North Vancouver man denied life-saving drug

A 22-year-old North Vancouver man has said he is facing a death sentence because B.C. will not fund the only medical treatment that could save him.

Garrett Shakespeare's red blood cells have a protein deficiency that causes his immune system to attack them, but the drug to treat it effectively costs $500,000 a year.

Shakespeare's rare blood disorder is known as paroxysmal nocturnal hemoglobinuria, or PNH, and besides the symptoms of excruciating abdominal and kidney pain, it causes blood clots that could kill him.

Shakespeare takes a variety of medications, including the steroid Prednisone, to manage the symptoms, but they don't work that well, he said. And one blood clot in the wrong place could be fatal.

"A report said that 50 per cent of people with PNH die within 10 years of diagnosis," he said.

Shakespeare was diagnosed when he was 11, almost 12 years ago.

Shakespeare said his doctor has petitioned the province's pharmaceutical board for an exemption that would allow him to get the drug, Soliris, through the B.C. Ministry of Health. Soliris is the only drug that can treat the underlying cause of PNH. The petition was denied.

Better outlook elsewhere

Barry Katsof, the president of the Canadian Association of PNH patients, has the disorder as well. But Katsof is living a normal life free of symptoms in Quebec, where the provincial government covers the cost of Soliris.

"It's incredible that governments in Canada are refusing to pay for this treatment when it's been proven to save lives," Katsof said.

Katsof said Canada is the only country in the developed world that refuses to fund Soliris because of the cost.

"You can't get a cost-effective model in a rare disease drug because there's so few people in the economic model," he said.

"The bottom line is: this drug has been proven to save lives. It's very black-and-white," Katsof said.

For now, Shakespeare tries to maintain an active life. He works as a DJ and is also a lifeguard and swim coach at the North Vancouver Recreation Centre.

"I try to stay really busy but it's hard because of the anemia," he said.

"I'm just tired a lot, and the pain is really frustrating to deal with all the time."