Dustin Keelty was plagued by one question long after he was diagnosed as having amyotrophiclateral sclerosis (aka Lou Gehrig’s disease). Why me?
Although he was not feeling sorry for himself the Milton man wasn’t. Keelty wanted to find out how cosmic forces combined to give him this rare, incurable, fatal illness.
Scientists have long struggled to answer this question, despite the fact that around 90% of ALS cases, including Keelty’s, have no genetic explanation. Researchers at Dartmouth-Hitchcock medical centerI was particularly interested to see if there is a link between the fatal illness and cyanobacteria which is the cause for the stinky algal blooms that plague some Vermont lakes every year.
Keelty was diagnosed at 41 in late 2016. He was an avid kayaker, and scuba diver. He was certain that he had been exposed. He knew of five other people in Milton with ALS, including his former assistant in the town’s Public Works Department.
“He just thought there were.” Had“There must be something out here causing it,” Karen Keelty, his wife of 21-years, stated last week.
A bill that is currently before the state legislature could help identify trends by mandating that health care providers report ALS diagnoses in a central registry. Researchers would have access to certain demographic details, including where the victims live. Researchers could use this information to investigate possible risk factors and correlations between cases. It could also help in the search for a cure.
“There are registries for cancer. There are infectious diseases registries,” said Dr. Elijah Stommel at Dartmouth-Hitchcock. “ALS is a fatal illness. It’s hard to track all these patients. If you want to do accurate and reliable epidemiology, you must be able identify all cases within a population. This shouldn’t be difficult in a state like Vermont.
ALS is a paralytic condition in which the affected person becomes physically disabled. They are unable to walk, talk, and eventually breathe on their own. In the United States, there are approximately 5,000 new cases each year. Stommel estimates that there are around 20 cases per year in Vermont. However, no one keeps track.
Despite the fact that many patients have lived longer and more productive lives in recent years, there is no cure. Patients usually die within two to five year.
Keelty was diagnosed with the disease in 2005, and he died five years later. His wife considered her family one the luckier ones since he was able to speak and breathe on his own until the end.
The U.S. Centers for Disease Control and PreventionKeep it up A national ALS registryIt is voluntary, and some cases are not reported. Massachusetts is the only state with a mandatory registry. It was established 20 years ago by ALS patients, their families, and advocacy groups. Registry of the Bay StateCollects data about patient demographics, symptoms, and treatment.
Researchers can apply to access the confidential database. This allows them to better understand the disease’s prevalence. A 2016 report from the state health departmentFor example, the study by CDC found that just two people were diagnosed for every 100,000, which is consistent with national estimates.
New Hampshire and Vermont lack a registry. But Stommel has mapped hundreds and is looking for clues in the list. Stommel said that the environment is a “key feature” in ALS, as only a fraction can be attributed to genetics.
He said, “I have a husband and a wife who both got ALS around the exact same time.” “I had a caretaker who was ALS-free and a man who lived in a Shelburne Point, Vermont house. They both died within a year. These situations are not something you can ignore.
Stommel’s research team is looking at a variety of risk factors including air pollution, pesticides, and heavy metals. But the most promising data center on cyanobacteria. Many ALS victims lived near lakes with poor water quality, he said.
Stommel and other researchers sought out airborne sources for patients who did not eat fish, swim or drink water from nearby lakes. Airborne neurotoxins were found in aerosol samples taken from blooms in New Hampshire and Vermont. Other studies also found neurotoxins in the brain tissue and lung fluids of some patients who died.
The researchers have taken particular interest in a specific cyanobacteria-produced toxin: BMAA, which some research has linked to human brain disease. One important study found that the toxin was responsible for a paralytic condition affecting a native Guam community. Scientists discovered that cyanobacteria was living on the roots of the Cycad tree and that it was producing BMAA which was then used to grow tree seeds. The seeds were eaten by fruit bats, while the native population consumed the fruit bats. The disease’s prevalence dropped as their diet changed over the years.
A 2016 study also found that monkeys who were given BMAA had neurological problems similar to Alzheimer’s disease.
Stommel stated, “Our feeling is it, as with many cancers, it’s probably due to multiple hits over many, many many years.” “The first exposure can happen when you’re young. This means that you may have been exposed in utero and then you might not be exposed again until you’re 10 or 15. Most ALS patients are between 55-70 years old. “It’s possible that it takes a few hits from toxins over the course of time before you break the camel’s back,” says Dr.
However, not everyone is convinced that there is a link between cyanobacteria blossoms and ALS. According to the Vermont Department of Health, a toxicologist told Seven DaysIn 2016, published research on the topic was considered “epidemiologically inadequate” and failed to account for other contributing factors, such as age and previous exposure to pesticides or metals.
The department hasn’t seemed to have changed much since. An FAQ on the cyanobacteria web site Stommel acknowledges Stommel’s cluster research, but says it is “very early and hasn’t been proven.”
Stommel claims that Dr. Mark Levine, Health Commissioner, has never supported a registry. He and Dr. Rup Tardan, a neurologist at the University of Vermont Medical Center sent Levine a detailed proposal more than a decade ago. Stommel stated, “It was Dr. Levine’s opinion that it wasn’t a common disease and there wasn’t enough interest to have a registry.”
Although lawmakers who support the registry bill don’t know the cost, Stommel estimated that it would cost less than Vermont’s.
A spokesperson for the health department issued a written response to specific questions.
It said that ALS is a deadly disease with no known cause. “The Department of Health believes that more research is needed in this area, and we support high-quality epidemiological research on ALS. It is not clear if a registry is the best approach to that research in Vermont.
Stommel believes the department’s reluctance to cooperate stems from more that healthy scientific skepticism. He said that a Vermont health officer who had criticized our research said it could have an impact on tourism and people who rent their houses on the lake.
He stated that he did not intend to alarm anyone. “My intention is not to alarm anyone,” he said. Registry is one part of that infrastructure.
The registry bill, which was introduced one year ago, but didn’t get anywhere in a COVID-19-dominated session this year, seems to have a better chance. The Senate Health and Welfare Committee, chaired by Sen. Ginny Lyns (D-Chittenden), will begin reviewing the proposal this week.
Karen Keelty, Dustin Keelty’s wife, didn’t know about the bill until she spoke with him. Seven DaysBut she said she fully supports this idea. She believed Dustin would have supported the ALS community, given his passion.
Dustin was a frequent participant in online support groups following his diagnosis. He shared his number with strangers and encouraged them all to call him with questions. The ALS AssociationA national nonprofit, started referring newly diagnosed patients to him. He then went on mentor to five to six others in this way. Karen said, “He was such an optimistic light.”
Dustin would talk to people about what to do in the first few days and weeks following the life-altering news. He would then encourage them to contact the national ALS Registry, explaining that it might help researchers to understand the causes of the disease. Numerous studies have cited the database over the years, which explore the link between ALS and environmental factors.
Keelty knew that he would be dead before there were any major medical breakthroughs. Karen stated that Keelty felt the same way. “But, he just felt like, If it can help somebody down the road then it was going be worth it all.”