Had she felt this before, she wondered? She may have had a bout of weakness a year earlier, but the memory was hazy. It was easy to brush off because the feeling left as quickly as it came.

But now, in the middle of the stairs, the weakness was undeniable, and the awareness of it mingled with confusion. “What is going on with my body?” If there’s one commonality in people diagnosed with amyotrophic lateral sclerosis, or ALS, it’s that no one thinks the worst at first, but everyone remembers the first sign of trouble.

A half mile away, on another Lakewood street, and just six months after Cara experienced her first troubling sign, Carol Shillace struggled to open a brick of cheese encased in a plastic wrapper. Her left hand felt clumsy and weak. When she began to slur her words eight months later, alarm bells went off and she immediately sought help, afraid she’d had a stroke.

Both Carol and Cara would receive an official diagnosis of ALS on nearly the same day in April of 2023. And now, a year later, both are struggling to manage a neurodegenerative disease called by one researcher, “A particularly devastating disease for both patients and families alike.”

OBSERVER File photo A harmful algal bloom is pictured on Chautauqua Lake during a past summer.

The incidence of ALS in Chautauqua County was called “worrisome,” by one leading researcher, with four known cases presently in southern Chautauqua County, and seven deaths reported from 2019 to 2024. The incidence may be higher, but New York state lacks a mandatory reporting system, and past and current cases in the county are gleaned from obituaries and from patients who are public about their diagnosis.

From 2013 to 2018, another six deaths were caused by ALS in Chautauqua County, a disease whose incidence rate is calculated at 1.5 to 2 cases per 100,000 people per year. In Lakewood alone, a village of 3,000, five people reportedly had ALS in the summer of 2023, verified by obituaries and private knowledge of the cases.

Dr. Elija Stommel is a professor of neurology at Dartmouth’s Geisel School of Medicine and a neurologist at Dartmouth-Hitchcock Medical Center. He’s been researching ALS for more than two decades, mapping out high occurrences of the disease that are found clustered around lakes with harmful algae blooms (HABs). HABs are considered a strong contender as a risk factor for ALS, although researchers from the University of Miami published a study in 2020 declaring a direct link, discovered when monkeys, who were fed cyanobacteria, developed early pathological changes similar to what happens to humans with the disease.

In correspondence with Dr. Stommel, he called the anecdotal incidence of ALS in Chautauqua County “worrisome,” but noted that a direct association of county cases with HABs and other suspected environmental toxins would involve concerted research and study.

“We don’t know if these people have been in your area their whole lives,” he noted. “Profession can be a risk factor, such as pesticide exposure and agriculture in general.”

Hard core studies, he said, would source more information about patients’ occupations, age, sex, the area in which the people live, and population density for that area. Migratory history can also be important. There is also a genetic form of ALS, which accounts for just 5 to 10 percent of cases, which must also be ruled out.

MONITORING WATER BODIES AND ALS CASES

Across northern New England, researchers have identified ALS hot spots–a large cluster in Vermont near Lake Champlain and a smattering of clusters among coastal communities in New Hampshire and Maine. They’ve found that living within 18 miles of a lake with high levels of dissolved nitrogen–a pollutant from fertilizer and sewage that feeds algae and cyanobacteria blooms — raised the odds of belonging to an ALS hot spot by 167 percent.

Lake Champlain in Vermont may well be the poster child for taking bold and decisive action to monitor HABs. Since 2003, the Lake Champlain Committee has trained 350 citizen volunteers to monitor for HABs at lakeshore locations. The volunteers, along with staff from the Vermont Department of Health and Environmental Conservation, file weekly online reports that are then displayed on the Cyanobacteria Tracker Map.

Both Massachusetts and Vermont have mandatory ALS reporting systems, requiring healthcare providers to report all new cases of ALS to state health departments. State-level registries are gaining traction in the Northeast, and New Hampshire and Maine are also considering legislation for mandatory reporting.

Just as cancers and infectious diseases have environmental risk factors and are tracked in registries, scientists are learning neurodegenerative diseases have similar risk factors, so a state registry makes sense for ALS, Dr. Stommel said. Diseases like the Zika virus and AIDS have mandatory tracking, so why not ALS? And while a National ALS registry already exists, it is strictly voluntary.

In New York, as in other states with harmful algae blooms, it is incumbent on residents living in lake communities to be mindful of the danger and to keep themselves and their pets away when signs are posted. There is also a New York State online reporting system and an interactive map residents can access to keep up to date on recent algae blooms.

LOCAL RESPONSE TO ANECDOTAL EVIDENCE OF ALS CASES

Chautauqua County health officials say they are sympathetic to the devastating diagnosis of ALS, but as a local health department, they are limited in scope in responding to information that has not risen to a level that would cause the state or federal government to react.

Past interim Public Health Director and Chief Medical Officer Dr. Michael Faulk told The Post-Journal that one of the biggest challenges regarding ALS is gathering the appropriate amount of data. “ALS is not a reportable illness within New York State,” he said. “Trying to wrap our head around the true incidence and prevalence of a disease like this is very difficult.”

Without data, Dr. Faulk admitted, the Health Department can’t endorse the notion that the incidence and prevalence of ALS is high in Chautauqua County. “We recognize that ALS is a devastating illness. We recognize there’s a growing body of research on the impacts of harmful algae blooms with ALS. We want to see more research. But we at the Health Department cannot make a broad blanket statement to say that the incidence and prevalence is higher in Chautauqua County than elsewhere.”

Dr. Faulk noted they are open to the idea of better state-wide reporting of ALS.

A community with a health or environmental concern sometimes needs a champion to create change. In New Hampshire, Dr. Stommel raised the alarm about the number of ALS cases he was seeing in his practice. It began as an observation and evolved into a study, which is one way in which data is generated.

LOCALS LIVING WITH ALS

During her initial appointments, Cara’s doctors in both Miami and at Duke University in North Carolina, where she travels for both traditional and non-conventional treatment, expressed a keen interest in her living near a lake with harmful algal blooms It’s one of several suspects the ALS research community points to in looking at potential causes.

“One day, late summer of 2022, I was walking my dog by the lake,” Cara recalls. “It was during the worst algae bloom. I’d never heard anyone say, ‘Don’t go near the lake.’ I’d heard that I shouldn’t swim in the water, or let my dogs in the water. I can’t help but wonder if that walk was the trigger for me.”

Cara points to a 2018 study by Florida Gulf Coast University that showed evidence airborne cyanobacteria toxins may travel more than a mile inland on the wind.

It’s important to note that science has not yet explained why some people contract ALS and others don’t. Recent research has suggested that genetic mutations might be part of the story.

Carol Shillace, who was diagnosed the same month as Cara and lives less than a mile away, looks to her past for clues as to what may have brought ALS into her life. She was in the army reserves for more than 18 years and was assigned to a field hospital where both training and sleeping took place outdoors. She also grew up in an agricultural area and has lived near Chautauqua Lake for 37 years.

Like Cara, she is worried about the risk the harmful algae blooms may pose for people and would like to see county or state health officials impose mandatory reporting for neurodegenerative diseases. Carol has been active in the community contacting local ALS patients and her own research has shown there are currently five active patients in southern Chautauqua County and two deaths from the disease within the past six months.

Both Cara and Carol have led full and active lives, and neither was prepared for the blow brought by their diagnoses. In the year that has passed, both women have experienced a progression of muscle atrophy. Carol has opted for a feeding tube to help keep weight on and is bravely dealing with the psychological ramifications of her illness.

“I was devastated when I got this diagnosis,” she wrote in an email. “I became very depressed and had panic attacks. I totally lost my appetite and quickly lost 20 pounds. I became more proactive and read a lot about the disease and treatment for symptoms.” Carol noted that the two medications prescribed to her haven’t slowed the progression. She says she stopped taking both because of side effects. “The one medication that I was given costs $158,00 a year and it’s now been taken off of the market.”

Carol says she’s learned to live one day at a time, and recently began a spiritual practice to help her cope.

“There is always that fear that creeps in because things can change quickly.”

ALS is a debilitating neurodegenerative condition that progressively impairs nerve cells responsible for muscle control, leading to muscle wastage, paralysis and, ultimately, death. At present, the average life expectancy of an ALS patient post-diagnosis is two to five years, although great progress by researchers is giving patients hope. In the past year, a team of Canadian researchers found that targeting an interaction between two proteins present in ALS-impacted nerve cells may halt or reverse disease progression. It may take years, however, before such a discovery is translated to human use in a clinical setting.

POLITICAL AND ENVIRONMENTAL ACTION

Chautauqua County Executive PJ Wendel hosted a Lake Symposium on June 2 in Celoron, focusing on the health and sustainability of Chautauqua Lake.

Several topics were discussed, including how local governments and organizations are working to combat harmful algae blooms. Stakeholders, residents, and elected officials gathered to pour over the latest data and hear updates on efforts to manage the complex environmental issues posed by the lake.

“We really need to look at what are the best practices and get an understanding on what causes these harmful algae blooms,” Wendel told Erie News Now before the conference. “We are really engaging and invested in getting as much knowledge as we can for the lake.”

Wendel underscored the amount of support the algae issue has received from agencies and organizations like Rensselaer Polytechnic Institute, SUNY Oneonta, The Jefferson Project, and the New York State Department of Environmental Conservation, among several others.

State and local officials have made it clear that understanding and managing harmful algal blooms is an important priority, and initiatives like the Jefferson Project and lake monitoring programs signal their investment in this complex problem. But for those concerned that HABs may have serious effects on human health, they hope more research will be done, not just on the management of toxic algae, but on the effects it may have on human lives.