This is an excerpt of a story that originally appeared in the Spring 2013 issue of Alpha-1-To-One Magazine.
Mary Pierce was diagnosed with Alpha-1 on her 40th birthday, Feb. 16, 1987.
She’d been home sick from work for about a week when she got a call from her new primary care doctor who ran a small practice in the little town of St. Joseph, Michigan.
Both her parents were smokers, and she began smoking when she was about 16. She had managed to quit a few months before she saw the doctor. Her father had died of lung disease and her mother was very sick and short of breath; she thought that most likely, she faced a similar future. “I knew the damage was done. I was ashamed. I believed I had done this to myself. My motive was to prevent anybody I worked with from knowing about it.”
At the time, she was working long hours setting up a self-insurance plan for a major manufacturer of heavy construction equipment. She spent a lot of time in airports, much of it finding excuses for why she couldn’t keep up with her healthy young co-workers. They didn’t know about her COPD. “They just thought I had a bad bladder.”
But her new doctor, Anthony Salvagione, MD, wondered why she’d been getting sick so often. He had asked a lot of family history questions. And he decided to test her for this rare condition he’d heard about. When Pierce took the call on her kitchen phone, he told her four things she would never forget:
- She had severe COPD.
- She had Alpha-1 Antitrypsin Deficiency.
- A new treatment for Alpha-1 was undergoing clinical trial.
- And successful lung transplants had recently been accomplished.
The news made her truly hopeful, for the first time in years. “He told me what was wrong. And he told me treatment was available. The enemy had a name now; I had a sense of relief. And he nudged me toward the idea that I could do something about it.”
DOING SOMETHING ABOUT IT
Doing something often meant running into discouragement. For example, she followed up the conversation with Salvagione by going to a pulmonary specialist.
Who told her, “You have severe COPD caused by a rare, degenerative, ultimately fatal lung disease and there’s nothing anyone can do.”
She and her husband Todd – “We did everything together that involved my healthcare” – gave up on the doctor, but not on the idea that she could improve her health with exercise and nutrition. Todd reminded her of an article he’d read about a nurse who worked with her own father, a COPD patient, and helped him exercise and achieve much better breathing and quality of life.
Todd and Mary Pierce
She and Todd decided to find a pulmonary physician who would work with her to help her live the healthiest life possible with her condition. And they found one: Geoffrey Grambau, MD, of Kalamazoo, Michigan, about an hour’s drive away.
Mary is 5-6. She had lost weight gradually as her breathing deteriorated in her 30s, and eventually dropped to just 99 pounds. “Friends and co-workers thought I was anorexic,” she says.
There was very little patient information available about Alpha-1 in 1987. But she was willing to do her own research, and very fortunate: she had access to her own company’s computer system. Her searches brought up many results, all in technical medical language. “It took us forever to understand them.”
But she and Todd began to learn and to get help.
She tried to get into the National Heart, Lung and Blood Institute (NHLBI) trial of augmentation therapy that her doctor had mentioned, but didn’t qualify because her lung function was so poor.
The commonly quoted measure of lung function is FEV1 – the amount of air you can blow through a tube in one second. Lung function is measured in percentages: for FEV1, it’s how much air you can blow out in that one second, compared to the predicted amount for someone your age, sex and size. Her score of just 14% FEV1 on pulmonary function tests was too low.
Mary just before her transplant
She believed that proper nutrition could help her, and by eating a lot of healthful fats, she regained some weight. She began to work with a group of physicians in New Jersey who were doing total parenteral nutrition (TPN) with children who had Cystic Fibrosis and people with severe lung disease who were on ventilators for long periods and couldn’t eat.
The doctors implanted an intravenous infusion port in her chest and gave her high-calorie, high fat, anti-inflammatory nutrients, at first daily, then less often as her weight stabilized, and eventually weaned her off the TPN entirely.
AT HOME WITH THE ALPHAS
In 1988, she was able to quit her job and go on disability, keeping much of her income and excellent health insurance. She was able to get augmentation therapy, now FDA-approved and on the commercial market. She was able to self-infuse through the same port she’d gotten the TPN.
Mary and Todd heard about a meeting to organize a national Alpha-1 Association in Minnesota, and they drove 500 miles to attend.
“I walked into the room and saw a bunch of people hunched over, breathing with their shoulders up to their ears, and felt immediately at home. Sandy Sandhaus, Jamie Stoller and Jim Gadek were walking around talking to people. It was wonderful.”
Now they had great resources. They knew some “Alpha docs,” real experts; they knew other Alphas with similar experiences; they had a treasure chest of information in Sandy Brandley, executive director of the new Alpha-1 Association; and they were getting the first Alpha-1 newsletters from Peter Smith.
And Mary was willing to work hard. With exercise, good diet and a strong response to bronchodilators, she was able to bring her feeble 14% FEV1 up to 17-18%. But that was as good as it got.
By 1993, she was on a lung transplant list, living attached by a 25-foot hose to a huge oxygen tank 24 hours a day. The family decided to clean out a downstairs room for a bedroom when she wasn’t able to climb the stairs anymore.
Their son Brandon recalls the day they ordered a wheelchair – a difficult thing for Mary to accept. That evening they were watching Star Trek on TV when they got The Call. A lung donor had died. “The first assignment she tasked me with was to make sure Star Trek got taped, so she could watch it after her surgery,” Brandon says.
She got her lung transplant at the University of Michigan on April 3, 1993. She was supposed to get a single lung, but the patient who had been intended to get the other one was in poor condition and the doctors didn’t believe he could survive transplant. So she got both lungs. The lung donor was a 27-year-old mother.
Mary wins gold in the World Transplant Games
She had always been athletic before she got sick. Now she began to train hard, lifting weighs and riding a bike. In 1994 she won a bronze medal in cycling at the U.S. Transplant Games; in 1995 she won the gold at the World Transplant Games in Manchester, England. In 1995, to leverage the publicity she and other Alpha athletes generated to promote awareness and raise funds for Alpha-1 research, she founded Team Alpha, Now called Team Alpha-1, it is still a program of the Alpha-1 Foundation.
Within a few years she became a well-known organ transplant athlete. She participated in the American Lung Association’s Big Ride Across America, from Seattle to Washington, DC; she carried the Olympic Torch she appeared twice on Good Morning America, did radio and TV interviews and became a public speaker.
“She had to work to develop a public persona,” says Todd. “Her comfort zone is not in front of crowds, but far away from them. The respect or adulation or whatever is simply not what drives her. Helping other Alphas is what drives her.”
Read the rest of her story in the Spring 2013 issue of Alpha-1-To-One Magazine on page 10.
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