For years, a machine pushed concentrated oxygen into Marcellius Sears's stiff lungs through a tube in his nose. It was hard to talk, hard to eat, and he could wander no farther than the length of tubing from the machine providing his oxygen. But that was the only way that Celli, as his family calls him, could breathe.
Toddlers get a lot of colds, but from the time he was 2, Celli, now 9, was in and out of the hospital regularly with pneumonia, bronchitis, and respiratory infections. Finally, when he was 3, his pediatrician recommended sending him for evaluation to the C. S. Mott Children's Hospital at the University of Michigan, two hours from the family's Saginaw home. Lisa Ortega-Sears, Celli's mom, vividly remembers the day she received the diagnosis: idiopathic pulmonary hemosiderosis, a very rare disease that causes bleeding into the lungs. The pulmonologist held her hands while she explained that Celli probably had about five years to live before his lungs became too damaged to allow him to breathe. A lung transplant wasn't an option, she said. The disease would return and destroy the new lungs.
Celli went home, on steroids to help him breathe and control his bleeding. Over the next few years, the family, including Celli's two siblings, tried to live a seminormal life. But it all came to an end one winter afternoon in 2006 when Celli, then 6, suffered a massive pulmonary hemorrhage. He was helicoptered to C. S. Mott and stabilized, but whether he would pull through was touch and go for many days.
In need of hope. In the weeks that followed, while Celli remained hospitalized, a family friend discovered the lung transplant program at Children's Hospital of Philadelphia. Impressed with its success with difficult cases like Celli's, the friend E-mailed Gary Visner, then the program's director. He reviewed Celli's medical records and said that even though Celli's condition was very critical, he should be evaluated for a lung transplant. Visner said that in his experience, idiopathic pulmonary hemosiderosis does not always return. "He was so optimistic, and I hadn't heard optimism in such a long time," Ortega-Sears says.
A handful of institutions perform the mere 50 to 60 lung transplants into children each year in the United States. Donated lungs deteriorate quickly, size is a problem, and organ rejection is more common with lungs than with other transplanted organs. Ten years after transplant, the survival rate for kids and adults is about 20 percent. But kids like Celli will otherwise die.
Celli was accepted into the lung transplant program and began several months of work to prepare for surgery. He needed physical therapy to get stronger after his long hospitalization, a tracheostomy to open a hole in his wind pipe so he could breathe more easily, and a new medication regimen that didn't rely as heavily on steroids.
The long haul was made bearable by the staff at the hospital, says Ortega-Sears, who lived at the Ronald McDonald House nearby. Whenever a frustrated Celli refused to do his exercises, a child development specialist would come up with games and rewards to persuade him to stick with it. As time dragged on and Ortega-Sears's spirits flagged, a staff member from the palliative-care unit took her out to get her hair done at a local salon.
After eight months, the family finally got the call—there was a pair of lungs for Celli. The surgery, performed by Thomas Spray, a pioneer in pediatric lung transplantation, was done in just over four hours, about half the usual time, with no complications. When she saw her son for the first time after surgery, Ortega-Sears was amazed. "He was pink," she remembers. "He'd never had any color to him before."
Celli would be in rehab at the hospital for an additional two months, but there came a day about a month after surgery when the tube came out of his windpipe and Celli took his first breath on his own since he was 3 years old. Out of the window he saw people passing by. "Look," he said to his mom. "I'm breathing just like them."
Celli is back home in Saginaw, with a new life. He started school for the first time ever in April, and he rides his bike, plays basketball, and goes swimming. Although he will be on a lifetime of antirejection medications, in many ways he's living the life of a normal 9-year-old. That suits him just fine: Normal is breathtaking.