Our sister site BradfordToday recently shared an update on cystic fibrosis (CF) patient Madi Vanstone and her mother Beth and their fight for orphan drugs in Canada.
This week, we sat down with another local CF patient, 17-year-old Victoria Vigneau and her mother Carol, to hear their story and fight for coverage on CF drug, Orkambi.
Orkambi, made by Vertex Pharmaceuticals, was approved for market in Canada as of January 2016, but access to the drug for many Canadians has been very limited due to its price tag, a whopping $250,000 a year.
Neither Carol nor her husband’s insurance benefits cover the cost of the medication for their daughter.
“There are a couple people I know whose kids are on it, but there aren’t that many because for a lot of us, our insurance companies don’t cover it,” Carol explained.
Victoria was diagnosed with CF at 18 months old. Her mother knew something wasn't right after her daughter kept coming down with colds almost every week, causing her to lose a lot of weight.
“She had six months of colds, antibiotics every week,” remembered Carol.
The family doctor was convinced that the colds were being brought home by Victoria's three older brothers, all of whom were at school.
Carol knew a friend-of-a-friend in British Columbia who had CF and suggested Victoria go for a sweat test after learning about her symptoms.Sweat tests are used to diagnose CF. Victoria tested positive in May 2004.
She was called back for a retest where the diagnosis was confirmed: she did have CF.
Carol said she had never known anything about CF prior to her daughter’s diagnosis.
“I was disheartened. She's my baby,” said Carol of that devastating moment.
After the diagnosis, Victoria was back at the hospital for two weeks, to get her weight back up to normal. Currently, Victoria takes 11 enzyme medications a day, seven with a meal and four for a snack.
“It helps me digest my food and helps me absorb all the nutrition from it because I can’t do it naturally,” explained Victoria, who has difficulty breathing and experiences regular coughing fits from the build up of mucus in her lungs.
Every morning, Victoria must plan her day accordingly with the right dosage of medications. Before taking each medication, she must clean her nebulizer throughly to prevent breathing in any germs, which could lead to lung infection.
Her her condition makes is harder to fight off the common cold or flu; she often ends up at Sick Kids Hospital for two-week stays, four times a year. She was just released from her most recent stay at the end of Februrary.
“I coughed up blood,” said Victoria of the reason for her last hospitalization. “For me, it doesn’t phase me but this time it did,” she said. “This one came out of nowhere. I was coughing up loads and loads of blood.”
It turnsout that three of the arteries in her right lung were leaking blood. “I had to get surgery for it,” she explained - but doctors were only able to plug one of them. The other two areteries are connected to her brain and spine.
Naturally, the hospitalizations affect Victoria's school work, but Victoria says that Bradford District High School has been very accommodating.
“It throws me behind sometimes... I can do work in the hospital but it’s always a catch up game afterward, me getting stressed out being two weeks behind,” she explained.
Typically, Carol will stay at the hospital with her daughter, which also puts a strain on her ability to work.
“I have to bring in notes to my work, saying my daughter is in the hospital,” said Carol.
Her previous employer even asked Carol to resign due to all the time she was taking off to tend to her daughter.
All of the hospitalizations, they say, could simply be avoided if they had coverage for Orkambi. The drug would help clear the mucus build-up, alleviate chest pain and reduce the risk of infection.
Carol and Victoria have been fighting for the past two years, trying to obtain coverage for Canadians and are now working with both MPP Caroline Mulroney and MP Scot Davidson to have their pleas heard, asking that life-saving medications be covered for all Canadians.
“Victoria has spent far too many days and nights over the years in the Hospital for Sick Children. I had the pleasure of meeting with Victoria and her family several times, most recently as of last week, and I sympathize with the struggles they face on a daily basis. Following our most recent meeting, I have written to the Minister of Health on Victoria’s behalf and highlighted the challenges she and many patients like her face," said Mulroney.
This spring, Davidson plans to bring mother and daughter to Ottawa, where they can address parliament.
“He’s (Davidson) behind us 100 percent to help us get these drugs,” said Carol.
"This is life or death for some people, so that's why it's important to me," said Davidson. "If I can get their story out to the powers that be and put some pressure on the government, that's what I want to try and do."
Ideally, he would like to to try and have the Vigneaus meet with two or three MPs and the shadow minister of health.
"I have to be the voice for the voiceless, that's my job," Davidson said. "I'd love the minister of health there if we can make that happen."
In the Legislative Assembly meeting on Feb. 26, another longtime advocate for the CF community, MPP Jim Wilson questioned Health Minister Christine Elliott on the status of the deal with Vertex, pointing out that 18 other countries have negotiated a deal with the pharmaceutical company in order to allow patients with cystic fibrosis to access life-saving medication.
"Canada is the only industrialized country that lags behind," he said.
"Will the minister make a firm commitment that she will sit down and negotiate a deal with the pharmaceutical firm Vertex for these life-saving drugs, save these patients all the stress and despair they’re going through, save the expense on the health care system and do the right thing?"
Elliott acknowledged that this is something that has been an important issue for a long time and that they are in discussions with health partners.
"There are very significant health consequences here. We are certainly very well aware of that, and we are doing our best to move this forward," she said.
But she noted that there are both provincial and federal requirements that need to be completed in order for a drug to be approved to be put on the formulary and to be available to people.
"It is something that I intend to bring forward at our next meeting this spring, to see if some of these approval processes can be run concurrently instead of consecutively to speed up the process."
"I’m very concerned about the time frames as well. I do intend to bring this forward," Elliott added.
While Victoria has not tried the Orkambi drug yet, Carol says they know it will work for her mutation.
“Her mutation is the most common one,” explained Carol. “There are 4,300 people in Canada with CF and about 90 percent of them have her mutation.”
“We’re not backing down. These are our children’s lives,” said Carol.
The ladies will also be rallying on March 25 at Queen's Park with other CF patients to raise awareness of the issue.
"It's going to be a big day. People will be coming from all over (the country)," explained Carol.
All over the country, there will be other rallies going on for the same cause, under the CF Get Loud campaign.
Victoria is very active in the CF community and even has her own Instagram account dedicated to raising awareness about the disease and the struggles patients deal with on a daily basis. Follow her @cystic_fibrosis_wont_stop_me.
