Heather Maeding and her seriously ill 9-year-old son, Luke, had just made the six-hour drive from UPMC Children's Hospital of Pittsburgh back to Nazareth, Pa., home when she received a phone call from the hospital transplant coordinator that would necessitate their immediate return.
As mother and son flew back to Pittsburgh, a procurement team was dispatched to retrieve the lungs in a race to transplant them within a four-hour window. Luke was in surgery within an hour of being admitted to the ICU.
Long Journey
Luke first came to UPMC Children's Hospital in 2007 after his mother met Geoffrey Kurland, MD, medical director of the hospital's Pediatric Heart and Lung Transplantation Program, at a Childhood Interstitial Lung Disease Foundation (ChILD) conference in Ohio. She had become involved in the nonprofit, which supports and educates families of children with rare lung diseases, as part of her search for answers about Luke's disease.
Heather and Glen Maeding had taken Luke to four eastern Pennsylvania hospitals, including The Children's Hospital of Philadelphia, but a precise diagnosis remained elusive. Prescribed treatment that included high-dose steroids had led to other serious health problems including seven fractured vertebrae, nonfunctional adrenal glands, and insulin-dependent diabetes. Meanwhile, his lung disease was getting worse.
Following the ChILD conference, she and her husband decided to bring Luke to Pittsburgh for a lung transplantation evaluation. They knew transplantation would be necessary if Luke's lung disease continued to progress.
"We felt comfortable because Dr. Kurland is part of that consortium," she says. "And we felt it was important to travel that distance to go to someone who knew about these rare lung diseases."
Physicians at UPMC Children's, like others before them, were unable to determine a precise diagnosis. "It is much better for us if we have a diagnosis that we know, that agrees with what the patient looks like, and that we understand the prognosis for," says Dr. Kurland, also of the Pulmonary Medicine, Allergy, and Immunology Division. "But that isn't always the case. We did not know Luke's precise diagnosis. But we knew a lot of things."
He Wasn't Really Living, and He Knew That
What they did know was that Luke's life had been difficult. He was born at 26 weeks gestation and spent three months in the neonatal intensive care unit of St. Luke's Hospital in Bethlehem, Pa., before going home on oxygen with the Maedings as a foster child. They later adopted Luke. From birth, he had chronic lung problems related to prematurity. As an infant, he developed influenza. He had undergone a lung biopsy and had abnormalities of formation of or damage to the lung. His need for oxygen continually increased, and although he could move air in and out of his lungs well, his oxygen delivery was poor.
The conclusion drawn was that his lungs had failed to mature. Luke grew in size, but his lungs just didn't catch up – like a tree that never bloomed.
The decision was made to postpone Luke's transplantation in favor of continuing treatment in the hope that his lung tissue would start to grow with him. But by 2010, he was wheelchair-bound and needed constant oxygen and BiPAP support.
"We did his lung transplant for his quality of life," his mother says. "Even though he was alive, he wasn't really living, and he knew that. He could only watch his brothers and sisters play."
Luke Just Started to Soar
The six-hour procedure began with the removal of Luke's diseased lungs, a process that usually begins once the transplant team receives word that the plane carrying the donor lungs has landed in Pittsburgh. Removing Luke's lungs required a bit more time due to scar tissue associated with a lobectomy that had been performed years earlier at another hospital. Luke was placed on a heart-lung machine.
The donor lungs were separated at a back table in the operating room and were implanted one at a time.
Luke spent the next seven weeks recovering, several of them in Children's state-of-the-art Cardiac Intensive Care Unit. Luke had his share of complications: He had to return to the operating room to have a blood clot removed from his chest. And when he had difficulty breathing post-transplant, an x-ray revealed that Luke's right diaphragm was lower than his left due to scar tissue that resulted from an earlier lobectomy (at another hospital). Once the scar tissue was removed, there was more space for his new lung to fully expand. After the procedure, Luke just soared.
Luke’s Next Stage in Care
Following his lung transplant, Luke began easing back into normal, everyday activities. But about a year after his lung transplant, he started experiencing intestinal issues. After additional trips to UPMC Children’s, the doctors determined that Luke needed an intestinal transplant.
“Throughout that entire process, Dr. Kurland and the team at UPMC Children’s were very supportive,” says Heather. “There was concern that an intestinal transplant would affect his lung transplant, but Dr. Kurland always had Luke’s best interest and quality of life in mind.”
Luke received his intestinal transplant in the spring of 2020 and quickly showed improvement.
"Ten days after his transplant, Luke ate a turkey sandwich for lunch and a piece of pizza for dinner,” says Heather. “He hadn’t eaten solid food in over three years prior to his transplant.”
Now, Luke continues to play baseball on his local Miracle League team, the Pirates. He graduated high school in the spring of 2022 and is an active participant of UPMC Children’s Camp Chihopi, an overnight summer camp dedicated to children who received liver and/or intestinal transplantation.
The Maeding family has been through a lot, and Heather extends her support to other families going through a similar experience.
“The team at UPMC Children’s was never afraid of the unknown,” explains Heather. “From the beginning of Luke’s lung transplant journey to his recent intestinal transplant, UPMC Children’s was always determined to provide care for Luke. The team took his high-risk case and were with him every step of the journey. I think that’s important for parents to understand when deciding where to take their child for care.”
Luke and his family attend routine appointments at UPMC Children’s to monitor Luke’s health. When he and his family are ready, Luke will transition care to the adult program at the UPMC Heart and Vascular Institute as part of an integrated, multi-disciplinary program. For now, the Maeding family welcomes their recent sense of normalcy by celebrating Luke’s newest milestone.