronic obstructive pulmonary diseases, such as emphysema. For children, teens and young adults, the most common condition is cystic fibrosis. Other conditions include:

    • pulmonic vascular congenital heart diseases
    • chronic lung disease of infancy (CLD)
    • pulmonary hypertension
    • heart disease or heart defects affecting the lungs
    • pulmonary fibrosis

    Potential complications

    A transplant can offer your child a longer and healthier life, but the procedure, like any surgery, does carry its own risks. Understanding these risks will help you to recognize signs early, which can lead to more effective treatment for your child.

    What is rejection?

    Rejection is a normal - and, under most circumstances, healthy - reaction of the body’s immune system to a foreign object. When new lungs are placed in your child's body, his immune system attacks it, not recognizing that it is useful. To prevent this, your child will have to take life-long medications to weaken his immune system.

    How can I tell if my child is having rejection?

    It’s not always easy – many children have rejection without any symptoms at all. Your child’s transplant team will watch for these signs and show you what to look for. Some of the most common signs include:

    • decrease in lung function
    • shortness of breath
    • fever
    • chills
    • flu-like aches

    If your child has any of these symptoms, you should call his doctor immediately.

    What medications are used to prevent rejection?

    Your child’s transplant pharmacologist will work closely with the rest of the transplant team to determine the best medications for your child. Some of the most common ones are:

    • cyclosporine
    • tacrolimus
    • mycophenolate mofetil
    • prednisone
    • azathioprine

    Anti-rejection medications work by suppressing the immune system, but that means that your child will be at higher risk for infections. Our team will continually monitor his health through blood tests and other exams.

    What infections will my child be susceptible to?

    Some of the infections your child may be especially susceptible to include:

    • oral yeast infections (thrush)
    • herpes
    • respiratory viruses

    This risk of infections is especially high during the first few months after the transplant. This is because higher doses of anti-rejection medicines are given, so the immune system is especially suppressed.

    Teens and transplants

    Being a teenager can be tough enough without having to cope with everything that comes with being a transplant patient. Along with the physical changes, teens also experience cognitive and emotional changes that influence their thoughts and behaviors, sometimes in unpredictable ways. Learn about the special challenges your teen may face as a transplant patient – and discover some ways you can help.

    Long-term outlook

    What’s the long-term outlook for my child?

    We cannot say for sure, because each child is different. According to the National Heart, Lung and Blood Institute (NHLBI), part of the National Institutes of Health (NIH), short-term survival from lung transplant has improved. Figures from 2005 on single-lung transplant show that more than 82% of patients survive the first year, nearly 60% survive 3 years, and more than 43% survive 5 years. Survival rates for double-lung transplants are similar.

    Finding a donor organ for your child

    Where do transplanted organs come from?

    Most transplanted lungs come from deceased organ donors. These are adults or children who have become critically ill (often due to accidental injury). A donor may come from any part of the United States.

    Who distributes the donor organs?

    The United Network for Organ Sharing (UNOS) is responsible for transplant organ distribution in the United States. They oversee the distribution of most types of solid organ transplants – in addition to lungs, they also allocate livers, kidneys, pancreas, hearts and corneas.

    How are donor organs distributed?

    UNOS keeps a list of all the people in the United States who need a solid organ transplant. In the case of lung transplants, people over age 12 are given an allocation score based on how urgently they need a transplant and the severity of their condition.

    Those who would benefit most are placed highest on the list, and given first priority when a donor organ becomes available. For children under age 12, lungs are allocated based only on the time they’ve spent on the waiting list.

    When donor lungs becomes available:

    1. A computer searches all the people on the waiting list and creates a smaller list of people for whom the lungs would be the appropriate size and blood type.
    2. The computer ranks the people on that smaller list according to their allocation scores.
    3. The person at the top is considered first for the transplant. If he is not a good candidate, the next person on the list is considered, and so forth.

    When the decision is made to put your child on the transplant list, his transplant team will send his medical profile to UNOS, and keep them updated them as his condition changes.

    How long is the wait?

    We know it’s agonizing to wait for donor lungs, but unfortunately, there’s no definite answer to this question. It can be hard to find the right-sized lungs for smaller children. Depending on your child’s age, lung allocation score and how many lungs are available, it may take between a month to more than two years. You will be given a beeper for us to be able to reach you at all times.

    What happens while we wait?

    We recommend that you use your waiting time to prepare for your child's surgery. A donor lung can become available at any time, day or night, and it is crucial to plan ahead for when this occurs. Here are some things to keep in mind:

    1. It’s important that you always leave a number where you can be reached if you’re away from home. Make sure the transplant team knows how to reach you at all times.
    2. When a lung becomes available, you will be expected to arrive at the hospital within two to three hours. Keep your packed hospital bag handy — including an extra 24-hour supply of your medications — and arrange transportation to the transplant center in advance.
    3. If you’re organizing your own transportation on the day of surgery, consider the possibility of inclement weather, distance and rush-hour traffic.
    4. Depending on how far you live from Children’s, you may need to relocate to be closer to the hospital if you cannot get to the hospital within two to three hours of our call.
    5. Plan well in advance for other factors including:
      • baby-sitting/child care for your other children
      • an alternative transportation plan in case the person driving you is unavailable
      • phone calls to family members - we suggest you call one family member so they in turn can contact other family members.
      • packing a bag for the hospital stay. This may include: toiletries, pajamas, personal items (pictures, favorite blanket, stuffed animal).

    Waiting for an available donor lung is often the most difficult and stressful time for our patients and their families. Please remember that we are always here to offer you support and resources should you need it.

    How will I be notified when a donor organ is available?

    When a donor organ that seems to be a good match for your child becomes available, we will notify you by phone or pager. You’ll be asked to come to the hospital immediately.

    When you receive the call to come to the hospital, be prepared to:

    • ask about any medications your child should take before coming to the hospital
    • let us know if your child has a temperature or is currently sick
    • not allow your child to eat or drink anything after receiving the call
    • leave your house as soon as possible

    Keep in mind that it is possible that you may be sent home again if one or both of the following things occur:

    • The team discovers a problem with the new lungs.
    • The team finds that your child has a condition that could jeopardize his health or the transplant’s chance of success.

    FAQ

    Q: Will my child be able to return to school after the transplant?

    A: Many of our children do quite well and are able to return to school after transplant. While precautions need to be taken, we encourage them to lead full and happy lives – to go to school, play sports, spend time with friends and enjoy hobbies.

    Q: How long will it take to get a new lung?

    A: Unfortunately, there’s no way to know. This may depend on the severity of your child’s condition, how long he has been on the waiting list, and the availability of organs. We know it’s hard to wait, and can direct you to resources and community support groups.

    Q: What will happen after the transplant?

    A: Your child will take medication to prevent the new lungs from being rejected for the rest of his life.  We’ll also have follow-up appointments here at Children’s, so we can monitor his health, change medications if needed and stay in touch with your family. Since your child’s immune system will be compromised, your family may need to make some adjustments to protect his health. Read more about how to help keep your child safe

    Questions to ask your doctor

    You and your family are key players in your child’s medical care. It’s important that you share your observations and ideas with your child’s transplant team and that you understand their recommendations.

    You probably already have some ideas and questions on your mind, but it can be easy to forget the questions you wanted to ask when you’re talking to your child’s doctor. It’s often helpful to jot them down ahead of time so to make sure that all of your concerns have been addressed.

    If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.

    Here are some questions to get you started:

    • Is a transplant the best option for my child? What other treatments might be available?
       
    • How many of this kind of transplants have been done at this institution and by my child’s surgeon?
       
    • Are there factors other than my child’s health that go into the decision of whether to move forward with the transplant?
       
    • Will our health insurance cover a transplant? What questions about finance will I need to have answered?
  • If it seems like a lung transplant might be an option for your child, we’ll ask you to come to the hospital for an informational visit. Your family will meet our transplant team, and you’ll be invited to ask questions and share any concerns you may have. We encourage you to bring family and close friends to this initial meeting.

    If you and the transplant team agree that a lung transplant is a good idea, your child will be scheduled for an evaluation. This is an extensive array of tests that are usually conducted over the course of four to five days. While this may seem like a lot, these tests are necessary in order for us to:

    • confirm that a transplant is the appropriate treatment
    • determine how urgent the need for a transplant is
    • make sure that the donor organ your child receives will be a good match

    The evaluation is usually done as an outpatient, but it can be completed with your child staying in the hospital if your insurance approves.

    Who will we meet with during the transplant evaluation?

    A transplant is a complex procedure that touches different medical specialties – that’s why you and your child will meet Children’s Hospital Boston experts from a number of different areas, including:

    • Cardiology - The cardiologist will monitor your child’s heart function and assess how well it is likely to stand up to the transplant.
       
    • Anesthesia - The anesthesiologist will review your child's medical and/or surgical histories and identify any risk factors associated with anesthesia.
       
    • Infectious disease - Our infectious disease specialist will perform a physical exam and review your child's vaccination records. We will want to know if your child has any particular environmental exposures or has recently traveled to a foreign country.
       
    • Otolaryngology - This consultation is required for all patients with cystic fibrosis in order to have their sinuses evaluated. The specialist may recommend sinus surgery, which requires an overnight stay in the hospital.
       
    • Psychiatry - A psychiatrist or psychologist will meet with you and your child to discuss coping strategies, stress management and family life.
       
    • Social service - The transplant social worker will meet with you and your family to identify support systems and discuss your feelings about transplant. He or she can provide you with information about resources related to finances, relocation expenses and support groups.
       
    • Nutrition - Our nutritionist will evaluate your child's diet and nutritional requirements. Maintaining a healthy lifestyle and good nutrition before the transplant is very important.
       
    • Physical therapy - The physical therapist will discuss the importance of exercising before transplant in a way that is appropriate for your child. The goal of developing an exercise plan is to help increase endurance and strength. The physical therapist will ask your child to take part in a six-minute walk test.
       
    • Dentist - Before a transplant can be considered, your child will meet with her own dentist or one here at the hospital. The goal is to treat any cavities, infections or tooth abscesses before transplant, since these conditions can be problematic once your child begins to take medicine to suppress his immune system.

    What tests are used during the evaluation?

    Your child’s specialists will order different tests to rule out infections, determine functionality of organs and make sure a donor match is compatible. These types of tests may include:

    1.Blood

    • blood tests to determine blood type (a donor and recipient must have compatible blood)

    2. Heart

    • electrocardiogram (ECG or EKG) to examine the electrical activity of your child's heart
    • echocardiogram (ECHO) to evaluate the function of your child's heart
    • cardiac catheterization to view your child’s heart and blood vessels
    • MUGA heart imaging to see how the heart walls move and how much blood is expelled with each heartbeat

    3. Lungs

    • pulmonary function tests (PFT) to measure lung volume and the rate of airflow in the lungs
    • chest x-rays to evaluate your child’s lungs
    • CT scan to examine the chest and sinuses
    • ventilation and perfusion scan (V/Q Scan) to compare the function of the left and right lungs
    • magnetic resonance imaging (MRI) to produce detailed images of your child’s lungs
    • lung biopsy to learn more about the condition of your child’s lungs

    4. Infections

    Some tests will check for exposure to viruses, bacteria and infections including:

    5. Other kinds of tests

    • liver and kidney function tests
    • abdominal ultrasound to look for masses, obstructions and structural abnormalities in your child’s abdomen
    • bone densitometry to determine the density of your child's bones
    • nocturnal oximetry to monitor oxygenation during sleep

    After these consultations and tests, the transplant team will meet as a group to determine whether your child is a good candidate for a lung transplant. Our multidisciplinary approach to care ensures that your child’s case will be given thoughtful discussion of every treatment possibility.

    If your child is a transplant candidate, we’ll make sure your family is fully educated about the risks and benefits, and your child will be placed on the United Network for Organ Sharing (UNOS) list.

  • Once you’ve been notified that a new pair of lungs has been found, things move very quickly. Our team will make sure that you and your family are comfortable and informed. It’s normal to be nervous, but you can rest assured that your child is in good hands with us.

    What happens when we arrive at the hospital?

    When you arrive at the hospital, you’ll go directly to the Transplant Unit, an inpatient unit designed for solid organ transplant patients. There, members of your the transplant team will perform a physical exam and run some tests to make sure your child is ready to go to the operating room.

    While this preparation is going on, a surgical team will be transporting the donor lungs to Children’s. The time of your child’s operation is planned to match the team's arrival back to the hospital. Ideally, lungs will be transplanted within four to six hours of being outside the donor’s body.

    What happens in the operating room?

    Often, the anesthesiologist will allow you to take your child into the operating room and stay with him until he’s given anesthesia and falls asleep. His chest will be thoroughly washed with a special cleansing solution to minimize the possibility of infection.

    Lung transplant

    The surgeon will make an incision across the middle of the chest, remove the diseased lungs, and place the new organ in its place. Then the surgeon will connect the main airway of the new lungs to your child’s main airway, and the main blood vessels of the new lungs to your child’s main blood vessels.

    Sometimes a heart-lung machine is used to regulate blood flow and oxygen supply during the procedure. This is a machine that takes over for the heart, and pumps oxygen-rich blood to the body.

    Heart-lung transplant

    If your child is receiving a heart-lung transplant, the diseased heart and lung are removed, except for a small portion of the heart to which the new heart will be sewn. The new organs are transplanted as a whole system — the donor heart and lungs aren't separated from each other. The airways of the new lungs are attached to your child’s main airway, and the main blood vessel of the new heart is attached to your child’s main blood vessel.

    How long will the transplant operation take?

    The transplant operation may take anywhere from six to 12 hours, depending on your child’s situation.

    What happens after surgery?

    After the surgery, your child will go to the cardiac intensive care unit (CICU) to be monitored closely. After his condition is stable, your family is welcomed to visit. Read more about the CICU.

    While your child is in the CICU, members of the transplant team will educate you and your family on all aspects of caring for your child after his operation. This will include information about medications, activity, follow-up, diet and any other specific instructions.

    Follow up

    After the transplant, your child’s team will want to keep a close eye on him through follow-up visits to make sure that everything is going well. These visits might include:

    • complete physical examination
    • lung function tests
    • chest x-ray
    • blood work
    • continued education for you and your child
    • medication changes

    These visits are essential to make sure your child’s medicine is at the right dosage, catch and treat any complications that might arise and make sure that your family and your child’s transplant team remain in touch. The frequency of visits will drop off over time, but your child will require life-long medication and contact with his transplant team.

    How can I help keep my child safe after the transplant?

    We understand that you will want to do everything possible to protect your child and his new lungs, and there are some steps you can take to minimize the risk of infection.  

    What’s the long-term outlook for my child?

    Each child is different, so there's no way to know for sure. Lung transplant are becoming increasingly successful as researchers learn more about how the body deals with transplanted organs and search for ways to improve children’s quality of life both before and after a transplant.

    Coping and support

    We understand how disruptive and frightening a lung transplant can be - not only for your child, but for your whole family. From your first visit, you’ll work with a team of professionals who are committed to supporting you.

    Patient education: Our nurses are on hand to walk you through the transplant process and help answer any questions you may have. They will also reach out to you by phone, continuing the care and support you received while at Children’s.

    Parent to parent: Want to talk with someone whose child has had a lung transplant? We can often put you in touch with other families who can share their experience.

    Faith-based support: If you and your family find yourself in need of spiritual support, we can connect you with the Children’s chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your treatment experience.

    Social work and mental health professionals: Our social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with issues such as coping with your child’s diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

    On our For Patients and Families site, you can read all you need to know about:

    • getting to Boston Children’s
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family
  • “I feel like I’ve been given a second chance at life.” Eleven simple words, but hearing them is a large part of why your child’s transplant team gets up in the morning. After her transplant, we want to see your child go to school, ride bikes, do chores — to have a full and happy life.

    But while a transplant can offer new hope and new opportunities, it also brings new obligations. To prevent rejection, your child will need to avoid certain situations, comply with restrictions and take medications for the rest of her life. That’s why our doctors sometimes describe getting a transplant as exchanging one disease for another.

    It’s also what our dedicated teams of transplant researchers are working to change.

    Research within the Lung Transplant Program at Boston Children's Hospital is aimed at improving the outcomes of lung transplant surgery. We focus primarily on three things:

    • preventing both short- and long-term lung transplant injury
    • developing transplant tolerance
    • creating therapies for long-term acceptance of transplanted lungs

    Here at Boston Children’s, we have the resources to conduct research in ways that other institutions can’t. For example, we are one of a handful of labs in the world capable of working with mice as models for lung transplant research, and we’re using mice to evaluate protocols with the goal of developing treatments that we can one day bring to our patients.

    Globally, lung transplant research has been slow because there are comparatively few of them performed. Boston Children’s is hoping to help solve that problem by conducting research as a member of the International Pediatric Lung Transplantation Collaboration, an organization founded to give lung transplant researchers as much data to work with as possible.

  • Zach's Story

    Zachary Nickerson was born with severe combined immunodeficiency disease (SCID), a life threatening disease, often referred to as "boy in the bubble syndrome," that causes you to have little or no immune system. There aren't many effective treatments for it, but the best one is a bone marrow transplant, and Zach had two of them by age 5.

    But the chemotherapy that was part of the treatment Zach needed after the bone marrow transplants caused him to develop pulmonary fibrosis, a progressive lung disease that could only be cured with a double lung transplant.

    Zack Nickerson

    Zach got his strength up and after two weeks on the transplant list, he received a life-saving double lung transplant at Children's on March 23, 2008, but his road to recovery has been long. He developed pneumonia after the transplant and was sedated for three weeks for pain management. But Zach fought back and did it with a smile on his face. "He has had health issues his whole life but he has the cutest smirk and still enjoys life," says his nurse, Cindy Campbell, RN.

    Both of his parents admire Zach's strength. "Never once has he asked me why he has to go through all of this." says Lloyd. "He's entitled to his crumby days, but it doesn't take much to distract him and he stays positive."

    Read more.

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