Bonnie Cameron and Cindy Waters-Goodman do not know each other, but they have much in common. Both are feisty, small-town Ontarians who were young, busy mothers when hit with diagnoses of pulmonary arterial hypertension, an incurable heart and lung disease.
Ms. Cameron's verdict came after the day she felt constantly winded and her legs grew so swollen that she had to scissor off her jeans. A family photographer, Ms. Waters-Goodman found out after a flight of stairs at work rendered her faint, dizzy and slurring her words.
Both were started on a regimen of drugs that are costly but slowed the pace of the disease, which is uncommon and often goes undetected. The province paid much of the tab, which the women estimate was about $100,000 a year each. Their lives improved drastically: They could play with their kids, cook meals and shower without fainting.
Then, last spring, out of the blue, both received letters informing them the province would no longer allow more than one medication. To continue receiving support, they'd have to stop taking combos.
After much lobbying, Ms. Waters-Goodman won a reprieve and can stay on her combination of drugs for one more year.
Ms. Cameron wasn't so lucky. Forced to give up her secondary medication, she has suffered greatly, and this week slipped into critical condition after being moved to hospital in Toronto. With an intravenous line in her neck and several failing organs, she seesaws between life and death.
No one knows exactly how many Canadians suffer from pulmonary arterial hypertension (PAH). Estimates range from 2,000 to 10,000 people. While the disease cannot be cured, by experimenting with drug combinations, specialists around the world are finding they can keep patients alive twice as long as is possible without medication, sometimes more than 10 years.
Ontario was one of several provinces to underwrite the cost of this therapy - until the practice was reviewed and letters went out informing patients and doctors there was a "lack of good quality safety and efficacy evidence" for the multidrug approach. And those able to secure funding for secondary medications on their own - through private insurance or by taking part in a drug trial - were told that this would nullify coverage of their primary medication.
The decision, made by the province's Committee to Evaluate Drugs, alarmed patients and frustrated their doctors.
"There's no question that we cannot do as well in Ontario now for our patients as other places in Canada," said Dr. Sanjay Mehta, a PAH specialist in London. "This is a human issue. If someone is in front of you doing badly on the first drug, do you just sit and watch until they die?"
DOCTORS WERE PUZZLED
It has been a decade since the bizarre ballooning of her legs and the feeling she was suffocating drove Bonnie Cameron to a small-town hospital in Hagersville, where puzzled emergency-room doctors sent her to specialists in nearby Hamilton. After a month of back-and-forth with more specialists in Toronto, her symptoms - dizziness, light-headedness, inability to catch her breath - were diagnosed as severe PAH.
The bedridden 22-year-old had never heard of the disease, but its description made her other problems - man trouble, money trouble and raising a toddler by herself - pale in comparison. "The doctors more or less said I probably had a month to live," she recounted this week after arranging for a lawyer to update her will. "Now it's almost 10 years later. I'm still kicking."
But her status has worsened since her doctor, Hamilton internist Clive Davis, weaned her off all but one medication.
When she was diagnosed, Dr. Davis saw what he describes as a "dramatic improvement" in her condition when she began taking Flolan, the popular PAH drug that helps to open constricted blood vessels. A sensitive compound, it must be kept cool and is administered by a constant intravenous drip that requires patients to carry around a Walkman-sized pump along with a cassette of medication and an ice pack.
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