Matt Pozsgai lives with the reality of cystic fibrosis every day. During the past six years, he’s spent the equivalent of 48 weeks — nearly a year — in the hospital fighting his disease.
He wasn’t alone in his fight. The Pozsgai family, who live in Salmon Creek, have spent so much time at Doernbecher Children’s Hospital in Portland that they jokingly refer to it as “Club Doernbecher” or “the club.” They play video games, card games and draw. Sometimes Matt strums his guitar.
“We try to make it feel as normal as possible,” said Matt, 21.
“It’s a family thing. It’s all of us,” said Matt’s mother, Mary Pozsgai. “He’s never going to be alone in this disease. Family time may have to be changed up a bit, but it’s the time you spend together, not where you spend it that counts.”
Cystic fibrosis is a progressive, genetic, life-shortening disease that causes persistent lung infections and, over time, limits the ability to breathe. There is no cure.
In people with cystic fibrosis, the defective gene causes a thick, buildup of mucus in the lungs, pancreas and other organs. In the lungs, the mucus clogs the airways and traps bacteria, leading to infections, extensive lung damage and eventually, respiratory failure, according to the Cystic Fibrosis Foundation.
Matt was diagnosed with the disease when he was 4 months old. A regimen of maintenance medications kept him relatively healthy. But just before his freshman year at Skyview High School, things changed.
“I colonized a nasty bacteria in my lungs,” Matt said. “I got pretty sick and ended up in the hospital.”
He was so sick his freshman year that he went to only 28 of 180 school days. He spent much of his high school years either in the hospital or recovering at home.
A difficult norm
Matt’s illness has been a learning experience for the entire family, he said. The disease requires constant treatment for him to stay healthy. When he first got really sick, he was on the couch a lot. Sometimes his parents weren’t available to help him with his treatments, so his brother and sister stepped in and learned how to mix his medications.
Every day he completes a rigorous regimen to keep his lungs clear of life-threatening mucus. He uses a nebulizer to inhale aerosol medications that open his airways, thin the mucus and fight infection.
In order for his body to absorb nutrients, he takes seven pancreatic enzyme supplement capsules every time he eats. That’s more than 28 pills daily and more than 800 every month. He also takes oral antibiotics and vitamins. He does physical therapy multiple times daily for his chest.
Because Matt’s body burns so many extra calories fighting infections, he needs 2,000 to 3,000 more calories per day than a healthy person his age. A gastrostomy tube, or G-tube, which was surgically inserted through his abdomen, delivers nutrients directly to his stomach.
Every night, Matt is hooked to an IV for 10 to 12 hours. It runs nutrition to this stomach and antibiotics and extra fat through his veins.
During his two-week stays in the hospital, Matt has clean-out treatments, including IV antibiotics to break up the mucus in his lungs. In the hospital, he does the treatments four times a day. At home, he does his treatments two to three times a day.
“This is the behind-the-scene reality of CF,” his mom said. “You do what you can to have some semblance of a normal life. Normal shouldn’t have to be this hard.”
Sibling solidarity
Toward the end of his sophomore year, Matt started going to school half days and did the rest of his work online. Matt met with a tutor regularly to keep him caught up so he could graduate on time in 2013.
Because Matt’s health prevented him from spending much time at school, he didn’t hang out a lot with his friends. Instead, he spent more time with his siblings, twins McKenna and Adam, 16, who will be juniors at Skyview in the fall.
“We became much closer after I got sick,” Matt said.
Sometimes they play music together. On a recent afternoon in their living room, Matt played guitar, Adam played a box drum and McKenna sang. Now Matt’s friends and his siblings’ friends all hang out together. They go bowling, hiking in the Columbia River Gorge or exploring in Portland, Matt said.
Often his siblings and their friends hangs out with Matt in the hospital. They’ve found green spaces on the Doernbecher campus to get outside, even when he’s hooked up to an IV pole on wheels.
Last month, McKenna traveled to Washington, D.C., to speak with members of Congress to advocate for cystic fibrosis. She was among 75 teens chosen by the Oregon chapter of the Cystic Fibrosis Foundation to be a voice for cystic fibrosis patients.
She emphasized the need for funding for the National Institutes of Health and the Food and Drug Administration. She talked about how the disease has affected her brother and her entire family.
Living with cystic fibrosis “means watching my brother get poked and prodded until his veins aren’t accessible,” she said. “He has to struggle just to maintain his health. He has to spend hours in the morning and hours at night doing treatment. It means he’s been in the hospital 24 times in six years. I’m tired of watching.”
She said she’s very close to her twin brother, Adam, “but I’ve always had a special connection with Matt. He’s always been my hero, not just a brother.”
Matt’s future
Mary and her husband, Steve Pozsgai, are among the 10 million Americans who are symptomless carriers of the gene that causes cystic fibrosis. They didn’t know they were carriers. A child is born with cystic fibrosis only if both parents have the gene mutation.
Matt is a carrier, and so is McKenna. Adam’s test was inclusive.
In 1955, children with cystic fibrosis rarely lived long enough to attend kindergarten. With medical research, new drugs have added years to the life expectancy for people with cystic fibrosis. When she was a teenager, Mary’s best friend, Annie, had cystic fibrosis. Annie died the day after her 18th birthday.
“Now life expectancy is up to almost 40,” said Mary Pozsgai. “But that’s not good enough.”
The Pozsgai family organized the first Great Strides Walk for Cystic Fibrosis in Clark County in 2000. Each May, Matt’s family and friends — who call themselves the “Matt Pack” — walk with him to raise awareness and money for cystic fibrosis research.
Six years ago, Matt’s lung function was at 100 percent, but now after so many lung infections, “we struggle to keep it in the 60 percent range,” his mom said. “Instead of a flowing river, it’s sludge,” she said of the lining of Matt’s lungs.
Some drugs being used in clinical trials correct what’s happening at the cellular level, but they don’t work on all gene mutations, Mary Pozsgai said.
“We need to continue to fight to support research,” she said. “The science is better than it was when my friend Annie was alive. I have hope that with these drugs developed and on the market, he’ll gain back some lung function one day.”
Matt is fighting his disease in his own ways. He’s created a video blog to educate people about what it’s like to have cystic fibrosis. He also writes poetry and songs about fighting his disease.
“I think that everything happens for a reason. Everyone has a purpose in life,” he said. “You don’t really know truly who you are until you’ve had some kind of adversity or struggle. Take the hand that you’re dealt and something beautiful will come of it.”
