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the littlesErin Little holding daughter Harper Little, with niece Merra, and Olivia Little (right) at their home March 12.

MARCH 28 UPDATE: After being denied twice, Olivia Little has been approved to stay on Cystagon for 90 days. Following the 90 days they will have to continue reapply until decisions are made.

Hub Staff

The Little family was put on a path to the unknown and now Erin Little, mother of 8 year old Olivia, said she will be travelling to Ottawa with Member of Parliament Ben Lobb to advocate for her daughter as Health Canada has restricted Olivia’s life-saving medication Cystagon, making Olivia and approximately 100 other Canadians living with Cystinosis switch to a more expensive Procysbi, a delayed release capsule made by Horizon Pharma.

Little, while grateful that a drug company like Horizon has chosen to come to Canada, admits that while Procysbi could be great for older patients she doesn’t believe it’s for her 8 year old.

Olivia was diagnosed with Cystinosis, a rare, inherited, metabolic disease that destroys the body’s organs, in 2011 after she presented with acute kidney failure and has been taking Cystagon every six hours since July of that year. “She lost all kidney function and then she recuperated 50 percent,” said Little. “She has maintained 50 percent for seven years, in all that time her kidney function has stayed the same... why would I risk that,” she added.

Little said her family has requested an extension of Cystagon for three months after their first two requests were denied. She has fears for Procysbi which uses the same medication as Cystagon but works with a slow release so would require a higher dosage for Olivia. “If she can’t tolerate it, am I going to have immediate access to go right back onto Cystagon? It’s not the same as switching from Tylenol to Advil, it’s not that easy,” she said, having encountered many different forms of Cystinosis. “These kids are all so different.”

Little, who is the chair of the Liv-A-Little Foundation which has raised more than $500,000 for Cystinosis research, is also on the board of the Cystinosis Research Foundation. Through the Cyctinosis community Little said she knows of two people who have started taking Procysbi in the United States. One is 27 years old and the cost per month for Procysbi is $85,000 to $87,000 while another patient, who is 13 years old, is taking the drug at a cost of $57,000 per month. “That’s the cost of buying a house every year,” said Little.

Little said that Canada’s drug review board, the Canadian Agency for Drugs and Technologies in Health (CADTH) is currently in talks with Horizon Pharma and understands that CADTH has asked that the price of Procysbi be reduced by 90 percent. “Pharma[ceutical] companies are taking advantage of our children,” she said calling it “unethical” and adding that her fight is not with big pharma but with Health Canada. “You're supposed to be standing up and being there for your Canadian patients but what you're doing is taking away my choice and potentially the treatment that my daughter has right now,” she said.

Little said that the fact that Health Canada’s decision trumps her and Olivia’s physician makes her angry. “Health Canada gets to say what’s best for my child and they don't even know her...the system is completely wrong,” she said.

See Update: Lobb's motion for life saving medication denied