Central Hudson Gas & Electric station at Route 199 in Pine Plains near proposed access for Carson Power’s planned 10 megawatt solar project.
John Coston
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Dutchess County Board of Elections
The official results of the 2024 election have been finalized in Dutchess County as Presidential Inauguration day approaches on Jan. 20.
Dover was the only town to vote for all three Republican candidates at the federal level among the eight towns in Northeast Dutchess County. In Dover 61% of voters favored Donald Trump for President.
In Pine Plains and Stanford 18 or 19 votes decided the presidential winner with 57 votes on the conservative line in Pine Plains putting Trump over the top, and in Stanford the 87 votes on the Working Party line making the difference to Harris.
The total results in our area in the tight Presidential election where neither major party candidate won the majority reflect the close national outcome with Harris with 50.7% of votes. In contrast Harris received 53% in Dutchess County overall and 56% in New York State.
Both incumbents, Kristen Gillibrand for the Senate and Pat Ryan for the House of Representatives, outperformed Harris in every town in their races, including Dover, the only town where both lost. Although Gillibrand won 54% of votes in Northeast Dutchess, this was not as decisive as her 58.9% statewide win. Likewise, Ryan’s result in our towns of 52.6% was also lower than his district wide win of 57.2%.
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Millerton Inn kicks off the series. Veterans Park planter lights courtesy of Townscape with arrangement by Joanne Scasso at Country Gardeners.
Photo by John Coston
MILLERTON — The North East Historical Society’s Dine Out for History is returning this year with six participating restaurants.
On select dates from Sunday, Jan. 26, to Wednesday, March 19, restaurants will be hosting “Dine out for History” night. Ten percent of the profits will be donated to the North East Historical Society.
The donations will help the organization perform further research, digitize more of their collections and create content available for the public, especially teachers.
In conjunction with the North East Historical Society, the Millerton Inn will kickstart the celebration on Sunday, Jan. 26. Attendees may participate in an optional short presentation and a local quiz, beginning at 5:30 p.m. Attendees of the presentation will be offered a free glass of wine to enjoy with their meal at the Inn afterward.
More participating restaurants are Taro’s on Thursday, Feb. 6; The Oakhurst Diner on Sunday, Feb. 16; Willa on Thursday, Feb. 27; Round III, on Monday, March 10; and the Golden Wok on Wednesday, March 19, takeout only.
To ensure a table is available for your party, reservations are preferred for most participating restaurants.
For a reminder of this upcoming event, please sign up for the North East Historical Society newsletter by contacting Ed Downey at eddowney12@gmail.com.
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Aevary Kiernan, left, with her mother Jill Kiernan.
Photo by Jill Kiernan
STANFORDVILLE — Shortly after her daughter’s birth 21 years ago, Jill Kiernan realized baby Aevary was not progressing in the same manner as many newborns generally do after birth.
Kiernan, at the time a general education/special education teacher who lived — and continues to reside — in the Pine Plains area, was baffled by what she was seeing. Describing Aevary as “a floppy baby,” the infant was large, 9 lbs. to be exact, with low muscle tone, her tiny feet were turned down, there were difficulties with feeding, and the infant was slow to develop. While Jill Kiernan didn’t realize it at the time, she was at the beginning of a decade-long quest to get some answers on her daughter’s condition.
Clarity did not come easily or quickly. At the time of Aevary’s birth, Tatton Brown Rahman Syndrome, the condition affecting Aevary, was not yet identified by the medical community. In fact, the condition would not be pinpointed until 2014, designating it as a more recently discovered disorder.
One of the first steps in understanding Tatton Brown Rahman Syndrome is the knowledge of what constitutes a syndrome versus a disorder versus a disease. By definition, a syndrome is marked by groups of symptoms which simultaneously occur while a disorder is defined as a group of symptoms disrupting normal and daily functioning, resulting in significant impairment. Disease is an already identified medical condition with a distinct cause and with measurable components. In the case of Tatton Brown Rahman Syndrom, with the research and data in place, though limited, it is now all three — a syndrome, a disorder, and a very rare disease indeed which, according to Kiernan, affects about 450 identified persons worldwide.
At Aevary’s birth, there was little to no information available in the medical journals as to the source of her condition. Aevary’s growing list of symptoms mystified everyone around her — including health professionals, who were unable to provide the Kiernan family with any definitive insight. With the passage of time during Aevary’s childhood, additional issues would present themselves. Aevary, of course, was large from birth, but her physical growth accelerated at an abnormal rate. Unusually bushy eyebrows sprouted above her eyes. Developmental delays and intellectual challenges surfaced. Still, the Kiernan family received no answers to their inquiries from Aevary’s healthcare providers.
When Aevary was about ten, progress was made on determining her condition, which Kiernan describes as “a long hard journey,” which was finally labeled as Tatton Brown Rahman Syndrome. The disease is a complex utosomal dominant disorder affecting children who, by chance, inherit one copy of a mutated gene from either parent. More specifically, according to the website of the Tatton Brown Rahman Syndrome Community, a research and advocacy organization founded by Kiernan in the wake of Aevary’s official diagnosis, the disease is “a rare genetic disease caused by pathogenic variants (previously called mutations) in the DNMT3A gene… It is also called DNMT3A Overgrowth Syndrome.”
First identified in thirteen cases in the United Kingdom in 2014 by doctors Katrina Tatton-Brown and Nazneen Rahman, who were researching genetic drivers of overgrowth in individuals, members of the Tatton Brown Rahman Syndrome population are physically characterized by height and weight as affected individuals tend to be tall and heavy with a large head circumference known as macrocephaly and may display unique facial features. They may present with mild to severe intellectual disability. Behavioral and mental health issues may also surface as well as cardiac defects, orthopedic issues and autism, but according to Kiernan, a range of individual presentation of symptoms is common, the severity of which can vary between persons. Additionally, the Tatton Brown Rahman Syndrome gene is also linked to incidences of leukemia.
For Kiernan, while the diagnosis shed some light, it also brought some uncertainty. “It was a relief to have an answer,” she said of pinpointing the source of Aevary’s condition. But with the diagnosis came initial vagueness attached to Aevary’s outcomes or life expectancy as very little was known at the time of Tatton Brown Rahman Syndrome due to the small number of affected persons. Undeterred, however, Kiernan realized she could play a part in guiding medical research by sharing information with other families affected by Tatton Brown Rahman Syndrome as well as researchers, hence the creation of the Tatton Brown Rahman Syndrome Community.
The Tatton Brown Rahman Syndrome Community began with the creation of a website and a Facebook page by Kiernan. Through these mediums, Kiernan connected with other Tatton Brown Rahman Syndrome-affected families who began exchanging information on particulars pertaining to their individual cases. Eventually, the families began to meet. “We began informally gathering for support,” explained Kiernan, acknowledging the value of strength in numbers. Family conferences began to be organized with one taking place in 2018 at Rocking Horse Ranch Resort in Highland, New York. “Families came from all over the world,” explained Kiernan. “Dr. Tatton-Brown came over from the U.K.”
As the Tatton Brown Rahman Syndrome Community expanded, it led to the creation of a registry, which was launched in 2021 as families began donating blood and skin samples. The registry, in turn, became a valuable tool to researchers, who previously had little information available to them. As word of the existence of the Tatton Brown Rahman Syndrome Community spread, even more cases began to be identified, which, in turn, fueled the medical community with what Kiernan terms as “patient-driven research”.
Sources of funding for further research for the Tatton Brown Rahman Syndrome Community remains a challenge due to the rarity of the condition. “It’s hard to find opportunities in the rare disease space,” said Kiernan, who added that the Chan-Zuckerberg Institute provided $600,000 in grant monies a few years ago, but the funds have since dwindled. Appropriate grants, Kiernan explained, are difficult to find due to the rarity of the disease.
While Jill Kiernan is proof positive of how one person can make a difference, she’s focused less on accolades and more on her daughter. Aevary Kiernan has defied medical expectations with her viability, given the seriousness of her condition. While she will not be able to live independently, Aevary lives the life of a young woman who, while navigating physical and emotional challenges, enjoys spending time with animals, telling jokes, acting and singing. She was even featured on a segment of “Good Morning, America” which showcased her performance in a production of “Beauty and the Beast.” Aevary has completed high school via special education through the Pine Plains school district, first at the Center for Spectrum Services and then at the Center for Discovery. She is currently attending the Tri-Form program for individuals with challenges in Columbia County. Aevary’s future is uncertain, given her medical history and the possibility of future medical challenges. She suffers from joint and spinal issues, and will soon need a hip replacement. Whatever is to come next for Aevary is, for Jill Kiernan, “a ticking time bomb feeling,” but Kiernan remains optimistic about the future given how far she and her family have come on their Tatton Brown Rahman Syndrome journey. “We’ve learned a lot living in the rare disease world,” she said.
Additional information on Tatton Brown Rahman Syndrome can be found at: www.tbrsyndrome.org.
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Troutbeck's sign at the intersection of Leedsville Road and Route 343 in Amenia.
Archive photo
AMENIA — Public hearings continued from the previous meeting concluded at the regular meeting of the Amenia Planning Board on Wednesday, Jan. 8. Having considered public comment, the board approved applications from Troutbeck for an indoor pool and from DaVinci Windows for a sign installation.
No public comment was heard in connection with the Troutbeck proposal to construct an outdoor pool as briefly described by project engineer Rich Rennia of Rennia Engineering of Dover Plains. The application is part of Phase 8 of Troutbeck’s adaptive reuse application, revised recently to reduce the environmental impact.
Plans call for renovation to the existing pool, the bathhouse and the snack bar — all contained within the wellness area — and construction of a new indoor pool inside the Amenity Barn, Rennia said, explaining that the wellness area will not expand in area, only configuration.
Planning Board attorney Paul Van Cott said that the Phase 8 plans remain consistent with previous planning board approvals and are consistent with the site plan. Board engineer John Andrews indicated that the application was in order and ready for planning board decision, clearing the way for the unanimous vote to approve.
Residents’ concerns about traffic safety were repeated during the continued public hearing to approve installation of a two by six sign at the Route 343 location of DaVinci Windows’ showroom. However, following discussion, the planning board voted unanimous approval of the sign permit, providing that a site plan be submitted showing details of the new sign.
Resident George Bistransin echoed neighbors’ concerns that the sign might pose a dangerous distraction for motorists negotiating a nearby highway curve. He also offered his opinion that conversion of a residential property into a commercial enterprise should have been subjected to further review.
Engineer Andrews reported on his visit to the site accompanied by town highway and building officials. Andrews said that the property is located in a mixed-use district and that the project renovates a single-family residence to add a commercial use.
Andrews added his findings that the sight lines would not be impacted by the sign’s location.
Atty. Van Cott advised that Andrews will need to sign off on the sign’s site plan, a plan that will show illumination details.
Board member Nina Peek suggested that approval be delayed until the site plan with sign details is submitted, but board member James Vitiello favored moving ahead with a vote to approve, pending receipt of the site plan.
Cascade Road affordable housing
The Planning Board heard a preliminary review of impending plans for a Cascade Road affordable housing development to be proposed by Hudson River Housing of Poughkeepsie.
Plans were introduced by Senior Planner Peter Sander of Rennia Engineering. The site would use the land for workforce housing, envisioning a potential layout of 28 small lots to hold single-family, 4-bedroom homes with on-site shared water and septic systems. Half of the land would be conserved, Sander said, asking the board to provide any preliminary comments to assist with preparing for the application process.
Engineer John Andrews asked for a detailed analysis of the conservation elements on the property and he said that the precise acreage needs to be determined.
Atty. Van Cott noted that the planning board is not going to be looking at the subdivision plans at the current early stage. Rather, the board will be considering the plot of land as a whole including the conservation value.
“The applicant’s job is to design around these land resources,” Van Cott added, indicating the presence of a stream corridor at the site.
Board member Vitiello asked about impact on any wildlife to which Van Cott responded that the applicant would need to acquire the services of a professional biologist who would define any habitats. An alternative, Van Cott noted, would be for the Planning Board to hire a biologist at the applicant’s expense.
Spotted salamander is present at the site, as noted by board member James Walsh who indicated that he has caught trout when fishing the site.
Water and septic concerns were of interest to board member Kenneth Topolsky, including water quality, wastewater treatment plans, and run-off from storm water and Cascade Creek and how it might affect surrounding properties.
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