Cystic fibrosis (CF) is a life-threatening disease that impacts the lungs and digestive system. Most people living with CF have persistent lung infections and lose lung function over time. Now British Columbians will have access to a newly developed treatment option, Trikafta, a drug shown to dramatically improve lung function.
Abby McFee, a 30-year-old woman from Victoria, was given early access to Trikafta through the drug manufacturer’s Compassionate Care program. “I’m not an athlete or anything, but since I started Trikafta, I go for walks every day. Like going on an hour-long walk was just something that wasn’t on my radar before. It’s been like night and day.”
CF is a genetic disease that affects approximately 500 people in B.C. It largely affects a person’s lungs, but also the pancreas, liver, kidneys and intestines. Studies show adults taking Trikafta have fewer hospitalizations for intensive care, an increase in body mass index, and prolonged improvement in lung function.
"It's a very dramatic medication. It really, truly, is a game changer for patients with cystic fibrosis," explains Dr. Mark Chilvers, provincial medical director of Cystic Fibrosis Care BC (CFCBC). "There are many patients who have been assessed for a lung transplantation and when they get started on Trikafta, they've actually improved and come off the transplant list."
Trikafta is used for the treatment of cystic fibrosis in patients 12 and older who have at least one F508del mutation. It will be covered through the B.C. Expensive Drugs for Rare Diseases (EDRD) process and is expected to benefit approximately 400 British Columbians.
Using Trikafta, McFee’s lung function went from 30 per cent to 45 per cent.
“Since I started taking Trikafta, I haven’t needed the intensive care that I used to. I know technically my lungs are not like a normal person, but I feel so good, it’s wild. That’s why I feel so much more active than I used to be.
The median age of survival for people with CF in Canada has increased from four years in the 1960s to 57 years in 2019. With scientific gains made in recent years, the adult CF population is expected to grow by approximately 50 per cent over the next 10 years. PHSA’s first-in-Canada provincial program for CF, CFCBC, aims to link CF care across B.C. and the Yukon to make it easier for patients to access efficient, high-quality care from specialists across disciplines.
Adds McFee, “now that we’re living longer, CF patients definitely need more support with mental health and social areas.”
Max Creighton, from Nanaimo, agrees.
“I think a provincial program is a great idea. Having additional support, especially from nurses and specialists who understand what you’re going through, is really positive for people in B.C. and the Yukon.”
Now 24, Max’s diagnosis came when he was 22. During a bout of myocarditis in the summer of 2019, a specialist considered Max’s history of lung and intestinal health issues and connected the dots.
The diagnosis was a relief for Creighton. “I didn’t know how being well was supposed to feel. I was coughing every morning, almost to the point of throwing up, and that was normal for me.”
“Growing up I was always a good athlete, but I was never as big as my friends. So I think anything I do now I get there on my own, based on hard work, not necessarily on size. It makes me appreciate the small victories a lot more.”
Max rode over 200 kilometres for a CF fundraiser in May