Even With Ataxia, a Rare Degenerative Disease, He’ll Ride Until He No Longer Can
Frank Orlowski has been an avid skier and cyclist for much of his life. But in 2014, he was skiing trails he knew well when suddenly, he felt like he had to hold onto something to stay upright. The sensation passed, so he shrugged it off.
But within a few months his walking gait felt increasingly off, and he began to limp on his right side. He felt as if he’d been at sea for a year and was getting reacquainted with stable ground. If he couldn’t walk, he wondered, how would he manage to get on his bike and ride.
“It was a bizarre and bewildering sensation,” Orlowski told Bicycling. “I had no idea what was going on. Cognitively, I felt fine, but my limbs were not cooperating.”
A physician initially thought Orlowski was suffering from peripheral neuropathy, a general term describing damage to the nerves in the hands and feet resulting in loss of motor control. But as Orlowski’s symptoms worsened over the following two years, his affliction appeared neurological.
In 2016, with imaging and MRIs, Orlowski’s doctors were able to rule out amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS). Due to the progressive nature of the symptoms, Orlowski’s doctors eventually landed on ataxia.
According to the National Ataxia Foundation’s website, “Ataxia is not a specific disease, but rather a symptom of many diverse conditions that affect the nervous system.” Some of those symptoms include loss of balance, mobility issues, voice, swallowing problems, spasticity, tremors, and loss of hand dexterity. According to Orlowski, people diagnosed with other neurological conditions often have symptoms of ataxia, making it a particularly difficult condition to diagnose.
Despite his diagnosis, Orlowski said his goal was and is “to stay active and moving,” and he didn’t want to “become immobile, bedridden, and non-functioning, as some with this disorder do.” He thought that if he could just keep riding his bike, as he had for the past twenty years, he’d be able to retain his sense of self. But riding was no longer that simple, he learned.
In 2017, about a year and a half after his diagnosis, Orlowski and his wife, Mary Ellen, headed out for a 25-mile ride on the Lamoille Valley rail-trail out of St. Johnsbury, Vermont, on a sunny-but-brisk, mid-August day. This was Orlowski’s first ride in weeks; his legs felt stiff, heavy, and clunky, and each pedal stroke was choppy and belabored, but he was determined not to give up. Luckily, after the first mile, Orlowski’s body warmed to the ride.
Riding in nature—listening to the rustle of leaves in the wind, feeling the warm rays of the sun—brought Orlowski back to the days before he’d begun to feel the effects of ataxia. He smiled and felt more like himself in the way that only a bike ride could make him feel. Over the course of the year since his diagnosis, Orlowski began to use a walking stick and then, even trekking poles for support when walking in his daily life. But he had been adamant about not giving up cycling, his main source of pure joy, mental and emotional therapy, physical pleasure, and escape.
As Orlowski approached an intersection where the trail crossed a road, he went to tap and feather his brakes as he’d done a thousand times before, but his fingers were stiff and unresponsive—another symptom of ataxia. A bolt of fear and adrenaline flashed through him. Instead of gradually tapping the brakes and coming to a stop, he squeezed the brakes, white-knuckling, gripping with too much force. Stopping so suddenly sent Orlowski flying over the handlebars and slammed him to the ground. He gasped, feeling the impact reverberate through his spine and sternum.
Dazed, scraped, bruised, his bike shorts shredded open along his hip, lying in the trail-side rubble, Orlowski’s body knew what his mind didn’t want to accept.
“That was it,” he thought. “That was my last ride.”
Orlowski was devastated. He’d lost the ability to stand or walk without assistance; he couldn’t ski, hike, or do any of the physical work his job as a store manager in the COVER Home Repair & ReCover store required. And now, the final link that most connected him to himself was gone too.
It’s difficult to pinpoint how ataxia can develop in a person. According to Dr. David Lynch, a neurologist at Children’s Hospital of Philadelphia and director of the Friedreich’s Ataxia Program, ataxia can be either genetic or acquired. This classification is important for doctors so they can diagnose and attempt to treat the symptoms of the disease. “There are many forms of ataxia within those two classifications,” Lynch told Bicycling.
Ataxia can result from autoimmune problems, head trauma, spinal cord damage, or vitamin deficiencies; or it can stem from environmental factors, such as exposure to toxic substances or drugs. A majority of patients suffering from the symptoms of ataxia remain undiagnosed, with no specific form of ataxia pinpointed.
Orlowski’s doctors believe his ataxia is of the autoimmune type, but to date, they don’t know how it developed. Orlowski has no family history of ataxia, and genetic testing has not been a match for hereditary ataxia, although they still cannot test for all gene involvement. Orlowski also has had many ataxia symptoms that one neurologist termed “spastic ataxia,” which is a neurodegenerative disorder.
All they know for certain is that ataxia is rare, all forms are progressive and life-shortening, they do not know how to treat it, and there is no cure.
“It is hard to treat because in many cases, it involves loss of brain cells—that is hard to reverse or even slow. And [ataxia] is hard to diagnose because in many cases the overt physical features are changing very slowly,” Lynch said.
Orlowski fought to keep depression from taking hold of him as his arms and legs stopped responding to his brain’s signals. He increasingly lost dexterity in his hands, arms, and shoulders; his fingers can no longer grip a pen well or pick up a fork easily. And as much as he tried to maintain the use of his limbs, Orlowski now must use a four-wheeled walker any time he wants to move. Much like mapping out a new ride, each movement he makes requires an intricate process of thought, preparation, and planning. He can’t make any spontaneous movements, or he could lose his balance and fall.
In the years after the crash, Orlowski continued to experience a steady loss of mobility, a decline of muscle control and coordination. He also began to develop difficulty speaking because of ataxia. His jaw feels as if he’s received a dozen Novocain shots in it, and his speech declines during the day. Although the doctors offered a muscle relaxant to treat the symptoms and try to keep his muscles from stiffening, the disorder progressed.
In 2018, Orlowski noticed a photo on social media of a man who suffered from ataxia and was riding a recumbent bike—something Orlowski had been eager to try. Through his online network of friends, formed through ataxia support groups, Orlowski was introduced to the Ataxia Athlete Initiative, which takes applications each spring for grants for recumbent bikes.
Orlowski gathered reference letters and applied. He waited five months without hearing anything. And then, he received a letter in the mail: Orlowski was awarded a grant that paid for a recumbent bike worth $2,750. The grant allowed coverage of the full cost of the bike, but Orlowski chose to pay $500 of his own money towards the recumbent to free up funds for other grant applicants.
A year after his fall, after what he thought was the last ride of his life, Orlowski received his first recumbent bike, a lava red Catrike Villager. It’s low-profile and easy for him to climb in and out by himself. Orlowski holds onto a porch railing, the ledge behind the tailgate of his pickup truck, or another stable structure, and lowers himself into the recumbent using his upper body. He takes daily rides on his Catrike, which he refers to as his self-prescribed, daily dose of medicine. “That bike is a godsend; it’s everything,” he said.
Lynch agreed. “[Exercise, such as cycling, is beneficial] because physiologically it can modify the pathophysiology of diseases by induction of protective mechanisms, such as mitophagy.” In other words, riding a bike or trike is “a pruning method for removing abnormal mitochondria,” which causes harm to the body.
“Cycling is useful [for people suffering from ataxia] because it is a form of exercise that does not require the same degree of balance as walking or running,” Lynch said. “On a recumbent [bike], the person is placed in a stable, essentially sitting position in which the bike frame takes the place of balance.”
Not only does riding keep Orlowski’s body from growing stiffer and more atrophied, but it also builds his community and support systems. He uses his trike as a vehicle to connect with others and spread awareness about ataxia. Even though his ability to speak has declined, every time he is out on his trike, Orlowski welcomes the many questions he receives about his recumbent bike and his disability.
Originally from Michigan, Orlowski, 62, has lived in Piermont, New Hampshire for thirty years, where he would ride bikes in the summer and ski every winter. But as his balance deteriorated, he had to give up doing both. Additionally, living in New Hampshire during the winter became no longer feasible with ataxia, so five years ago, Orlowski and his wife began renting a home in Florida for three to five months out of the year.
“This condition causes me to stiffen up in cold, damp weather, making movement even harder,” Orlowski said. “Plus, ice and snow on the ground makes falling even more common.”
While some with ataxia, such as Orlowski, lose the ability to balance or walk, others decline cognitively. In this regard, Orlowski considers himself lucky. He believes that his mind was spared so that he could spread hope and help others to stay positive. He writes poetry and blog posts as a means of consoling, uplifting, and inspiring others who are suffering from the disease or, really, from anything.
“We all go through difficulties in life,” he says, “mine just look different. … Being disabled doesn’t mean you’re done.”
Orlowski’s mind is as sharp as ever and as of this writing, he works part-time remotely in ad sales, writes poetry, spends time with his wife, is working on his first novel, and rides his beloved trike as much as he can.
Orlowski has also created fundraiser rides to benefit others. During the winter of 2020, near his rental home in Punta Gorda, Florida, Orlowski rode 500 miles in three months. Supporters donated between one and ten cents per mile. Sometimes Orlowski rode alone. Sometimes others joined him—cyclists riding standard two-wheel bikes including strangers, friends, and his wife. In three months, Orlowski raised over $2,200 for the National Ataxia Foundation—the same foundation that awarded him his grant. He’s hosting his fundraiser ride again this year, with a goal of riding 800 miles in three months.
Orlowski kept the torn bike shorts from his fall four years ago. He wore them when he rode an exercise bike at the gym. After getting the recumbent bike, he wore them again outside for three years until they were too sheer to wear. The shorts were a reminder to himself. “Don’t quit. Don’t give up. I’m still here.”
“Even though I am disabled, even though I’m not riding a two-wheel bike anymore, I can still ride,” he said. “In your face, ataxia.”
Orlowski is pedaling his Catrike to fundraise for the National Ataxia Foundation. If you would like to find out more or support his campaign, visit here.
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