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Wheeler Makes Strides in ALS Research

1/8/2019

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By Carol Lannon

​“Life,” John Lennon wrote, “is what happens to you while you’re busy making other plans.” Those words could have been written for Josh Wheeler. He and his brother, Jake, grew up on Signal Mountain near Robert’s Mill, next door to his grandparents’ home and 2,000 acres of protected land. “We spent the summers swimming in the lake and roaming the woods,” Josh says. “It was a fantastic place to grow up.”

Josh’s father, Dawson Wheeler, owned Rock Creek Outfitters; his family was active, always outdoors, and Josh also wrestled and ran for Rock Creek’s team. His grandfather was a pediatrician, and his mother, Sugar Holliday Wheeler, is a guidance counselor who later became a pediatric oncologist. Helping others to be fit, strong, and healthy was a family endeavor. But as a student at McCallie, Josh was interested primarily in English and history and thought he’d pursue those subjects in college. Then Life Happened.

In his senior year of high school, Josh was diagnosed with a brain tumor. To say this was life-changing is an understatement, but this strong, determined, and intensely curious young man used a life-threatening experience as a launching pad into an entirely different field of study. His treatment made Josh think about the causes and development of illness.

The avid athlete went off to Appalachian State, where he majored in exercise physiology, focusing on the ways in which the body, especially the skeleton and muscles, respond to physical stress. He found he liked research and moved on to Duke to a graduate program in molecular genetics, a field combining biology, biochemistry, and genetics to explore the structure of DNA and its connection to illness, particularly hereditary diseases. There he also met his wife, Kristen, who was pursuing a degree in physical therapy. After completing their studies at Duke, they moved to Colorado; Josh was chosen for a highly selective and rigorous program at the University of Colorado to become both a doctor and a scientist. When he graduates, he’ll have both a M.D. and a Ph. D. in neuroscience, qualified to work as a research scientist and treat patients, as well.

As his studies progressed, Josh found himself focusing more and more on a specific disease: Amyotrophic Lateral Sclerosis – ALS. (The name comes from Greek words that translate “no muscle nourishment.) “It was a natural progression,” he says. “Getting my undergraduate degree in exercise physiology, I spent time studying how skeletal muscles respond to stress and that led to ALS.”

ALS is a disease of gradual muscle weakening when motor neurons begin to die in the brain and it can no longer control muscle movement. As this process progresses, movement is impaired until muscles cease to function at all, causing paralysis and eventually destroying the ability to speak, eat, move, and breathe.

Many people call ALS Lou Gehrig’s Disease, named for a famous baseball player who died of the disease in 1941. The brilliant Steven Hawking, who died just this year, lived with ALS for 35 years, an unusually long time. How quickly the illness progresses varies, but the average life-expectancy is much less than Hawkings’ since there is no cure. In 2016, Tim Green, who played with the Atlanta Falcons, was diagnosed with ALS, one of a growing number of professional football players who are developing the disease. Green is being treated with drugs to slow the progression. In November 2018, the creator of SpongeBob SquarePants, Stephen Hillenburg, died of ALS. Though it has been considered a rare disease, that may be changing. “We don’t know yet what causes this neuro-degeneration,” Josh says. “This is what interests me – how and why are these neurons dying.”

Josh’s education is preparing him to be part of a new approach in healthcare. The National Institute of Health (NIH) has identified a need for physician-scientists (which Josh will be) who are researching a specific disease and also seeing patients diagnosed with that disease. Throughout his studies in Colorado, he’s been doing this. “What we learn through research can be used to improve treatment, and what we learn from patients will inform the research,” Josh explains. “The university protects our time in each area,” so he can give adequate attention to each. “My ideal would be an 80/20 split – research being 80,” he says. “Medicine is grounded in technology now,” he points out, which allows research and treatment to interface more easily. And he has also learned the protocol to conduct clinical trials. This cross-training is the future of medical research, training, and treatment.

Still to come is residency, though where that will be hasn’t been determined yet. Josh has been in remission for 12 years, and odds are that we’ll be hearing much more from him in the years ahead. “Our research sheds light on the normal processes that cells use to handle stress. If they get too revved up, they begin either attacking themselves or losing the ability to fight off stress.” Perhaps Josh’s research into ALS will someday discover not only the causes of that devasting disease, but expand understanding of other diseases, as well. Meanwhile, when there’s a little free time, Josh and Kristen are skiing, both loving cross country and back country with a little downhill thrown in. “It’s important to keep fit,” Josh says, “especially if it’s a ‘bluebird day.’”


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