A Kamloops woman with a rare metabolic disease has gone public with her struggle for medical coverage from the provincial government.
Amanda Cosburn, a nurse who lives in Kamloops, has been lobbying on behalf of the support group CanPKU for provincial funding of an expensive drug called Kuvan, which is touted as a treatment for PKU.
The group is funded by the drug’s manufacturer BioMarin.
B.C.’s Health Ministry isn’t paying for the drug despite its Health Canada approval and its coverage in Ontario and Saskatchewan.
Kuvan is also publicly funded in many European countries and in the U.S.
The B.C. health ministry, however, states there’s no scientific evidence that Kuvan provides “clear and cost effective benefit” for those suffering with PKU.
The annual cost per patient for the medication can be as much as $180,000 per year for an adult, and the available clinical research does not show clear benefits, said Ryan Jabs, Ministry of Health communications manager, in an emailed statement.
“Our drug coverage decisions are made only after a thorough and rigorous review of the scientific evidence available,” he said.
Approximately one in 12,000 to 15,000 infants in Canada is born with PKU. In Kamloops, it affects Cosburn and five-year-old Hayden Knox, whose mother has also been lobbying for the coverage of drugs and special low-protein food needed to stave off brain damage.
Cosburn says her life is entirely wrapped up in trying to manage the disease.
“I was too busy to keep up with my health and wellness goals over the summer… it impacted (me) for sure,” she said.
“I have had a very big increase to my pain over the past few weeks. My medications have all been increased. I am stiff and sore as soon as I wake up. Trouble sleeping or staying asleep and most of all getting comfortable.”
PKU (phenylketonuria) sufferers are born without the enzyme that converts one amino acid into another amino acid needed for the brain’s neurotransmitters.
The unconverted amino acid accumulates in the brain, causing neuro-cognitive impairments, depression, anxiety, decreased ability to focus and delayed or affected fine motor skills.
To avoid the severest symptoms, PKU sufferers must follow a strict, low protein diet. This creates additional costs, which the B.C. government is also largely unwilling to cover.
“It usually costs me $220 to $240 every two months on top of my regular grocery bill. It is fair to say… my compliance to the PKU low protein diet is affected greatly by my financial situation and income,” said Cosburn.
All other Canadian provinces cover some of the medical foods, but the only financial help from B.C. for such foods is $40 month and goes only to income assistance recipients.
The government is reviewing low-protein food subsidies for PKU patients, said Jabs.
He added that the province’s health authorities do provide a metabolic formula that PKU patients need for proper body development.