Formula to treat rare blood disorder costs hundreds of dollars per month
The rare blood disorder phenylketonuria (PKU) affects one in 12,000 people according to the non-profit group Canadian PKU and Allied Disorders (CanPKU).
It used to take John Robinson of St. John’s only a day and a half to use a full can of the dietary supplement Phenyl Free 2 to treat a rare blood disorder he was born with called phenylketonuria. No longer able to afford the formula since losing his subsidized coverage, Robinson has not been taking it for the last two months. — Submitted photo
For Newfoundland and Labrador, that would equate to approximately 42 people who lack the necessary enzyme to break down protein in food.
That’s why people such as John Robinson and Andrew Ryan cannot understand why the government will not guarantee full coverage for the costly dietary supplement they are supposed to take to avoid the consequences of leaving PKU untreated.
“For the low amount of people who are diagnosed with PKU on the island, why can’t we get coverage and yet every other province does?” Robinson said.
Robinson uses Phenyl Free 2, a synthetic formula that comes in a one-pound can in powder form. A can costs $45, and Robinson would typically make his way through its contents in a day and a half. He combines that with a low-protein diet that’s consistent with what would be considered a vegan diet.
“It’s a very strict diet. It’s fruits, vegetables ... basically anything that’s not meat or dairy.”
Babies are screened for PKU. The disease can cause an intellectual disability if not treated following birth. If left untreated later in life, PKU can lead to intellectual disabilities, seizures and other health problems.
In Newfoundland and Labrador, there are two formulas approved for coverage up to the age of 18 through the provincial health plan. Beyond that age, PKU sufferers can get formula costs subsidized depending on their level of income. Other than P.E.I., Newfoundland and Labrador is the only province to not guarantee formula coverage for adults, according to CanPKU.
Ryan, who at times has gone years without taking the synthetic formula since turning 18, has experienced mood swings he attributes to leaving his PKU untreated.
“If I don’t have the formula, your life is deteriorating,” he said. “It affects your moods. It affects your motor skills. Pretty much everything. ... It’s stressful living with it.”
The 26-year-old would like to regain coverage for PKU, but has not been able to do so, even after Ryan’s employer recently laid him off.
“I’m not able to afford it given my situation,” he said. “It’s not right and it’s not the way it should be.”
Robinson lost his coverage last summer because he was making too much money — he’s not aware of the exact cut-off point for coverage. For the last two months, Robinson has not been using the formula.
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“They’ve basically made it unaffordable for me to live, to the point now that it would actually be a better idea for me to move away, even to somewhere as close as Nova Scotia, where I could basically save myself $900 a month and live a healthier lifestyle.”
He now worries what the consequences will be for his health to not be using Phenyl Free 2.
“It might not happen to me today. It might not happen to me tomorrow. But it could happen.”
Liberal Health critic Andrew Parsons met with CanPKU president and CEO John Adams last fall and wrote Health Minister Susan Sullivan shortly thereafter requesting information on PKU services provided by the province.
“We’re basically dealing with approximately 40 people in this province, so there’s not a great number, and I certainly don’t think there’s a great cost,” he said with regards to the notion of government fully funding formula coverage.
An inquiry was also made about the potential to fund KUVAN, a prescription medication that lowers Phenylalanine levels, through the provincial drug program.
Parsons received a response letter in January that indicated the government — through the Atlantic Common Drug Review — had contacted the makers of KUVAN about the possibility of covering the medication, but asked the manufacturer to provide additional information. It did not receive the requested information.
Robinson would also like government to consider covering other formulas to treat PKU that have lower fat content. He also notes some provinces fund the cost of specialty foods for PKU sufferers (Newfoundland and Labrador does so for people younger than 18).
The Telegram contacted officials with the Department of Health and Communities Services requesting comment on the feasibility of expanding formula coverage and funding additional formulas. It did not receive a response prior to deadline.