It was in the final game of the 2010 Miracle League of the Lehigh Valley Baseball season last October when 9-year-old Luke Maeding, playing for the Pirates, achieved a milestone that would have been impossible a year earlier. He ran to first base.
Less than two months before, Luke, who lives in Nazareth, Pa., had received a double-lung transplant at Children’s Hospital of Pittsburgh of UPMC, freeing him from the inactivity and respiratory support demanded by his severe lung disease. “He wasn’t able to do much this summer, but he made it to his last game and he ran to first base,” says his mother, Heather Maeding. “He was so happy.”
Luke’s life had been difficult from the beginning. 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 Heather and her husband, Glen, as a foster child.
Heather, a nurse practitioner at St. Luke’s, and her husband adopted Luke when he was 20 months old. Being born so early typically means a child will struggle with chronic lung problems, and Luke was no exception. To make matters worse, he had respiratory illnesses as an infant, including influenza. Infants born as early as Luke often have a temporary need for oxygen as their lungs mature. But Luke’s need increased as he got older. At age 1, he relied on oxygen only at night. At 30 months, he needed it around the clock. “By that time,” his mother says, “we realized this was something more than just premature lung disease.”
In Search of a Diagnosis
Luke spent his first six years of life undergoing treatment and examinations at four hospitals near his home, including The Children’s Hospital of Philadelphia, where he had a lung biopsy. For his parents, those years were marked by frustration, disappointment, and growing concern. For all of the consultations and hospital visits, a precise diagnosis for Luke’s lung disease could not be determined; the prescribed treatment led to other serious health problems; and his lung disease progressively got worse.
“He was on high-dose steroids for two and a half years, which caused bad side effects,” says his mother. He fractured seven vertebrae in his back. His adrenal glands no longer worked. And he ended up with insulin-dependent diabetes. “A lot of bad things were happening with the treatment, but it seemed that no one was pursuing what was wrong with his lungs and whether we were doing the right things for him.”
A visit to Pittsburgh brought the family hope. Luke’s mother became involved in the Childhood Interstitial Lung Disease Foundation, a nonprofit that offers support and education to families of children with rare lung diseases. At one of the group’s conferences she met Geoffrey Kurland, MD, medical director of Pediatric Heart and Lung Transplantation at Children’s Hospital of Pittsburgh of UPMC, and discussed Luke’s case with him. In July 2007, Luke made his first six-hour trip to Pittsburgh for an evaluation.
“We felt comfortable because Dr. Kurland is part of that consortium,” Heather Maeding says. “And we felt it was important to travel that distance to go to someone who knew about these rare lung diseases.”
Her instincts, she says, were confirmed during the first visit to the hospital. “Right from the start we felt that we were part of the team, that what we thought mattered. They truly cared about Luke — that he was a little 5-year-old boy going through this — and about our family’s experience and not just about what the medical outcome and statistics would be.”
“A Tree That Never Bloomed”
Doctors in Pittsburgh, like others before them, could not point to a precise diagnosis for Luke’s lung disease. But from their evaluation and information from Luke’s previous tests and evaluations, it appeared the boy’s lungs simply failed to mature, which likely was the result of being born prematurely, illness, and other factors.
“He grew in size, but his lungs didn’t catch up,” says Jonathan Spahr, MD, Luke’s pulmonologist at Children’s and part of the
Division of Pulmonary Medicine, Allergy, and Immunology.
“The air sacs in Luke’s lungs never developed properly. So while Luke could get air in and out of his lungs easily, oxygen didn’t pass to the bloodstream as well as it needed to. The best analogy I heard was that Luke had lungs like a tree that never bloomed.”
The decision of whether to list Luke for a transplant did not come until three years after his evaluation at Children’s because his doctors tried to buy time with treatment, such as optimizing his nutrition and oxygen, in the hope that his lung tissue would start to grow with him. About half of the patients who receive lung transplants survive five years or longer, so it is important to try to delay the operation for as long as possible without further compromising the child’s health.
But Luke’s lungs did not recover as hoped. “It took us a few years to get a better handle on where he was headed,” says Dr. Kurland, who also is in Children’s Pulmonary Medicine Division. “We came to the point where it looked like where he was heading was not where we wanted him to go. A lung transplant is always the last option— but we felt at that point a lung transplant was our only option.”
Luke’s family agreed. He was wheelchair-bound, needed oxygen and breathing support day and night, and could do little more than read, play video games, and sleep. “That’s why 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.”
A Whole New Life
The Maedings got the call at 6:31 p.m. on Friday, Aug. 13, 2010. Donor lungs were available for Luke. As an organ procurement team from Children’s flew to get the donor organs, Luke and his mother were flown to Pittsburgh in a race against the clock. Donor lungs no longer receive blood once they are removed, so surgeons want to transplant them as quickly as possible, usually within four hours.
Peter Wearden, MD, PhD,of Children’s Division of Pediatric
Cardiothoracic Surgery, led the transplant team. Dr. Wearden and
Victor Morell, MD,, surgical director of the Pediatric Heart and Lung Transplantation Program and chief of the Cardiothoracic Surgery Division, typically perform three to six double-lung transplants at Children’s each year. That Friday night, the surgical team gave Luke a new set of healthy lungs during a six-hour operation.
Luke spent the next seven weeks recovering in the hospital, several of them in Children’s state-of-the-art Cardiac Intensive Care Unit, or CICU. Complications are common after lung transplantation, and Luke had his share. When, for instance, he had difficulty breathing after his transplant, Dr. Wearden noticed on an x-ray that Luke’s right diaphragm was lower than his left. He took Luke back into surgery, where he discovered scar tissue on the diaphragm from a previous surgery and removed it. “Once we did that,” says Dr. Wearden, “Luke just started to soar.”
Getting himself back into shape after having been inactive for so long is something Luke continues to work on with walks and workouts on a treadmill. He has to take strong medication to prevent his body from rejecting his new lungs. He has to try to avoid colds, flu, and other respiratory infections, as his weakened immune system leaves him vulnerable to illness. But for the first time in his life, oxygen tanks are nowhere to be found in the house. And his brothers and sisters — five of whom live at home — no longer have the advantage of following his air tubing when playing hide-and-seek. “Twice now I’ve had the kids come to me and say they couldn’t find him,” his mother says. “We are all getting used to a whole new life with Luke, and he is able to live life again.”