Rare disorder won't slow this boy

Five-year-old child must eat every three hours

Catherine Litt / Kamloops Daily News
February 26, 2013 01:00 AM

Bonnie Clarke prepares her son's nightly mixture of milk and cornstarch. Adam, age 5, has a rare disorder that prevents his body from converting fat into energy. He must eat every three hours. The cornstarch mixture slows his metabolism so he can sleep without interruption.

At five years old, Adam Clarke has a boundless energy about him - a kind of whirlwind excitement that would leave a marathon runner breathless.

Monday morning was a prime example. It was just after 9 a.m. and the Dallas boy was eager to show a visitor his collection of stuffed animals.

So, he dashed off to his bedroom and came sliding back down the hallway and onto the couch with three favourites: Scooby Do, a Dalmatian and a blue-and-white dog with big, floppy ears.

"This one's name is Patches," he said, holding up the Dalmatian.

"And this one's name is Scooby Doo."

The blue-and-white dog was Woofy, the boy proudly proclaimed, barely a breath between words.

Bonnie Clarke smiled at her son.

"Oh, good, you've named them all," she said, playing right along.

Adam is the only child of Clarke and her husband, Steve.

If they were to have another child, there's a good chance the baby would be born with medium-chain acyl-CoA dehydrogenase deficiency, as Adam was.

"My husband and I both are both carriers of the gene," said Clarke.

MCAD is a metabolic condition that prevents the body from converting fats to energy, vital to sustaining life.

Adam was diagnosed days after birth, during routine newborn screening.

"Looking at him, you wouldn't know anything is wrong," said Clarke.

Yet her son's life is far from ordinary.

Adam must eat every three hours to manage his MCAD. If he doesn't, he becomes lethargic; his blood-sugar levels dip dangerously and he goes into hypoglycemia.

If he goes without food for too long, Adam's MCAD could lead to brain damage or death, said his mother.

"It was pretty scary at first," said Clarke. "We didn't know what any of it was. The first year was kind of a blur; it was definitely the hardest."

Thursday is International Rare Disease Day, a day when dozens of countries will be raising awareness of the 6,000-plus rare disorders known worldwide.

Adam's MCAD is certainly rare. He is believed to be the only child in Kamloops with the disorder, the only one who must eat a carbohydrate every three hours and drink a life-saving, but unappealing, blend of milk and cornstarch every night before bed.

Five tablespoons of cornstarch in 200 millilitres of milk, with a few drops of Strawberry Quik for favouring, slows Adam's metabolism enough that he can forgo food until morning.

But, as soon as morning comes, he must drink a glass of juice and eat breakfast before doing anything else. The rest of the day follows a pattern of snacks and meals leading to the bedtime drink.

Adam won't need to take the cornstarch drink for much longer, though. Clarke said it's expected his next visit to B.C. Children's Hospital will clear him for the next stage: a simple snack before bed.

But Adam will have MCAD forever.

Next fall, he enters kindergarten, a new world of excitement for Adam and a new world of worry for his parents.

Clarke will have to meet with the school principal and kindergarten teacher to explain Adam's specific food routine. For the first time in five years, she won't be there to ensure her son manages his condition.

"We had (school) nurses in our day, but I know that's not there anymore," said Clarke.

Leaving her son alone is a frightening prospect, but Clarke is confident Adam will be able to lead a relatively normal school life.

And she's hoping the family's story will prompt other expectant parents in Kamloops to be aware of the diseases and disorders that could affect their children.

For more about rare disorders, visit www.rarediseaseday.org.

© Copyright 2015 Kamloops Daily News

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