Saturday, March 13, 2004
By Mary MacKay, The Guardian
©The Guardian, Prince Edward Island

As the crow flies, Stan MacNevin of Clyde River and Ron MacLennan of Fredericton, P.E.I., don’t live far from one another.

But until recently they hadn’t crossed paths.

Then again, there’s an age difference — MacNevin is 51 and MacLennan is 36. And while MacNevin’s job as a long haul trucker with Seafood Express had him on the road all the time, MacLennan’s position with Farmers Dairy delivering milk saw him travel a more local route.

But one life-changing event — their dual diagnosis of amyotrophic lateral sclerosis (ALS) or Lou Gehrig’s disease — has brought these two Prince Edward Islanders together to participate in the Canadian portion of an international clinical trial for a new drug compound, TCH346.

“We’re going down a road where nobody knows what’s at the end yet. It would be just like going to the moon,” MacNevin says of the phase two study being conducted at the Montreal Neurological Institute and Hospital, the objectives of which are to establish the efficacy and safety of TCH346 and explore the dose response relationship of the compound.

“Lou Gehrig’s (disease) has been around a lot of years and this is the first time they actually have something they think might work. Can you imagine (what that means) to be a part of that process?” MacNevin adds.

The process by which the two P.E.I. men came to be involved in the Montreal study began after their diagnosis of ALS or Lou Gehrig’s disease, which is a neurodegenerative disease that attacks nerve cells and pathways in the brain and spinal cord that affect voluntary muscle control and movement. In the later stages of the disease, people are totally paralyzed, yet in most cases their minds remain sharp and alert. The average lifespan from the diagnosis of ALS is two to five years.

MacNevin was an ever-alert truck driver with 35 years on the road when his hand began to cramp and his speech slurred a little when he was tired during the spring of 2003.

“It never even crossed my mind that it was serious. I never took a pill in my life. I never missed a day’s work in my life, so why would you ever think . . . ?” MacNevin says.

Then he had a major dizzy spell while driving through Maine. By chance, a neurologist was at a hospital there and the verdict was dire.

“He was 99 per cent sure I had (ALS), but he said I should get another opinion,” MacNevin remembers.

“I guess I didn’t know enough to be scared. Well, I’m actually not scared, but I’m not saying I’m any Superman either. I mean I was fighting this right from the start, the first day, the first minute.”

He started on riluzole, the only medical treatment available to people with ALS at the moment. His family — wife Maureen and children, Tanya, 28, Tara, 25, and 17-year-old Tim, as well as friends locally and up and down the entire eastern seaboard of Canada and the United States rallied to help him in any way they could.

Then MacNevin heard about the drug study in Montreal. Eligible patients had to be between the ages of 21 and 80, have a diagnosis of ALS and have shown symptoms for no more than three years.

He went to Montreal for an assessment interview in November 2003, the first of five trips he has made to date.

“The first time I went there I only had one thing on my mind — fighting this — and when I got there, I got my eyes opened. I never saw anybody with ALS before that I really could relate to,” he says.

“And when you go to this place in Montreal, it’s right there in front of me. There could be 15 or 20 people there with ALS in a lot of different stages. A lot of them are in wheelchairs, a lot of them can’t talk. You couldn’t even imagine. When I came home from that trip, I didn’t even know if I was going back.”

However, with encouraging words from family and friends, he was back in his usual fighting form a few days later.

Six Canadian centres are involved in the Novartis Pharmaceuticals Canada-sponsored study which involves 60 Canadian patients. Worldwide, there are approximately 500 patients involved in the international trial.

This is the second phase of the clinical trial. The compound TCH346 has already been through the pre-clinical stage where the compound is tested but not in humans. Phase one showed the medication is safe in healthy humans.

“Phase two is meant to establish parameters such as the right dosage for these patients as well as establish the medication’s efficacy in treating the disease.

“This phase tends to be in a smaller group of patients,” says Jason Jacobs, the director of communications for Novartis Pharmaceuticals Canada Inc.

In phase three, the medication is typically provided to a larger group of patients with the disease. TCH346 may slow down the development and/or progression of ALS symptoms, but this will not be established conclusively until all the clinical trials are completed.

“I still don’t think it’s going to be the be-all cure, but I think it might help some,” says Ron MacLennan of Fredericton, who is presently taking over-the-counter vitamins recommended by the neurological institute.

MacLennan, who is husband to Colleen and father of Kandace, 16, and Chad, 13, was the picture of health until October 2002 when he noticed his arms were weak.

“I’m a volunteer fireman (with the New Glasgow Fire Department), so I worked all day and then we went to a barn fire. I was there until about one o’clock in the morning so I just figured it was fatigue,” he remembers.

As time went on, his symptoms slowly progressed. Physicians did tests and came up with the ALS diagnosis.

“At that point, I didn’t really believe it, but when we went to (see a neurologist in) Saint John in May (2003) that’s when we found out what it was. I’m a sports fan so I knew what Lou Gehrig’s was all about,” MacLennan says, referring to the baseball player with the New York Yankees in the late 1920 and ’30s, who was forced to retire in 1939 as a result of the loss of motor control caused by the disease.

“I was a little upset, but I never really got too worked up over it. Things happen, there could be worse things.”

MacLennan’s wife works with MacNevin’s daughter, Tanya, at the liquor commission in Cornwall. The two men met through this connection. That’s when MacLennan heard about the clinical drug study in Montreal. Through the couple’s efforts and that of MacNevin, who worked behind the scenes talking to specialists in Montreal, the 36-year-old was put on the clinical trial list.

The caveat is that for every four patients receiving one of four prescribed doses of TCH346, one person will be given a placebo.

“So your chances are one in five. You don’t know until the end of the study. It’s the only way they can find out if it works or not,” MacLennan says.

Marc Lemieux, clinical research co-ordinator at the Montreal Neurological Institute and Hospital, says the placebo is an essential part of a clinical study.

“The way to really get a good result is to have everyone . . . coming into the clinic at the same time, they all fit the same criteria (and) they all think that they could be getting a drug,” he adds.

“So they could be on any dose or a placebo, but they all think the same thing. No one knows what it is, it’s the only way to standardize the whole procedure.”

Lemieux also notes the placebo effect must be considered in clinical trials because some patients, even in the absence of any drug, think they are on one and can exhibit positive results or even side effects.

“(Volunteers are) extremely important, we can’t do it without volunteers, obviously. And in ALS it’s very easy to find volunteers because there’s nothing really out there for them,” he says.

“Riluzole is the only other approved medication which is in minimal usage. You could say it doesn’t do a whole lot. So we’ve got to find something better, that’s what we’re hoping to do.”

MacLennan had to stop delivering milk for Farmer’s Dairy a year ago.

“My arms are pretty well done, my legs are getting there, too. I can’t walk very far. My wife or daughter has to assist me to get dressed. I can still feed myself, I just can’t lift anything heavy . . . ,” he says.

“It’s just that every day you notice there’s something you can’t do, so you get frustrated or upset. But there’s always hope and I always figured just live as normal a life as possible. I have good people around me, so I’m not too concerned.”

“We’re muddling through, taking each day as it comes,” his wife, Colleen, adds.

His great-aunts and great-uncles pooled enough money for them to travel to Alberta last July for a get-away-from-it-all trip.

Others sold tickets on a quilt, the New Glasgow firefighters sold tickets on a side of beef and a portion of the proceeds from a local concert raised money to help the Fredericton couple.

Unfortunately, because there are no clinical trial sites in the Maritimes, MacNevin and MacLennan must travel at their own expense to Montreal to participate. MacLennan has been to the institute three times. He begins the actual clinical study in May.

MacNevin, who begins the clinical trials in April, has been overwhelmed by the support from his area and beyond.

A recent fundraiser attracted more than 1,000 people and raised more than $34,000 from the local community and throughout the Maritimes and the United States. Some contributed a generous amount of Air Miles and other frequent flyer points so he, his wife or another travel companion can fly to Montreal.

MacNevin says the day he was accepted into the clinical study was one of the happiest he’s ever had, but the night of the fundraiser was on par with that.

“I needed people behind me, just to know that they care enough. I will fight but need people to say, ‘Let’s go, Stan, let’s do this!’ Along with the (medical staff in Montreal), those are the two biggest things.”