Man’s ride for rare disease ends in St. John’s

Campaign advocates for increased coverage for treatments of PKU

Published on August 18, 2014

An Ontario man is hoping the Newfoundland and Labrador government will take notice of his recently-completed bike ride across Canada and find a way to increase funding for people who suffer from a rare metabolic disease.

Kevin Dube completed his two month journey Sunday in St. John’s, a ride he took on in aid of phenylketonuria (PKU). One year ago, his newborn niece Avery was diagnosed with PKU. The ailment makes it difficult to break down protein containing phenylalanine, an amino acid that’s toxic to the brain. It is believed to affect one in 12,000 people.

“It’s lifesaving to have it detected early on,” said Dube. “If (Avery) wasn’t diagnosed, there could be huge issues for her with her brain.”

All babies are screened for PKU, and in the case of Avery, the Ontario government helps cover the cost of the synthetic formula she drinks to stay healthy. If not treated following birth, the disease can lead to a severe intellectual disability. Later in life, it can cause seizures, intellectual disabilities and other health problems.

In Newfoundland and Labrador, coverage for PKU treatments vary.

The provincial health plan offers coverage for two formulas up to the age of 18 years.


Beyond the age of 18, formula costs can be subsidized depending on an individual’s level of income.

A one-pound can of synthetic formula such as Phenyl Free 2 can cost $45. John Robinson, a local man suffering from PKU, told The Telegram earlier this year it takes him a day and a half to get through the contents of one can.

A prescription medication called KUVAN can lower phenylalanine levels in a person’s blood and is the first prescription drug of its kind. Approved by Health Canada in 2010, it is not covered by many provincial drug programs.

“Three provinces have listed it — Ontario, Saskatchewan and Quebec,” said John Adams, president and CEO of CanPKU. Dube’s ride, dubbed “Avery’s Ride for PKU,” aided the non-profit organization to the tune of over $60,000.

According to Adams, new scientific evidence due to come out in the fall should prove helpful in convincing other governments to get on board with covering KUVAN under a provincial drug program.

“Ontario and Saskatchewan have negotiated the best price in the world for it, and that price is available to other provinces,” said Adams, who noted the prescription cost is generally unaffordable on an individual basis — a two-week supply can cost more than $9,000.

“The drug is expensive. It’s a budget buster for a family unless you have private insurance.”

Adams has seen first-hand the benefits of the drug through his adult son. He started taking KUVAN eight years ago.

“He had a learning disability and went through high school taking an extra year. Had trouble with organization, focus, attention and multi-tasking. He went through university with a reduced course-load, typically four instead of five courses (per term). After being on the drug for a year, he was two years behind his peers in school, and he felt more capable than he ever did before in his life. In his final year, he was eight credits short ... and he didn’t get Bs and Cs, he got straight As. We know his brain works much, much better. You have a better chance

at reaching your potential as a human being.”

Adams met with Liberal health critic Andrew Parsons last fall to discuss PKU. Parsons later wrote to then Health minister Susan Sullivan to request information on PKU services.

Speaking with The Telegram earlier this year, Parsons said he received a letter informing him the province had been in touch with the makers of KUVAN through the Atlantic Common Drug Review.

At that time, it was waiting to receive additional information from the manufacturer.