Lilia Zaharieva wants to share her medical good fortune

High points tempered with immediate devastating lows are part of life for Lilia Zaharieva.

But the undeniable high of learning she can now access a life-changing medication, tempered by the low that not everyone can share in her good fortune has steeled her resolve for change.

The University of Victoria student wants the medication — which she now accesses thanks to a special compassionate exemption from at drug company — to be covered by provincial medical for everyone who needs it.

Zaharieva lives with cystic fibrosis, a fatal, autosomal recessive genetic disorder that until fall 2016 left her “hungry for air” every day and kills half of those with it before their 29th birthday.

“It was getting worse so quickly that I couldn’t even think of myself in the future tense,” she said. “I was living life where it was painful to breathe; where it was painful to walk for 10 minutes and I was looking to sort of wrap things up.”

She started a medication called Orkambi in September 2016 after learning it was covered by her university insurance plan.

“I had my life handed back to me again. I went from going 45 days in the hospital to the next year I had 10 and I haven’t been in the hospital for a year now.”

But last fall that coverage ended.

“From that point I had this horrific realization that I had eight days to find the $250,000 worth of coverage that I would need,” Zaharieva said. “I’ve essentially been in a fight for my survival ever since.”

Her friends, peers, classmates and strangers came through with a gofundme page.

“They understood exactly the values and came through and raised $20,000 in less than three weeks and were able to keep me healthy enough to keep advocating.”

But given the cost of the drug, even $20,000 only goes so far. Looking at her dwindling supply, she was weighing her scant options when, after multiple applications, she learned she’d earned compassionate coverage from Vertex.

“I haven’t actually been given word of how long it will last but I haven’t been given an end date either. As far as I know it’s going to be assessed as it goes,” she said. “I’m basically at the mercy of corporate generosity from a U.S. company at this point, so I’m hoping for the best.

But even that high point meant a low. It meant a phone call to her closest ally, Melissa Verleg of Vernon, a wife and mother of two.

Like Zaharieva, Verleg has the same strain of CF that responds best to Orkambi, and also lost her private insurance coverage. Together they lobby Health Minister Adrian Dix for coverage.

Unlike Zaharieva, she still isn’t covered. Verleg is the reason this story isn’t over for Zaharieva.

“Cystic fibrosis is progressive and Melissa is really in danger right now. We have a person in our province who’s been prescribed a drug that works for her, lost private insurance coverage and now has to go off of it. We should really not be OK with that.”

She has the support of Cystic Fibrosis Canada.

“Orkambi has the potential to benefit 2,100 of the 4,200 Canadians who live with cystic fibrosis,” said Dr. John Wallenburg, Chief Scientific Officer at CF Canada. “Since it treats the basic defect, as opposed to treating symptoms, its benefits can be dramatic in those who respond to it well. Orkambi does not equally benefit everyone who takes it, but for those who it does benefit, it can absolutely be life-changing.

“About one in eight people who take Orkambi are ‘super-responders’ who will see such dramatic improvements in their health that they are fully able to return to work or school, reduce the time spent in hospital from three months to zero days, or no longer need a lung transplant.”

During a press conference last November, Dix said Orkambi would cost $87 million over three years and Canadians are being asked to pay 30 per cent more for Orkambi than other developed countries.

Cystic Fibrosis Canada advocates continue to meet with elected and non-elected officials across the country to convey the urgency of the situation.

“Our efforts now aim to ensure that the Canadian Agency on Drugs and Technologies in Health fast-tracks its review process of Orkambi, as well as keeping pressure on provincial governments to expedite their review and reimbursement processes,” said Kim Steele, Manager of Government Relations and Advocacy at CF Canada.

“I think that right now today specifically is one of the most important junctures in the story and that’s because the resubmission is going in. This is one small victory I’ve had, but this is a big battle and it’s so important to keep that momentum going forward.”

Zaharieva too continues the fight. And with the medication’s help she has a lot to look forward to, for example her role as a bridesmaid and continuing her research into youth who have been in the foster care system and are now in post secondary education, even co-editing an international child and youth care journal on the subject.

“This is a privilege I’ve been given and I feel it’s my responsibility to carry this forward and carry this momentum,” she said. “Health Care is one of the most important things in our Canadian identity, right up there with hockey, Celine Dion, poutine, and I was shocked to hear how far behind we are on this.”

Did you know?

• Approximately 1 in 25 Canadians is a carrier of the defective gene with approximately 1 in 3600 live births diagnosed with cystic fibrosis.

• There is no cure for CF. Eventually, the effects of the damaged lungs increasingly limits their opportunities to live a normal life. For those at the end stage of their disease, the only hope of survival is to receive a double lung transplant. Unfortunately, the demand for donor lungs far exceeds the availability. Many people on the waitlist for new lungs will die before organs are available.

• CF causes various effects on the body, but mainly affects the digestive system and lungs. The degree of CF severity differs from person to person, however, the persistence and ongoing infection in the lungs, with destruction of lungs and loss of lung function, will eventually lead to death in the majority of people with CF.

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