What to Expect

Waiting
Once your child has been officially listed with UNOS as a candidate for a lung transplant, a waiting period follows until a suitable donor becomes available. The waiting time varies for each child based on organ availability. Unfortunately, no one can predict how long you will wait. The average waiting time can vary from 18 to 24 months. Waiting for an available organ is often the most difficult and stressful time of the transplant process. As a parent, you may feel a sense of helplessness and loss of control. The pediatric transplant team will do whatever they can to decrease your anxiety and to allow you to continue your normal activities.

Waiting for a transplant can also be a stressful time for extended family members as well. There is a great deal of uncertainty that occurs, but the donor organ offers hope and a new beginning. A child with lung disease requires many special therapies (medications and nutrition) during the waiting period, which may add a financial burden to the family. Because everyone in the family is affected by the long wait, whenever possible take time for other children in the family, your mate and yourself.

Although the transplant will change the course of your child’s lung disease, it is not a “cure.” One family expressed it simply by saying that you need to respect the new organ and take care of it for the remainder of your child’s life. This places a great deal of responsibility on the family to recognize when their child is ill, to obtain medical care for him and to make sure he continues to take the medications on a regular basis.

In order for lung transplantation to occur, it requires the death of one person for the preservation of another. Guilty feelings regarding the death of the donor are naturally associated with transplantation. Talk with members of the transplant team about how you are feeling, and remember, organ donors are giving us one of the greatest gifts — the gift of life.

Preparing for the Transplant
When an organ becomes available, the transplant coordinator will first call your home or any other number you have provided (i.e., your work number). You should never leave the area with your child without first informing the transplant coordinators or the doctors. Organs are hard enough to procure, and it would be a tragedy if a lung became available for your child and you could not be found. Always keep the transplant coordinator informed of your whereabouts.

There are things you can do to prepare yourself and your family for transplant while you wait. Medical technology has improved dramatically in the last few years. A lung can be stored for up to 4-6 hours out of the body. Therefore, you do not always have to leave at a moment’s notice. However, it would be helpful if you do the following:

•  Make a list of things you may want to bring with you: phone numbers of family and friends you may wish to contact, a book to read (surgery can take anywhere from 8 to 14 hours), and change for the vending machine and phone calls. It may help to develop a telephone tree if you have a large family.
• Once you are “on the list,” keep your bags packed. You will not have time to think about what you should take with you.
• Make suitable arrangements for other siblings or family members.
• Inform your employer of your child’s situation. Most employers will be understanding if you are up-front with them from the beginning. You may be out for a couple of days or a couple of weeks, depending on how your child is doing.
• Bring a toy, special photograph, special blanket or anything that has meaning to your child to put in your child’s room after the operation. Sometimes these special things are more for us than for the children.

Alternative living arrangements can be coordinated with the transplant social worker. As long as the child is not in the Pediatric Intensive Care Unit, a parent may sleep by the bedside. Housing outside the hospital, such as a local Ronald McDonald House, 410-528-1010, or the Children’s House at Johns Hopkins, 410-614-2560, is often available and coordinated through the social worker. Also hotels in the vicinity often offer a discounted rate.

The coordinator will ask you several questions regarding your child’s current health: does he have a cold, fever or rash or has he recently been exposed to a communicable disease (i.e. chicken pox)? Children who currently have a virus or the flu may not be good candidates for transplantation because the immunosuppressant medications used in the post-transplantation period to prevent rejection may prolong recovery from these ordinary illnesses.

Once you are told to come to the hospital do not give your child anything to eat or drink. When you arrive at the hospital, go directly to the pediatric emergency room. Blood work will be obtained and an IV will be started if your child does not have a central line.

Although it is rare, it is possible you could arrive at the hospital and find out the surgery has been canceled. Although many tests are done on the donor, it is only after the lungs have been examined that the transplant surgeon can determine whether the donor organ is acceptable.

Visitors to the PICU are limited to two people at a time. Parents can visit any time during the day or night except when the nurses are changing shifts (7:00 a.m. to 8:00 a.m. and 7:00 p.m. to 8:00 p.m.). You may call the unit at any time to ask about your child’s condition, 410-955-5260 or 800-999-PICU (7428). To maintain privacy and confidentiality, the PICU staff will not release information about your child to anyone but the parents. Because of the extensive equipment and the one-on-one nursing care, parents are not allowed to sleep in the child’s room. However, arrangements can be made to sleep on another floor of the Children’s Center as space permits. Please leave a phone number with the nursing staff so they can contact you if there is a change in your child’s condition overnight. Sometimes a visit may not be possible because a doctor is performing a sterile procedure, or the staff is making rounds. Always check at the front desk before entering the unit.

Over the first 24 hours your child will be in protective isolation. Everyone entering the room must wear a gown, mask and gloves. On the second post-operative day, and throughout the hospital stay, good hand washing should be adequate enough to avoid an infection.

Your child will return from the operating room with a breathing (endotracheal) tube in his mouth. The breathing tube is connected to a ventilator to assist your child’s breathing. While the breathing tube is in place, your child will not be able to speak or cry. If he is awake, he can be given a communication board or paper and pencil to communicate his needs. The tube is usually removed within a few days after surgery. However, children who have a problem with the functioning of the transplanted lungs require the breathing tube for a longer period of time. After the breathing tube is removed, most children need extra oxygen, which is provided using a face mask or nasal cannula.

Your child will have a large dressing covering his or her chest. In addition, four chest tubes are inserted to drain fluid from the chest and keep the lungs expanded. The nurse carefully monitors how much fluid comes out each chest tube. The chest tubes are removed one by one during the week following surgery. Your child will also have multiple intravenous lines (IVs) to provide medications, fluids and blood products as well as to monitor laboratory values.  If your child becomes very active when he awakens from the surgery, he may need to be restrained to protect the IVs and chest tubes.

A nasogastric tube (NG), which passes from the mouth or nose into the stomach, is inserted while your child is asleep in the operating room. It will be left in place several days to drain secretions from the stomach and to prevent vomiting. It may also be used to give medications. Once your child has bowel sounds or passes stool, the NG tube is removed.

A catheter, or a tube to drain urine, is inserted into your child’s bladder while he or she is asleep in the operating room. The nurse measures your child’s urine output to monitor his kidney function.

Initially your child will not receive anything to eat or drink (NPO). All of his fluid and nutritional needs will be met through intravenous fluids. Once your child is able to eat again, he will be offered clear liquids and then an age-appropriate diet. Some children require supplemental feedings, which can be provided via a nasogastric (NG) tube as formula or through the IV line as TPN (total parenteral nutrition). Children with a gastrostomy tube can resume their pre-operative feeding regime.

Many parents ask about pain medication. Pain medication is provided to the children to keep them as comfortable as possible. Since it is very important to assess neurologic function children are not given medication to make them sleep all of the time. The Pediatric Pain Service which is staffed by specially trained physicians and nurses will oversee the administration of your child’s pain medications.

Complications
Many parents describe the first few days of the post-transplant period as a roller coaster ride. Initially the new lungs may not work well; this is related to the long surgery and to the lungs recovering after transfer from one person to another. In rare cases, the lungs may not work at all. This condition, known as primary graft failure, is often fatal. Other complications which can occur over the first few days and weeks include bleeding, problems with the airway or blood vessel connections to the transplanted lungs, infections and rejection. If excessive bleeding or an airway complication does occur, your child may have to return to the operating room so the transplant surgeon can identify and correct the problem.

Rejection
Rejection is the most common post-transplant complication. More than 90 percent of the children experience have at least one episode of rejection. Don’t panic if this happens to your child! The first episode of rejection usually occurs about seven to ten days after transplant. However, even after months at home, your child may experience rejection. The typical signs of rejection include a fever (temperature greater than 38.5^oC or 101^oF), shortness of breath, cough, increased oxygen requirement and decreased pulmonary function tests. Usually a transbronchial biopsy is performed during a bronchoscopy by the pediatric pulmonologist to confirm the diagnosis of rejection. If rejection is confirmed then more immunosuppressive therapy is administered. The usual treatment for rejection is large doses of steroids. Occasionally children need a stronger medicine, called OKT3, to help fight the rejection episode. Children are usually hospitalized during an episode of acute rejection.

Infection
The same medications that prevent rejection also decrease the body’s ability to fight infection. As a result, care must be taken to limit your child’s exposure to common infections such as colds or the flu. You must be alert to signs of infection in your child, including fever (temperature greater than 101^oF), sore throat, cough that lasts longer than 24 hours, rashes and/or diarrhea.

Cytomegalovirus (CMV) is a common viral infection that can present serious problems for a child following lung transplantation. The lungs, liver and gastrointestinal tract are frequent sites of CMV infection. Typical symptoms include shortness of breath, night sweats, low-grade fever and diarrhea.

A first time exposure occurs in many children who are CMV negative at the time of transplantation because they may receive a CMV positive donor organ. Should this occur with your child, they will receive intravenous immunoglobulin (IVIG) or Cytogam on an eight-week schedule to prevent CMV infection. In addition they will receive a 12-week course of intravenous (IV) ganciclovir.

Even with this therapy many children will still develop a CMV infection. Treatment involves administration of IV ganciclovir for 4-12 weeks and adjustment of immunosuppressant medications. Viral shedding may persist for several weeks to months after the initial infection occurs. Good hand washing helps prevent further spread of this virus.

After transplantation your child is at risk for a number of fungal and bacterial infections. Initially, aerosolized amphotericin is administered to prevent fungal infections. In addition an antibiotic such as Bactrim^Ò is prescribed to prevent specific pulmonary infections. Most children take Bactrim^Ò three days a week. Your child will routinely undergo bronchoscopy to monitor for lung infections. If an infection is identified an appropriate antibiotic is prescribed to fight the infection.

The most common fungal infection that occurs in an immunosuppressed child is candida or thrush. This fungus often grows in the mouths of children on antibiotics, such as Bactrim^Ò, because the antibiotics eliminate bacteria that are normally present in the body. The primary symptom of thrush is the appearance of white spots on the tongue, gums and inside the lips and cheeks. Your child may also develop a fever, cough or digestive problems. Oral nystatin is prescribed to prevent or treat thrush.

Transfer to the Intermediate Care Unit and the Children's Ward
Children are transferred from the PICU once they are stable, usually after a week, but possibly after just a few days. It is important to remember that every child has a slightly different course in and out of the intensive care unit so predicting length of stay is difficult.

Some things that you can do to help your child during the recovery period:

• Encourage your child to walk around the room; this will help him build strength.
• Participate in coughing and deep breathing exercises; use the incentive spirometer or blow up a glove placed on a large syringe.
• Encourage your child to eat. The body requires many calories to heal after surgery. A nutritionist will meet with you to help plan the appropriate diet.
• Play is an important part of your child’s recovery. A child life therapist will help you find appropriate toys to assist your child in adjusting to the hospitalization.

Medications
In preparing for discharge, it is important for you and your child to understand why and how each medicine is given. Discharge will not occur until all of the medication levels are stable, and parents understand how to take care of their child at home. The transplant coordinator will help obtain all necessary medications and medical equipment for home.

Spirometry
Your child will receive a portable spirometer to be used at home. This device measures basic pulmonary function values (FVC and FEV1). Your child will be instructed in the use of the spirometer prior to discharge and he or she will have to use it several times a day after discharge. This testing is very important since decreased pulmonary function values can detect mild rejection allowing it to be treated in a timely manner.

Some Important Points to Remember

• Do not increase or decrease your child’s medication without discussing it with the physicians first.
• Get more medication from the drug store before the supply runs out. Plan ahead for weekends and vacations.
• When your child no longer needs a specific medicine, the transplant physician will discontinue it.
• Do not give over-the-counter medications for colds or flu without consulting your physician.

The staff will teach you how to recognize and report signs of potential complications. Initially clinic visits will be scheduled twice a week. If your child is stable after a month, visits can be scheduled once a week. Eventually this will decrease to twice a month and then finally monthly.  On the day you come to clinic, do not give your child a dose of Cyclosporine (Neoral^(R)) or Prograf⁽. Bring the dose to the hospital and give it to your child after blood work is drawn.

When to Call the Doctor/Clinical Nurse Specialist

• If your child repeatedly vomits medicine after it is given
• If several medication doses have been missed
• If too much medicine is accidentally given
• If your child has any of the side effects mentioned
• If your child is vomiting or has diarrhea (more than five runny stools)
• If your child has any symptoms of rejection
• If your child's FEV1 falls by more than 10 percent
• If your child develops a cough or fever

Outpatient Follow-up
After being discharged from the hospital your child will need to return to transplant clinic two to three times per week for the first month and then weekly for several months. At each clinic visit your child will undergo a complete physical examination. In addition, your child will have a number of tests performed to monitor the function of his or her new lungs. A typical schedule of testing is listed below. Chest x-rays and PFTs will be performed during clinic visits, other tests will be scheduled to minimize your visits to the hospital. Generally, all testing can be performed on an outpatient basis.

Bronchoscopy and transbronchial biopsies are an integral part of your child's post-operative care. The biopsies allow the transplant physicians to look for subtle signs of rejection. A bronchoscopy will also be performed if your child has any clinical deterioration. Bronchoscopy is performed in the operating room. Sedation for the procedure will be administered by a pediatric anesthesiologist. The procedure takes about 60 minutes. After the bronchoscopy is completed your child will be taken to the pediatric recovery room until he or she is fully awake.

Post-Operative Testing