Next May, Beth Vanstone and her teenage daughter, Madi, will participate in an eight-day trek to Machu Picchu in the Andes of Peru. It’s a fundraiser for Cystic Fibrosis (CF) Canada.
It would be a challenge for anyone; it’s all the more impressive because Madi has CF.
“I’ve always wanted to travel and hike, but I never thought that it would be realistic,” said Madi.
Cystic fibrosis impacts both lung function and digestion, resulting in difficulty in absorbing nutrients, a heightened risk of infection and respiratory problems.
Since she was a toddler, Madi has had episodes of infection, weakness and difficulty in breathing that kept her out of school and sent her to hospital. A trek in the mountains would have been impossible.
But Madi has benefited from new genetic research and drug therapies. Kalydeco, a drug developed by Vertex Chemicals, has dramatically improved her lung function from less than 70 percent, to 100 percent. She was able to graduate from elementary school, attend high school, and now can plan for a trip to Peru.
She had to fight to get the drug, though, and at one time it looked like that was a battle she couldn’t win. The Ontario government had refused to add Kalydeco to the list of covered medications, citing cost: $360,000 per year.
CF is a genetic disease that affects an estimated 4,200 Canadians. It is a degenerative disease that leads to an early death, and there is no cure. Kalydeco is not a cure, but it is an effective treatment for the three to four percent of CF sufferers, like Madi, with a specific genetic mutation.
While Madi and her family petitioned the Ontario government, and spoke eloquently in public, they also launched fundraising campaigns to help cover the cost of treatment. Vertex and private insurance paid the bulk of the annual cost, and the community rallied around the Vanstones to help make up the difference.
“It was tiring, but looking back it was really cool,” Madi said, noting many people sent both money and messages of encouragement.
She started on Kalydeco before the issue of coverage was decided, and the effect was dramatic. It transformed Madi from a kid with a chronic disease into an ambassador for Cystic Fibrosis, helping to educate the public on CF, and supporting others with rare diseases, for whom treatment might be out of reach.
“We’re not done just because Madi is doing well,” said Beth.
Her latest fight is to have the new drugs, Orkambi and Symdeko, which are proving to be effective for some with CF, to also be covered by OHIP. And rather than fight the same battle every time a new therapy comes on the scene, the Vanstones are encouraging the province to work with drug companies, and the Canadian Organization for Rare Diseases (CORD) to promote a better model for the development and certification of drugs that can provide hope to patients.
“For some, being in a holding pattern is awesome, because this is a degenerative disease,” said Beth. “There has to be a better way, a bigger picture. We’ve been working for 16 years. We are sharing our passion and trying to get out there.”
She calls Madi a “warrior.” Not only did she win the fight over Kalydeco, the Vanstones pushed the government to introduce newborn screening for CF, which has meant an earlier diagnosis and treatment for many sufferers.
Madi wasn’t diagnosed with CF until she was eight months old. “Kids that are diagnosed late are at a disadvantage,” Beth said, noting starting treatment at a later date means experiencing more damage from the disease and more infections.
Madi’s appearances in the media have had an impact; a Tottenham family only realized their two sons had CF after hearing her describe the symptoms.
Madi must take the drug and undergo treatment for the rest of her life. She also faces the possibility of setbacks – but it has made it possible to live a normal life, and participate in next year’s Machu Picchu Walk to Make CF History.
Only 30 people will be part of her group, and she is the only walker in the group with CF.
“Four years ago, Madi wouldn’t have been able to do it,” Beth noted.
Both Madi and Beth must raise $4,200 for Cystic Fibrosis Canada to participate and hope to raise another $5,000 to cover their costs. They have set up fundraising pages and are selling ball caps embroidered with the words, “just breathe” on the front, and the number 65 on the back.
Sixty-five is a reference to “sixty-five roses,” which is how some young children pronounce the difficult name of the disease. And “just breathe” is not only a reference to CF’s greatest challenge, the build-up of mucus in the lungs that can lead to infection and death, but a comment on life itself.
“It applies to everybody, and, of course, it’s personal to us,” said Beth.
Both Madi and her parents have “just breathe” tattoos.
The hats are available for $25 thanks to a contribution by the Canadian company New Era. There are other fundraisers planned in the months leading up to the May 2019 Trek.
They are also busy preparing for the challenges of hiking in Andes. “Hiking, working out, eating healthy,” said Madi, who has now signed up at a Bradford gym. “It’ll be interesting. I want to start running, just to get my lungs in shape.”
They have been getting tips from Nick Talbot, a mountaineer who has not only tackled Mt. Everest, but who has CF, as they gear up for this new challenge.
As Beth said, “You’ve got to have fighters out there willing to battle.”
If you would like to help, click on the fundraising pages:
Sponsor me on my Walk to Make Cystic Fibrosis History Page (Madi)
Sponsor me on my Walk to Make Cystic Fibrosis History Page (Beth)
