It started with a slight twitching in Gary Jarvis’ left arm. “Then it moved to my right arm, and I started losing strength in my left arm,” the 77-year-old Hermon resident said. “I knew I had to find out what was going on.” The subtle symptoms had been noticeable for about five years when he finally saw a neurologist.
“Diane and I had kind of surmised what was going on by then,” Jarvis said, glancing over at his wife. “I had a cousin who died with ALS.”
So when the diagnosis of amyotrophic lateral sclerosis was confirmed, first by the local neurologist and then by a specialist in Vermont, Jarvis said, it wasn’t really a shock.
“I said to him, ‘You know, Doc, half the battle is knowing what you have.’” That was in 1999, and Jarvis was told then that he likely had three to five years to live. But ALS — also called Lou Gehrig’s disease after the major league ballplayer who in 1939 put a public face on what had been an obscure and fearsome diagnosis — runs an unpredictable course.
“I told that doctor, ‘It isn’t up to you or up to me how long I live,’” Jarvis said. “It’s up to God.”
ALS is a neurodegenerative disease that affects nerve cells in the brain and spinal column. It progressively destroys the brain’s ability to initiate and control voluntary muscle activity, including the ability to walk, hold an object, speak, swallow and breathe. Many people don’t lose cognitive function, though 40 percent do experience some loss. The drug Riluzole slows the progression of the disease in some people, and a new intravenous medication, Edaravone, recently approved by the U.S. Food and Drug Administration, promises to lessen the loss of daily function — at an estimated cost of about $145,000 per year. There is currently no cure for ALS.
About 20,000 people in the U.S. are living with ALS at any given time. It strikes most often between the ages of 40 and 70 and affects more men than women. About 10 percent of cases have a heritable genetic basis, but the remaining 90 percent are thought to be caused by some combination of genetic factors and environmental exposure.
Military veterans, even those who serve in peacetime, are twice as likely as the general population to contract ALS. Servicemembers who served during the Gulf War are at an even greater risk. Though not understood why this connection exists, this association is so strong that a diagnosis of ALS is considered a service-related condition that qualifies a veteran for full health care and disability benefits through the U.S. Department of Veterans Affairs.
Research, advocacy and visibility
All these unknowns, enigmas and variables underscore the challenge of understanding ALS, preventing its onset and developing effective treatments, according to researcher Gregory Cox, who studies the genetics of ALS and other neuromuscular disorders at The Jackson Laboratory in Bar Harbor.
“ALS is not one disease, but a constellation of symptoms,” Cox said. Recent progress in decoding the gene makeup of living organisms and in the technological ability to analyze and track complex genetic information has allowed researchers to identify 21 specific gene variants that are linked to the development of ALS in humans and animal models, he said.
The hope is to discover “modifier genes” that suppress the development of ALS in the presence of these variants, and to develop drug therapies and other interventions that mimic the effects of the modifiers. But the work is slow, Cox said, and since these heritable genetic factors appear to be responsible for only about 10 percent of ALS cases, it’s important to support research into other aspects of the disease.
Cox, members of his research team and others at The Jackson Laboratory participate each year in the Walk to Defeat ALS in Bangor, a 2.5-mile trek that raises public awareness and helps fund research and services such as equipment loans, support groups and political advocacy. The annual event is in its 10th year. The next walk is scheduled for 10 a.m. Saturday, Aug. 26, at Hayford Park. All funds raised at this event support research and local services for ALS patients and their families. For more information, call 207-712-3091.
Among its founders is Lisa Kingsbury, whose husband, Jim, was diagnosed with ALS in 2007, shortly after the birth of the couple’s son, Jack. At the time, the family lived in Bangor.
Now 51, Lisa Kingsbury said Jim’s disease progressed to the point that she could no longer care for him at home. He opted for a feeding tube in 2010 when swallowing became too difficult and a mechanical ventilator in 2012 when his breathing became badly compromised. When his personal care became too much for her, she looked for a nearby nursing facility.
“But most nursing homes won’t accept ventilators,” she said. “They didn’t tell us that when he went on the vent.”
Eventually, a facility in Biddeford agreed to take Jim. He’s been there since November 2012. Lisa and Jack visit once a month or so.
“His mind is completely intact,” she said, but he has no way to communicate except by using a letterboard, raising an eyebrow to choose a letter and laboriously spell out what he wants to say.
Unable to afford the cost of paying for his care, Lisa declared bankruptcy, allowed the bank to foreclose on her home and moved to Dover-Foxcroft, where she works for a home care organization.
“I would have given all that up if I could have kept him with me,” she said. “But it turned out I gave it up and couldn’t keep him anyway.”
Guided by gratitude
Although the life expectancy of a person with ALS averages two to five years from the time of diagnosis, more than half of all people with ALS live more than three years and up to 10 percent live 10 years or longer, according to the ALS Association. It depends on how quickly the disease progresses and in what order it affects muscle function; both factors are highly variable.
For Gary Jarvis, whose disease has confined him to a wheelchair for the last four years, the progression has been slow. That’s fine by him. “I believe the reason I’ve lived so long is my attitude and my faith,” he said. A devout Methodist, Jarvis feels called to share his story with others who are living with ALS, to provide information, hope and support in the face of a devastating diagnosis.
Many doctors, he said, do not refer patients to support groups, occupational therapy or other resources that can help them come to terms with their illness and maintain their independence as long as possible. But that help is available.
He and Diane regularly attend a monthly support group in Bangor whose membership changes frequently as newly diagnosed members come in and others become too debilitated to make the effort or are forced to move out of the area for their care. He also invites his doctors in Maine and Vermont to refer new ALS patients and their loved ones to him.
“I go and talk with them,” he said. “I find it very rewarding. If I can do anything to help someone ease their mind and give them some support, that’s what I want to do.”
He’s grateful that his disease has progressed so slowly; grateful that his modern, ranch-style home accommodates his changing mobility needs; and grateful that, as an Army veteran, the VA has provided him an abundance of help, including two electric lifts, a fancy electric wheelchair and some in-home modifications to meet his current and future needs. He’s grateful that he has good friends and family nearby and that Diane, who has had some serious medical issues of her own, will stand by him for the long haul, come what may.
Mostly, though, Jarvis is grateful for his faith. “This is never going to get better,” he said. “I’ll keep sliding, but whatever is in store, I’ll be fine. I really do believe this is all in God’s hands.”