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New state-of-the-art research lab at Ste-Justine Hospital to fight rare genetic disease in children

Click to play video: 'New Montreal research lab offers hope for those with rare disease'
New Montreal research lab offers hope for those with rare disease
Wed, Nov 8: A new world-class research lab investigating Sanfilippo syndrome was inaugurated at Montreal’s CHU Sainte-Justine Hospital on Wednesday. As Global’s Dan Spector reports, the lab was made possible thanks to a generous donation by a Foundation run by the Linton family, who has firsthand experience with the disease – Nov 8, 2017

Montreal has become a world-class research centre for a rare disease called Sanfilippo syndrome. It’s a fatal genetic disorder affecting the brains of children.

The new facility at Ste-Justine Hospital is called the Elisa Linton Sanfilippo Research Laboratory, named in honour of Elisa Linton.

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When Elisa Linton was born, all seemed normal. But her parents Randall and Elizabeth realized something was wrong.

“She was diagnosed with a rare, genetic, terminal regressive illness called Sanfilippo syndrome,” said her mother Elizabeth.

It’s a fatal disorder that affects 1 in 70,000 children.

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“The child slowly loses its ability to think, to speak, to understand,” said Dr. Alexey Pshezhetsky, who runs the new lab at Ste-Justine.

With Sanfilippo, the body can’t break down certain molecules. Those molecules build up in the brain and cause severe brain damage. It’s been called children’s Alzheimer’s.

“Generally, they’re in a wheelchair by the age of 10, bedridden by 12 and then they succumb to their condition in their mid-teens,” Elizabeth Linton told Global News.

After Elisa’s diagnosis came about two decades ago, the Toronto family refused to accept that there was no hope.

“We decided we were not going to take that as an answer. We were going to create hope for our daughter,” said Randall Linton, Elisa’s father.

Nineteen years ago, Randall and Elizabeth launched the Sanfilippo Children’s Research Foundation to fight the disease. Today, their foundation has raised over $7.5 million and has funded dozens of research projects all over the world.

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Elisa passed away last year, and on the first anniversary of her funeral, the Lintons were in Montreal to celebrate the launch of the Elisa Linton Sanfilippo Research Laboratory at Ste-Justine Hospital.

“The research that is going to come out of this laboratory and the hope it’s going to give families is amazing,” said Elizabeth Linton after touring the new facility.

The laboratory was made possible because of a $1 million donation from the Lintons’ foundation.

“They are really heroes,” said Pshezhetsky, pointing out how hard it is to get funding for research from the government.

Because of the awareness and funds brought by the Linton family, progress is being made.

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“I hope lines of research end up in clinical trials in about two years,” said Pshezhetsky.

The Lintons hope the work done in Elisa’s name will help Sanfilippo patients all over the world.

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