Gabbie needs your help

Gabbie Strauss will slowly die from the inside out if a cure for an extremely rare disease can’t be found.

The 19-month-old infant may look the picture of perfect health, but 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.

Her 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 chambers as Gabbie’s family brought their message of help to the city Monday night.

Grandparents Wayne and Janice Gillette, and Jody’s sister and brother-in-law, Jill and Bill Jeffs, all of whom live on Scriver Road in Quinte West, are doing their part to raise awareness of Gabbie’s plight. They were all in council chambers Monday.

City council is supporting 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 with the Cystinosis Research Foundation based in California.

The foundation has raised about $9.3 million.

Gabbie has to be medicated 10 times daily, between 6 a.m. and 12-midnight.

“It’s a privilege to take care of her,” said Jody. “We try to make her life as good as possible. With the medication and all the treatments that Gabbie has gone through for the last few months, we literally live by the clock. We hope and dream they will find a cure for Gabbie.”

In an emotional presentation to council, Bill and Jill Jeffs said their neice will need a kidney transplant.

While some people with cystinosis are lucky to live to 40, there are no guarantees for Gabbie.

“She looks healthy, but on the inside the disease is ravaging her,’’ said Jill.

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.

A drug, Cystagon, has been developed to slow the disease’s progress.

Although Cystagon has not been approved by the Canadian government, the Strausses are able to obtain it through the Health Canada Special Access Program. The program 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 with more money and more research, specialists now believe 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.

City council got the message loud and clear.

“It’s a message we all need to hear,’’ said Mayor John Williams, his voice straining with emotion.

“I had no idea there was such disease. We all have our struggles, but sometimes we don’t know how lucky we have it. Our prayers are with you.’’

Kiwanian Terry Broderick, chairman of the Gabbies Wish Campaign, said fundraising details will be finalized in the next few weeks.

In the meantime, those wishing to make donations right now can call Broderick at 613-392-9351.

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© 2009 Sun Media