It was announced on June 18 that the British Columbia (BC) government will end subsidies for expensive medication for Shirley Pollock, a 9-year-old resident of Vancouver Island.
Shirley suffers from a rare genetic disease called Batten disease (neuronal ceroid lipofuscinosis type 2). This disease causes epilepsy seizures multiple times a day, eventually causing severe brain damage. It is an extremely rare disease for which there is currently no cure, and Shirley is the only person in British Columbia who has been diagnosed with Batten disease.
The drug that was being discontinued was called Brineura, a treatment that slows the progression of the disease and costs $1 million a year. The family was shocked by the notice they received on the 18th. The family revealed that the last dose was administered on the 19th.
British Columbia’s Minister of Health, Josie Osborne, said on the 19th that the decision to end funding was “an extremely difficult decision,” and that it was not because of the price of the drug, but because Shirley’s condition had progressed to the point where she met the “criteria for stopping Brineura. “There is clinical evidence that once motor and speech function have declined to a certain extent, Brineura no longer has the effect of slowing progression. But her mother, Jori Fails, denies that her daughter’s condition has worsened.
Dr Ineka Whiteman, director of the US-Australia-based Batten Disease Support and Research Association and the Global Batten Disease Research Initiative, also told CBC Online that the decision to stop funding was based on old clinical trials and called the state government’s response “shocking.”