B.C. woman receives long-term supply of life-saving drug — but vows to fight for coverage for all

Lilia Zaharieva was sitting in a Victoria restaurant having "the world's saddest happy hour" when she received an email that changed her life.

'I have the energy to fight for more than just my life'

Zaharieva at a rally in December. (Lilia Zaharieva/Facebook)

Lilia Zaharieva was sitting in a Victoria restaurant having "the world's saddest happy hour" when she received an email that changed her life.

Just that morning, the 31-year-old had started a "drug diet," cutting one of her life-saving pills in half in the hopes she could wean herself off her cystic fibrosis medication — or at least make her supply last a little longer.

When, in September 2017 she lost her coverage for Orkambi  — a little pink pill that costs $250,000 per year — she said she felt like she was "living on borrowed time." 

She campaigned for B.C. Health Minister Adrian Dix to make the drug publicly available through PharmaCare, and lived week to week, depending on donations to buy her own supply.

'I might not die anymore'

But her funds and energy were running low.

Then, just like that, her pharmacy told her that Vertex, the company that manufactures Orkambi, had decided to provide her with a long-term supply of the drug, under a so-called "compassionate coverage" program.

"I was dying, then I got the medication I needed, but then I lost it, then I was fighting the government for it, but now I have it again and so I might not die anymore," she frantically told a stunned server, who hurriedly sent over a bottle of champagne, on the house.

Zaharieva said she's elated to now be receiving the drug. For the first time, the University of Victoria graduate can plan to be a bridesmaid in her best friend's wedding, and present her academic research at a conference in May.

But with that overwhelming relief also comes a kind of survivor's guilt — the compassionate coverage she's been granted does nothing for others in B.C. living with rare diseases like cystic fibrosis, who are unable to access the live-saving drugs they need.

"Now I feel like I'm the last one standing,"​ she said.

Health Canada has approved Orkambi for use in Canada. However, individual provinces decide if they will cover the drug. (Vertex/ORKAMBI)

Hungry for air

To be eligible for Vertex's "compassionate coverage" program, patients have to fit a set of criteria decided by the manufacturer. 

Previously, patients were required to have less than 40 per cent lung function, a healthy liver function, and could never previously have had coverage for Orkambi.

With just 37 per cent lung function, Zaharieva met all the medical criteria necessary for the program. But because she'd previously received coverage under her student health plan before it was abruptly cancelled, she didn't qualify for the program.

She learned she's now been given coverage because Vertex axed the rule that stated patients could not previously have been covered.

But other patients who either experience complications with their liver or have slightly higher lung function find themselves in the position Zaharieva was in just days ago, depending on donations to fund their medication, or simply going without it, and facing the possibility of total lung collapse.

Coverage approval process

Health Canada has approved Orkambi for use in Canada. However, individual provinces decide if they will cover the drug.

B.C. relies on the recommendations of two committees: the national Common Drug Review and B.C.'s Drug Benefit Council. Both those committees have chosen not to recommend Orkambi for provincial coverage.

Decisions about funding for rare disease drugs are made based on cost effectiveness and clinical results, which can be hard to prove when there are so few patients using them.

Vertex is currently in the process of resubmitting additional clinical data to support the drug's effectiveness.

Zaharieva said B.C. needs to come up with a policy framework that accounts for funding expensive drugs that are used by a small portion of the population — and that she won't give up her advocacy work until all cystic fibrosis patients are covered.

"The feeling of being hungry for air is horrible. This access is a matter of principle to me," she said.

"I have the energy to fight for more than just my life and I have the breath to march for the things that matter."