CF affects lungs and digestive system
It affects mostly the lungs and digestive system, causing symptoms such as a persistent cough with thick mucous, wheezing and shortness of breath, frequent chest infections, bowel disturbances and weight loss.
Cystic fibrosis is a genetic disease that occurs when a child inherits two abnormal genes, one from each parent.
About one in every 25 Canadians carries an abnormal version of this gene. Nathan and Lesley are carriers, but do not have cystic fibrosis themselves.
When two carriers have a child, though, there is a 25 per cent chance that child will have CF.
There’s a 50 per cent chance the child will be a carrier. And a 25 per cent chance he or she will be neither a carrier or have CF.
Casey likens his family to a legendary Pokemon. (A group of incredibly rare and often very powerful albeit fictional characters in the world of Pokemon, for the uninitiated.)
“I think it’s better to think positive than negative,” he says.
The Chown family was one of several people to join runner Emily Allan on the final leg of her 895-kilometre adventure to run the entire length of the Bruce Trail in 22 days. She finished by arriving at the stone cairn in Queenston Heights Park on Canada Day and raised some $28,000 for Cystic Fibrosis Canada.
Emily ’s cousin, Sara Bloomfield, lived with CF and died in 2005 at age 23.
Casey and Lesley ran the final seven kilometres alongside Emily. Riley, who also lives with autism and does not talk, walked with her father.
Afterwards, Nathan wrote an emotional post to Facebook, in appreciation of Emily’s efforts to raise awareness of a disease that is part of their lives every day. “We put everything we have to maximizing our children’s health and are just plain drained most of the time to fight CF on other important fronts, as you guys are so aptly doing,” he wrote.
“It’s about knowing we’re not alone,” says Nathan. “Sometimes you do think you’re in a little bubble.” “It’s a lot of hope,” says Lesley. Keeping their kids healthy takes a lot of work, even though Nathan says it has become their normal.
Morning and night, both Casey and Riley undergo therapies designed to loosen built-up mucus in their lungs to make it easier to cough up. It involves breathing in medications through a mask, repeated banging on their chest and back with a special cup-like device, and for Casey, blowing air into another mask.
“It’s like lifting weights for your lungs,” says Nathan.
The thick, sticky mucus makes their lungs more susceptible to bacterial growth, and chest infections. In fact, CF Canada recommends that people with CF avoid close contact with other people who have CF.
They also take digestive enzyme pills with every meal. The enzymes are produced in the pancreas, but the small channels through which they flow become blocked with mucus.
Vacations and day trips take extra planning.
Physical activity is also part of their therapy. It improves lung function and can lead to fewer hospitalizations. It can also help clear airways and reduce stress.
Staying active isn’t a problem. Nathan and Lesley are members of the St. Catharines Cycling Club, and can easily clock a 100-km ride. The club supports the family by donating funds and displaying a CF Canada logo on its jersey. And Nathan rides with Team CF, a group of competitive cyclists committed to the development of a cure for the disease. They use cycling as a platform to increase awareness of CF, raise money for research and promote fitness for people with the disease.
Meanwhile, Lesley competes in triathlons. She does it for fitness and mental health.
As much as they try to live in the moment, there can be a lot to worry about when you’re a parent of two children with CF.
They worry about colds, germs and bacteria growing in lungs. They worry about weight gain. And about compliance with therapies as their children grow older. Casey is very independent and has taken on a lot of responsibility for remembering to take his pills and keeping himself healthy. Riley, because she lives with autism, will likely always need help.
“We hope for a cure. That would be amazing,” says Lesley.
There are many unknowns. And that can be scary.
“We’ll always be there for them,” says Nathan.
“We hope they’ll have a good life,” says Leslie. “That they’ll stay healthy. And that their health won’t be a barrier to them doing things.”
Eight-year-old Riley has climbed up onto a pile of dirt, then onto the lid of a garden composter, then onto the top edge of a wooden fence in her backyard.
There she balances, smiling.
“She’s good at balancing,” calmly notes her 11-year-old brother, Casey, who confides that he doesn’t possess his sister’s sense of equilibrium.
Next thing, she’s down off the fence, running circles around a backyard pool. She climbs up a small step ladder at the side of the above-ground pool and dips her fingers in the cool water. It was just filled a couple of days ago, from the tap. And it’s cold. She wants to jump in. “Oh, just let her,” relents her dad, Nathan Chown.
In she goes, splashing, dunking under the surface multiple times as piercing giggles burst through the air. Both kids love the water. Casey has just returned home from a swim practice. This year, he was a mere half-second off qualifying for a Niagara regional meet in the 50-metre freestyle. Through the summer he swims six days a week.
Indeed, if anyone were to notice the activity in their backyard, the assumption would be regular kids at play. Casey explains: “You can know me all my life and think I’m a normal person,” he says.
“I’m this normal kid that has to have a mask on my face and has to have pills.
“In my opinion, it’s just more sticky stuff in my body.”
Both Casey and Riley live with cystic fibrosis. According to Cystic Fibrosis Canada, it’s the most common fatal genetic disease affecting Canadian children and young adults. At present, there is no cure.