Cliff Barr has no illusions about how his life is going to end but he still has hope.
The 70-year-old from Okotoks, Alta., is one of 100 patients taking part in new Canada-wide clinical trial to treat ALS — a debilitating and ultimately deadly neural disease that has few treatments and no cure.
“It is a difficult and an awkward disease,” Barr said Thursday at the University of Calgary, which is running the trial.
“I found the idea of the clinical trial promising. It gives you a little more hope.”
Barr was diagnosed with amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, in October. It causes paralysis because the brain is no longer able to communicate with the body’s muscles.
Over time, as the muscles break down, an ALS patient loses the ability to walk, talk, eat, swallow and, eventually, breathe. Experts say one in 400 Canadians will die of ALS.
Even on good days, Barr said, the disease is always there.
“The disease kind of reared its head and I’m a little weaker than normal,” said Barr, who retired 10 years ago. “This morning, I couldn’t do my pants up. I couldn’t brush my hair. I needed help doing the zipper up on this sweater.”
Dr. Lawrence Korngut from the University of Calgary’s Cumming School of Medicine is running the clinical trial over the next 1 1/2 years at nine different Canadian universities.
He said an anti-psychotic drug called pimozide slowed down the disease in zebra fish, worms and mice, as well as in humans with ALS in a limited six-week trial a couple of years ago.
Korngut said the drug doesn’t address the primary cause of the disease, which destroys nerves. But he adds that it’s now believed that there’s an electrical failure that accompanies the breakdown.
“This treats that electrical failure. We’re hoping by preserving that electrical function, even if the cable keeps breaking down, that will buy people time.
“It will prolong life.”
Korngut said people shouldn’t jump to any conclusions about how well the trial will turn out and he’s only “cautiously optimistic.”
“We’ve been through this before. We know that sometimes animals behave very differently from humans and we just have to do things properly and find out these answers.”
Barr said he has been told he is likely to have between three and five years to live. The research is a double-blind study so only half the participants will receive the drug. The rest get a placebo.
“I am a fighter. You pay your money. You take your chances,” Barr said.
“It can’t make it better. It can’t repair the muscle damage … but it can slow down the progression which would in effect help me maintain the quality of life for longer than I would have.”
Korngut said it could be years before all the results are known and he grateful for those willing to volunteer.
“ALS is a disease of weakness but these are the strongest people I know. These people fight this disease so courageously.”