Lung Transplantation

The Waiting List

Many parents ask questions about what it means to be “listed” on the transplant list.  A national procurement network, the United Network for Organ Sharing (UNOS), was established for the fair distribution of donor organs.  Potential lung recipients are listed by the transplant coordinator with the organ procurement agency for the region

For children age 11 and under:

Lung transplantation is performed based on the total time spent waiting for a transplant. Several factors are taken into consideration for the assignment of donor lungs including blood type, lung size and single versus bilateral transplantation. Once the evaluation has been completed and the transplant team feels that your child is eligible for a lung transplant he or she will be placed on the waiting list. The sooner a child is evaluated and listed, the more advantage a child has of attaining a transplant. 

For children over the age 12 and over:

Each patient will receive a “lung allocation score,” ranging from 0 (less ill) to 100 (gravely ill).  Patients with higher scores will receive priority over those with lower scores.  The score will be updated every six months based on regularly scheduled testing.  The score can also be reassessed by additional testing at any point if a patient’s clinical status changes.  Therefore, it is important to come to Johns Hopkins Hospital every three months to evaluate a patient’s status.  The lung allocation score is based on the following tests:

• Forced vital capacity - This is a lung function test that measures the maximum amount of air you can breathe out after you breathe in as deeply as possible. 
• Pulmonary artery pressure - This is the pressure the heart must generate to pump blood through the lungs.  This pressure may be high in some patients with serious lung disease.
• Oxygen at rest - This is the amount of oxygen needed by a patient at rest to maintain adequate oxygen levels in the blood.  Patients with severe lung disease may need additional oxygen.
• Age - This is the candidate’s age at the time lungs are offered.
• Body mass index - BMI is a ratio of a patient’s weight to height that, when interpreted with other medical test results, helps to evaluate health status.
• Insulin dependent diabetes - Diabetes may be a predictor of health status in some patients with lung disease.
• Functional status - The New York Heart Association classifications measure effects that lung disease may have on a patient’s function in everyday life.
• Creatinine - Creatinine levels are a measure of kidney function. 
• 6-Minute walk distance - In the 6-minute walk test, a patient is asked to walk as far as s/he can in 6 minutes.  The distance walked is a measure of functional status.
• Ventilator use - The use of a ventilator to assist breathing may be a measure of disease severity and may affect success after transplant.
• Pulmonary capillary wedge pressure - PCW or “Wedge Pressure” is the pressure blood returning to the heart from the lungs must overcome.  This pressure can become increased when the heart is not pumping effectively.
• Diagnosis - Research has shown that urgency among patients needing a transplant and success after transplant vary among patients with different lung diseases due to the specific progression of the particular disease.  Therefore, for every patient, diagnosis factors into the calculation of the lung allocation score.

Single Lung Transplant
This surgery replaces one of your child’s lungs with a donor lung. Once the blood vessels and airway of the donor lung are attached the transplanted lung will begin to function. This operation is rarely performed in children

Bilateral Lung Transplant
This surgery replaces both of your child’s lungs with donated lungs. The lungs are implanted one at a time during the same operation. In some cases only a single lobe of the donor lung is used to replace your child’s entire lung. This procedure is called a bilateral lobar transplant. This surgery is an option for younger children where the function of one lung can be replaced by an adult lung lobe.

Living Donor Lung Transplant
This surgery is an option for smaller children where the function of one lung can be replaced by an adult lung lobe donated by healthy adults with normal lung function. This surgery requires two adults, with the same blood type as your child, to undergo surgery to donate portions of their lungs. Living donation is usually performed only in dire circumstances. If you and your physicians feel that living donation is appropriate for your child then you will be asked to identify at least five potential donors. These potential donors will then be screened and evaluated by an adult pulmonologist at Johns Hopkins.

Organ Donation

Donation from deceased donor
Typically, lung donors are people that have suffered severe brain injury rendering them “brain dead”. The families of these children or adults have given their consent for organ donation. Potential organ donors are evaluated for blood type compatibility, lung function and infections. Donor lungs are matched to potential recipients based on blood type and size. The donation process is managed by the Transplant Resource Center of Maryland (TRC). All organ donations in the United States are governed by the protocols of the United Network for Organ Sharing (UNOS).

You may have many questions about the person that donated your child’s new lungs. However, due to patient confidentiality we can only supply you with a limited amount of information. It is natural to feel grateful to an organ donor and his or her family. If you would like, you can write to the donor’s family via the Transplant Resource Center (TRC), who will gladly forward your letter to them.

Living Donation
In some cases, a portion of a lung from two living adult donors is used for a lung transplant. Each donor would have a single lobe removed to replace each of your child’s lungs. At least five potential candidates will need to be evaluated as potential donors for this type of transplant. Potential donors are evaluated for blood type compatibility, lung function and infections.  Since living donor transplantation requires two healthy adults to undergo surgery it is reserved for children whose chance of survival without transplant is low.

Lung transplantation is performed based on the total time spent waiting for a transplant. Sicker children do not receive preference for lung transplantation. Several factors are taken into consideration for the assignment of donor lungs including blood type, lung size and single versus bilateral transplantation. Once the evaluation has been completed and the transplant team feels that your child is eligible for a lung transplant he or she will be placed on the waiting list. The average time on The Johns Hopkins Hospital waiting list is 18-24 months.

Since donor lungs are allocated to children based on the total time spent on the waiting list, and not on severity of lung disease, the timing of the transplant evaluation is critical. The best time for referral is before your child’s lung disease has affected other organs. Given the current waiting time for a lung transplant the evaluation process should be started at least 2 years prior to the anticipated need for a transplant. If your child's health improves he or she will be transferred from the waiting list to an inactive status (status 7). Your child will not lose their time on the waiting list. If he or she needs to be reactivated, the earlier time on the list will be added to your child's wait for a transplant. This system necessitates early evaluation for transplantation to provide the best possible outcome.