John and Kelvin Dyck may be brothers, but the two men share more than a last name.

They also have in common a rare condition called Pompe disease. The genetic disorder affects only about one in 40,000 people, and causes a progressive weakening of heart and skeletal muscles. Over time, it can be fatal.

But although the Dycks have the same condition, Kelvin, 64, lives in Manitoba, and the province covers the cost of the expensive drug infusions he receives to treat Pompe. John, 61, lives in B.C., and his drug treatment is not covered by the province. The drug, called myozyme, can cost more than $600,000 a year, and John can’t afford it on his own.

John says the situation isn’t fair.

“I should be offered the same type of treatment in B.C. as is offered in other provinces,” he said.

Laura Heinze, a spokesperson for the B.C. Ministry of Health, said she couldn’t comment on John’s case specifically.

But she said the ministry considers requests to cover myozyme “on an exceptional last-resort, case-by-case basis.” Although some Pompe sufferers in B.C. have their myozyme treatments paid for, Heinze said she could not disclose how many due to privacy concerns.

A spokesperson for the Manitoba government said less than five people in that province have been approved for myozyme coverage.

Cases like the Dyck brothers’ raise questions about inequities between provinces’ approaches to treating rare or so-called “orphan” diseases. Rare disease patients and their advocates have long charged that provincial drug policies lack consistency, leading to unequal outcomes for patients in different jurisdictions. For years they have been pushing for a national strategy on orphan conditions.

According to a 2015 study by University of Alberta researchers, access to drugs for rare diseases varies significantly across provinces and territories. Five provinces—Alberta, Ontario, Saskatchewan, and British Columbia—have created specific programs to decide which patients should receive coverage for orphan disease drugs. Patients in other jurisdictions can apply for coverage through the regular drug program. Criteria for who qualifies for coverage vary depending on the province, however, and health ministries don’t even agree on how to define a rare disease.

“Access to drugs for rare genetic disorders is very complicated in that there are 13 different jurisdictions across Canada. So there’s different access based on where you live. And every provincial government has in place a specific pathway by which you access certain drugs,” said Dr. Chantal Morel, a physician who treats patients with rare genetic conditions at Toronto’s Fred A. Litwin Family Centre in Genetic Medicine.

Audrey Laporte, director of the Canadian Centre for Health Economics, said it’s inevitable that provinces reach different conclusions about which drugs to cover, because each jurisdiction has a unique population with its own set of health issues, and has to work within a limited health care budget.

If a province denies drug coverage for a patient with a rare disease, it doesn’t mean the government is “heartless,” Laporte said. “The issue is ... OK, we can fund this drug for people suffering from this (rare) illness, or we can fund drugs to treat so many hundreds of people, or thousands of people, with this other (more common) illness that’s also deadly.”

According to the Canadian Organization for Rare Disorders, provinces spend about 2 per cent of their drug coverage budgets on treatments for orphan diseases. In the fiscal year of 2014-2015, $63 million of the Ontario public drug program’s $4.8-billion budget went toward drugs for rare conditions.

For Kelvin Dyck, who is a Mennonite pastor in Winkler, Man., having his treatment paid for has been life-altering. He was originally misdiagnosed with muscular dystrophy in 1992, but after tests five years ago confirmed he had Pompe, the Manitoba government approved him for treatment with myozyme.

The effect has been dramatic. Before he began receiving bi-weekly infusions of the drug, he had to use a wheelchair to get around. Now he hardly ever uses one. His lung capacity has improved, and his heart is functioning normally, he told the Star. “I’ve been very, very pleased with the level of care,” he said.

John’s symptoms haven’t been as severe as his brother’s. He was diagnosed with Pompe in 2011, and although he has difficulty walking long distances, climbing stairs or carrying things, he is still ambulatory. But he said his doctor has told him that he could end up on a respirator and might eventually need a tracheotomy and daily medical care.

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According to John, who is a professor at Trinity Western University in Langley, B.C., the province has twice turned him down for drug coverage. With the province considering myozyme requests on a “last-resort” basis, he worries that he will only be able to get treatment once his condition worsens, and his quality of life dramatically declines.

“I’m just simply ineligible, until my health deteriorates,” he said.

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