By Haven Orecchio-Egresitz, The Berkshire Eagle
PITTSFIELD — Scott Osleeb pulled into a dirt parking lot off the side of a Dalton road one recent morning and unloaded his two chow dogs, YaYa and YoYo, from his back seat. Donning a hat that read YNWA, an abbreviation for “You’ll Never Walk Alone,” he let the dogs off their leashes and made his way to a nearby trail.
Next month, the Pittsfield man will wear the same hat during his ascent to the top of Mount Kilimanjaro, a trek he’ll embark on to raise money for and awareness of a rare muscular disease that makes every day a challenge for his best friend’s two young daughters.
“It’s the worst thing I’ve ever heard of,” Osleeb said of Friedreich’s ataxia, a life-shortening genetic disease that causes difficulty walking, a loss of sensation in the arms and legs, and impaired speech. “Only 5,000 people in this country are diagnosed with this, and both of his daughters are.”
The girls, Grace and Stella Smith, both were diagnosed with Friedreich’s ataxia in 2016. For Grace, 12, early symptoms were cardiac issues and a lack of coordination. For Stella, 9, it was a Type 1 diabetes diagnosis that prompted testing for the double recessive trait.
Now, both girls rely on wheelchairs to get around at least some of the time, and their symptoms are getting worse with each passing year.
Osleeb, who underwent chemotherapy for his Stage 4 leukemia shortly before the girls were diagnosed, hopes his 19,341-foot journey to the top of Africa’s highest peak will raise the same number of dollars for the Friedreich’s Ataxia Research Alliance, an organization that funds research, education and family outreach about the debilitating disease.
“Everyone wants to donate to cancer,” Osleeb said. “For this, nobody even knows what it is. It’s so hard to raise money for this. It’s so hard to raise awareness. It’s crazy.”
Osleeb, who moved from Austin, Texas, to Pittsfield about a year ago, has become like an uncle to the daughters of his good friends Scott and Susan Smith.
He described as “horrifying” the moment he heard that Grace and Stella were diagnosed with a degenerative illness for which there is no cure or treatment.
“It was terrifying,” their father said of the diagnosis, during a phone interview this month. “I was in denial.”
While Friedreich’s ataxia generally has no effect on a patient’s intellect, its symptoms can include unsteady posture, frequent falling, slurred speech and a curved spine.
The onset of the disease usually occurs in children ages 5 to 15.
“Their muscles are just going to stop working,” Osleeb said. “They know what’s going on. They will know the whole time.”
Today, Grace, who is petite and has high arches in her feet, has difficulty walking without assistance. Her speech has begun to slow, according to her father.
“She has symptoms every day of her life,” he said.
Stella can walk on her own, using a wheelchair only during long walks at an airport or hospital, her father said. For the most part, her symptoms mostly are related to her diabetes, he said.
Osleeb, who tries to FaceTime with the family at its Austin home each week, said that seeing the girls, and their parents, face their challenges has been inspirational.
“They really are trying to keep some sense of normalcy. As heartbreaking as it is, I don’t hear them complain about anything,” Osleeb said of the girls’ parents. “They’re still trying to give them the life that children should have.”
Smith said that with Friedreich’s ataxia, it’s essential to live in the present.
Looking back in time is a reminder of how quickly his daughters’ symptoms are worsening. Looking forward is scary, too.
“My fear is that Grace won’t make it out of her 20s,” Smith said.
“We’ve lived with it long enough that we know when we have good days, to lean into them, and when you have bad days, you lean into each other,” he added. “We don’t really make super-long-term plans. You live your life to the fullest.”
Both girls still attend school as much as they are able.
Every year, the family takes a trip, sometimes two, to the Philadelphia Children’s Hospital, where the girls see specialists. The Smiths recently added a detour to New York City as part of the routine, so the girls could attend Broadway shows.
“They just love it,” he said. “It’s the most accessible activity in New York.”
Getting involved with the Friedreich’s Ataxia Research Alliance, which connects families affected by the disease, also has been helpful, he said.
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Both girls are part of ongoing studies though FARA. As a part of them, they wear motion monitors that collect data about how they move, and how their coordination is changing, and scientists use it in their research, according to Smith.
“FARA raising money for these purposes,” he said.
Osleeb has wanted to climb Mount Kilimanjaro since he was 15, when he heard that one of his sports idols had done it to raise money for charity.
In March, he decided to get the ball rolling, and hike in honor of Grace and Stella.
His friend, Rocio Ildemaro, who lives in Dallas, will join him.
“I’m 46 years old, and even though I still have cancer, my life is darn good,” he said. “Now, it’s not just me climbing a mountain. I can start raising funds for this super-rare disease.”
To prepare, Osleeb visited a travel doctor and received some vaccines for the trip. Because of his cancer, he couldn’t be vaccinated for yellow fever, so he made sure to avoid countries with active outbreaks during his travel to and from Mount Kilimanjaro.
He also has been training by regularly hiking in the Berkshires and, of course, fundraising.
So far, their team has raised just over $2,000 for FARA.
Osleeb said he has been surprised with how challenging it has been to raise money for the cause but said FARA staff assured him that it’s par for the course with rare diseases.
Originally, the men hoped their journey would bring in at least $50,000, but they have since reduced that goal to slightly less than $20,000.
“We had much bigger ambitions,” he said.
The Friedreich’s Ataxia Research Alliance has an annual budget of about $8.5 million, according to spokeswoman Felicia DeRosa. Of that, about $1.5 million is raised by people like Osleeb who host about 40 fundraising events, including 5-kilometer road races, cycling tours or dances, she said.
“We have predominantly been funded by the families affected by Friedreich’s ataxia and the families that surround them,” she said.
Osleeb, who has hiked all over the United States and parts of Europe, said he expects to experience all four seasons during his hike next month.
“On the climb, there will be every climate,” he said. “You start off in a rainforest, and by the time you hit the top, you’re at glaciers.”
While it’s a nontechnical mountain, and doesn’t require the use of ropes, altitude sickness could be an issue, and he plans on taking medication to prevent it.
“There are two types of people. For one, the goal is the summit. For the other, the goal is to get back safely,” Osleeb said. “I’m in that latter camp. I’m going to do everything I can, but if it looks like I’m going to die up there, I’m not willing to die up there.”
Still, if it came down to it, Osleeb said he’d rather risk his life doing something he loves than never having the experience.
“I would rather die on top of Mount Kilimanjaro than die here being afraid of dying,” he said. “I’m fine.”
Smith said that his family has been moved by Osleeb’s decision to make the hike in support of their daughters.
“It was so emotionally impactful, but not surprising at all,” he said. “He has such a zest for life.”
Not only does Osleeb’s effort help raise awareness of the disease and money for research, Smith said, it also builds confidence in his daughters.
“It helps the kids know it’s OK to have this condition,” he said.
Haven Orecchio-Egresitz can be reached at [email protected], @HavenEagle on Twitter and 413-770-6977.
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