PKU patients demand $2.8M food and drug strategy in B.C.

Svenga Forstrom is one of only 170 British Columbians who suffers from phenylketonuria, a genetic metabolic disorder commonly referred to as PKU. - Todd Coyne
Svenga Forstrom is one of only 170 British Columbians who suffers from phenylketonuria, a genetic metabolic disorder commonly referred to as PKU.
— image credit: Todd Coyne

British Columbians touched by a rare, potentially brain-damaging disorder are once again calling on Premier Christy Clark’s government to fund food and drug treatments for phenylketonuria (PKU).

The genetic disease affects an estimated 170 British Columbians, causing a dangerous build-up of the amino acid phenylalanine in the brain.

Without an expensive drug called Kuvan (sapropterin) and protein-restricted foods not covered by the province, the phenylalanine build-up can cause damage ranging from mild cognitive impairment to severe retardation in some patients.

Following up on a similar albeit unsuccessful campaign for Kuvan funding last fall, the Canadian PKU and Allied Disorders group (CanPKU) wrote to the Premier this week asking for $2.8 million for what it calls its Comprehensive Brain Protection Strategy.

The three-year plan, rolled out at last weekend’s PKU Day conference at B.C. Children’s Hospital, includes funding not only for Kuvan and low-protein medical foods, but for things like travel expenses and for the hiring of four new PKU specialists at B.C. Children’s.

Dubbing the strategy “From Worst to First,” CanPKU says B.C. lags behind other provinces in funding for the potentially debilitating disease, especially as it pertains to Kuvan, a drug approved by Health Canada in 2010 and fully covered under Ontario’s health plan as of today, Feb. 28.

“It is our view that your government is the worst government in Canada in providing treatment for patients who suffer from PKU,” CanPKU told Premier Clark in the letter. “We believe the B.C. Ministry of Health has not only turned its back on PKU patients, but it has engaged in a deliberate and systematic campaign to avoid providing improved treatment for PKU patients in B.C.”

In a statement to The Outlook Monday, the B.C. health ministry denied those allegations, saying its decisions on drug coverage are based on rigorous scientific review.

“Both the national Canadian Agency for Drugs and Technologies in Health and the B.C. Drug Benefit Council reviewed Kuvan to treat PKU and recommended against provinces covering this drug because of unclear benefits and high drug costs,” said health ministry spokesman Ryan Jabs.

He went on to say the ministry is “open to reassess this decision if additional evidence is introduced showing that this drug provides a clear benefit for PKU patients.”

One patient for whom Kuvan does offer a clear benefit is Svenga Forstrom, 18, of North Vancouver.

He began a trial of the drug in September 2011 and very quickly his tolerance for protein more than doubled and his dietary options broadened.

Now for the first time ever, Forstrom says he can go into a Subway restaurant and have a six-inch vegetarian sandwich; a lunch that could have put his brain in medical distress before Kuvan.

But at a cost of $18,000 a month for 16 little pills per day, the Forstroms, like many other B.C. families, couldn’t afford the drug if their private third party insurer wasn’t covering a good chunk of the cost. And, as Forstrom prepares to go off to college this year, his coverage under his parents’ health plan ends when he leaves school or when he turns 25, whichever comes first. So, upon graduation or at the end of his 24th year, a $216,000-a-year Kuvan bill will be his to afford.

Because of this fact, and because there are already PKU sufferers in B.C. without access to private insurance, the Forstroms and other PKU families want the province to step up its funding.

CanPKU spokesman John Adams, whose 21-year-old son suffers from the disease, told The Outlook his group has met with the province more than three dozen times since 2009. The most recent visit was on Feb. 4, when Adams and CanPKU vice-president Nicole Pallone traveled to Victoria to meet with an assistant deputy minister for the health ministry’s pharmaceutical services division, only to be told “in no uncertain terms that the file was closed,” Adams said, calling the decision unacceptable.

“Premier Clark came to power on the theme of ‘families first’ and we say it’s time to include PKU families,” he said. “We don’t want to be orphaned by the political or health bureaucracy in B.C.”

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