Raising awareness about a medical mystery

Published in the Ottawa West edition of The News EMC.
Apr. 20, 2006

Kelda Whalen recalls a beautiful day in 1997 when she walked 10 blocks up Elgin Street.

“I was smiling at strangers,” she says. “It was the happiest I’d been in 15 years.”

That walk was the result of a temporarily successful operation on her brain – one of 12 to treat a disorder known as dystonia, which causes sufferers to constantly jerk and move involuntarily.

However, the surgery’s effects only lasted a few months before the deep brain stimulators implanted in her skull malfunctioned and had to be removed.

Ms. Whalen has had the condition since taking a fall in 1982, when she fainted several times in a sauna, which deprived her brain of oxygen.

Today, Ms. Whalen’s dystonia makes it difficult for her to walk long distances because the constant jerking of her torso is very tiring and painful, although her legs are not themselves affected by the movement disorder.

“I’m always in motion, all the time,” she says.

She estimates that about 160 people suffer from dystonia in Ottawa. The total number of sufferers in Canada, however, is unknown because of frequent misdiagnosis and ignorance about the disorder.

“I joke that whenever I tell people I have dystonia, they say ‘Dystonia? Isn’t that a country in Eastern Europe?'” Ms. Whalen says, adding that doctors thought the initial pains in her neck were imaginary and prescribed anti-depressants.

Stu Higdon of the Ottawa Dystonia Support Group says around 50 percent of general practitioners have never heard of the condition, and the same goes for most people in Ottawa and beyond. This lack of awareness, he notes, makes fundraising difficult.

“The problem is that people don’t seem to believe you,” Mr. Higdon says. “It’s a medical mystery.”

There are many different kinds of dystonia: focal dystonia, for example, affects only certain parts of the body, most commonly the neck.

Mr. Higdon’s brother Gordon has had generalized dystonia, which affects the entire body, from birth. However, no one knew what he was suffering from until he was 19 years old.

Part of the problem, says Dr. David Grimes, is there aren’t many tests available to determine if a person has dystonia.

Dr. Grimes, who is the director of the Parkinson’s Disease and Movement Disorder Clinic at the Civic campus of the Ottawa Hospital, is one of only two dystonia specialists in Canada, and one of 16 in North America.

“Unfortunately, the only test right now is to observe someone who has involuntary movements,” Dr. Grimes says.

He adds that while a person with dystonia may look like they have Parkinson’s disease or Tourette’s syndrome, there are differences.

“Dystonia is a symptom, whereas Parkinson’s is a disease,” Dr. Grimes explains. “A person with Parkinson’s may have dystonia, but a person with dystonia doesn’t necessarily have Parkinson’s.”

Dr. Grimes says dystonia does not affect a person’s longevity, unlike Parkinson’s which is a degenerative brain disease.

He adds that the posturing and jerking in dystonia is uncontrollable, unlike tics or what a person experiences with Tourette’s syndrome.

Botox injections and deep brain stimulators – a sort of “pacemaker for the brain,” Dr. Grimes explains – have had some positive effects on the disorder, although they don’t work on everyone.

Ms. Whalen says she has tried everything, but with few or temporary results.

“I’m much better than I was before, but I’m not cured,” she says.

For now, Ms. Whalen is working with the Dystonia Support Group to educate MPs about the issue. The group is trying to ensure that Botox treatments will be covered by insurance, as the injections currently cost $800 to $1,200 a session.

They have raised $60,000 over the past 10 years for dystonia research through their annual Walk-and-Wheel, which will be held this year on May 27.

The event takes place on Parliament Hill, and participants walk a few times from the flame to the top of the Hill. Those interested can contact Mr. Higdon at 613-749-7401 for more information.

Ms. Whalen still enjoys a rather active lifestyle, despite her condition: she swims every day, takes yoga classes and is a member of Toastmasters.

However, she says she still hopes her dystonia will one day be treated, and asks her doctors regularly about a cure.

“I’ll just keep asking if a miracle has been invented,” she says. “It’s all I can do.”

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