A.L.S.

Local organizations provide support and care for manitobans and their families suffering from the fatal neuromuscular disorder. It’s still sometimes referred to as Lou Gehrig’s Disease–after the legendary Yankee first baseman who famously called himself “The luckiest man on the face of the earth” in announcing his retirement from baseball before dying of the properly-named Amyotrophic Lateral Sclerosis (ALS) in 1941, just before his 38th birthday. 

Fast forward to 2010, and former Montreal Alouette player Tony Proudfoot donated his brain for research in a final act before joining the increasing numbers dying of this motor neuron disease caused by the degeneration of neurons located in the spinal cord. ALS isn’t just afflicting athletes, of course, but in the interim between the passing of Gehrig and Proudfoot, science hasn’t learned much more about the cause of the incurable disease.

What it does know amounts to this: The disorder is a progressive, fatal, neurodegenerative disease characterized by rapidly progressive weakness and muscle atrophy that eventually affects the diaphragm. As a result, most patients die of “respiratory compromise” and pneumonia within five years, although some survive much longer (while some die within weeks of diagnosis). It affects about 3,000 Canadians and roughly 200 Manitobans, although numbers are growing, with 10 new cases confirmed in one recent 11-day stretch.

About 10 to 15 per cent of ALS is inherited, according to Dr. Wenre Ilse, neurophysiologist and Assistant Professor of Medicine (Neurology) at the University of Manitoba, and in a small fraction of those patients, a gene has been identified that correlates with it, but he says it’s speculative whether the product of the malfunction of that gene is actually responsible for the ALS. In the remaining patients, ALS just happens.

That randomness is one of the scariest aspects of the disease–which can take an alarming number of forms, and which is diagnosed as ALS only because certain common denominators are present. Although science knows what happens, it has few clues as to why, meaning that its research efforts can be akin to trying to grab smoke with your hands and put it into a bag. 

“There has been all sorts of speculation about what (the contributing factor) is,” says Ilse, “including increased environmental toxins, but the fact is, no industrial toxin has been demonstrated to be relevant. Other theories include more exposure of people to each other, more international travel and more distribution of other agents that might be relevant, but in the end, we don’t know.”

Riverview Health Centre is surprisingly cheery given its reputed role as a palliative care facility (although rehabilitation and long-term care are also offered). At the door of a room about halfway down one second-floor hallway, I am ushered in by Carole Wright to meet her husband Ian, who greets me warmly from his large, motorized chair facing a huge window and the sunlit trees beyond. The room is appointed with a large TV–on which he has been enjoying the hockey playoffs, despite the exit of his Habs from the picture. 

Wright’s story is like so many others. “I was diagnosed in 2008,” explains between breaths facilitated by the ventilator tube in his neck, in the halting way familiar to many who saw actor Christopher Reeve interviewed, “but I really noticed that something was wrong about three years earlier. My golf game went into the tank, but I always put it down to physical fitness, so I began a program to improve that. It didn’t, so I finally realised that something was wrong. I went to a doctor, then to a neurologist who diagnosed me. That process took a couple of meetings and an MRI, so it took a long time”, and also encompassed trips to Minnesota’s Mayo Clinic and to Toronto. All opinions led to the same conclusion. 

“That’s it. You know, I had it”, says Wright of his reaction to the diagnosis. “I went into a period of my own research, talking to people throughout North America who had the disease–two of whom had had it for a period of 13 years or so–and then I went into an analysis of different institutions that offered cures, and I found that none of them really cured anything.”

When the diagnosis was confirmed, ALS Manitoba stepped in right away. “We’re involved with the client right from diagnosis to six months after death,” explains Diana Rasmussen, Executive Director and Client Services Coordinator for the not-for-profit organization, “staying in touch with the families. I go out and do home visits with them to see how they are managing.”

ALS Manitoba offers a number of programs to those living with ALS including an equipment program–for anyone who has equipment prescribed for them–the LVR Program, which is a breathing bag it purchases which it then trains clients and caregivers to use, a tube-feeding bag program, buying them by the case to supply to the client at no cost. “All of this is for the client,” says Rasmussen, “but ultimately it helps the family because it doesn’t have to worry about the cost of purchasing equipment. ALS is one of the most costly diseases because there is so much that needs to be done. Families might end up in a situation, for example, where they might have to modify their home in some way, and we try to provide input as to how they might use available resources to limit some of these costs.”

ALS Manitoba offers education and counseling for families and clients, including a children’s program to provide education and discussion for children of ALS clients, who can also take advantage of a bursary program to go to camp, for example. 

The organization also operates Brummet-Feasby ALS House, the only home with care and support in North America. The four-bed home on Sturgeon Creek provides a place where clients can come for respite–to spell off the family or for care while the caregiver is out of town, for example–or they can live out their lives at the facility, cared for by a mix of professional and non-professional staff to look after their needs.

The annual June Walk for ALS and numerous other fundraising initiatives are essential in enabling ALS Manitoba to offer these services and support that are so invaluable to both patient and family. Wright says his experience with the medical system (particularly the nursing staff at HSC, where he was hospitalized for over a year), ALS Manitoba and Riverview have all been very positive. “It’s amazing what can be done today for people as far as the occupational therapy,” adds Carole. “They’re one step ahead of you all the way. They know what Ian’s abilities are as far as movement so that he can make (his chair and room electronics) work. It’s really quite incredible.”

Meanwhile, the relentless pursuit of more knowledge about the disease continues. “We certainly know much more than we did in the past,” concludes Dr. Ilse when asked about research progress toward a possible cure (or more likely, a slowing or reversing of symptoms), “and we know more about the features of what happens with ALS, so I think it would be reasonable to say that we’re closer; how much closer, it’s hard to say.”

On the web: www.alsmb.ca