Jim Best, a 34-year-old Canadian pharmacist with cystic fibrosis, is also a talented photographer who rides his motorcycle across the country every year to raise funds for CF research.
In addition to generating funds, the trips help the Sackville, Nova Scotia, resident spread awareness about the incurable disease that affects him but does not define his life.
Marie-Pier Emery, a Montréal lawyer who specializes in labor and employment issues, has CF as well. She’s 26. At one point doctors said she would live only until she was 20.
Emery struggles through daily medical treatments, and takes two dozen pills a day. But she still finds time to cook with her husband and do volunteer work on weekends.
Another attorney, Chris MacLeod of Toronto, was diagnosed with CF at the age of 2. Now 44, he credits a breakthrough therapy called Kalydeco, developed by Vertex Pharmaceuticals, for saving his life. And he’s going to court to try to help uninsured CF patients in Canada obtain access to the $300,000-a-year drug.
Best, Emery and MacLeod feature prominently in a holiday fundraising campaign that Cystic Fibrosis Canada (CFC) has launched. The campaign, “31 Reasons to Give,” tells 31 patients’ stories — one for each day in December. The idea is to offer a more personal reason to support CFC.
Morgan Davidson is senior manager of digital marketing at CFC, which has raised $244 million for research since it was established in 1960.
She said the goal of the holiday fundraiser is to exceed $60,000, the amount CFC generated in last year’s campaign.
“We’ve been really focused these last couple of years on trying to reach newer audiences in Canada,” Davidson told Cystic Fibrosis News Today in a telephone interview. “Only one in 25 Canadians carry the gene, so most people have no idea what CF is until it impacts them.”
Davidson said the campaign’s strategy is to focus less on the medical complexities of the disease, and more on how CF affect daily living — an approach that healthy Canadians can relate to.
“These impacts are as simple as interrupting play time with your siblings, or not being able to make it to your soccer practice, or not being able to plan your wedding because you’re waiting for a lung transplant,” she explained. “CF patients have lives like everyone else, but they also have to balance those lives with treatments and medications. We wanted to highlight the way those living with CF touch the lives of others, and how they’re changing the world.”
At last count, 4,246 Canadians are living with the disease, says the CFC, which bills itself as “one of the world’s top three charitable organizations committed to finding a cure or control for cystic fibrosis.” The figure includes 122 patients diagnosed in 2016.
CFC has 81 staff, about 25 at its Toronto headquarters and the others at regional offices in Vancouver, Winnipeg, Ottawa, Montréal and Halifax.
Sixty-one percent of Canadians with CF are adults, and 89 percent have the F508del genetic mutation that also accounts for most cases in the United States. Fifty-nine percent of Canadian patients were diagnosed by their first birthday. Adults diagnosed after the age of 18 account for only 7 percent of patients.
The largest number of CF patients live in Ontario — 1,473. Québec has 1,198, Alberta 587 and British Columbia 424, according to the Canadian Cystic Fibrosis Registry’s 2016 annual data report.
But the provinces with the biggest increases in CF growth rates in the past 20 years have been Manitoba, where cases were up 77 percent; Saskatchewan, 59 percent; and New Brunswick, 41 percent.
“Our campaign features people from across the country — from Nova Scotia and Québec to Ontario and Alberta,” Davidson said. “We don’t have a lot of people who live in the far north or in abnormal climates. Living with CF in Canada is very similar to living with CF in the States. The difference in Canada is our healthcare system.”
Longevity statistics seem to bear that out. CF patients in Canada live a median of 53.3 years — at least a decade longer than in the United States. One reason, a study asserts, is that 10.3 percent of Canadian patients have received lung transplants, compared with 6.5 percent of American patients.
The research, “Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States: A Population-Based Cohort Study,” was published in the journal Annals of Internal Medicine.
A step that has helped a lot is that most Canadian provinces now screen newborns for CF. When Alberta joined the program in 2007, fewer than 10 percent of all CF diagnoses came from newborn screening. The figure is 40 percent now.
The increase reflects the fact that most of the provinces screen newborns for the disease these days. Québec will add CF screening in 2018.
Another sign of Canada’s commitment to CF patients is that the country boasts 42 specialized clinics for the disease.
A symbol of the investments’ payoffs is that the oldest Canadian with CF is nearly 80 years old.
In 2016-17, CFC awarded $5 million to 52 studies, 18 fellows and students, and eight targeted research programs and scholarships. This includes $1.9 million for 19 studies dealing with the infections that many patients experience. The studies’ topics range from antibiotic resistance to how to predict lung infection flare-ups.
Despite the advances, 85 percent of Canadians with CF must take pancreatic enzymes to digest food and absorb nutrients, and 23 percent have CF-related diabetes.