By Meredith Cohn, The Baltimore Sun
7:18 PM EDT, April 26, 2012
For the first time in the six years since Victoria Chakwin was diagnosed with a deadly lung disease, the gown she wears won't be hospital issue.
The 18-year-old from Martinsburg, W.Va., will go to her senior prom Saturday night in a red-and-black number she found on the Internet.
A rite of passage for most teens, the event is more momentous for Victoria — who's known as Tori — because people diagnosed with pulmonary fibrosis generally live only three to five years.
That she is headed to her prom demonstrates not only the possibilities of modern medicine but the will of the teen and her mother, according to Tori's doctors at the University of Maryland Medical Center, who in late January replaced her scarred, dysfunctional lungs with a donor set.
"We can do a lot with technology, if we're not afraid to use it," said Dr. Aldo T. Iacono, medical director of Maryland's lung transplant program, one of the few in the country that will transplant scarce organs into someone so sick.
He approved the Jan. 30 surgery after another area transplant center took her off the waiting list because, he said: "We sensed an extreme willingness to live and a lot of family support."
After a checkup on Thursday, less than three weeks after she was released from a rehabilitation hospital, Iacono declared Tori a normal teen — lip ring, blue nail polish, red hair and all.
Why Tori developed pulmonary fibrosis is a mystery. The disease affects up to 200,000 Americans and kills about 40,000 a year, but most patients are decades older than Tori, according to the Pulmonary Fibrosis Foundation.
Diagnosing the disease can be difficult, and indeed Tori was told she was asthmatic, allergic or seeking attention before doctors at a Washington hospital realized she was seriously ill.
Government research shows about 10 percent of sufferers have a genetic predisposition to the disease, and other cases have been linked to such disorders as lupus and rheumatoid arthritis or to environmental contaminants such as asbestos or coal dust.
Doctors believe the scarring, or fibrosis, is an inflammatory response to microscopic injury. The lungs become so damaged they can't properly pass oxygen into the bloodstream. Corticosteroids are used to try to stem the inflammation and scarring, but not everyone responds to the treatment.
Transplants are considered the only long-term means of survival, but many patients don't qualify because they have other health conditions or are too old. Some die while waiting on the donor list, doctors say.
Tori was put on a transplant list at another hospital on Jan. 5 but was removed a day later when she suffered respiratory failure. But her mother, Robyn Schonhans, a retired New York City police officer, would not accept that they were going to give her 10 days "to go to sleep."
"I said, 'The hell you will!'" said Schonhans, pushing doctors, who found her a place at Maryland, where she was transferred in mid-January.
She went into respiratory failure several more times, her ventilator no longer able to push enough oxygen into her lungs. Doctors hooked her blood vessels directly to a machine — known as ECMO, for extracorporeal membrane oxygenation — that removes carbon dioxide and infuses oxygen, the tasks normally performed by the lungs.
Tori was unconscious and clinging to life two weeks later when an anonymous donor was found and she was taken into surgery by Dr. Bartley P. Griffith, a chief of University of Maryland Medical Center's division of cardiac surgery and director of heart and lung transplantation.
The surgery was complicated because Tori's immobilized body had deteriorated, she was on blood thinners and the donor lungs were too big. Griffith said he had to remove the old lungs, reduced to the size of teacups, and sew in as much new lung tissue as he could. Her wound was left open and she returned to the operating room three or four more times to complete a little more of the operation as her chest expanded.
By March 1, her 18th birthday, her surgeries were behind her and she was sitting up and enjoying some frosting from her red velvet cake. Physical and occupational therapy helped her advance from a wheelchair to a walker and now to a cane.
"I was really pushing it," she said.
The outcome is remarkable, say doctors familiar with the disease, including Dr. Norman H. Edelman, chief medical officer for the American Lung Association and a lung specialist at Stony Brook University Medical Center.
The disease is growing in prevalence, he said, perhaps because people are living longer or because they are exposed to more contaminants. It's still uncommon in children.
Patients are all treated the same way, with corticosteroids and immunosuppressants. Those who qualify go on the transplant list.
"But there are far more people who need a lung transplants than there are lungs," Edelman said. "Centers are very picky."
Edelman said he has a patient who was a good candidate for a transplant but was rejected because he had no family nearby to support him.
The survival rate five years after a lung transplant is 70 percent, and after 10 years it's more than half. There are no separate statistics for children because so few have had lung transplants.
Tori, he said, "will be someone to watch."
Griffith, Tori's transplant surgeon, will be watching with hundreds of others who helped care for the teen.
University of Maryland Medical Center performed 375 lung transplants in 2011, more than any other hospital in the state, according to data from the federal Organ Procurement and Transplant Network. Just four were performed on children.
"There is a small but needy group of young people," Griffith said. "We have to make the decision if they are too sick to benefit from a transplant, and few centers will take someone once he's been on ECMO because the outcomes are well known to be not as good."
Tori, he said, is cured of pulmonary fibrosis, though she now will face regular monitoring and a lifetime of anti-rejection drugs.
"The proof is in the pudding," he said. "She has a prom dress, and she looks marvelous."
Tori said she's ready with her dress and red Chuck Taylor sneakers. Her mother is throwing a barbecue before the dance for some friends and family. Her escorts will be her two best girlfriends, Caylee and Breanne.
She hasn't been to her high school in more than a year and is a little overwhelmed at the thought of seeing her hundreds of classmates. She hopes they won't make a fuss over her.
"I'm going to stay as long as I can handle," she said.
Tori kept up with her studies and will graduate with her class, though she plans to go camping instead of attending the ceremony. She's become a bit shy from years of feeling a little different.
She hid her illness, and long ago gave up on dances, sports and cheerleading, which took too much of her breath away.
She plans to take a year off and travel before going to college for a degree in psychology. She hopes to personally thank the family from Tampa, Fla., who donated her new lungs.
"I finally have the ability to make plans for the future," she said. "Amazing."
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