A mother in Windsor, Ont. is worried about what could happen to her daughter if she loses access to a medication that is one of the most expensive in the world but one that she says prolonging her daughter’s life.

Nicole Lamont’s two-year-old daughter, Lilah, has an incurable, rare condition called spinal muscular atrophy, or SMA. It’s a genetic disease that destroys motor neurons in the spinal cord, damaging children’s ability to move, leading to paralysis and often death.

Lilah has been taking an injectable drug called Spinraza, or nusinersen, which Health Canada approved for sale in June, 2017. The drug delivers synthetic pieces of DNA to increase a key protein that’s missing in people with SMA and is the first medication to show effectiveness at halting and sometimes reversing SMA.

Lamont says the drug has already helped her daughter.

“Lilah was one of the ones who came from nothing. She couldn't move; all she could do was wiggle her fingers, and now, she's starting to sit on her own,” she told CTV Windsor.

The drug’s producer, Biogen, approved Lilah for its Early Access Program, which covers the $750,000 cost of the first year of treatment, as well as the $375,000 cost of each subsequent treatment year.

But that coverage could end if provinces follow the recommendations of CADTH, the Canadian Agency for Drugs and Technologies in Health.

CADTH advises the provinces on whether to cover new medications and in December, it recommended that Spinraza be used on only the youngest SMA patients who have the most severe form, SMA Type 1.

Lilah has Type 1, but because her disease has progressed, she also now needs a breathing tube. CADTH’s recommends covering the medication only for those patients not currently requiring “invasive ventilation.’

Lamont says she was devastated when she heard Lilah wouldn’t qualify under CADTH’s recommendations.

“I was able to run outside and I screamed at the top of my lungs, you know? And I just dropped to the floor and (cried)," she said.

For now, Biogen will continue to pay for Lilah’s doses through its early access program.

The company is now in negotiations with the pan-Canadian Pharmaceutical Alliance (pCPA), which brings all the provinces together to negotiate prices on the drugs that CADTH recommends receive funding.

Biogen’s executive director, Marina Vasiliou, says all SMA Type 1 patients who currently have special access to the drug will continue to have their costs covered until a funding decision is made.

“While we are negotiating a more sustainable way of access with governments, we want to make sure that these babies that are at risk for death are covered,” Vasiliou told CTVNews.ca.

“It isn’t ethical for these families to have their children at risk of death while we are engaged in negotiations,” she said.

Lamont worries, though, about what will happen if the provinces follow CADTH’s recommendations and decide that patients like Lilah should not receive provincial coverage. Once a funding agreement is reached, Biogen’s Early Access Program for the drug will end.

“We're being told that we can't have it for our children, and we have to stand here and either make ourselves go bankrupt in so many ways, or sit here and watch our children pass," she said.

Lamont is working with others facing SMA diagnoses to pressure the provincial governments to provide more access to the drug.

"I’m optimistic because I’m not going to stop fighting for this. We’re not going to give up,” she said. “We’re a tough community, the SMA community, and we're not going down without a fight, that's for sure."

With a report from CTV Windsor’s Rich Garton

Correction: This is a corrected version that notes Biogen will continue to offer coverage for Spinraza under its Early Access Program while negotiations with pCPA continue.