Saxtons River youth with rare disease shoots first buck
DUMMERSTON -- Austin Leclaire, his father and some family friends sat patiently near the access road of a power line on a recent Saturday, and waited.
Then at 8 a.m., after an hour and a half, they saw it -- a beautiful 8-point, 158-pound buck walking along, unaware of the hunting party a short distance away. Austin, of Saxtons River, aimed his 30-06 Springfield, pulled the trigger and killed the deer on the land his family has hunted for more than 30 years.
It was a cause for celebration -- Austin's first buck (an 8-pointer) was quite an accomplishment and victory for a young man stricken with Duchenne muscular dystrophy.
The 13-year-old started hunting with his family a couple of years ago despite the physical limitations of his ailment and plans to continue for as long as possible. Duchenne muscular dystrophy (DMD) is a rare disease that limits mobility and causes muscle deterioration. Austin, whose younger brother Max also suffers from the disorder, was diagnosed at 3 years old and has been confined to a wheelchair since he was 10.
Austin's dad, Craig McNary, said he holds the end of the gun while Austin aims and fires.
Austin said he'll remember the shot for the rest of his life and looks forward to more. "I was pretty excited because it was my first deer," he said, adding that he also hunts turkeys.
Though simple tasks are becoming increasingly more difficult, he said he loves hunting with his family. "I like to go out into the woods with my all-terrain (wheel)chair," he told the Reformer.
McNary said it was a proud day all around. "I've shot a lot of deer and I have never been so excited," he said. "I think I was shaking more than he was."
McNary said his Austin's condition is getting worse while Max is one of 12 children throughout the country participating in a clinical trial testing the effects of a drug that can reverse the symptoms of DMD.
Eteplirsen, the drug created by Sarepta Therapeutics, helps Max's body create dystrophin, a protein he lacks because of Duchennes, and alters his genetic code, replacing the missing chromosome, to provide structural stability in his muscle tissue. He qualified to get weekly treatment at Nationwide Children's Hospital in Columbus, Ohio.
McNary worries Austin won't be physically able to pick up a gun by this time next year and, even if he can, it will be more difficult for him to shoot. He said the average life expectancy of someone with Duchenne muscular dystrophy is late teens to early 20s. "We do everything we can for him," McNary said, adding that Austin is driven up to Burlington every week to play on a wheelchair soccer team called the Vermont Chargers. "That's why (shooting the buck) was so special to him."
He said trying to get the drug for Austin has been a battle but the drug companies are being as supportive as they can be. He said he has started a letter to be sent to the Food and Drug Administration and is asking for signatures.
Kelly Price, a Vermont Fish and Wildlife game warden, said Saturday was a big deal for Austin and his family. "This rare genetic disorder has kept him in a wheel chair most of his life. However, it has not crushed his spirit or desire to experience all the world has to offer," Price said in an e-mail to the Reformer.
The family has also started a website, www.dmdhero.com, to help raise awareness for the cause and offer information about the disease.
Jenn McNary said she and her husband are now hoping to support Sarepta in its quest for accelerated approval of the drug Eteplirsen at the FDA later this year. The letter to the FDA can be found on the homepage of dmdhero.com. "This is the fastest way that Austin and other boys will get the drug that they so desperately need," she said.
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