roud parents watch as 4-year-old Gavin carefully writes first his name on lined paper in marker, and then his dad's name, Jeremy, and finally, his mom's name, Joy.
Such simple things for a family of three, except for this Altoona family, nothing is taken for granted anymore. Jeremy Weyandt, Gavin's father, almost lost everything when Jeremy came as close to death as anyone could come, his doctor said. Jeremy doesn't remember anything of that harrowing time, he said.
"The last thing I remember is the nurse saying I think we need to talk about a transplant,'' Weyandt said.
The next thing he recalled is Joy whispering in his ear, telling him he had new lungs. What he didn't know, but she later told him, is that over a week had passed and he'd had a double lung transplant. Before that, he'd been hooked up to a heart-lung machine called an Extracorporeal Membrane Oxygenation machine that kept him alive for over a week while doctors searched for compatible donor lungs.
They almost ran out of time, said Dr. Joseph Pilewski, medical director for the UPMC lung transplant program in Pittsburgh. There's a limit to how long people can be on the ECMO machine and Jeremy had almost reached that limit, he said.
"It really highlights the critical need we have in this country for more organ donors,'' Pilewski said, "that we have to go to such heroic measures to keep people alive.''
Jeremy, 36, had a double lung transplant on Sept. 27, 2012, at UPMC Pittsburgh and recently celebrated a one-year anniversary of the surgery.
"That's a great sign,'' said Pilewski. "The most risk is in the first year.''
But Jeremy is not risk-free. He has a genetic disease, cystic fibrosis, which has not gone away with the surgery. His chance for survival, even with the surgery, is still about 60 percent if he makes it to five years, Pilewski said.
"There are a wide variety of outcomes,'' Pilewski said.
Cystic fibrosis is a "life-shortening'' disease, he said. The median life expectancy for someone with the disease is 37 to 38 years old.
However, that can vary to a great extent, from the low end of the spectrum, with people dying in childhood, to people living into their 70's, Pilewski said.
"It's the most common life-shortening disease among Caucasians,'' he said. "The majority of patients get transplants and they either get transplants or they die.''
UPMC was one of the first hospitals to pioneer double lung transplant surgeries, Pilewski said. The first were performed in the early 1980s at UPMC in Pittsburgh, and also in St. Louis, Mo., and Toronto, Canada. One of Pilewski's patients who had the procedure performed in 1989 is still alive but others, about 15 percent, don't make it through the first year, he said.
"The most risk is in the first year,'' said Pilewski, who described risk factors that include infection and the body's tendency to reject new organs.
People who have cystic fibrosis receive double lung transplants, rather than just one lung transplant, because of the risk that one old lung would infect the new lung, Pilewski said. Cystic fibrosis is a disease that affects about 30,000 people in the U.S. or 70,000 worldwide. It strikes predominantly Caucasians, and both males and females, Pilewski said. The disease causes the body to build up excess mucus in the lungs, making it hard to breathe, but it also affects the sinuses, liver and pancreas, he said.
Jeremy said he was diagnosed as a child and he remembers his mother learning how to do breathing treatments for him, massaging his chest to help break up mucus in his chest. He used a nebulizer, similar to what a person with asthma uses, but he was able to play some sports as a child such as hockey. Jeremy said his disease was manageable and he really didn't show too many symptoms.
That's a common scenario among people with cystic fibrosis, that they do relatively well for several years and then go into a rapid decline, he said. He has talked with fellow transplant recipients in a support group since his surgery, he said.
"I was very healthy, not really having any symptoms until that last summer when things kind of went downhill,'' he said.
He was able to work in industrial sales for several years, and Joy, as a medical secretary, understood a lot about her husband's disease. She admitted she was apprehensive when they got married about the obstacles they might face, but she was ready for the challenges.
"It was scary, it was definitely scary,'' she said. "But I loved him. I knew we'd just take it one day at a time.''
A few years into the marriage, Gavin was born, and the family was handling things as normally as possible. Joy would help her husband when he needed medications intravenously or had to stay in the hospital if he had an infection, but nothing seemed too serious. However, starting in 2012, he seemed to be in and out of the hospital more and more. He just didn't seem to be getting better, Joy said.
Then finally multiple hospital stays culminated in his system all but shutting down, and Jeremy ended up on a ventilator. The situation had turned critical. Eventually Jeremy had the transplant surgery that took about six-and-a-half hours. He was heavily sedated for several days afterward.
"I could only move my pinkie when I woke up,'' Jeremy said.
One of the first steps in his recovery was seeing his son, who thought his father had died, despite reassurances from Joy.
Gavin had to put on gloves and a paper gown to see his dad, but that was OK with the little boy, just so he could see his father. It had been so long since he'd seen him.
"He tells everybody now that his daddy has new lungs,'' Jeremy said.
Gradually, Jeremy made his way back to health through physical therapy. His recovery was hampered because the many hospital stays before the surgery had weakened his muscle tone and it took awhile to get that back, the couple said.
Jeremy has frequent tests to see if his body is rejecting his new lungs, and three times, the tests have come up positive. But if caught early, the rejection can be treated with medicine, he said. The best part of this post-surgery period is that he feels great.
"I've not been sick,'' he said, taking a deep breath. "It's nice to be home. We're looking forward to this year.''
At present, there is no cure for cystic fibrosis, but researchers are working on several drug trials. Some of the drugs treat symptoms, but others target gene mutations, Pilewski said.
"So the future looks promising,'' he said.