A Port Elgin, Ont., family says the drug needed to keep their nine-year-old daughter from going blind is about to become unaffordable after Health Canada approved a plan that would increase its cost by nearly 2,000 percent.

Olivia Little needs eye drops every hour to help treat her Cystinosis, an ultra-rare genetic disease that causes crystals to build up in the eyes and impacts various organs.

The Little family has been paying an estimated $5,000 per year for the drug, which Olivia has been on since she was diagnosed at 18 months.

The family was left scrambling after Health Canada approved an official version of the drug, which is slated to sell for more than $100,000 per year.

"I don't know why they're going up so much," said Erin Little, Olivia's mom. "We need to question why these drugs are so outrageously expensive."

Little said her daughter's current drug supply will last up until Sept., 14. She doesn't know what to do after that.

Erin Little has used prescription eye drops to treat her young daughter's eye condition since she was a toddler. The drops used to cost a few thousand dollars a year, but Little says that price is rising to over $100,000, which she can't afford. 1:26

Drug approval process

The family has been purchasing eye drops from a compounding pharmacy in London Ont., before a tweaked formula of the drug was made available last year in Quebec.

Those pharmacies will no longer have the authority to sell the drug after Recordati, an Italian pharmaceutical company, applied to have its own formula approved by Health Canada.

"When Health Canada approves a new drug, other companies have to stop making compounds … it's a Health Canada rule and it's one of the things that we as a family feel has to change," said Little.

Olivia Little, right, is living with an ultra-rare genetic disease called Cystinosis. (Submitted by Erin Little )

It's the second time within two years that another pharmaceutical company upgraded the drug and began selling a newer version of it in Canada, said Little.

"It is not the patient who is going to bankrupt our country, it is the pharmaceutical companies," she said.

Financial help

Little said the family previously received financial help through a provincial fund.

However, with the soaring cost of the drug, it's difficult to determine whether the family will get any relief at all, she said. The family's insurance company hasn't decided whether it will provide any coverage.

With Olivia's current drug supply running out in about two weeks, Little said she hopes media attention will pressure Health Canada to make changes.

CBC News has reached out to Health Canada and Recordati officials for comment.