Heart Transplants in Children

A heart transplant is offered not only to help a child live longer, but also to improve their quality of life. Heart transplants are offered when no other medical or surgical options are available to fix a failing heart without serious risk. A transplant can work well in
these situations, but it is not a cure and comes with many new responsibilities and challenges.

A child may need a heart transplant for several reasons, including:

• cardiomyopathy (weak heart muscles)

• congenital heart disease (heart disease that a

• child is born with) that cannot be operated on without serious risk

• continued heart problems following surgery for congenital heart disease, such as the heart not working properly or the valves being too leaky

• life-threatening abnormal heart rhythms that cannot be controlled any other way.

Your child’s heart problems fall into one of these categories, and your child’s cardiologist has referred your child to our heart transplant team to be assessed for a heart transplant. Among the team members are pediatric cardiologists who specialize in the care of children with heart failure who may need transplantation, as well as children who have already received a transplant.

At this stage, your child’s cardiologist is only asking the transplant team for their opinion. They will consider if your child:

• needs, or qualifies for, a heart transplant

• needs a transplant right at that moment

• is able to have a heart transplant.

• Rejection may happen even though your child takes medicine to prevent or stop rejection.

• Increased risk of infection (special care must be taken to avoid contact with other people who are sick).

• Increased risk of cancer

• Joint and bone disease

• Weight gain

• Diabetes may be caused by immunosuppressants or steroids (medications taken to prevent rejection).

• Your child will need intense and ongoing monitoring and treatment for the rest of their life, inc

Each child is different. In general, Medicaid or most insurance plans cover the cost of transplant for the recipient. The coverage for the cost of a heart from a donor depends on the individual’s insurance policy. A financial coordinator or your social worker is available to help you through this involved process.

• You must notify your coordinator if you lose your insurance coverage immediately.Transplant is expensive and a big commitment for life.

• Transplant requires insurance coverage.

• Medications for the first month after transplant can cost up to $15,000.

Transplants are life changing. This is a serious lifetime commitment and it is expensive. The decision for transplant is a partnership between your family, your child’s doctors, and the transplant team.

It is important to keep insurance coverage to support the cost of the transplant and it is required for ongoing care and medications.

If your insurance changes or is cancelled, please call our transplant financial coordinator, immediately.

If you know it will be cancelled, call the financial coordinator before it is cancelled.

Your failure to do so can result in an insurance denial. You will be responsible to pay all charges for the transplant and test(s).

Not notifying your transplant team of insurance changes also can change listing status.

Your transplant financial coordinator can help you review all of your options.

Before we contact you, our financial coordinators contact the insurance company and make sure the visit will be covered at Children’s Mercy for a transplant evaluation. Often gathering the medical records and getting answers from the insurance company can take days to weeks. If your child is gravely ill, the process is expedited. If we are having difficulty getting information, we may ask for your help. Once dates are chosen for the evaluation, our social worker will contact you and help you arrange travel and lodging if needed.  

A heart transplant evaluation lets health care professionals decide if a transplant:

• is possible

• is the best treatment

• is the right option for your child at this time

It also helps the team to see if your child will have any special needs at the time of or after a transplant.

Where will my child have the evaluation?

Most patients have their evaluation as an outpatient of the hospital over one or two weeks. This means they have appointments in the hospital but do not stay overnight.

We may prefer to admit your child and evaluate him/her over two or three days. If your child already needs care as an inpatient, we may perform the evaluation while they are in the hospital. Sometimes children are so sick they are in the intensive care unit (ICU) when they need an evaluation. In these cases, evaluations are often shorter and may not include all of the testing that you will read about in this handbook.

Every child is different. Your transplant nurse or cardiologist will discuss your child’s evaluation with you in detail.

How is my child evaluated?
Your child will have a number of medical tests, and you will be asked questions about your child’s medical history by different members of our transplant team. You also will meet with several other health care professionals to ensure your child is physically ready and emotionally prepared for a heart transplant.

The results of the tests will give the transplant team an idea of your child’s overall health. The tests your child will have may depend on:

• age

• what is wrong with their heart

• how long they have been sick

• how sick they are

• whether they have been seen at the transplant center before

A heart transplant is offered not only to extend a child’s life, but also to offer a better quality of life.

Someone who is being considered for a heart transplant has no other medical options available. A child may need a heart transplant because he/she was born with a heart defect that cannot be fixed with surgery, or his/her heart muscle is not working. In rare cases, a heart transplant may be an option when a child has a serious, abnormal heart rhythm that cannot be controlled any other way.

What are the steps of a transplant evaluation?
Once financial coverage has been verified from your medical payment source (private insurance, Medicaid, etc.), the transplant evaluation can begin.

1. Medical Evaluation: Your child has a complete medical evaluation including medical history, physical exam and a series of tests to help decide if he/she is healthy enough to receive a heart transplant. These tests will be explained in more detail in the next section.

2. HLA antibody testing/ABO Blood Type

   • HLA antibody testing is a blood test, used to determine HLA antigens (your child’s genetic make-up.) Blood is taken from your child and any possible organ donors on life support. This how we find the best match between donors and recipients

   • ABO blood type controls blood group compatibility. The donor and recipient must have compatible blood groups.

3. Psychosocial Team Evaluation: Your child and family will meet with members of our psychosocial team. This part of the evaluation measures your understanding as parents of what’s involved with a transplant and the effects a transplant may have on your family. Other areas that may be assessed include: outside support systems, i.e., church, school, neighbors, friends, and other family, relationships with brothers and sisters, home setting, financial resources/work history, how you handle stress, medical and mental health history and your relationship with your child. The psychosocial team has members with different educational backgrounds and expertise. The team includes a pediatric transplant psychologist, clinical social worker, and a child life specialist. The discussion with your child’s psychosocial team is important, as everyone in the family will likely be affected during the transplant process. The transplant team requires that two adults be present during evaluation meetings with social work and psychology. At least one of the adults must be the primary caregiver.

4. Financial Team: It is important to know the benefits and limits of your insurance policy, along with the possible out-of-pocket costs for items not covered by insurance. The transplant financial coordinators are here to help you understand extra costs and help you make choices about financial coverage. During the transplant evaluation, you will meet with our financial coordinator who will help explain transplant-related insurance benefits and answer any questions you may have.

5. Presentation to the Transplant Selection Committee: After your child’s evaluation is done, the patient will be presented to the Transplant Selection Committee to be sure that everyone agrees transplantation is possible and likely to be successful based on all the information gathered during the evaluation. Once financial clearance has been received and approval has been given by the Transplant Selection Committee, your child will be registered with the United Network for Organ Sharing (UNOS) – the national waitlist for patients waiting for an organ transplant.

The two main goals of the medical evaluation before transplant are:

1. to be certain that a heart transplant is needed (i.e., that no other medicines or surgeries will stabilize or help the illness)

2. to be certain that your child’s other body systems are healthy enough to survive a transplant and the side effects of all the medicine after transplant.

Many of the standard, pre-transplant tests are listed below. A member of the transplant team can tell you which tests will be needed for your child.

Cardiac catheterization
This test checks the pressure inside the heart and lungs. Small tubes called catheters are put into blood vessels in the groin or neck. The small tubes are passed through the blood vessels into the chambers of the heart and out to the lungs to measure pressure and oxygen levels. If the blood pressure in the lungs is high (also known as pulmonary hypertension), one or more medicines may be given during the test to lower the pressure. If the blood pressure in the lungs cannot be lowered, then the heart transplant would not be successful. The donor heart would not work because it would not be able to pump against the high pressure in the lung vessels. This test takes about two to four hours. Your child will be sedated for this test.

Electrocardiogram (EKG)
This test measures the electrical system in the heart. Several “stickers” (also known as leads) will be placed on your child’s chest and connected to a machine that receives the electrical impulses from your child’s heart and records them on paper. When the test is over, the stickers will be removed. This test only takes a few minutes. It is not painful. No medicine is needed to help your child sleep.

Echocardiogram (Echo)
This is an ultrasound scan of the heart. This test uses sound waves to draw a picture of the heart that can be seen on a TV screen and recorded on videotape. The echocardiogram gives doctors another way to look at how well the heart muscle and valves are working, and measures the size of the heart. Your child will lie on a bed and lubricating jelly will be placed on his/her chest. The technician will slide a transducer (which is like a microphone) over the chest to obtain the pictures. This test uses no needles and does not hurt, but it does take about 20 to 40 minutes. During the test, your child will need to lie still. For this reason, babies and toddlers may be given medicine to help them sleep for this test. Older children will be able to watch a video during the test; we have several from which to choose.

Exercise stress test
This test will show how your child’s heart rate and blood pressure respond to exercise. It will take about an hour. Your child’s exercise capacity will be measured while he/she walks and/or runs on the treadmill. The test is supervised by a registered nurse and an exercise physiologist. If your doctor requests, a cardiologist also will be present.

Radiology studies
A chest X-ray (CXR) allows us to look at the size of the heart and the condition of the lungs. Spinal X-rays and hand X-rays will be done to measure and assess bone structure before transplant. A child’s “bone age” helps us to measure your child’s potential for future growth. Each X-ray takes only a few minutes and is not painful.

Pulmonary function tests (PFTs)
These tests show how well a child’s lungs can expand and how well oxygen is carried to the blood. If your child is tall enough (about 40 inches) and old enough to cooperate, he or she will be asked to breathe into a tube connected to a machine. The test takes about 20 minutes and it is not painful.

Head magnetic resonance imaging (MRI)
This test takes special pictures of the brain to see if any defects are present. Because some transplant medications can have side effects on the brain, these pictures can also be compared to those taken after transplant to help the team know if changes occurred. The MRI camera looks like a short tunnel. Your child will lie on an exam table that will move slowly through the “tunnel” while the pictures are taken. Your child should be aware that the camera is noisy, but it will not touch his/her body and it will not hurt. He/she will need to be still for this test to get the best picture, so babies and very young children are usually given medicine to help them sleep. This test can take 25 minutes to one hour. Children with pacemakers or other metal implants will not be able to have an MRI, but will have a head CT (computed tomography) scan instead. The CT scanner is like the MRI camera, except that it looks like a large donut rather than a tunnel. A head CT takes less time than an MRI, 10 to 30 minutes.

Kidney testing
The transplant team needs to assess your child’s kidney function. Kidney function may not be normal before transplant, but can become normal after transplant. However, if kidneys are permanently damaged, a heart transplant will not repair kidney function. Some of the medications after transplant can cause damage to the kidneys. While this damage is usually small, we must be certain that your child’s kidneys will be able to handle the medicines. The kidney function will be measured by testing the urine, blood, and collection of urine over a 24-hour period. Testing may also include X-rays or pictures of the kidneys.

Blood testing
Blood samples will be taken to measure kidney function, liver function, immune system response, blood type and prior exposure to certain viruses, including HIV, which is the virus that causes AIDS.

Consultations
Several specialists will examine and work with your child. These may include any or all of the following: a transplant surgeon, neurologist (brain doctor), pulmonologist (lung doctor), nephrologist (kidney doctor), psychologist, psychiatrist, dietitian, social worker, child life specialist and a financial counselor (insurance specialist). 

Heart transplantation is a procedure that requires the knowledge of many pediatric specialists trained in medicine, surgery and immunology (the body’s response to infection and foreign substances). For this reason, we use a team approach to coordinate your child’s care. In addition, all of the heart team members are pediatric experts.

The professional members of this team include: doctors, advanced practice nurses, transplant coordinators, nurses, social workers, psychologists, dietitians, pharmacists, child life specialists, chaplains and others who have been trained to meet the special needs of pediatric heart transplant patients and their families. The transplant coordinators and doctors are always available to answer your questions about transplant. A brief description of some of the transplant team members follows.

Transplant cardiologists (heart doctors)
The transplant cardiologists are pediatric heart specialists with expert knowledge in heart transplant. They will work with your child’s primary pediatrician(s). After heart transplant, the transplant cardiologists will become your child’s heart doctors. They will manage your child’s heart care, and will work closely with you and with your child’s pediatrician to be sure all your child’s medical needs are met. An APRN may see the patient in conjunction with the cardiologist or on their own.

Transplant surgeons
The transplant surgeons are the doctors who will perform the operation to place the new heart into your child’s body. The transplant surgeons are specialists in pediatric heart surgery. During the transplant evaluation, the surgeons will explain the operation, discuss the associated risks, and answer any questions you may have. The transplant surgeon also will care for your child on a daily basis during the first few days following transplant. The surgeon will make rounds to check on your child and review lab results and other procedures.

Transplant coordinators
The transplant coordinators are experienced registered nurses who are responsible for organizing all parts of the transplant process, from referral by the local doctors, to evaluation, and on through post-transplant, discharge and follow-up care. The transplant coordinator works with the cardiologist, your local doctor, transplant doctor and surgeons to coordinate or organize your child’s care. The coordinator always is available to answer any questions you may have about transplant, and the transplant process.

Financial coordinators
A Financial Coordinator is available to help families with financial concerns throughout the transplant process. The financial coordinator will call your insurance company to get a benefit summary and find out if approval is required for evaluation and transplant. At the time of evaluation, a financial coordinator will review the details of your benefits and identify other financial resources. The financial coordinator will help you when applying for state/federal programs (i.e. Medicaid, Supplemental Security Income). If you have any questions about your health insurance, we encourage you to call your financial coordinator.

Social workers
A social worker will help you deal with the many stresses that come with the transplant and hospitalization of your child so you won’t have to face the difficult times alone. A social worker can help you find the resources you need before transplant surgery like transportation, lodging and financial assistance. A social worker also can give counseling and emotional support to help lessen any emotional distress you feel. The social worker also will help arrange your child’s discharge from the hospital and make the right appointments to outside community resources if needed.

Psychologist
As part of the evaluation process, all children and families will see the transplant psychologist. The psychologist is an expert in child development and behavior and in helping children and their families deal with the stresses from heart transplant. Many children with long-lasting illnesses show small developmental (age-related) delays, and if these are found, more help will be needed. The psychologist will ask about any concerns you have about your child’s emotional status and behavior. Many parents find it helpful to talk about their concerns with an expert who may be able to make some suggestions that can make a difference to them and their child’s adjustment to the transplant.

The team psychologist also is there to help other members of the family. A transplant is not just surgery; it is a special experience that affects every family member. The psychologist has worked with many transplant patients and is qualified to help you and your loved ones get the most from the new opportunities that transplant offers. 

Dietitians
A registered clinical dietitian will help evaluate your child’s nutritional status before the transplant and follow him or her throughout the transplant period and after surgery. The dietitian will keep detailed records of your child’s height, weight and muscle growth. The dietitian also will work closely with you to develop the best diet for your child. For younger children, the dietitian will tell you how to mix formulas, which may need to be changed from time to time as your child’s nutritional status changes. Like the other members of the transplant team, the dietitian is experienced in developing special diets for children with heart disease. The dietitian provides expert nutritional advice for your child’s needs.

Transplant pharmacist
The transplant pharmacist is responsible for identifying and preventing possible and actual medication problems. He or she is a resource to the medical team while your child is both in the hospital and after discharge. The transplant pharmacist’s role also is to teach your child and family about the medications and help you create a medication regimen that is both safe and manageable.

Child life
Your child will need help to emotionally prepare for the transplant surgery and the hospital stay. A child life specialist is someone that will explain all the pieces of medical equipment and procedures to your child in words he or she can understand. This process includes “medical play,” a time when the child can see and touch different pieces of medical equipment, under the child life specialist’s supervision, and pretend to be a doctor or nurse for a stuffed animal. This allows the child to express his or her fears and worries about the hospital and the child life specialist can address those worries with the child.

Other activities with the child life specialist may include a tour of the surgery area and the intensive care unit (ICU) and viewing children’s videos about what to expect during a hospital stay. During your child’s hospital stay, a child life specialist will be available to direct supervised play time in the hospital’s playroom and organize special events for the children at the hospital.

Chaplains
Hospital chaplains are available 24/7 to help patients and families with the faith and spiritual questions that may come up during the course of the transplant experience. At Children’s Mercy, pastoral care provides daily chapel services, regular pastoral visits, supportive counseling and parent support groups.

The pastoral care department helps persons from all faith traditions. Their goal is to help you find the spiritual support you need during a hospital stay. The chaplains also can help you by contacting your minister or other clergy from home or a clergy person from your faith tradition.

Other staff members
Many other specialists trained in pediatrics will help care for your child. These specialists include intensivists (ICU doctors), pathologists (microscope doctors), radiologists (X-ray doctors), infectious disease doctors (germ doctors), renal doctors (kidney doctors), respiratory therapists (breathing specialists), and physical and occupational therapists (body movement specialists).

Other staff members, such as interns and residents (doctors in school), often are with your doctors on rounds. These are graduate, licensed doctors who are getting more training in specialized medical or surgical skills under the supervision and direction of the transplant team. 

Your transplant team expects you to be involved in the care of your child. It is important for you to ask the team questions you may have and tell them about any changes in how you feel. The best way to remember your questions or concerns is to write them down in a journal or on a notepad when you think of them. When the doctors make rounds, you can discuss each item on your list with the transplant team.

Patient and family
The most important member of the transplant team is YOU!

By the time a child has completed the transplant assessment and has been placed on the heart transplant wait list, they will already have undergone a lot of medical testing.

During this time, however, you will be asked to bring your child to the Transplant Clinic for follow-up visits. These visits are intended to check that your child’s condition remains the same and that they still need and are ready for a new heart.

You will need to call the transplant coordinator anytime your child has a fever or any other signs of illness such as a runny nose, cough, vomiting or diarrhea. If your child develops an infection while waiting for a donor heart, he/she will not be able to have the transplant until the infection is cured. This is due to the high doses of immunosuppressant medications that are given at the time of transplant which can allow infection to overwhelm your child’s body.

Waiting in the hospital
A child waiting in the hospital for a heart transplant will have the same tests as a child waiting at home, but may also have additional blood work, X-rays or other testing. This depends on the health problems they have while they are waiting.

Sometimes a child may need to have repeat heart catheterization to measure the pressure in their heart and lungs. This usually happens if the child has been waiting for a long time or if there has been a major change in their health.

How long does it take to get a heart?

There is no way to know how long your child needs to wait for a donor heart; it could be a few days or many months to years. Your child’s wait time can depend on their age, weight, blood group and status on the waiting list.

The wait for transplant can be an anxious and emotional one. It is important to continue to find balance and a sense of normalcy for yourself, your child and the rest of your family.

Waiting at home
Transplants can happen at any time, day or night, depending on when a suitable donor organ is found. It is extremely important for the transplant team to have all your contact phone numbers (home, cell, work and school) so they can reach you. If you have a cell phone, keep it charged and with you at all times. If a family does not have a cell phone, the team will help you make other arrangements.

The family must be within a two-hour drive of the Children’s Mercy Adele Hall Campus at 2401 Gillham, Kansas City, Mo. at all times while waiting for a donor heart. This will allow enough time to get to the hospital when a heart becomes available. If your home is outside this two-hour limit, your child and one parent may be required to live in the Kansas City area while waiting. Our social worker can help with housing during this time.

You will need to be ready to leave your home as soon as possible after the transplant coordinator tells you an organ is available. Plan well in advance for this by:

• arranging reliable babysitting or child care for

• any other children

• lining up other transportation if the person driving you is unavailable

• organizing how to tell family members – we suggest you call one member who can then contact others

• packing a bag for the hospital stay ahead of time. The bag may include toiletries, pajamas and some of your child’s personal items (such as pictures, a favorite blanket, and a stuffed animal)

• have cash available for gas money, cab fare, etc., so you will be able to leave at a moment’s notice.

Waiting in the hospital
If you are preparing to wait for transplant in the hospital, talk to the transplant team about bringing in personal items (such as a computer, gaming system, movies and personal photos) to make the hospital room feel more like home. We also recommend you bring enough clothing and toiletries for at least two to three weeks at a time. 

What happens while my child waits for a new heart?

Emotions and Feelings
Many families say the waiting period is the hardest part of the transplant journey. It is important to recognize that the serious illness of one family member affects the whole family in different ways. To prevent burnout, it is essential to care for yourself and your other family members as well as you can.

While you wait for a new heart for your child, it is natural to experience a range of feelings, including anxiety, hope, anger, sadness and powerlessness. When so much is out of your control, use the supports available to you, whether family and friends, your faith community, professional supports (such as a counselor or therapist) and the transplant team. Also try to exercise, do activities you enjoy and take time for yourself.

Illness and hospital stays are both stressful, and a stay in the hospital can be difficult for a child at any age. Hospital stays disrupt a child’s life and can interfere with their normal development. While they are in the hospital, children may miss their friends and be bored or afraid. They might also not understand why they are in the hospital or have false beliefs about what is happening to them.

Talk to the transplant team about meeting another transplant family with a child of similar age. This might help an older child to find out how they will look and feel after a transplant and give you the chance to ask questions about their past experiences on the transplant journey.

Activities

It is important that your child and family do as many normal activities as possible during the waiting period. All activities will naturally depend on your child’s health. Your cardiologist will help you decide what your child can or cannot do.

If your child is waiting at home, it is important for them to go to school, even for only half days. The goal is to keep as normal a schedule as possible so your child can maintain their physical and emotional well being. If your child’s physical therapist has provided any exercise routine, follow it to keep your child as strong as possible before the heart transplant.

Waiting for a transplant in the hospital can be particularly hard, especially if you are from out of town. It often feels like your whole life has been put on hold. During this time, your child will follow a set schedule that often involves physical therapy, occupational therapy, speech therapy, therapeutic recreation (games or drawing) and school tutoring if applicable. Staff members at the hospital will work with you and your child to deal with the difficulties of a long hospital stay.

Vacations
The question of going away for a vacation may arise while your child is listed for a heart transplant. This is often possible, but you will need to discuss it with the transplant team. Sometimes going on vacation means your child will be put “on hold” on the transplant waiting list while you are away. This could delay the matching of a donor to your child, but you and your family may decide you can manage this risk if a vacation is needed to maintain a certain quality of life for the whole family. Your transplant team will help you to make this decision.

Nutrition
Patients waiting for a heart transplant often find it hard to take in enough energy (calories) to grow. For instance, infants and young children may breathe very quickly. This both burns more calories and makes it hard to drink. Children may also be limited in the amount they are allowed to drink. In addition, poor heart function can cause gut problems such as vomiting, gagging and retching in some children. Patients with cardiomyopathy (weak heart muscles) often develop heart failure quickly. Usually these patients need more calories as their heart is working harder, but they may be unable to take them if their appetite is small and they are having medical therapies. The dietitian will use various methods to help your child stay nourished before and after transplant.

Boosting nutrition for children of different ages
For infants and young children with heart disease, breast milk or formula may be “concentrated” to provide more calories and nutrients in less volume. This is usually done by adding some infant formula to your breast milk and/or following a recipe developed by the dietitian. Many types and flavors of supplements are available to improve the weight of an older child on the waiting list. To help your child take supplements, try offering them in small quantities throughout the day rather than in a large portion. Also try offering them cold rather than at room temperature and pour them into a glass or cup instead of leaving them in the can. Your dietitian can advise you about different supplements.

Feeding tubes
Sometimes your child might need to be fed through a feeding tube. This tube can be placed in their nose or directly into their stomach. Feeding tubes are helpful if your child gets tired before they drink enough fluid or if there are strict limits on the fluids they can drink.

The dietitian will work with you and your child to develop a feeding schedule allowing your child to eat and drink if they wish and still get enough energy and nutrients to grow. For example, it may be possible to allow your child to eat and drink during the day and then get the rest of the nutrition they need through the feeding tube overnight.

What happens if my child gets sicker while waiting for a heart?

Once your child is placed on the transplant waiting list, they will be reviewed regularly by the transplant team. If your child’s heart becomes sicker and your child needs more medical care, they may be moved to a more urgent listing status. This can mean:

• being admitted to the hospital to wait

• needing continuous intravenous (IV) medications

• getting help with breathing from oxygen or a ventilator machine

• getting support from machines called ventricular assist devices, which take over the work of the heart

If a change in your child’s condition makes a successful heart transplant less likely, your child may be removed from the list either for a short time (for example, while they receive treatment for an infection) or permanently (for example, if there is major organ failure). If this happens, the transplant team will explain this to you and your child and give you a plan.

How will a transplant change my child’s life?

A lot depends on what your child’s life was like before transplant. If they are a “normal” kid and have never taken medicine a day in their life, a transplant will make a big difference. On the other hand, if they have struggled with heart disease in the past, they may be familiar with medications, blood tests and frequent visits to the doctor.

The biggest change in your child’s life is they now have a new heart and a chance for a full life. This gift of life is not without cost, however.

• Your child will have to take medicines every day for the rest of their life to make sure their body does not reject the new heart.

• They will need to have blood and other medical tests for the rest of their life to make sure their medicine is working well, to look for any side effects and to look for any signs of rejection and infection.

• They will need to develop relationships with different health care professionals and learn to be responsible for their own heart health as they get older and move from pediatric to adult care. Good communication with the transplant team is essential to their success.

When a donor organ becomes available for your child, it will be a day filled with overwhelming emotions. You and your child may feel nervous, anxious, excited or scared. At this time it is very important for you and your child to ask any questions you feel have not yet been answered.

Finding out about a donor heart
The coordinator will call you when a donor heart is available for your child. The coordinator will give you specific instructions about when to come to the hospital and where to go when you arrive. The transplant team member also will ask you some questions about any recent symptoms that would cause concern for infection. 

Note: It is very important your child does not eat or drink anything once you have been called. Please follow any instructions carefully. Not following instructions could result in delayed or cancelled surgery.

Arriving at the hospital
Once your child arrives at the hospital, they will be admitted to the Pediatric Intensive Care Unit (PICU). A nurse will examine them and take vital signs (such as their heart rate, blood pressure and temperature). They will review the information they have on file about your child’s allergies or medications and update your child’s medical history with you.

To prepare for transplant, your child will need to have blood tests, and have an IV inserted into their arm or back of their hand. Your child will also have an X-ray of their chest. All these tests help us make sure your child is ready and safe for transplant.

Many people will see you and your child before the surgery and can answer questions. Our transplant surgeons will have you sign a consent form for the surgery and answer any other remaining questions. An anesthesiologist (the doctor who gives your child the anesthesia, or sleep medicine for surgery) will also meet you, examine your child and have you sign a consent form.

During this time, we will communicate to you through your child’s nurse, the actual operating room time (OR time). Generally speaking, your child is in surgery AT LEAST two hours before the “new” organ arrives at the hospital. The coordinators are constantly updated on any changes that may affect the quality of the organ and communicate this with the transplant surgeon(s) and team members. The “timing” for the transplant may also change and as a result we may change the time your child goes to the operating room or the time the organ will arrive at Children’s Mercy.

Your child is now ready to go to the OR. A member of the OR team will tell your child’s nurse they are ready and then the OR team will transport your child to the OR. The team will also escort your family to the OR waiting area. The nurse or a member of the surgical team will discuss how and when they will give you medical updates during your child’s surgery. You will be told where to wait to receive these updates. 

If my child is called for transplant, is the donor heart healthy and ready to be used?
When a suitable heart is offered for your child, the doctors will accept it, let you know about the quality of the heart and prepare your child for the transplant. 

Preparations for the transplant take place even before the doctors have inspected the donor heart. When the doctors actually see the donor heart, sometimes it is too damaged to be used. In these situations, the transplant must be called off. Usually this happens when you and your child are already at the hospital, but before the operation has started. Unfortunately, it is not uncommon for this to happen after your child is already asleep in the operating room. We call this a “dry run.” If this happens, the team will tell you to wait for another organ to become available.

This can be very disappointing, but it is more important to make sure your child receives a healthy donor heart than to take risks because a heart is available.

What happens in the operating room?

Assuming the donor heart is healthy, the transplant operation involves removing the failing heart from your child’s body and attaching the new heart. This takes about four to eight hours. For children with complex congenital heart disease, the operation is more complicated. 

Several members from the surgical team (nurse, surgeon and other doctors and staff) will be with your child during surgery. The surgical team will describe your child’s operation to you in detail.

Preparing for surgery
When your child enters the operating room, they lie on the special operating room bed while the nurse attaches ECG stickers to their chest, an oxygen saturation probe to their finger and forehead and a blood pressure cuff to one of their arms.

These devices are all designed to help the team monitor your child’s condition during the surgery. 

Your child may be given medication to keep them calm. Then, within a few minutes of entering the OR, they will have anesthesia to put them completely to sleep. A breathing tube, special IV and urine catheters will then be inserted to support your child’s bodily functions during and after surgery.

Removing your child’s heart
The surgical team will open up your child’s chest and start a heart and lung bypass machine, which does the work of the heart and lungs during the operation. The surgical, nursing, anesthesia and perfusion teams monitor your child carefully during the surgery.

Transplanting the new heart
When the new heart arrives, the surgical team works to safely implant it in your child. When this is done, the heart and lung bypass machine is stopped, and the new heart begins to take over the work.

After the bypass machine is stopped, the teams in the operating room watch the new heart to make sure it is working properly. Often an echocardiogram is done to see how the new heart is working and to make sure there is no narrowing where the new heart is attached to your child’s blood vessels.

When the surgical team is sure everything is functioning as it should, your child will be prepared to be transferred to the Pediatric Intensive Care Unit (PICU) to recover.

When your child first arrives in the PICU, they will require a period of “settling in.” This usually takes at least one to two hours. Your child’s nurse will tell you when you can visit.

What to expect when you first see your child

Your child will be attached to many tubes and monitors, which can be an overwhelming sight at first. Your child’s team will explain all the monitors, tubes and IVs to you in detail.

Monitors
Your child will be on a ventilator (breathing machine) until they are ready to breathe on their own. Your child will remain sedated while they are on the breathing machine so they are relaxed and comfortable. This usually takes a day to two for older children and teenagers, but it can sometimes take a bit longer for babies. In addition to the ventilator, several monitors in the room will be used to check your child’s heart rhythm, blood pressures, blood oxygen level and possibly other signs, depending on your child’s specific situation.

Devices and tubes 
Most children return from the operating room with a temporary pacemaker in place. This is an electrical device to control the rate of the heart beat. The pacemaker wires will be attached to your child’s heart and come out through their skin. 

You child will have a bladder catheter, a soft plastic tube, which will continuously drain urine. This tube is inserted during surgery.

A nasogastric tube will also be in place. This tube is inserted through a nostril and then passed down into the stomach.

Your child will also have chest tubes to drain fluids that collect during and after surgery. These tubes will come out through the skin just under your child’s ribs.

Because your child will still be asleep or very drowsy at this time, they will get fluid, medications and nutrition through intravenous (IV) drips. These will normally go into your child’s arm or the back of their hand.

Your child’s nurse will be able to explain all monitors and tubes to you. Feel free to ask any questions.

Your child’s incision
Your child will have an incision (cut) along the length of their sternum (breastbone). This is called a “sternotomy” and will be covered with a dressing. After two or three days, the dressing will be removed.

Depending on their surgeon’s preference, your child may have staples (wires) or a suture (thread stitches) on the incision. Sometimes, a child returns from the OR with the “chest open” because they are more stable that way. In this case, the incision is covered with a dressing and then closed in the PICU or the OR after a few days, once your child is more stable.

As your child recovers from surgery, the medical team will reduce their medications and begin to remove the invasive lines and monitors. The breathing machine will do less work as your child wakes up from sedation. When your child is finally disconnected from the breathing machine, they will be able to speak, and, in time, be able to drink.

While your child is being closely monitored by the nurses and doctors around the clock, the PICU care team typically has more structured “rounds” between 8 a.m. and noon. During this time, your child’s doctors, nurses, respiratory therapist, pharmacist, dietitian, and any additional team members will review all of the clinical data at the bedside and form a plan for the day and create goals for your child. As parents or primary caregivers, you are considered an important part of the care team and are encouraged to be present during rounds. This is a great opportunity to understand the plan and ask questions.

Visitation in the PICU
Parents/primary caregivers are welcome at your child’s bedside 24/7. On rare occasions, you may be asked to step out for procedures, especially those that require sterile conditions. Your nurse can explain all of the options for sleeping arrangements. For infection control reasons, parents and visitors are not allowed to eat in the room but drinks are permitted. Keep in mind that your child may not be able to eat or drink at this time. Therefore, it is best not to drink in front of them.

To help prevent infection in your child and also to encourage rest and recovery, it is recommended that you limit visitors to immediate family only. Also, there are restrictions on children visiting. Your nurse can explain this in more detail. 

When does my child leave the PICU?
Depending on their condition, a child can stay in the PICU for a few days or even a few weeks. When your child no longer needs intensive care, they will be moved to the cardiology floor (4 Sutherland Tower.) 

Although most of the monitoring equipment will have been removed, your child may still have chest drains or intravenous infusions. Over the following days, these will be gradually removed as your child’s condition improves.

Working with your child’s health care team
Once your child is on the floor, the nurse who looks after them regularly will also have other patients to care for, unlike in the PICU. Because of this, you will be encouraged to play a greater role in your child’s care.

At this point, too, other transplant team members, such as the physical therapist, occupational therapist, and dietitian, become involved again in your child’s care. It is important you and your child follow their recommendations and instructions so your child can begin to experience life with their new heart.

This is also a time when the transplant coordinators and pharmacists work closely with you to make sure you are prepared to care for your child at home. Prior to going home, you will be able to practice taking your child’s vital signs, giving medications, and performing any other required care with the help of a nurse.

Your child’s emotional and psychological recovery

The physical recovery from a heart transplant operation is only one part of your child’s transplant journey. A heart transplant can be an emotionally challenging experience for you, your child and the rest of your family.

It is not unusual for children who have received a transplant to experience depression, self-esteem and self-image issues, and/or attention disorders after their surgery. Being aware of the warning signs and providing support and counseling for new issues will help to reduce their impact on your child’s life.

The transplant team is prepared to support your child’s emotional concerns and needs at this time. You can get support from the transplant psychologists, psychiatrists, social workers and child life specialists.

A heart transplant generally improves the overall quality of life for a child and their family. It allows seriously ill children to feel well and take part in age-appropriate activities. It has helped many children get back to the normal routines and tasks involved in growing up. However, a heart transplant is not a cure. Children and their families are trading a life-limiting heart disease for lifelong medications and their side effects, close medical follow-up and invasive procedures.

Going home after the transplant

Before you and your child are discharged from the hospital, you will start to learn about life after transplant. The transplant nurse coordinator, dietitian, pharmacist and other team members will teach you how to keep your child healthy at home.

The next section of this handbook has information about things that are important after a heart transplant.

It is important to remember each child’s situation is different, and your child may not experience everything mentioned.

Medicines are important to the success of your child’s heart transplant. Without these medicines, the body will reject the new heart. The body’s reaction to the new heart is to fight it off as it would a cold or virus. Medicines are given for your child’s specific needs to stop the body from fighting or rejecting the new heart. The medicines must be taken as directed; do not change or stop giving your child’s medicines unless instructed by a member of the transplant team. Over time, we expect you and your child to understand the actions of each medication. Understanding the medicines helps you to recognize the side effects when they happen.

Prior to going home, you will begin to give medicines to your child, with the help of your nurse. With help from the transplant team, you will develop a medication schedule. This schedule may change with adjustments in the medications.

Before going home from the hospital, we will teach you:

• The generic and brand names of each medicine.

• What each medicine looks like.

• The purpose and action of each medicine.

• How to determine the correct dose of each medicine.

• Safety measures required for each medicine.

• How and when to take the medicine.

• The common and uncommon side effects of each medicine.

The medication schedule should be updated with each change to dose or administration time. Any changes that are made to the schedule should be done using a pencil. If a change is made during a hospital stay or clinic appointment, the nurse will inform you and you can mark the change on the schedule. Should you have any questions, please verify the change with your nurse. This process will help you feel comfortable making changes at home.

Once you are at home, medications should be given by your schedule, not according to the instructions written on the medication bottle.

Be sure to keep all the medicines in their original containers. This will be helpful in keeping up with the expiration dates, who prescribed each drug, etc. Keep all medicines away from heat, direct light and moisture, each of which can cause the medicine to go bad. Always give the medicine at the same time every day. Each of the regular medicines needs to reach a stable level in the blood. Watching these blood levels at certain times and the test results will help make changes in medication dosages. Therefore, it is important that medicines are given at the same time every day.

Your child must take all their medicines as they are ordered. If your child has nausea, vomiting or diarrhea and cannot take the medicines, please call the transplant coordinator immediately for instructions. If a dose is not given at the ordered time, please give the dose as soon as possible. Do not give the missed dose close to the next dose; instead call the transplant coordinator for instructions on how to get back on a safe schedule.

Many medicines will affect the level of the immunosuppressive medicines or may react with them in a bad way. Therefore, do not give your child any over-the-counter medicines or medications ordered by a doctor who does not know your child’s medical history without first talking to the transplant team.

Always bring your child’s medicine schedule to the clinic so that changes can be made if needed. You may also want to give your child a copy of medicines to keep with them. Should your child need hospital admission, please bring all your medicines with you along with your medication schedule to make the admission process easier.

If you have any questions or problems, contact the transplant office immediately. There is a transplant coordinator available 24 hours a day to answer questions.

General medication information
Prescription refills are done during normal business hours, Monday through Friday, 8 a.m. to 4 p.m. Please request refills early before you run out. Please notify the pharmacy seven days before a refill is due to make sure you receive it on time.

• Generic formulations of medications are available. Your doctor may prescribe the generic forms when and if it is appropriate.

• Always take medicines as prescribed.

• Follow the dosage instructions given by your transplant doctor, not what is printed on the label of the medicine, since the dose may be changed before your next refill from the pharmacy.

• Do not stop taking any medications without being instructed by a member of the transplant team.

• Do not add any over-the-counter (store-bought) medicines or supplements, including herbal remedies, unless your transplant doctor is aware of it.

• Contact your transplant doctor if you notice any physical or mental changes in your child.

• Take medicines at the same time every day to avoid missing doses.

• If your child vomits within 30 minutes of taking their immunosuppressive medicine, call the transplant coordinator. If your child vomits after 30 minutes of taking their medicine, do not give a second dose, and call the transplant coordinator for more instructions. Please contact the transplant coordinator if vomiting continues.

• Store medicines away from heat, light and moisture. Avoid storing medications in the bathroom or kitchen.

• Contact your pharmacy if any medicine doesn’t look “right” for any reason.

• Do not try to make up for missed doses unless told to do so by your transplant team.

• 1 cc is the same as 1 mL.

• Check expiration dates on all medicines.

• Throw away and replace medicines that have expired.

• Avoid NSAIDs (non-steroidal anti-inflammatory drugs) such as Ibuprofen, Motrin, Advil, Naproxen and Aleve. These can cause problems with your child’s kidneys after transplant. These will be listed as medication allergies on your child’s medical record.

Immunosuppressive medications
Some commonly used medicines in transplantation are Prograf (Tacrolimus), CellCept (Mycophenolate Mofetil), Neoral (Cyclosporine), Prednisone and Imuran (Azathioprine). They all work to suppress (lower) the immune system and prevent rejection of the new heart. Your child will need to take immunosuppressant medications after transplant for the rest of his/her life. These are powerful medications with many side effects, and they must be taken exactly as prescribed.

If your child’s immune system is not suppressed enough by the medicines, it will be able to destroy (reject) the transplanted organ. If your child’s immune system is suppressed too much by the medicines, it will not be able to protect him or her from infection. These medicines also can cause unwanted and dangerous side effects. Therefore, you must follow the medication schedule exactly as directed. Under no circumstances should you change the dosage of these medications without instructions from your child’s doctor or the transplant office.

The following is a list of medicines which are commonly given to patients after transplant. The medicines prescribed for your child will be personalized to meet his or her needs. This list is for informational purposes only. Your child’s transplant doctors and transplant coordinators will help educate you and ensure that you understand the medicines ordered.

How long will the new heart last?
It is not clear how long a new heart will function well in a child. Survival has improved greatly over the last 20 years, with most children having a good chance of surviving into adulthood. This depends on the age of your child when they have a transplant. In 2013:

• more than 50 percent of infants survive with a transplanted heart until 20 years after their transplant

• more than 50 percent of children survive until 15 years after their transplant

• more than 50 percent of teenagers survive until 12 years after their transplant.

However, it is difficult to know exactly how long a transplanted heart will last. This uncertainty can be a source of stress for parents and older children.

There are many reasons a child can develop heart failure after a heart transplant. The most common ones include rejection and transplant coronary artery vasculopathy (CAV). Other reasons include nonspecific allograft failure, which means the new heart function decreases without any clear reason why. This is a type of rejection that still needs to be better understood.

To give your child the best chance of success with a new heart, make sure:

• They take their medications as prescribed.

• They follow a heart-healthy lifestyle (by eating the right foods and getting enough exercise that is suitable for them).

• You keep all of your follow-up appointments at the Transplant Center.

Despite all of this, it is still possible a new heart will unexpectedly fail. If the reason for this is known, the heart transplant team will explain it to you.

In cases of heart failure, your child may need to be treated for rejection or put on heart failure or heart rhythm medications. They may even need to be assessed or re-listed for another heart transplant.

These things will be explained to you by your heart transplant team if they happen to your child.

When can my child return to school or other daily activities?
As your child recovers from the transplant and has more energy, encourage them to take part in all normal family activities. There is no specific time children are expected to stay home; each child’s situation is unique. Please talk to your transplant team about what is best for your child.

Before your child returns to school, talk to the school about the best way to continue class work. Your child’s teacher may provide some work or, in some cases, tutors can be arranged.

Children can play outside, go for walks and see friends. If relatives or friends are sick, it is best to ask them to visit once they are well. Other ways to keep your child healthy include regular hand-washing, which will help to prevent spread of infections. Children returning to school (or work if they are in their teens) might find it useful to carry hand sanitizer and anti-bacterial wipes with them to clean any work surfaces.

It is very important your child’s teachers (or coworkers) realize a transplant patient is healthy and no longer ill when they receive a new heart. As such, they should treat the patient as normally as possible.

The transplant team will help with your child’s return to school or work once your child is ready.

Does my child need a special skin care routine?
Transplant patients generally don’t need a special skin care routine, unless they develop an unusual skin condition or rash. Showering and bathing with regular soap is generally okay, but mild soap and lotion after bathing are recommended if the skin is dry.

During the first year following transplant, the sternotomy scar should be protected from the sun since the skin is more delicate.

A number of children will have dry skin or eczema after their transplant. This can be cared for by having regular baths with hydrating oil, using Vaseline® on damp skin and, if necessary, using cortisone cream from your family doctor or pediatrician. The eczema can sometimes be so severe that your child may need to see a dermatologist or take medications.

Immunosuppressants prescribed after transplant can increase the risk of certain viral skin conditions, such as warts or herpes. Teens that have had a transplant also may be at increased risk of acne, which is already common in teenagers, due to steroids.

Will medications affect my child’s hair?
If your child is on prednisone, the texture of their hair likely will change, possibly becoming dryer or coarser. Your child’s hairdresser will probably suggest a good conditioner to help with this.

Chemical treatments like highlights, hair dye, permanents and straightening can increase hair breakage. Your child should avoid these until their prednisone dosage is lowered. Medications also may cause more facial hair. A hair removal cream can be used, but the instructions should be followed carefully to avoid skin irritation.

Teens may also try bleaching, waxing or electrolysis.

What type of dental care should my child have after transplant?
Regular dental care and check-ups are important for transplant patients.

Dental problems such as abscesses or mouth pain can lead to or be signs of serious infection because your child’s immune system is weaker.

• Some transplant medications can cause gum problems.

• Tell your child’s dentist that your child has had a transplant.

Right after transplant, your child’s teeth and gums should continue to be cleaned every day. Preventive care is more important than ever.

The American Heart Association currently recommends heart transplant patients do not need antibiotics before dental procedures. There are exceptions to this, so check with your child’s transplant team to be certain. Your child will usually only need antibiotics if dental work is needed within the first six months after transplant.

Can my child go out in the sun as usual?
Transplant patients are more likely to develop skin cancers, so it is extremely important that you protect your child’s skin.

• Protect your child’s sternotomy scar from sun during the first year after transplant.

• Avoid the sun between 10 a.m. and 2 p.m.; this is when ultraviolet rays are the strongest.

• Have your child wear protective clothing outdoors, including a hat.

• Encourage your child to sit or play in shaded areas.

• Apply sunscreen and lip balm daily to uncovered areas of your child’s body.

• Remember to re-apply sunscreen every few hours – more often if the child is swimming or sweating – because it wears off.

• Use skin products with at least SPF 30.

• Check your child’s skin for abnormal spots (irregular moles or growths) and report them to your doctor.

What if my child wants a tattoo or piercing?

• Piercings are only allowed in certain parts of the body due to the high risk for infection. Please speak to the transplant team if you are considering any piercings for your child.

• Tattoos are absolutely not allowed due to the high risk for infection and potential transmission of contagious diseases.

Can my child have a pet?
You can safely have a pet in your home if your family follows a few simple guidelines.

• Have your child wash their hands well after handling pets. Avoid cleaning cages or litter boxes and handling feces (also known as stool or “poo”).

• Make sure your pet is regularly seen by a vet and is up-to-date with all vaccinations.

• Birds, reptiles and rodents are not safe pets for your child because they can carry diseases.

If you have any questions about a specific species, please talk with the transplant team.

Can my child travel?
Your child can travel after transplant if they have been well, without medical complications. Please talk with your transplant team before you make any travel plans, especially if traveling abroad.

Tips for safe travel

• Always carry your child’s medications with you – never pack them in your checked-in or stowed luggage in case it gets lost.

• Keep medications in their original containers (bottles or pill packages) with the pharmacy labels on them.

• Consider buying travel health insurance if you are planning international travel. Check if it covers pre-existing conditions.

• Well before your trip, talk with your transplant team and a travel clinic about any vaccines your child needs for traveling abroad.

• Ask your transplant center for a letter about your child’s condition and how to contact the transplant team in case your child needs medical attention while away.

• Know the location of the nearest hospital at your destination and ask your transplant team if they can recommend doctors in the area.

My child still has trouble sleeping. Is this normal?
Many children who have a heart operation have difficulty sleeping and concentrating afterwards and may show signs of hyperactivity. This also is true for children who have had a heart transplant.

They have been found to have more schooling and behavior issues compared to healthy children. Help is available through psychological counseling and/or medication.