One in four children in the U.S. who need lung transplants is on the waiting list at St. Louis Children’s Hospital, one of the few hospitals that performs the surgeries. Their waiting time is expected to get shorter after new rules go into effect this year that will expand the geographic range in which donor lungs can be shared.
The rule change is a response to the case of a dying 10-year-old Philadelphia girl who was added to the adult transplant waiting list in 2013 after a federal judge intervened. Sarah Murnaghan went on to receive two lung transplants from adult donors because donor lungs from children are in short supply. The next year, the organizations that oversee organ transplants changed the rules to allow children to be added to waiting lists for adult lungs.
But because implanting adult-sized lungs into children’s bodies is not ideal, the groups decided to change the rules again. If a set of lungs from a child become available, doctors will now look for a suitable pediatric transplant candidate throughout an expanded 1,000-mile region.
“Today if a lung is available in Alabama or Florida, I’ve got to compete with every adult between here and there,” said Dr. Stuart Sweet, director of the pediatric lung transplant program at Children’s. Under the new rules, “the only competition will be children. It levels the playing field and gives my children that are waiting here a much better chance at transplant.”
Organ transplants require one of the most sensitive and complicated equations in all of medicine. The donor organs have to be compatible with the recipient, who can’t be either too healthy or too sick to receive them. Transplant surgeries themselves are risky, with about a 50 percent survival rate for lung patients after five years. Most tragically, for a child to receive a chance at life, another child has to die.
Sweet wrestles with those decisions in his role at the hospital and with the United Network for Organ Sharing, the nonprofit group that oversees transplantation in the U.S. Later this year, Sweet will become president of the organization’s board of directors.
The rule change for pediatric lung transplants is not expected to have an effect on the waiting times for adults. There are currently 38 children waiting for lung transplants nationwide, compared to about 1,500 adults. Nine of the children are on the waiting list at Children’s, where the median wait time for surgery is 10 months.
Heather Gwin, 9, has been on the waiting list for new lungs since August. Like Sarah Murnaghan, Heather has end-stage lung disease caused by cystic fibrosis. Since joining the waiting list she has traveled from her home near Indianapolis to St. Louis to spend two weeks in the hospital every month.
Cystic fibrosis is a chronic genetic condition that causes mucus to build up in the lungs and other organs. Because of the progression of the disease, Heather also will need a liver transplant. The wait has been grueling on the family. Heather’s grandparents take turns traveling to St. Louis along with her mother, Jennifer Gwin.
Cystic fibrosis is one of the most common conditions in children that can lead to lung transplants. Treatments for the disease have improved in recent years, reducing the number of children who require transplants. But in cases like Sarah and Heather’s, the disease becomes so severe that transplant is the only option. In court arguments that led to Sarah being placed on the adult waiting list, her parents said she was a few weeks from death. The rule changes that resulted from her case were unprecedented in transplant medicine, doctors said.
“There has never been a case like this where the U.S. judicial system has made such a statement that led to an urgent evaluation of policy,” said Dr. Kevin Chan of the University of Michigan, chairman of the lung subcommittee.
The priority for the new rules is reducing the number of children who die before receiving a transplant. About 25 percent of children who need new lungs die on the waiting lists, according to the Scientific Registry of Transplant Recipients. Just adding kids to adult waiting lists isn’t enough to solve the problem, doctors said. When adult lungs are downsized to fit into children, the procedure is more complicated and the body’s natural infection fighting abilities are not preserved.
Using simulation models, the members of the national group’s lung transplant and ethics committees plotted out ways to increase opportunities for transplants for children and teenagers, without significantly changing the outcomes for adults. Doctors said they feel like the new rules are a fair way to increase the number of transplants for anyone who needs them.
“One way is to increase broader sharing of organs,” Chan said. “We want the organs offered to similar age groups rather than being taken by an adult.”