By Mat Edelson | Photographs by Keith Weller
t’s a muggy Maryland countryside dusk and a pony-tailed teenager stands giggling in the batter’s box. "Hey Stefanie! Hit it for your boyfriend!!" yells a softball teammate. Then, as her latest beau looks on, 15-year-old Stefanie Allen complies, lining a sizzler into left field. Suddenly, she’s a neon green streak racing toward first when, mid-stride, she purposely pops her batting helmet off. "If you throw your helmet, you’re OUT," warns the ump.
"It’s bothering me!" Stefanie shouts. "It messed up my hair!"
From a lawn chair behind the first base dugout, Stefanie’s father, Dave, can only laugh. "I’m sure there has to be a bad hair day rule," he drawls. The loving look in Dave Allen’s eyes says, "Whatever you say, Stef, it’s all right by me." To the average dad, antics like hers are just part of watching a little girl become a young lady. To Dave Allen, these are moments he thought his daughter would never live to see.
When Stephen Yang stopped by Stefanie Allen’s Hopkins Hospital room just after Christmas in 1996, one thing was horribly clear: The child was dying. Yang, director of the Lung Transplant Program at Johns Hopkins, could see the signs. Cystic fibrosis, the lethal pulmonary disease, his patient had been born with, had ravaged her lungs. She was on a ventilator, going into kidney failure."They were having trouble blowing the carbon dioxide off her lungs," Yang recalled later. "When one spoke comes off the wheel, then the wheel starts falling apart." By Yang’s estimate, Stefanie Allen had days, perhaps hours, to live. Her only hope was a lung transplant. Yet despite months on a transplant list, there were no new lungs to be had.
Stefanie Allen’s mother, Anita Harmon, remembers that night, too, with the clarity of a nightmare. In fact, she remembers all of 1996 like that. Sitting in the living room of her Fallston, Md., home early this summer, she pointed to a picture of a rosy-faced Stefanie taken at the beginning of that year at her uncle’s wedding. Dressed in a pretty gown, mugging it up for the camera with her brother, the 11-year-old had no idea what was in store for her. Until then, she’d been lucky. Her cystic fibrosis had required few hospital visits. She was relatively active and kept up with her classmates. But when the hammer fell, it fell hard. In February 1996, Beryl Rosenstein, Hopkins’ premier CF specialist and Stefanie’s regular pediatrician, examined her and quietly told her mother that she was dying. She needed lungs, Rosenstein said. Badly.
"That’s how fast she went from being healthy to sick," Harmon remembers. "That’s why it was so shocking." That night when Anita and Dave told their daughter that she needed a lung transplant, Stefanie, who had spent her life refusing to give an inch to her disease, asked a thousand questions. Why a transplant? What does it involve? What if I don’t do it?
Tears welled in her father’s eyes. "Baby," said Dave Allen, "if you don’t do this, I’m not sure how much longer you’re gonna be here."
But where to do it was a question. Hopkins’ lung transplant program was just getting off the ground four years ago, so Anita Harmon spent that spring researching established transplant programs nearby. What she learned was devastating. In North Carolina the waiting list was more than a year, in Pittsburgh, 24 months. Then Anita’s sister, a physician’s administrative assistant, chanced upon an article about Steve Yang. It said that he’d been recruited to Johns Hopkins’ new Comprehensive Transplant Center specifically to head up the lung transplant service, and was one of the few pulmonary surgeons in the country with experience in transplanting lungs into pediatric patients.
Ask Andrew Klein why the Johns Hopkins Comprehensive Transplant Center (CTC) came into being five years ago, and Klein will immediately put on his administrative hat and reel off a list of medical economic realities—changing insurance payment structures, competition from other area transplant programs, the sizable revenue that transplant referrals generate for a hospital’s overall budget. As the center’s chief and one of its co-founders, Klein can wax long and eloquent about the "economies of scale" generated by bringing all of Hopkins’ transplant programs under one banner.
But sit with Klein for awhile and the physician beneath the administrator emerges. As surgical director of the liver transplant program, Klein can relate all too well to the major plight of transplant surgeons. Despite all the physical talent in the world, these technical wizards often lack the raw material—the donor organs—they need to save lives. Klein’s seen too many patients face death before their time because they simply didn’t have any options.
To Klein, the CTC is all about options. It’s about developing research, educational and surgical relationships among transplant specialists and creating life-saving alternatives for patients. Before the center, "everybody sort of revolved in their own sphere," he says. "We had a lot of talented people here who didn’t have a sense of what others in their field were doing. Cross-fertilizing from one transplantation area to another was a matter of simple logic, because a lot of the models we all use are very similar, no matter what organ is being transplanted."
To Anita it made sense to have Yang do Stefanie’s transplant, but she knew Stefanie had no time for wait lists. Her condition was going rapidly downhill. Rosenstein, Yang and Paul Colombani, Hopkins’ director of pediatric transplantation, met with Stefanie in early September to decide if she was a good transplant candidate. Aside from her physical condition, uppermost in Colombani’s mind was Stefanie’s psychological state. What he needed to know was whether she truly wanted to undergo this huge operation or if she was doing this for her mother.
The physicians were struck by Stefanie’s mature approach to the procedure. She voluntarily watched a lung transplant on TV to learn what to expect. They also were amazed by her zest for life. Despite being on constant oxygen and huge amounts of medication, Stefanie refused to be slowed by CF. When her younger brother, John, went bike riding with friends, she would grab her oxygen tank, run out the door and head for her bicycle. Nothing stopped her.
Stefanie was an ideal candidate for a transplant, Yang and Colombani agreed, but by now she was desperately ill. Even with her home oxygen canisters turned up to the maximum, her lungs could no longer absorb the vital gas. By Thanksgiving she was hospitalized. On Christmas Eve, when she was allowed a brief leave from the hospital, the message to her parents was clear: the doctors were sending her home so she could spend her last Christmas there. Stefanie was too weak even to open her presents. Within hours, she was readmitted to the Pediatric Intensive Care Unit, anesthetized and put on a ventilator. Her time was running out.
Then, on the night of January 3,1997, Paul Colombani was at the University of Maryland Medical Center harvesting a liver from an adult male who’d just died. At the same time, a Maryland thoracic surgeon cracked the cadaver’s chest to harvest the lungs and immediately let out a disgusted sigh. The upper lobes of both lungs were irreparably damaged from smoking with fluid-filled bubbles called bullae.
"I can’t use these lungs," muttered the Maryland surgeon, who had planned on taking the left lung for one of his patients.
Colombani saw the damaged lobes, then let his eyes drift further south.
The lower lobes were perfect.
"You can’t use that lung?" asked Colombani.
"No. I can’t justify it for my patient."
"What about the other lung?"
"Pittsburgh Medical Center’s supposed to get that. I’m supposed to take it for them."
But a few minutes and a phone call later, Pittsburgh too passed because of the bullae. The lungs were about to go into the trash.
Suddenly, Colombani turned to his colleague and announced, "I know someone who can use these lungs."
"What do you mean?"
"We’ve got a little girl who’s dying. She can’t ventilate. She’s half the size of this guy here. I think these lower lobes would fit her and we could implant them. Those lower lobes look good to you, right?"
"We’re going to do it."
What Colombani was proposing was unprecedented. He knew Steve Yang was gearing up to do living-related lung transplants, in which living adults would be able to donate part of their lower lobes to save the life of a relative with lung disease. Why not adapt the procedure for a child? It would be a similar procedure and might work. At another institution, in a more conservative atmosphere, Colombani knew this grab at life might meet with resistance. It would be considered too controversial. Let someone else go first. Follow established protocol. But this moment was exactly why Paul Colombani had joined Hopkins’ Comprehensive Transplant Center. The group fosters a sense of medical adventure. Innovation is not only welcomed but encouraged. Every one of Colombani’s colleagues reached out to moments like these, to bring transplantation to places where it hadn’t been before.
Paul Colombani rushed to a phone and paged Yang. "Steve, we have two lower lobes," he said, explaining what had just happened.
On the other end of the line, Yang contemplated turning theory into reality. "We haven’t done this before," he said slowly, knowing that while he’d done a full lung transplant involving two children, what lay before him—adapting part of an adult lung to a child’s needs—was uncharted territory.
"Well, we have two choices," retorted Colombani. "We either do it, or she’s going to die in the next 24 to 48 hours."
"You’re right," Yang said. "Let’s do it."
Moments later, Anita Harmon was stepping out of a Pizza Hut in Westminster, Md., with her son John when her beeper went off. Dave Allen had remained in the PICU with Stefanie, and the page was Dave’s number—along with 911.
Panicked, Anita reached for her cell phone and dialed Dave. She fully expected to hear that Stefanie had died. John Allen saw his mom put the phone to her ear and watched tears fill her eyes. "She’s got lungs?" Anita said in almost a whisper, then started yelling and hugging John. "SHE’S GOT LUNGS! SHE’S GOT LUNGS!"
As Stefanie’s family converged on Hopkins on that January morning three and a half years ago, Colombani and Yang met over the little girl in the OR and literally split the surgical chores. Yang separated the upper and lower lobes from the donor lungs, Colombani worked on Stephanie to remove her diseased lung. The lengthy cut-down time had both surgeons concerned about potential ischemia, the damage that can occur to transplant organs while they sit on ice awaiting implantation.
As they prepared to transplant the lobes, Stefanie was attached to a heart-lung machine—sometimes called a cardiopulmonary bypass because it literally takes over the job of putting oxygen into the blood and removing carbon dioxide—as her own damaged lungs were removed. Yang reached for the first healthy lobe and placed it gently into the gaping cavity in Stefanie’s chest. Then he connected it to her pulmonary arteries and inflated the lung. He saw with relief that it was passing oxygen into the child’s bloodstream. He could begin implanting the second lobe.
The 10-hour surgery was arduous, but in the end, it went better than anyone had expected. Early that evening, Yang burst through the door into the family waiting room with a grin. "It went pretty much the way I’d read it would," he told Anita and Dave.
But Stefanie’s toughest hours were yet to come. In the Intensive Care Unit, her new lungs refused to kick in. "We just couldn’t oxygenate her," Colombani remembers. Even with a ventilator, the transplanted lungs refused to inflate fully. The results were immediate and potentially deadly. Stefanie’s carbon dioxide levels soared, and pneumonia loomed. Colombani, examining the chest X-ray, saw that so much fluid had built up on the lungs the ribs behind them weren’t visible. All that showed was a huge white mass.
For eight hours, Colombani and pediatric anesthesiologist Dave Nichols fiddled with the vent settings and the oxygenation and pressure dials, looking for the spark that would make Stefanie’s new lungs start filling with air. Nothing worked. Putting her on the heart-lung machine to keep her breathing was out of the question. Patients on the machine require anti-clotting medication. Stefanie, who was covered with sutures, would bleed to death. After 24 hours on his feet, Paul Colombani had reached the limits of medicine. The rest was up to Stefanie’s body.
In the next eight hours she would live or die. Early the following morning the lungs began working.
Nearly four years later, Stephen Yang sits in his colleague’s office and gets very quiet. It is bring-your-child-to-work-day, and in his office next door, Yang’s daughter, Kristin, is working on the computer, replete in green hospital scrubs. Yang nods toward the office where his child waits and begins to talk softly of Stefanie Allen.
"It was a good save," he says, then thinks for another moment.
"No. It was a great save."
In the years since Stefanie Allen’s lungs took hold that night, Yang has seen the CTC take hold as well. His Living-related Adult Donor Program has shortened waiting lists, and his research into the changes in patient swallowing patterns after a lung transplant may soon apply to a wide range of disciplines, including GI work. But time and again Yang comes back to the Stefanie Allen case as a symbol of what the CTC represents.
CF still affects Stefanie’s nasal passages
and her digestion to a minor extent, but the skill and improvisation
of Yang & Co. have made the condition manageable instead
of life-threatening. "We couldn’t have done it without
the teamwork," says Yang. "Paul and I worked together
on everything on that case. I just happened to be the technician
who sewed in the new lungs."