Cystic fibrosis drug gives young Yukoner hope for a future

Avery Kearns lives with cystic fibrosis. She said the new drug she's on has allowed her to
Avery Kearns lives with cystic fibrosis. She said the new drug she's on has allowed her to

16-year-old Avery Kearns is looking forward to the future. She wouldn't have said that a year ago.

Kearns lives in Watson Lake, Yukon, and was born with cystic fibrosis (CF).

According to the Cystic Fibrosis Foundation, "cystic fibrosis is a progressive, genetic disease that affects the lungs, pancreas, and other organs."

As Kearns explained, "it's where cells don't work as normal as the average person's cells. Because of this problem there's a whole bunch of mucus build up in my body which is clogging my organs causing them to not work as well and eventually, overtime, shut down."

"It's a disease that affects the lungs and the digestive system but affects my whole body," she said. "It affects me everyday."

Kearns said she grew up believing she wasn't going to live a long life.

"CF is progressive meaning it's going to get worse over time," she said. "The future was never something I thought about and my parents would always tell me 'No you're going to live a long life,' but I kind of never really got to see any of that."

That was until she started taking Trikafta — a costly but groundbreaking drug for people living with cystic fibrosis. Kearns said it's changed her life.

"With ... Trikafta I'm able to imagine my life in ways that used to sound impossible," she said. "In a way I could say I was living to do my treatment rather than doing my treatment to live."

Cystic Fibrosis Canada/Handout/The Canadian Press
Cystic Fibrosis Canada/Handout/The Canadian Press

Last year, the Yukon government announced it would cover the drug, which costs roughly $300,000 a year at its list price under publicly-funded drug programs.

Kearns said there is no cystic fibrosis clinic in the Yukon so her treatment entails going to the BC Children's Hospital in Vancouver every 10 weeks.

There she meets with a long list of medical professionals that she calls her CF team.

"We'll talk with the doctors, nurses, pharmacists, dietitians," Kearns said. "We'll talk to discuss how my health has been and what needs to be improved or worked on in terms of how I'm doing."

Then there's the tests.

"There's the pulmonary function test which show how well my lungs are working. Lots of lung exercises. Blood work. ECG. There's so many tests."

Kearns said she has only been on Trikafta for nine months, and as her health improves she hopes things will start to get easier for her.

"For example I have a feeding tube because with CF it's very hard to gain weight. You don't absorb the nutrition from food and so there's been discussion on taking out my feeding tube, whether or not I need to take certain pills anymore or my respiratory therapy, if I still need to do that."

Kearns said that she knows Trikafta isn't a cure, but she isn't going to let CF get in the way of her future plans.

"I'm at that age now where I have to start applying for school and stuff," she said."It's stressing me out because I've never had to think about it. I didn't think it was an option. I'm definitely excited to go away to school and try new things. I really want to go to school in Victoria."

"The journey through CF and Trikafta coming with it has definitely given me a different perspective on life and I think it's beneficial compared to the ones that don't have as many struggles. If that makes sense."