ALS robbed George Smith of his ability to walk, eat and speak before it eventually took his life. But it never took his spirit.

Jodie Mosher-Towle, right, helps George Smith prepare before a Community Voices session at the Ostrove Auditorium on May 14, 2019, at Colby College in Waterville. Smith, who used his column to detail his struggles with ALS and became an advocate for those struggling with the disease, died in February 2021. Michael G. Seamans/Morning Sentinel, File

Quite the opposite, in fact. Diagnosed with the mysterious, always-fatal disease, George, a pioneering Maine outdoorsman who for decades wrote a weekly column for the Kennebec Journal and Morning Sentinel, threw himself into the fight against ALS with the same vigor he tapped to fight for land conservation and Sunday hunting.

With him in mind, we support the creation of a statewide registry for Mainers with ALS, or amyotrophic lateral sclerosis, a proposal now before the Legislature, so that we can learn more about this devastating disease.

Otherwise known as Lou Gehrig’s disease, ALS attacks the neuromuscular system, shutting it down little by little. As patient David Doane of Bucksport told a legislative committee, ALS is a “cruel, insidious disease, taking away your ability to walk, talk, eat and breathe.”

ALS affects roughly 4 to 6 people per 100,000. There is no known cure, and most people diagnosed with ALS die within four years, during which time they typically lose their motor skills and require a high level of care, even as their minds stay sharp.

Even as the disease progressed, George continued to file columns for the newspaper, typing first using one finger, then with voice-recognition software as he lost the ability to use his hands.

Advertisement

During this time, he used his column to detail his life with ALS. It became another one of his causes, as he talked about his struggles and victories. He became as much an advocate for the people suffering from ALS, and those trying to help them, as he had been for the Maine outdoors.

Those people still need help. The registry is part of that.

To find a cure or treatment, we need to know more about ALS. It’s more common in men than women, and usually develops when a person is in their 50s. Genetics is likely a large risk factor. Exposure to environmental toxins is another. Military veterans are more likely to be diagnosed, with an exceptionally high prevalence in people who fought in certain conflicts.

In New England, 11 clusters of diagnoses have been identified, with two in Maine: one in Bangor, and the other on Mount Desert Island.

But besides those strands, researchers are largely in the dark.

A registry, formed by information from health care practitioners, would establish a record of the 60 or 70 people in Maine living with the disease at any one time. Based on the state’s registry for cancer diagnoses, it would show who has it and where they live, perhaps giving researchers a better idea at how environmental conditions affect the disease.

Advertisement

Massachusetts, which is the only state with such a mandatory registry, also collects patient demographics, clinical symptoms and laboratory data, and information on diagnosis and treatment.

All of that information would help paint a more clear picture of who is getting ALS and why. It would get researchers closer to a cure that has so far been elusive.

Any cure or treatment will arrive too late for George, a fact he and others with ALS understand as soon as they are diagnosed.

But that doesn’t stop them from using their short time left to fight for a cure for all those who will get the disease in the future.

A statewide registry would honor those folks, and their loved ones, while giving hope to all those that come after them.


Only subscribers are eligible to post comments. Please subscribe or login to participate in the conversation. Here’s why.

Use the form below to reset your password. When you've submitted your account email, we will send an email with a reset code.