CORNER BROOK Debbie Mullins feels like a price tag has been put on her life.
Unfortunately, she says she can’t afford the roughly $40,000 a year that it would cost for a new drug to treat her idiopathic pulmonary fibrosis.
The Corner Brook woman was diagnosed in 2004 with the rare disease, which involves scarring of the lungs. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker and causes an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream.
There has also been no effective treatment or a cure for the progressively fatal disease — until recently, when InterMune released the world’s only treatment in the form of a drug. Esbriet — pirfenidone — was approved by Health Canada in October and made available in January for treatment of mild to moderate idiopathic pulmonary fibrosis in adults.
Mullins’ diagnosis came early, the result of hereditary testing. Her father, John Smith, died because of complications from the disease 26 years ago, as did her brother Brian in 2006. Her brother John, who lives in Alberta, has also been diagnosed.
The diagnosis is basically a death sentence, with life expectancy following symptoms between five and seven years.
Mullins was ecstatic at the news of the first treatment. She monitored the progress, asked her doctors and a local pharmacist to watch for it, and waited for an opportunity at an improved life.
The drug was released in Canada in January — as generic versions of it have been in other countries around the world recently. It is not available at regular drug stores.
Mullins went through the appropriate steps to receive a prescription, and filed a claim with her insurance company, but coverage was turned down.
“The drug is almost $40,000 a year,” said Mullins, who has not been able to work in recent years because of the illness.
“My husband (Gerry) doesn’t make that, let alone pay for it.”
She was not surprised by the denial. It is a new drug and very costly, she said, but that doesn’t make it easier for her. The drug is relatively unknown right now, but she believes the denial was mainly due to cost.
“They are putting a cost value on somebody’s life, that’s what they are doing,” she said. “I said to my doctor, ‘So, I am going to die if I don’t get it?’
“They have it out there, and they are going to let people die because they can’t afford to get it.”
Mullins is now hoping to meet with local politicians and get her story to provincial Health Minister Susan Sullivan. The next progression for this treatment, according to her, is to have provincial governments in Canada cover it.
She has been in contact with a national advocacy group, and has signed up with InterMune’s “Inspire” program.
“I am doing whatever I can do,” she said. “I don’t think people in Newfoundland with idiopathic pulmonary fibrosis even know this is out there, and what the cost is going to be, or that they are not going to get it.”
Pirfenidone has been shown to improve the diffusing capacity of people with this disease by up to 25 per cent, Mullins said.
In recent years, the Corner Brook woman has lost a lot of weight and finds it more difficult to breathe.
A couple of years ago, she was enrolled in a drug study by Gilead Science. She believes she was receiving the drug being studied — not a placebo — because her pulmonary functions tests improved every 10 weeks, except once when she had a cold. But that study was discontinued because the company said there were no significant differences noted, and Mullins had to send back the medication.
It was a disappointment, since she had noticed improvements.
Mullins is a suitable candidate for a lung transplant, but she is still not sure whether that is an option she wants to take.
The considerable risks of the surgery and transplant compatability make her uncertain, but she is close to being forced to make that decision. She also said the many medications following the transplant are extremely expensive, and said many of them she would not receive coverage for.
Right now though, a transplant is something she should not even be considering.
“Why should I have to go through a double lung transplant if this treatment is out there?” she said via telephone, trying to catch her breath as she makes her bed.
“It could give me probably two more years before I even have to decide that. It is crazy.”
If she has the lung transplant, she said she will have to move to Toronto, Ont. for two years. She says MCP will cover some expenses, but not all. She doesn’t know how she would be able to afford it. She said it will cost the province a lot of money in medical expenses, if she undergoes the transplant, guessing it will be more than she needs to cover the new drug.
Mullins hopes more people suffering with the disease will come forward and help her lobby for coverage.
“I wish I knew more people in Newfoundland who have it,” she said. “I would love to talk to them, get together and fight. It’s no good to be private about it because you are only going to sit there and die.”