Devin Scullion: Hamilton teen fighting rare rapid aging disease
Most people with rare genetic disorder don't live past 13
In many ways, Devin Scullion is no different from any other teenager who just celebrated an 18th birthday.
But the Hamilton teen has already survived health scares that normally affect older people, because he's living with progeria, a rare genetic disorder that causes rapid aging.
Still, Scullion is defying the odds, as most children with progeria don't live past 13. His 18th birthday Thursday makes him one of the oldest alive in the world with the disorder.
An experimental treatment has helped the teen reach that milestone age, but it has been a long road, says his mother, Jamie Madley.
People are always staring at me out in the street or in stores. And I always think, "Put yourself in my shoes for just one day. Just try to be me."- Devin Scullion, 18 with progeria
“We almost lost him back in February,” she told CBC Hamilton from her east-end home.
That was the day before the Family Day holiday, and they had spent the day in Niagara Falls, Ont.
After returning home, Devin seemed a little off, so the family checked his blood pressure. It was 212 over 120 — a normal reading is 120 over 80 — meaning Scullion was heading for cardiac arrest.
“The doctor said if he had gone to bed that night, he wouldn't have made it to the next day,” Madley said.
He spent five days in intensive care as doctors worked to keep him alive.
Scullion can now add dodging a massive heart attack to his impressive, odds-defying resumé, along with surviving two strokes just days apart.
“My theory with him is nothing is going to kill this kid,” Madley said. “He's either one hell of a fighter, or he's just stubborn.”
Just a normal teenager
According to Devin Scullion, he is neither.
“I'm just an average teenager. I like to play games. That's me.”
It's easy to get that impression after speaking to for just a few minutes.
He's shy and soft-spoken about a lot of things, but opens up quickly if you ask him about video games. He'd rather spend his time thinking about Grand Theft Auto 5 or Minecraft over his own mortality.
“I just don't think about all of that,” he said.
He knows he has defied insurmountable odds to stay alive, but doesn't much care.
Scullion is also passionate about airplanes, and wants more than anything to do something linked to aviation.
“If I could get into Mohawk and do something with planes, I'd be in heaven,” he said.
Physically, Devin knows he's not the same as everyone else.
The progeria causes decreased muscle and bone density. There's also a higher risk of heart attack and stroke, which is how most people with the disorder die.
Scullion is much smaller than most 18-year-olds, at just under four feet tall. He has hip dysplasia and gets around with help from a walker. He doesn't have much hair, and has a very distinctive face.
“People are always staring at me out in the street or in stores,” he said. “And I always think, 'Put yourself in my shoes for just one day. Just try to be me.'”
Madley says she's convinced part of the reason she gave birth to Devin was to help educate people about the disorder, which affects only about 200 people in the entire world.
“You can come up and ask us questions,” she said. “We won't bite.”
You weigh how much?
A drug trial by Boston Children's Hospital has also given Scullion hope.
He has been on Lonafarnib for over five years now, and it has reversed his hardening of his arteries by 35 per cent. Ordinarily, a child with progeria may have vascular stiffening like that of an 80- or 90-year-old.
He's also experiencing some weight gain – but how much depends on who you ask.
During an interview, the teen and his mother couldn't decide just how much he weighed, so they hauled out the bathroom scale, hoping to prove each other wrong.
He stepped on it and it read 38.4 pounds – heavier than he has ever been. He was only 24 pounds when he started the drug trial.
“Holy s--t!" his mother screamed. “What the hell have you been eating? I have to tell everybody.”
He and his mother plan to head back to Boston before the end of the year so the teen can undergo a series of tests to ensure he's still doing OK. He'll go through 80 appointments in a week – roughly a year's worth for someone with a regular heart condition.
But for now, the two are still enjoying Scullion's birthday celebrations, which have always been a big deal, his mom says, because they never know how much longer her son will be around.
But that's changing as time goes by.
“We might have to look at scaling things back a little,” Madley laughed. “He just keeps going.
“This kid seems indestructible.”