Relentless disease

Ayr woman's fight with ALS inspires fundraiser

JOHANNA WEIDNER
(Jun 8, 2007)


Heather Snell can still play piano, still golf, still walk. But talking is tough, and so is swallowing. Eventually, and inevitably, more abilities will slip away.

The 44-year-old Ayr woman has amyotrophic lateral sclerosis, or ALS.

The disease's trajectory is as devastating as it is certain.

People with the neurodegenerative disease, also known as Lou Gehrig's disease, become progressively paralyzed as the neurons in the brain and spinal cord deteriorate.

Eighty per cent of people with ALS die two to five years after diagnosis, unable to breathe or swallow. There is no cure, and treatment can only slow the disease.

Snell focuses on the things she can still do.

"So far it's only in my speech," she said. Her words are slurred and often difficult to decipher unless the listener is paying close attention.

But that trouble with her voice means she can no longer sing.

Losing that was tough for Snell, who has been a musician all her life -- singing in choirs, playing as an accompanist and teaching youngsters.

"I've always been involved in playing or singing or both," Snell said. But, "you just have to keep going."

Her husband, Bob Snell, is amazed by her resilience. The couple have three children, ages 15, 18 and 21. "She's handling it very well. Extremely well," he said.

FACE MANY UNKNOWNS

The couple face so many unknowns. Not even doctors can tell them what to expect since the disease's progression varies so much from person to person.

"They have no idea. It's all a crapshoot," she said. For now, the pair try to carry on as before.

"They just told us to live your life day to day. What can you do?" her husband said.

"Sure they say it's progressing slowly, but that can change, too. Who knows?"

Snell is taking the only drug available, "but apparently it only adds like three months to your life expectancy," her husband said.

She was diagnosed in summer 2005. The disease can be difficult to diagnose because early symptoms are vague or mimic other conditions. For Snell, it "just started with some slurred speech. We didn't think too much of it," her husband said.

"We just thought it was too much red wine," she joked.

She visited the emergency department at the insistence of friends worried about her worsening speech and difficulty swallowing. From there she was referred to Freeport to investigate a possible stroke, then to a Toronto neurologist for testing.

The cause of ALS is unknown. That's why raising money for research is so important to Snell and her family. The family of five will be walking in an ALS fundraiser in Stratford tomorrow. June is ALS month, and fundraising walks are being held all over the country.

FAMILY FUN DAY PLANNED

A group of Ayr residents and Snell's family and friends are organizing Heather's Hootenanny for Hope. The family fun day -- with lots of music, of course -- will go from 3 p.m. to midnight on Aug. 11.

"Come and bring a lawn chair and have fun," said Snell's sister, Lynn McRuer, one of the organizers.

All proceeds will go to ALS research.

"At this point, that's what they need," McRuer said. "They don't know the cause. There's all types of theories."

"You have to do something," Snell added.

Along with raising money, they want to raise awareness of the devastating disease that none of them had heard of until Snell's diagnosis.

"It's an event for hope," McRuer said.

Snell works for her sister at the Kitchener music store Music Plus, although with her trouble speaking she's moving away from customer service and more into paperwork. This spring she'll stop piano lessons because it's hard to communicate with her students.

"They don't want her to quit," her husband said, "but she can't speak to them."

Her speech is worsening, and she often depends on e-mails to stay in touch. Even her husband has trouble understanding sometimes.

"It's getting tougher for me now," he said. "I really have to concentrate now on what she's saying."

Snell goes to a London ALS clinic regularly for testing to see how her nerves are responding, how quickly they're deteriorating.

"She woke up the other day and she couldn't straighten her fingers," Bob said.

But she can still play piano, for now. All they can really do is wait and see.

"That's about all you can do with this disease," he said.

jweidner@therecord.com

ABOUT ALS

Amyotrophic lateral sclerosis, also known as Lou Gehrig's disease, is a devastating neurodegenerative disease.

ALS destroys upper and lower motor neurons in the spinal cord and brain stem through which the brain controls voluntary muscles throughout the body.

ALS affects muscles throughout the body -- leg, foot, arm, hand, finger, speaking and swallowing. The disease does not normally affect the mind or senses.

People with the disease become paralyzed as the neurons degenerate, and four out of five die within two to five years of diagnosis.

Other neurodegenerative diseases include Alzheimer's and Parkinson's.

About six people out of 100,000 develop ALS every year. About 2,000 Canadians currently have ALS. It most commonly occurs between 40 and 70, although it can occur in older people and rarely in teenagers.

SOURCE: THE ALS SOCIETY OF CANADA AT WWW.ALS.CA

HOOTENANNY FOR HOPE TO BE HELD AUG. 11

Heather's Hootenanny for Hope is Saturday, August 11 from 3 p.m. to midnight.
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