Living with ALS

David Keller has been diagnosed with ALS, but he has hope researchers will some day find a cause and cure for the disease

LONDONDERRY — David Keller is as hopeful as he is brave.

The Londonderry man was diagnosed with amyotrophic lateral sclerosis (ALS) a year ago.

“It’s a difficult thing to put your mind around, that you’re slowly dying,” Keller said.

ALS, also known as Lou Gehrig’s disease, is a progressive disease which attacks the nerve cells that control voluntary movement. No one knows for sure what causes ALS and there is no cure.

The average life expectancy of a person with ALS is two to five years from the time of diagnosis.

Keller said his first symptoms were twitches. He thought it was just a sign he was getting older.

“Then it started to affect my left hand. I had been a rock musician in Chicago. I couldn’t play the guitar. I picked it up and I couldn’t play,” Keller said.

Keller said it took a year to get a diagnosis because of all of the testing involved.

“As I went on, it progressed over the year and the symptoms grew to my legs and then my arm became progressively harder to move,” Keller said.

Keller is now on Riluzole, a drug approved by the Food and Drug Administration for ALS treatment. It slows the disease and prolongs survival.

Despite the deadly diagnosis, Keller — who moved from Connecticut to Londonderry 20 years ago and has six children between the ages of 29 and 48 — has hope.

With warmer weather on its way, Keller is looking forward to each new day as it comes. As the owner of Maxwell Street Labradors, he and his wife, Pamela, are excited about a potential litter of puppies in May.

And Keller hopes some day doctors will be able to cure ALS.

“I’m thankful and hopeful for all the work that’s being done in this very difficult area,” Keller said.

Research in NH

Scientists are working on finding a cause and cure right here in New Hampshire. Researchers are working to determine if there is an environmental factor that may be connected with ALS.

Elijah Stommel, a professor of neurology at Dartmouth’s Geisel School of Medicine and a neurologist at Dartmouth-Hitchcock Medical Center, has teamed up with James Haney, a professor of biological sciences at the University of New Hampshire’s College of Life Sciences & Agriculture.

Researching ALS factors

Dr. James Haney shows off his equipment to collect aerosols from cyanobacterial blooms at his University of New Hampshire lab in Durham. He is working to determine if ALS can be triggered by environmental factors.

They are exploring whether cyanobacteria, formerly known as blue-green algae, in the state’s waterways could be a potential environmental trigger for ALS. Blooms of cyanobacteria produce many toxins, including the neurotoxin BMAA.

“We have been looking very closely at aerosol exposure,” Stommel said. “When we look at post-mortem tissue of patients’ brains and compare it to what we see in the lungs, most people harbor some degree of cyanobacteria in their lungs.”

Stommel said he developed an interest in the theory that cyanobacteria could be linked to ALS after learning about clusters of patients in New England living near water bodies, including Mascoma Lake in Enfield. He reached out to Haney about eight years ago.

At UNH in Durham, Haney and his team have developed a system to collect aerosols produced by cyanobacteria. He said they have been gathering and testing samples for about six years.

Haney said although there is no smoking gun, there are links between cyanobacteria exposure and ALS.

“Right now, we’re doing the simplest thing, which is to measure how much is out there,” Haney said. “Is it at a level that would cause a problem? We just don’t know what would cause a problem.”

The hypothesis that cyanobacteria is a potential environmental trigger for ALS has received pushback. A 2017 report by scientists at the U.S. Geological Survey concluded that a causal link between ALS, Parkinsonism and dementia and exposure to BMAA is not supported by existing data.

Haney says the potential connection is still worth exploring. Only five to 10 percent of ALS is familial and caused by mutations in one of several genes.

“That’s why there is a lot of interest in seeing what causes this,” Haney said. “These are lethal diseases and they’re widespread, and that’s why it’s a compelling field to be in, because we know so little about it.”

Finding support

For Keller and others currently living with ALS in New Hampshire, there is support. The Northern New England Chapter of the ALS Association in Concord has people who can meet with patients and their families at their homes and at clinics. They also have an equipment loan program.

“I personally think the biggest thing we’re known for is the care services,” Executive Director Mauret Brinser said.

Brinser said that on average in New Hampshire, there are 70 patients registered at any given time. Maine typically has about 70 patients registered, and Vermont has about 45, she said.

Amber Stalker, director of care services at the ALS Association chapter in Concord, said support for patients and their families is the most important thing they can provide as they advocate for more research and funding to help people with the disease.

“We are starting some new programs. We’re starting a bereavement group for family members called ‘Hope and Healing,’” Stalker said.

Dartmouth-Hitchcock has two ALS centers and clinics in Lebanon and Manchester. The Lebanon site has been recognized by the national ALS Association as a certified center for managing affected patients.

They offer speech therapy, physical therapy, occupational therapy, neurology, pulmonary medicine, nutrition, nursing and social work assistance, as well as opportunities for patients to have their blood checked for abnormalities and biomarkers that may assist in the search for an underlying explanation of the disease.

The U.S. Centers for Disease Control and Prevention estimates that every year doctors in America tell about 5,000 people that they have ALS.

The ALS Association estimates that as many as 30,000 Americans may have the disease at any given time.