Lung Transplantation
We consider lung transplantation if a child has end-stage pulmonary (lung) disease, his or her life expectancy is limited, and no other treatment options are available. Lung transplants can now be performed in all ages — from newborn to adult. A child may need a transplant if he or she has certain conditions, including:
- Cystic fibrosis
- Primary pulmonary hypertension
- Interstitial lung disease
- Bronchiolitis obliterans
- Surfactant protein deficiencies
- Congenital malformation with inadequate lung development
- Pulmonary veno-occlusive disease
- Heart disease or heart defects (may require a heart-lung transplant)
Whether your child could benefit from lung transplantation is a complex issue that must be carefully and individually evaluated. If you are considering lung transplantation, request an evaluation as early as possible.
Evaluations and referrals
If lung transplantation is being considered, please request an evaluation at Children's Hospital as early as possible by calling Tara Chestnut at 267-426-0664 or sending us an e-mail at Lungtransplant@email.chop.edu. If you are a physician and would like to refer a patient, please call 215-590-0388 or send an e-mail to Samuel Goldfarb, MD, Medical Director, Lung and Heart/Lung Transplant Programs.
Evaluating your child for transplantation
Typically, we evaluate your child for transplantation over the course of two to three days. In some cases, inpatients evaluations are performed. We do a complete review of your child's condition and determine whether it is medically and otherwise appropriate to proceed to transplantation. Appointments for evaluations can be made promptly. The medical review may include:
- Lung function tests, such as spirometry and measuring lung volumes
- Measurement of your child's nutritional status and exercise testing
- Blood work to assess blood typing, kidney, immunologic, nutritional and infectious disease status
- Heart function tests such as echocardiography and in some instances heart catheterization
- Lung computed tomography (CAT scans) and ventilation-perfusion scans
Our social workers and psychologist participate in your child's evaluation and play an important role in helping you and your family prepare for transplantation.
Financial advisors are also available to help families and to work with insurance companies.
Once we complete your child's evaluation, the entire transplantation team meets to decide if we should place your child on the transplant list.
Patient listing
The listing procedure is coordinated through the United Network of Organ Sharing (UNOS). It is important for you and your child to remember that:
- Your child is matched with donor lungs based on her body size and blood type
- If your child is 11 years of age or younger, his or her name will move higher on the list based on how long your child has been listed, not how sick he or she is
- If your child is 12 years of age or older, listing is based on a lung allocation score through UNOS that takes several factors into consideration
- UNOS allocates lungs on a regional basis; the region in which the donor is located is offered the organs first.
Waiting period
During the waiting period, your child will be followed by both your primary care physician and by our team. Arrangements will be made to contact you, and you will need to be within a short travel time (usually within 3-4 hours) to Children’s Hospital.
When you receive the call that an organ has been located, you must immediately come to the Hospital so your child can be admitted. In the meantime, a member of our surgical team will be sent to evaluate the donor and, if appropriate, retrieve the donor organ. Once our team is notified that the transplantation will proceed, your child will be taken to the surgical area and prepared to receive the donor lungs.
Surgery
Lung transplantation surgery typically takes five to seven hours. Our surgeons will make an incision that extends from side to side under the breastbone. Your child will be placed on cardiac bypass, his or her lungs removed, and the donor lungs implanted. Our Cardiothoracic Anesthesia team will anesthetize your child and remain with your child until he or she returns to the Cardiac Intensive Care Unit (CICU) for recovery.
Recovery and post-transplant care
After surgery, your child will be brought back to the CICU with the following equipment in place:
- A breathing tube connected to a mechanical ventilator
- Chest tubes to drain fluid from the surgical site
- Small catheters inserted into blood vessels for monitoring and to deliver medication
- A catheter inserted through the urethra to the bladder to monitor urine output
In general, the breathing tube will be removed within a few days after surgery, once we are certain we can administer adequate pain medication without suppressing your child’s drive to breathe. This is often accomplished by delivering pain medication through an epidural catheter, like those used for women in labor.
Usually within one week of surgery, most children are ready to be transferred to the General Pulmonary floor. Chest tubes are usually removed a few days later and by two to three weeks after surgery, your child should be ready for discharge.
Rejection prevention
The transplanted lungs are foreign tissue and the body’s natural immune response is to reject them. To prevent rejection, your child must take immunosuppressive medication. This type of medication greatly decreases your child’s ability to fight off infection. As a result, post-transplant care focuses on monitoring your child for signs of infection or rejection and for any side effects related to the medication.
This requires frequent visits to the transplant clinic – at first every other week or so, extending to every three months by the end of the first year. If your child experiences a fever or any change in symptoms, we encourage you to contact our Transplant advanced practice nurses, who are on call 24 hours a day, seven days a week.
The earliest sign of rejection is a decrease in lung function. You will be given simple testing equipment to monitor your child's lung function at home each day. In addition, surveillance bronchoscopies and biopsies are scheduled one month, three months, six months, one year, 18 months, and then yearly or as needed, depending on the patient's rejection history.
While our outcomes are among the best in the world, survival rates for lung transplantation vary depending on a child’s diagnosis and general condition at the time of surgery. We will talk with you at length about your child's specific situation and prognosis.
Contact Us
For more information on our services or to schedule an appointment.
- 215-590-4040
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