Pediatric Lung Transplantation | For Parents

What happens after the initial evaluation?

When the pretransplant evaluation has been completed, the SLCH Lung Transplant Team will review the results of the studies and decide whether or not your child is a candidate for lung transplantation. Your child will then be listed with the United Network for Organ Sharing (UNOS) which maintains a national computerized list of organ transplant candidates, listed by blood type, size and date of listing. When your child moves towards the top of the list, we ask that you relocate to St. Louis (some younger or sicker patients may relocate sooner). After relocation, you will come to transplant clinic appointments weekly and enter a pulmonary rehab program. Prior to relocating to St. Louis, we usually recommend return visits every 4-6 months.

 When should I call the transplant team?

• Whenever there is any significant change in your child’s health status.
• Whenever your child develops an infection, is started on antibiotics, or there is a major change in medication, which includes being started on prednisone.
• Whenever your child is admitted to the hospital for any reason.

 Who do I call?

Your primary contact will be one of our three lung transplant nurse coordinators:

• Pegi Shaner
• Debbie Springhart
• Donna Watkins

 What can I do to help my child prepare for surgery?

Patients who are on the waiting list for lung transplantation should participate in the pulmonary rehabilitation program. (If you do not live in the immediate St.Louis area, our physical therapist will instruct you on an exercise program or make arrangements at a pulmonary rehabilitation program near your home.) This regular exercise program is a great way to get in the best possible shape before having transplant surgery. The more fit a patient is going into surgery, the easier the recovery is likely to be. For patients with pulmonary hypertension, the exercise program will be tailored according to condition in conjunction with the recommendations of our pediatric cardiologists and pulmonologists.

 What about support group meetings?

The support group meets virtually every month on a weekday evening. Any patient being evaluated for transplantation, those patients on the transplant list, and patients who have received their lung transplant, plus family members are welcome to attend. This is an informal meeting to exchange information about transplantation. The format for the meeting may include speakers, but open discussion is always encouraged. You are not required to talk. You may come and just listen if you prefer. There is also a simultaneous support group specifically for the patients and siblings led by Child Life Services.

 What about counseling?

Individual or family counseling is often recommended both before and after transplantation. Because there are so many stresses and adjustments with transplantation, it is helpful to discuss these with a therapist or counselor. Counseling may help individuals and families to communicate and best prepare for or cope with the many changes and uncertainties that go with transplantation.

 Where do the donor lungs come from?

Organ donors are individuals in whom all brain function has ceased due to severe brain injury, (also known as "brain death") and consent has been obtained for organ donation from family members. The evaluation of potential organ donors includes their medical history, blood tests, and studies looking at function of the specific organs (e.g. heart, lungs, kidneys, liver, pancreas). These tests are done by protocol at the site of the donor.

The lungs in brain-dead donors are particularly vulnerable to disease, and function may rapidly deteriorate due to fluid buildup in the lung tissue, infection, or trauma. In only 20-25% of organ donors are the lungs healthy enough to be recovered for transplantation. Therefore, the number of donor lungs available is lower than the number of donor kidneys, livers, or hearts.

Uited Network for Organ Sharing (UNOS) operates the Transplantation Network and keeps a registry of all patients waiting for any type of organ transplant in the United States. UNOS is a government-authorized organization which was established to provide fair distribution of organs to programs throughout the U.S.

Mid-america Transplant Services (MTS) coordinates all donor calls and possible transplant matches in St. Louis and the surrounding counties. All areas of the country have an organ procurement organization like MTS to evaluate and coordinate organ donation.

When a lung or heart-lung becomes available, the UNOS waiting list is checked (by blood type, size and date of listing) to see which patient is at the top of the list. The donor coordinator then contacts the transplant program to see if they would accept the organ(s) for that patient. If not, the next patient’s center is contacted. This process continues until the organ(s) are accepted by one or more transplant centers. The three thoracic organs (the heart and two lungs) may be used in three different patients from three different transplant centers. This complex process requires utmost efficiency since the lives of several potential organ recipients are at stake.

How long is the waiting period for a lung transplant?

Waiting times vary by age, blood type and height. Due to minimal competition, the waiting time for infants and toddlers ranges from days to a few months. Small children generally wait approximately six months. Waiting times are generally shorter for patients waiting for single lung transplantation compared to those waiting for double lung or heart-lung transplantation. Adolescents usually wait longer due to competition with adults. The length of time your child will wait depends on:

• the availability of donor lungs.
• blood type
• size (height)
• chest x-ray measurements
• location of transplant center (St. Louis is a fortunate place to await lung transplantation)

In addition to the excellent quality of the pediatric and adult lung transplant programs at Washington University, there are other advantages. Our central location puts us within reach of many major population centers. Since there is preference given to distance from donor to recipient center, we have advantages over coastal locations. In addition, MTS has a national reputation for efficiency and interest in thoracic organs. Our regional lung procurement rate is among the highest in the nation. Lastly, because of our high volume and reputation, we sometimes get offered lungs when time is running out for placement.)

There is no priority status with the lung patients based on the severity of disease. Priority goes by how long you have been on the waiting list (*in contrast to the situation in heart and liver transplants). You are matched with the donor based primarily on your blood type and height. Our physicians have years of experience in judging disparity in lung size between recipient and donor.

 What information can I know about my donor?

Curiosity about the donor is natural. Some people may think or dream about their donor. Some recipients worry that they may take on the emotional, psychological or physical characteristics of the donor. Some patients and families may wish to know about their donor, and some do not. It is natural to feel grateful to the donor and the donor family. However, because of the rules of confidentiality, we cannot provide information on the identity of the donor. If you wish, you can write an anonymous letter to the donor family and give it to your transplant nurse to pass through MTS and on to the donor family. Donor families are often extremely appreciative of letters coming from individuals who have received organs. If you write a letter you can include first names only, no phone numbers or addresses, and make no reference to location. You may send pictures.

 What happens when a donor becomes available?

When a suitable donor becomes available, we will call you at home. If you are not at home we will beep you. We will supply you with a beeper when you relocate. If you are beeped, please call the St. Louis Children’s Hospital operator at 454-6000. Tell the operator your child is waiting for a lung transplant and that you were beeped. The operator will connect you with the transplant nurse coordinator on call.

When we call you, we will tell you not to let your child eat or drink anything and if your child takes coumadin we will instruct you not to give your child any. You should make arrangements to arrive at the hospital as soon as possible, (without breaking any laws!). You will first go to the admitting department on the first floor. If it is after 11:00 P.M., go through the emergency room because the front doors of the hospital will be locked.

FALSE ALARMS - Sometimes the beeper goes off due to interference in the area and we call this a false alarm. Call 454-6000 anytime your beeper goes off. The operator will let you know if it was a false alarm.

REMEMBER - the transplant can be canceled after your child is admitted if any problem develops with the donor organs. We only accept suitable organs. The evaluation process at the site of the donor harvest is a dynamic one.

You and your child will be admitted to 7 West prior to the transplant. Your child will have blood work drawn, a chest x-ray (if not done recently), and a history and physical examination.

How long does a lung transplant operation take?

• about 1 hour for the anesthesiologist to put your child to sleep and insert the necessary monitoring lines.
• about 4-6 hours for double lung transplant and heart-lung transplant operation.
• add 1-3 hours if your child has had prior chest surgery.

How is the surgery done?

Heart-lung transplant:

After the chest is entered, the patient is placed on a heart-lung machine. Since the heart-lung machine is able to do the work of both the heart and the lungs, we can proceed with removing the heart and both lungs. We receive the donor heart and lungs all together as one unit. It is sewn in place by just attaching the main airway (trachea) of the donor to the recipient, the main artery coming off the heart (aorta) of the donor to the recipient and attaching the upper chamber on the right side of the heart (the right atrium) of the donor to the recipient. We then gradually wean the heart-lung machine so that the new heart and lungs slowly take on the work necessary to support the circulation.

Single lung transplant:

This is also generally done using cardiopulmonary bypass or the heart-lung machine. After the chest has been entered, the patient is placed on the heart-lung machine.

The lung is removed and the new lung is sewn into place beginning with the bronchial tube, next the artery to the lungs, and finally the vein returning the blood from the lungs to the heart.

Double lung transplant:

After the chest has been entered, the patient is placed on the heart-lung machine. Both the right and left lungs are removed. We then sew the left lung in first, beginning with the bronchial tube, next the artery to the lungs from the donor is sewn to the artery of the lungs of the recipient, and finally the veins returning blood from the lungs of the donor is attached to the upper chamber on the left side of the heart of the recipient. The same is done for the right lung when it is sewn in place.

We always leave anywhere between two and four drainage tubes in place to remove any blood that collects in the chest cavity. These are removed between 2 and 7 days following the transplant.

A broviac catheter will be inserted at the time of the transplant so we may have continuing IV access and the ability to draw blood for several weeks after transplant.

The surgical incisions are as follows:

Heart-lung	Double-lung	Single-lung

 What about blood transfusions?

Your child will receive blood products in the operating room and in the immediate postoperative period after transplant. Current screening technology and added precautions in the transplant setting puts the danger of transmitting infection by way of transfusing blood products at an extremely low level. It is not practical for you or your family members to donate blood for transplantation because we rarely know when the transplant is going to take place. If your child should need blood after the immediate post-operative period, there may be an opportunity for you to have designated donors (i.e., family members). Call the American Red Cross on Lindell to donate.

 What should I expect after surgery in the intensive care unit?

Most patients begin to wake up 2-10 hours after surgery is over. We generally keep most patients heavily sedated the first 24 hours after surgery. Your child will have:

• A breathing tube through your child’s mouth into the trachea (the main airway): Your child cannot talk or eat with this tube in place. The PICU nurses will communicate with your child by asking yes and no questions to which they can nod their head. It is difficult to clear mucus with the tube in place so the nurse will insert a smaller tube (catheter) every 2-4 hours to suction out lung secretions. After the breathing tube is removed, it is very important to cough and breath deeply. All patients will be on oxygen supplementation temporarily by mask or nasal prongs. Once the breathing tube is removed the nurse will give your child ice chips and then advance their diet to clear liquids, then soft foods, etc., depending on stomach function.
• Wrist Restraints: Your child’s wrist’s will be gently tied down as a precaution so they do not pull or dislodge the breathing tube or other monitoring lines as they are waking up.
• Chest tubes (four drainage tubes coming out of your child’s chest): These drain the blood and fluids from surgery in the chest cavity. In most cases, they are removed within 2-7 days.
• Foley Catheter: This drains your child’s bladder. It is critical to monitor how much urine is produced in the first 24 to 48 hours. Generally this is removed within 2-4 days.
• Large intravenous (IV) catheters: Will be in your child’s arms. There may be one in the jugular vein in the neck also.
• Broviac catheter: A semipermanent silastic catheter inserted into the large vein behind the clavicle or collar bone. We can draw most blood test in the days and weeks after surgery by this route to minimize needle trauma!
• Isolation: Post-transplant patients are placed in isolation to protect against infection. Your child will be in a single patient room during the transplant hospitalization. When family members or medical staff come to see your child, they will need to wash their hands and put on a gown, masks, and gloves.
• Visitors: Because recovery in the PICU is stressful and tiring to the patient, we request that visitors be limited to the most immediate family only.

How will my child feel after the surgery?

After surgery, it is common to feel any or all of the following:

• Lack of Sleep:
  Because your child is being constantly monitored, uninterrupted sleep is inconsistent in the pediatric intensive care unit (PICU). Often, days and nights will get confused . The nurses will try to allow time for naps to make up for lack of sleep. Sedation to enhance sleep may be needed for the first week after transplantation in most patients.

• Strange Dreams /Nightmares/Hallucinations:
  Anesthesia, medications, lack of sleep, and other reasons often cause patients to have strange dreams, nightmares, or even hallucinations. We need your help in interpreting your child’s mood and needs.

• Pain/Discomfort:
  In the first 24 hours, pain is not usually a problem because the anesthesia works as pain medication. After the first day, when your child begins to move and sit in a chair, they will feel incisional pain and even more so the discomfort of the breathing tube and chest tubes. Pain medication will be ordered.

• Nauseated/Poor Appetite:
  Medications, anesthesia, and surgery often make your child feel nauseated. Medication to promote emptying of the stomach and bowel activity is often needed. Inactivity and pain medications predispose to a sluggish gastrointestinal system.

• Difficulty Concentrating:
  This is temporary and will improve with time.

• Weak or Dizzy:
  The first few times out of bed (the nurses and therapists will always assist your child until they are stronger and comfortable enough to walk on their own).

• Difficulty with Coughing:
  One of the unavoidable effects of lung transplantation is the cutting of the nerves to the lungs thereby compromising the cough reflex. There is also chest wall pain and muscle weakness. We will try to give your child adequate pain medication because coughing and deep breathing is extremely important. Accumulation of secretions requiring replacement of the breathing tube is a common, usually minor, but always discouraging event in the early days after transplant.

(These are not meant to scare you. They are to let you and your child know what is normal after surgery so you are both prepared for what you might experience)

 What happens when the patient transfers to 7 west?

• It is an adjustment period going from the PICU, where your nurse is almost always in your room, to the ward, where your nurse will likely have 2 or 3 other patients to care for as well your child. Fortunately, family visiting privileges are more liberal.
• Visitors and staff are required to wash their hands and put on a mask before entering your child’s room. Your child should put on a mask when they leave their room to protect themselves from infection during the first week or so. Because your child will still tire easily, we request that visitors and phone calls be limited.
• Physical therapy is very important although it may be tiring for your child. We have experienced therapists who know how far to push each patient. Exercise will increase circulation, help healing, aid in keeping the lungs clear, increase leg strength, and make everyone feel better!
• You will be expected to start learning about your child’s post-transplant care, including medications, Broviac care and blood drawing from the Broviac(you should receive a booklet on Broviac care), blood pressure measurement, pulmonary function testing, pulse oximetry, signs and symptoms of infection and rejection, and general issues related to lung transplantation.
• You will begin to record your child’s weight, blood pressure, pulse, temperature, pulmonary function test results, and pulse oximetry in your home journal every day. There are also three important blood values to monitor (CSA level, white blood count, and creatinine level).
• Blood tests and a chest x-ray will be taken as directed by the transplant surgeons and pulmonologists.
• You and your child will be taught how to use a home spirometer by Deborah White R.R.T., chief technician, in the Pulmonary Function Lab. This device measures airflow and volumes similar to the pulmonary function tests done in the lab.You will be checking this daily at home and recording the readings in the journal.

 What do I need to know about going home?

It is another huge adjustment going from the hospital to your St. Louis home. While most people look forward to leaving the hospital, actual discharge may be met with mixed emotions. You may feel overwhelmed by the medication schedules, blood pressure monitoring, etc. Remember that you will be in close contact with the transplant team. You will be seen in clinic each week and can call us at anytime if questions or problems arise. Prior to your departure we will do a "head to toe" check as to how you are feeling and go over your medications and any side effects your child may be experiencing. You can bring any questions to the transplant team at this point. We will take as much time as you need. We will try to balance the increasing financial pressures for discharge with your individual needs.

Everyone recovers from surgery at a different pace. It is normal to have good days, as well as some not so good days, as your child recovers. Your child’s body is going through many adjustments as he recovers from the surgery, gets used to new medications and resumes activities. Some recipients may have forgotten or never learned how to be healthy. This challenge, although daunting, is what organ transplant seeks to achieve.

 What are the instructions for discharge after transplant?

• The child should not lift anything greater than ten pounds for six weeks after surgery and not perform strenuous activity for six weeks.
• If applicable, the patient should not drive four weeks after surgery.
• Check your child’s chest incision daily. If there is any unusual redness, swelling, pus, drainage, or pain, contact your child’s transplant nurse. Clean the incision by showering with warm water and soap daily.
• Check your child’s Broviac site daily. If there is any unusual redness, swelling, pus, drainage, or pain, contact your child’s transplant nurse. Broviac dressing’s are changed weekly.
• Remember to fill out your daily journal.
• Call the physician or transplant nurse immediately if your child has any of the following signs or symptoms:
  • fever over 99.6 F
  • redness, swelling, drainage, pus, or pain at the incision site or broviac site
  • flu-like symptoms
  • cough or shortness of breath
  • nausea, vomiting or diarrhea
  • blood in the stool
  • chills

Home monitoring helps prevent problems and may signal complications when problem solving is relatively safe and easy. We try to preach prevention and early intervention for problems which may develop. Your daily journal entry should include the following (you will start this in the hospital after surgery):

• Weight in the morning after urinating and before eating breakfast.
• Blood pressure twice daily.
• Temperature (or whenever your child feels feverish). Do not let your child have anything to eat or drink ten minutes before taking their temperature or it may cause a false reading.
• Pulmonary Function Testing measurements with special attention to FVC and FEV1.
• Pulse Oximetry

The journal should be brought to each clinic visit. Additional information you will want to keep in your notebook: CSA levels, serum creatinine levels, WBC count, medications your child is currently taking. Also, it may be helpful to write down questions, concerns, or comments you want to share with us in clinic. It is a good idea to bring your blood pressure cuff to clinic occasionally (every 3-6 months) to check against the clinic blood pressure cuff (to make sure it is accurate).

Before you leave the hospital, the transplant pulmonologist and nurse should establish a "threshold of concern" for FEV₁ and SaO₂ (pulse oximeter reading) below which you should call.

 Whom and when do I call?

For non-urgent problems, you should call:

• your lung transplant coordinator for medical issues. Each nurse has voice mail and will return your call.
• for regularly scheduled evaluation timing post-transplant call Vicki Kirtlink at (314)454-2694.
• for regularly scheduled evaluation timing pre-transplant call Briant Mitchell at (314)454-4278.

For urgent problems during weekdays, call your nurse transplant coordinator. Before 8am and after 5pm, and on weekends and holidays, call the hospital operator at (314)454-6000 and ask for the pulmonogist on call. Drs. Cohen, Mallory, Mendeloff and Huddleston, will be available through the on-call doctor as needed for emergencies.

Post-transplant Clinic Protocol for Lung Recipients

First Month:

• Clinic appointment twice weekly
• Blood tests (electrolytes, creatinine, cbc, csa level) with each clinic appointment as needed
• Chest x-ray as needed
• Spirometry (PFT Lab) twice weekly
• Pulmonary rehabilitation program three times weekly as arranged by physical therapist
• Surveilance bronchoscopy and biopsy as needed (heart and lung recipients generally undergo endomyocardial biopsy at the time of each transbronchial biopsy)

Second and Third Months

• Clinic appointment one time weekly
• Blood tests and chest x-ray as needed
• Spirometry (PFT Lab) with each clinic appointment
• Pulmonary rehabilitation program three times weekly
• Surveillance bronchoscopy and biopsy as needed

At the end of the third month, a three month evaluation will be performed which includes complete PFT’s, V/Q Scan, RVG, Six-minute walk test with physical therapy, extensive blood work, and a Surveilence bronchoscopy with biopsy.

If your child is clinically stable at the end of the third month, you may return home. We will give you mailers so you may mail CSA levels back to us as needed. We also request you purchase a copy of your child’s most recent chest x-ray to take back home with you so that your referring physician will have a baseline chest x-ray. A letter related to your child’s transplant course will be mailed to your child’s physician with a copy also sent to you. Your child will need to see your doctor shortly after returning home.

Your child will come back to St. Louis Children’s Hospital every three months the first year and then every six months thereafter for routine re-evaluations. This schedule is based on a uncomplicated course. The frequency of various tests may increase with episodes of rejection or infection.

Bronchoscopy

All lung transplant patients require surveillance flexible fiberoptic bronchoscopy with lavage and transbronchial biopsy. We will also perform bronchoscopy when we suspect airway problems, and when rejection or lung infection is suspected at any point after transplant. The procedure is performed with the child sedated and the parents present (if preferred).This procedure consists of passing a flexible tube through the nose into the trachea (main airway) and down into the smaller airways. The lung(s) will be "washed" with a salt water solution and suctioned to diagnose infection. Small pieces of lung will be taken to diagnose rejection. In general, bronchcoscopies will be done in the Ambulatory Procedure Center on the first floor. The doctors and nurses will explain everything they need to do, and will help keep your child relaxed.

 What about my family?

It is common for family members to feel exhausted by the time of discharge from the hospital. It is therefore important to make sure that family members take care of themselves, get plenty of sleep, and eat a healthy diet. The time of discharge is a particularly important for both parents to be present, if at all possible. Family members may feel some anxiety about their vulnerable child leaving the hospital. It can be overwhelming to learn about new medications, a new lifestyle and a new complications. However, the transplant team is available to address your concerns. You will be redefining what life is like after transplantation. This is a time of change and adjustment which may take several months or longer. The recipient hopefully will now be well enough to do activities he or she may have been too sick to do before the transplant. The readjustment to the new lifestyle may be difficult for everyone in the family. It is important that you talk with each other about your feelings and perceptions. Please discuss these issues with the transplant social worker and in support group meetings.

 What is acute rejection?

Acute rejection is the term used to describe the body’s normal reaction of trying to rid itself of foreign tissue. We have come to expect at least one rejection episode in each patient. Acute lung rejection usually occurs within the first three months. Rejection is most often detected at a mild stage. Remember, acute rejection is usually treatable. Your child’s new lungs will continue to work well even if it has had episodes of rejection.

Acute lung rejection is most often detected by or manifested as:

• New infiltrates (white areas) on the chest x-ray.
• Decreased FEV1 on your spirometery.
• Increased shortness of breath.
• Increased temperature.
• Non-specific symptoms of fatigability and low energy.
• Sometimes low grade fever.

However, your child may not have any symptoms at the time that mild rejection is detected. That is why we consider close attention to spirometry measurements and surveillance bronchoscopy procedures to be so important.

Depending on the severity of rejection, treatment may include:

• A three-day course of intravenous Solumedrol, or an increase in your child’s daily prednisone dose.
• Admission to the hospital for intravenous anti-rejection medications.
• Addition of an additional anti-rejection medication.
• If very mild, possibly no treatment.

For heart-lung recipients it is possible also to have heart rejection. Therefore, periodic heart biopsies are required. A heart biopsy consists of inserting a catheter (small, thin tube like an IV) into the neck, or groin, until it reaches the entrance of the heart. A bioptome, which is a special instrument to get the biopsies, is passed through the catheter into the heart under x-ray guidance. Several small pieces of heart tissue are taken and examined under a microscope to detect rejection similar to the transbronchial biopsies. Washington University has some of the most experienced pathologist in the world interpreting our biopsies specimens.

Complications are rare with a heart biopsy. However, you should be aware of them:

• Bleeding at the entrance of the catheter. A bandage on the neck or groin must stay in place until the following morning. Please check for any bleeding.
• Pneumothorax, a collapse of the lung (from the catheter nicking the lung), may result in chest pain and shortness of breath. A chest x-ray is usually checked after the biopsy.

Symptoms of heart rejection the patient may feel:

• Fever
• Palpitations, irregular heart beats or sudden increase in heart rate, (around 30 beats faster).
• Unexplained tiredness or lack of energy.
• Symptoms similar to an infection or flu.

The treatment of acute heart rejection depends on the severity:

Mild rejection: No treatment, observe for any changes in condition; we may schedule your next clinic visits, chest x-rays, and PFT’S earlier

Moderate rejection: A three day course of intravenous Solumedrol (similar to prednisone) or increase oral prednisone dose gradually weaning back to your normal dose

Severe rejection: Generally treated with intravenous steroids for three days, or stronger antilymphocyte serum for 10 to 14 days.

 What is chronic graft dysfunction?

Chronic graft dysfunction (in lungs referred to as Bronchiolitis obliterans) is usually related to cumulative graft injury but sometimes occurs with little warning. It may occur as early as three months after transplant or many years later. Bronchiolitis obliterans causes problems with air flow and oxygen exchange. Bronchiolitis obliterans results in a gradual and progressive deterioration in lung function. The diagnosis is made by PFT’S ( a drop in FEV1), radiographic studies, and biopsy of the lung tissue.

In heart-lung transplantation chronic graft dysfunction may also affect the heart. In the heart, chronic rejection is manifested as accelerated coronary artery disease, that is, narrowing of the arteries which supply blood, oxygen, and nutrients to the heart. Chronic rejection in the heart can be detected by cardiac catheterization (injecting dye into the coronary arteries). Eventually, chronic rejection causes failure of the transplanted heart and lungs.

At this time, all of the factors leading to chronic graft dysfunction are poorly understood. Our program is involved in basic and clinical research to learn more about bronchiolitis obliterans. Medications are not always effective in reversing chronic rejection as they are in acute rejection. The only definitive form of treatment for severe chronic rejection is retransplantation. Retransplantation has higher risks than primary transplantation and is not a good choice in every patient.