Amish targeted over environmental problems

Once she skipped school; now she helps others thrive

PITTSBURGH (AP) — Knowing that fateful day someday would arrive, Paul McGuinness sat with his family as his doctor explained the life-and-death decision he faced. Neither option held much hope. Continue with available cystic fibrosis treatments and he'd likely be dead within a year, with round-the-clock struggles to breathe. Or get an experimental double-lung transplant at UPMC with a 10 percent chance of living five years. "The choice was to die or get a lung transplant, and, of course, I wanted to live longer," said McGuinness, who chose the transplant. "If I didn't make it, I'd help advance science." His double-lung transplant occurred Oct. 19, 1988 — 28 years ago. He not only beat the odds but also obliterated them and now is considered one of the longest surviving double-lung transplant patients with cystic fibrosis in the United States, if not the longest. "I'm still around," he said. "You can't get rid of me that easily." The 65-year-old Avalon resident spoke to the Post-Gazette recently to help celebrate UPMC's 2,000th lung transplant, which occurred on Dec. 7. The center made the announcement after Michael Keller, 32, of Bellefonte, Centre County, was discharged on Jan. 3 from UPMC Presbyterian. Also requiring a double-lung transplant due to cystic fibrosis, Keller said he's now going on long walks and feeling well. Soon after the transplant, UPMC transplant doctors dubbed him "Mr. 2000." "I'm amazed how quickly things started to improve," he said. "Breathing is much clearer, with no crackles or wheezing. I'm able to finish full sentences. I listened through a stethoscope, and it was a very emotional moment." Jonathan D'Cunha, UPMC's surgical director for lung transplantation at UPMC Presbyterian, said reaching the 2,000-transplant mark — and being the first transplant center nationwide to do so — means that "when you come here, there is nothing we haven't seen at this point. We use a team-based approach to navigate and get the best outcomes for the most complex patients." Lung transplants remain the most fragile of all organ transplants, but it's improving with a 90-plus percent rate of survival after one year and up to 60 percent of all patients surviving five years. On the contrary, consider that patients like McGuinness likely wouldn't have survived a year without transplantation. Cystic fibrosis is an inherited disease of the secretory glands that make mucus and sweat, according to the National Institutes of Health, resulting in thick, sticky mucus that fills the lungs. The condition, however, doesn't affect the transplanted lungs. "One of the greatest things we do in medicine and surgery is giving breath again," Dr. D'Cunha said. "These are patients carrying oxygen tanks — and not the person at the grocery store with an oxygen tank, but people who can't leave the house or even get up without turning blue. They are very sick patients, and transplantation gives them hope for the future." The United Network for Organ Sharing, which manages organ procurement and advances organ availability, among other roles in organ transplantation, congratulated UPMC and provided statistics showing it has done more lung transplants than any other center in the United States. From 1988 through 2016, UPMC did 1,874 lung transplants, but those don't include transplants prior to 1988, which helps explain the difference in UNOS vs. UPMC numbers. Dr. D'Cunha also said UPMC's total includes 130 heart-lung transplants, which traditionally are included in lung-transplant totals but aren't included in UNOS totals, explaining the 2,000 milestone. Allegheny General Hospital has done 17 lung transplants since 1988 but none in recent years, the records show. Through 2016, Duke University Hospital is second with 1,704 total lung transplants, with the Cleveland Clinic Foundation having performed 1,546 transplants. In recent years, various centers nationwide have done more annual lung transplants than UPMC, but its legacy is assured. "This is a wonderful milestone to celebrate. It may help provide hope to people waiting for a lung transplant to hear that one center has surpassed the 2,000 mark and that more than 33,000 lung transplants have been performed in the United States since 1988," said Anne Paschke, UNOS spokeswoman. Nearly three decades ago, McGuinness' decision to undergo the lung transplant was a media event, with television stations showing him being pushed into UPMC Presbyterian in his wheelchair and with an oxygen tank. A Post-Gazette story said he "was shaking and scared" but gave a thumbs-up when wheeled into the operating room to become the third person at UPMC to receive a double-lung transplant. (The first two have since died.) A family member told reporters he wouldn't have lasted another six months without new lungs. And he's still going strong with twice-daily workouts on a stationary bicycle or Spinning classes at the North Boroughs YMCA in Bellevue and long walks in parks. "He's the kind of patient we champion and brag about to other patients about what can happen," Dr. D'Cunha said. "It's not out of the question, if you take your immunosuppressant drugs, see your transplant doctor and get a good set of lungs. There is no reason why you cannot live 10 to 15 years." McGuinness said he's paying his benefits forward by advocating for donor awareness while recommending "a lot of exercise and clean living." "Anything I can do to promote organ donations, I'm up for it," he said. "I like to help as many people as I can. It's not about me but future organ donations and future transplants." Keller, the 2,000th patient, said he's hoping to return to his Penn State University position in communications and human resources within a month and is thankful that people like McGuinness blazed the trail and helped develop procedures for lung transplantation. "Without him, who knows what level of success we'd have now," he said, noting he had a choice of transplant centers. "The fact that UPMC is so experienced, and I knew they were good at it, was the deciding factor to go there." ___ Online: http://bit.ly/2js3ZHf ___ Information from: Pittsburgh Post-Gazette, http://www.post-gazette.com

UPMC celebrates 2,000th lung transplant, 28-year survivor