By Kristi Wright

 

Santa Clara High School junior, Matt Rupel, age 17, has Friedreich’s ataxia, a rare, life-threatening disease that causes progressive damage to the nerves and muscles. As reported last year in a Santa Clara Weekly article, he was optimistic that he would make it through high school without the use of a wheelchair. Unfortunately, his ataxia worsened more rapidly than he anticipated and now he’s using wheels during school hours, which he labels, “not cool.” No question he was disappointed. “You don’t look people in the eye,” he says. “You’re a lot shorter; a lot of people don’t talk to you directly.” In addition, “getting through doors is tough.” But he’s quick to add, “The wheelchair isn’t 100% bad.” He’s made new friends even in the wheelchair (something he had wondered about). “I prefer it to walking around like I’m constantly drunk; it’s nice to get from class to class without help.”

 

He recounts a story where he was recently at Santana Row with friends. Instead of using a wheelchair, he relied on his friends’ shoulders. A security guard approached them and questioned whether he had been drinking. “I tried to say I had a neurological disease, but it came out like a slurred response.” Matt laughs. “When you’re a child, no one assumes you’re drunk, but now that I’m older it becomes a problem.”

 

Along with ataxia, scoliosis, and heart disease, slurred speech is another common symptom of FA.

 

Ironically, Matt takes way too many prescribed drugs on a daily basis for liquor to ever be in the picture. Since last year, he’s added a shot in his stomach three days a week of EPO (Erythropoieten)—an energy booster that may increase frataxin levels over time. (FAers produce dangerously low levels of the frataxin protein.) In theory, if enough frataxin is produced, Matt could regain some coordination. In addition, he continues to take Idebinone for his heart (heart disease is the deadliest symptom of FA) and a hefty handful of vitamins three times a day—vitamin E, vitamin C, and a myriad of others. He’s considering taking the drug Deferiprone someday—currently being used in Europe and not yet available in the U.S.—that anecdotally is showing great results. One FAer on the drug recently broke his leg playing soccer.

 

Matt thought that was great—not that the boy broke his leg, but that he was playing soccer! He would happily sign up for any medicine that might make it possible for him to play sports again. “Every time I look out the window in English [class], I want to play baseball.” He has recurring dreams where he easily walks down stairs or crosses a room, simple, throw-away actions for most people, but that’s what he dreams about. Now that school is winding down for the summer, Matt is looking forward to getting serious about his exercise program. He and a good friend have made a pact to be workout buddies to motivate each other to stay on track. “I’m hoping to devote 90% of my time to physical exercise and 10% to sleep,” he says with a grin.

 

Matt’s not a whiner. “There are things that could be worse than FA. I could be a starving child in Africa in the middle of some kind of conflict over water rights.”

 

All in all, his junior year is pretty normal. He admits that his full load of AP and honors classes this year has “kicked my butt.” For winter break, he went with family and friends to Flagstaff, Arizona and viewed the Grand Canyon from the edge. He and his two best friends spent spring break in New York on their own, having a great time exploring the city. This summer the same threesome is heading to Alaska for some salmon fishing. Not only does he push to be independent (like any teen) but he also likes to push his physical limitations. For instance, he prefers to drive a stick shift despite his mom’s offer to buy him a new car if he’ll switch to an automatic.

 

He’s contemplating universities, “as long as it’s in an area with nice weather,” and is planning on a Bachelor’s in Fine Arts. Eventually, he hopes to find financing for an independent film. One way or another, he’ll be working in the film industry as an adult. And he’s adamant that he won’t be living at home—“That’s out!”

 

But in the same breath, he also states that while life has been tough on the FA front, the best thing in his life is his family and friends—his support group—his base. “That’s never going to change,” he says. “It’s always going to be the best part.”

 

The FAITH event to raise money for Friedreich’s ataxia research will be held May 31^st at 6:00pm at the Sunnyvale Community Center. Tickets must be purchased in advance. Details can be found at www.fa-ith.org. Come join the fun and help make Matt’s future the brightest it can be.