Organizers for a major fund raising campaign are hoping Saturday’s event raises enough money to help make Gabbie’s wish come true.Toddler Gabbie Strauss is suffering from an extremely rare disease.
While the 19-month-old infant might look the picture of perfect health, sadly that’s not the case.
Gabbie is suffering from cystinosis, an incurable metabolic disease where the amino acid cystine accumulates and crystalizes in cells due to abnormal transport.
The buildup of that amino acid eventually destroys all the body’s organs, including the kidneys, liver, muscles, white blood cells, eyes and central nervous system.
Gabbie is one of an estimated 50 people in Canada with the disease.
Gabbie’s parents, Jody and Trevor, are carriers of the mutant gene that causes cystinosis. The odds of their first child being born with the metabolic disease was one-in-four.
Her kidneys are already starting to fail.
There was barely a dry eye in Quinte West city council June 15, when Trenton resident Terry Broderick and the Strauss family brought their message to the city.
At the June 15 meeting city council got behind a local fundraising effort spearheaded by the Kente Kiwanis Club of Trenton. Council declared July 25 as Gabbie‘s wish Day.
The goal is to help the Cystinosis Awareness and Research Effort (CARE) raise money that will fund research in the hopes of finding a cure for the horrible disease. CARE works works with the Cystinosis Research Foundation based in California.
The foundation has raised about $9.3 million.
Kente Kiwanis will have wishing wells staffed by volunteers at the Quinte West Wal-Mart, Price Choppers, Giant Tiger, Zellers, Canadian Tire, and Smiley’s Independent Grocer.
Saturday’s event will include entertainment at the Trenton Town Centre, next to the Bulk Barn, from 10 a.m. to 4 p.m., featuring the band Madison Avenue, the Bay of Quinte Cloggers and various vocalists.
At 2 p.m. Northumberland-Quinte West MP Rick Norlock, MPP Lou Rinaldi and Quinte West Mayor John Williams, as well as other dignitaries, will be on stage to say a few words.
“There will also be some other surprises including, it’s rumoured, some vocalizing by Mayor Williams. We can only hope,’’ said Broderick. “We hope to have Gabbie with us along with her parents and grandparents Janice and Wayne Gillette, plus aunt and uncle Jill and Bill Jeffs.’’
All donations will go entirely towards funding research for a cure for cystinosis.
All administrative costs will be underwritten by Kente Kiwanis, the sponsors of this event.
The Jeffs say cystinosis is an “orphan disease” that the pharmaceutical industry has chosen not to adopt because it’s not lucrative to serve a small market. According to the Canadian Medical Association Journal, it costs between $1.3 billion and $1.7 billion to develop a new drug.
One drug, Cystagon, is able to slow the disease’s progress. Although it has not been approved by the Canadian government, the Strausses are able to obtain Cystagon through the Health Canada Special Access Program. It provides access to non-marketed drugs that treat serious or life-threatening conditions when conventional therapies have failed, are unsuitable, or unavailable.
The Jeffs say specialists believe that, with more money and more research, they can find a cure within three to five years.
But it’s going to take a lot more money.
“We just hope people will hear Gabbie’s story and be touched, and will want to help,” said Jody in an emotional presentation to Quinte West city council last month.
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