Brody is a brave little boy!
By Joan Janzen
An Oyen resident is hoping to raise awareness of a genetic disorder called Dechenne Muscular Dystrophy (DMD). Kelli Smigelski’s 9-year-old son Brody was diagnosed with DMD when he was just five years old. One in every 3500 boys is afflicted with the disorder, which is characterized by progressive muscle degeneration and weakness.
Brody and his mom live on their grain farm near Oyen, along with Brody’s dad and younger sister. “Brody adores his dad. Any time he gets to ride on the tractor, he’s there!” Kelli said. “He has a mechanical mind and likes anything that has to do with motors.”
9-year-old Brody Smigelski from Oyen is hanging out with his dog Cooper. Brody was diagnosed with Dechenne Muscular Dystrophy (DMD) when he was 5 years old. PHOTO SUBMITTED
Brody’s parents didn’t know anything about DMD at the time of his diagnosis. It was shocking to hear there is, as yet, no cure, which is why they want to raise awareness. Muscular Dystrophy Canada (MDC), a non-profit organization, raises funds for research.
Although there are online support groups that give Brody’s parents a sense of community, there are not many people who know about the disease. It primarily affects boys and, in rare cases, girls, with symptoms becoming evident in early childhood, as early as age two or three. Initially, muscles close to the core of the body are affected, which later spreads to the extremities. The affected child might have difficulty jumping, running and walking. The disease can be both physical and cognitive, as the lack of dystrophin can also affect the brain.
Additional symptoms that may occur are enlargement of the calves, a waddling gait, and an inward curve of the spine. Later on, the heart and respiratory muscles are affected.
Brody sees a specialist in Calgary semi-annually, but the treatment only serves to delay the progression. He attends Assumption Catholic School in Oyen, where he is in Grade 4. While at school, he uses a power wheelchair, which gives him a lot of independence, and the ability to do more things.
“He’s ambulatory and can walk short distances, but there’s all kinds of challenges,” his mom said. “It affects pretty much everything we do.” Even though Brody has undergone many medical tests, his mom says her son is pretty resilient.
Until the 1980s, little was known about DMD. At that time, it was discovered a particular gene on the X chromosome, when flawed, leads to DMD. In 1987, the protein associated with this gene was identified and named dystrophin. The lack of dystrophin protein in muscle cells causes them to be fragile and easily damaged. Thanks to advances in cardiac and respiratory care, it’s become common for survivors to reach their mid-30s. Researchers are actively pursuing strategies such as gene therapy, gene repair, and FDA-approved drugs.
Meanwhile, the Smigelski family is busy renovating their home. “We’re in the process of making our home wheelchair accessible and making our vehicle wheelchair accessible. We’re trying to do that by this summer so Brody can use the wheelchair at home,” Kelli explained.
Brody’s mom says her son is quite inquisitive, always asking questions. “He’s a pretty brave little boy,” she said. He may be brave, but he’s just like every other kid who enjoys hanging out with friends, building with lego and playing video games.
As the search for a cure continues, an organization called Defeat Duchenne Canada works alongside MDC to provide leadership in research, advocacy and support as patients and their families wait for treatments to be approved in Canada.