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HELPERS: Daughter's cystic fibrosis diagnosis turns into a lifetime of patient advocacy for local mom

'I’m fighting hard for access and have been for the last 10 years and I will continue to fight to make change ,' says local mom, advocate for cystic fibrosis patients

Local resident Beth Vanstone and her family had their lives turned upside down when they discovered their youngest daughter had Cystic Fibrosis (CF) at the tender age of eight months old; a diagnosis which led Vanstone into a world advocacy for Cystic Fibrosis Canada.

Originally from Bradford, Vanstone has resided in Beeton for the past 10 years with her husband of 25 years, Glenn, and three daughters; Jessica (Jessie), Madi, and Carly. 

When their youngest daughter Madi was diagnosed with CF in 2002, Vanstone says it changed the family’s entire life as they desperately looked for ways to combat the disease while learning more about Cystic Fibrosis.

“It was really hard,” said Vanstone. “It’s a chronic disease that’s genetic. Both my husband and I passed down a defective gene we were unaware of – we had no idea anyone in our family had it!”

One in 25 people carry the defective gene and it is recessive; it takes two parents to pass down the defective gene and one in every four pregnancies could end up with CF if both parents carry the gene.

Today, the life expectancy of those who live to adulthood with Cystic Fibrosis is approximately 44 years old.

“It attacks your whole body,” explains Vanstone. “The lungs generally have the most problems. Lots of people have double-lung transplants in their teens or early twenties. It attacks your digestive system and pancreas too.”

When Madi was first diagnosed, her pancreas did not work at the time, so they had to give her enzymes to help her digest food. And as the list of medical issues began to rise, Vanstone had no choice but to give up her career as a financial planner so she could take care of her daughter who would endure a lifetime of hospital visits and endless checkups.

“It’s a horrible disease,” she says. “Immediately, I learned how to be part of a solution for the CF community. We started doing the CF walk every year… then I started holding my own events.”

Vanstone shares she and her daughters organized several walk-a-thons in support of CF while trying to bring more awareness about the disease.

In 2019, Vanstone and Madi held a fundraiser for Cystic Fibrosis Canada and planned a trip to China to walk the ‘Great Wall’ through an organization called Charity Challenge Canada. The mother-daughter duo raised a whopping $32,000 for the cause and spent five days hiking the famous wall together.

“They took us to different parts of the wall that were all crumbled… we would spend seven or eight hours every day for five days going up and down different sections – I think it killed my knees!” she recalls about the charity trip.

Over the years, Vanstone allowed Madi to participate in trial drug studies performed at Sick Kids Hospital to try and alleviate any of the symptoms that were preventing her from living a normal life.

She shares that when a ‘gene modulator’ became available to try on CF patients, it was amazing and something they had been waiting for.

“When we started this drug, her lung function went from 73 per cent to 115 per cent in a matter of days, and she gained 15 pounds too,” she shares.

The drug was called Kalydeco, and as excited as the family was to see a new medication finally working for Madi’s condition, that excitement was quickly diminished when funding for the drug became an issue.  Despite the drug being approved in Canada, no drug companies would fund it.

Once the trial stopped, Madi lost access to the drug that was keeping her alive, which would cost $350,000 per year to stay on it.

“That’s a lot of cupcakes to sell," she said. “I ended up calling my insurance company and begged for them to help. They agreed to pay 50 per cent... and the manufacturer gave us 30 per cent off. So, if we raised $6,000 a month, Madi could live normally.”

Vanstone reached out to her community for help and shares that residents from Bradford, Beeton, and people across Canada began fundraising to help keep Madi on the drug treatment, but Vanstone knew this wasn’t sustainable.

So, she started advocating for access and provincial coverage of the drug and met with MPP Jim Wilson, Minister of Health MPP Christine Elliot, and then-premier Kathleen Wynne. 

“We were at Queen's Park a number of times… eventually, the drug was covered for Madi and others who could benefit from the drug,” she says. “But that particular drug was only effective in three to four per cent of the cystic fibrosis population because there are over 2000 faulty genetic mutations, so only people who had certain mutations would benefit from that drug – that left 96 per cent of the population without a modulator (drug).”

Vanstone began a decade-long fight to gain better access to more life-saving drugs for cystic fibrosis patients and continued to advocate for different drugs that were approved but sitting on a shelf unattainable due to costs.  

Finally, a new drug called Trikafta was tested and proven to help those other 96 per cent of the CF population – a drug that was approved and funded in the United States in 90 days.  But once it arrived in Canada, funding issues came up.

“This was like the golden goose,” she describes. “The US (CF patients) are thriving thanks to Trikafta… people were regaining their lives again!”

Vanstone wanted that same lifestyle for her daughter so she again continued to advocate with local politicians and petitioned the government for a change in how the system approves these drugs.

“In Canada, we have such challenging regulations and pricing issues,” she says. “I’m pushing for quick advocacy and trying to be loud in the CF community – this is about trying to get access to drugs that will save lives; people are dying because of a lack of access, so I will continue to fight!”

Currently, Trikafta has been approved by Health Canada for Canadians aged 12 and up with cystic fibrosis, however, it is not yet accessible to those who need it until it is funded by provincial drug plans and private insurers.

“There is a whole community fighting this – I am one of many – I’m fighting hard for access and have been for the last 10 years and I will continue to fight to make change – all patients should have access to this drug!” she says.

A film crew from the US recently reached out to Vanstone and Madi about their journey with CF and contracted videographers from Toronto to interview them next month about the differences in accessing drugs in Canada versus the United States.

“If people realize how much of a difference they can make just by standing up and putting their hands out… the value they could bring is so rewarding,” she concludes.

Today, Madi is 19 years old and currently dealing with water on her lungs. She was admitted to the hospital last month but continues to keep her head up and never lets the disease own her.