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Aevary Kiernan, left, with her mother 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.
MILLERTON — Edward Downey, President of the North East Historical Society, recently reflected on his role in the community as town historian, how to access the archives and Millerton’s rich history.
The North East Historical Society
The North East Historical Society is a non-profit organization which aims to promote greater appreciation of the history of the Town of North East and the Village of Millerton.
“The function of the Historical Society is to essentially collect, curate, and make available to the public information about the history of the world,” Downey said of his work which also involves programming and grant-sourcing.
As Downey explained it, history can be both personal and possessive. “The two largest areas of interest, first of all, genealogy. People are trying to find family members and the history of their families,” Downey said. “The other thing people are often interested in is maybe the history of a property they own, when it was built, how long it’s in existence.”
The organization is in possession of archives dating back to the 18th century, with a handful from the late 1700s. Downey and Edith Greenwood, the vice-president of North East Historical Society, provide access to the archives through appointments Downey encourages interested parties to reach out to him for appointments by email.
The archives are located on the second floor of the NorthEast-Millerton Library. They cannot be checked out from their location.
“It’s not a museum. There’s a distinction between a museum and an archive. A museum is a space where there are exhibits,” said Downey.
Downey explained assistance is available while reviewing the archives. “We have an area where someone can sit and we can go through things with them,” said Downey, “but it’s more like research library.”
History of Millerton
Before the revolution, the boundary lines relating to the Town of North East varied over the years. In 1823, the current boundary lines for the towns were created.
“Millerton was really just kind of farmland until the New York and Harlem railroad decided to build a rail system from Grand Central Station up through Hudson, through the Harlem Valley, all the way up to the town of Chatham in Columbia County, where it linked with a railroad coming from Boston to Albany,” Downey said. “That line was started in about late 1840s. By 1851, it had gotten to what is now Millerton. And what was then just kind of farmland along the Weevitut Creek, because of the development, the arrival of that railroad, suddenly, people were building commercial buildings and they were building residential buildings all because of the railroad.”
Between 1851 and 1888, Millerton was surrounded by four different railroad systems, three which came from the western part of the county.
“It enabled the transportation, particularly of coal, to and from the western part of the county, probably from mining and other areas that were brought to those railheads to be loaded onto cars and shipped over into Connecticut where there [was] a lot of manufacturing going on,” said Downey. “Millerton became a very active railroad center because of all that. So in 1851, after the railroad had just gotten here, a group of people got together and decided to name this new hamlet Millerton.”
The Village of Millerton, named for railroad project engineer Sydney Miller, was created in 1875 as an incorporated village, creating a political subdivision for the town. The Village would ensure residents had their own municipal management, safety and more.
“At that time you couldn’t create separate districts to provide special services such as a water district or a fire service district, but Millerton... the feeling began to develop that, ‘We should have those sorts of things,’” Downey said. “And the only way you could do it then, under New York State of Law, was to create what’s called an incorporated village. It’s also still part of the town of Northeast, but it’s a special village, or certain special units of political subdivisions.”
According to Downey, there are three distinct things which have created what Millerton is known for today — landscape, talent and proximity to New York City.
“There’s a very important landscape... but it’s a rich, resourced landscape,” Downey said. “It’s supported in the early years of iron ore mining. It then supported wheat farming and apple farming... and now agriculture is more for the large sort of crops such as hay but also for vegetables and direct sale. So there’s this whole history of the community because of what its landscape is.
“The second thing that’s been important is that landscape... has attracted periodic ways of talented commercial people... whether it’s been in farming or in operating businesses in the village.” Downey said.
“The third factor... We are 100 miles from one of the largest and wealthiest municipalities in the world.”
In 2026, the 175th anniversary of the creation of the hamlet is being celebrated, thanks to the railroads which helped establish this settlement, but also thanks to its scenic features.
“Another feature of this landscape is its beauty,” marvelled Downey. “There’s just extraordinary views throughout the town and different areas.”
For more information, email Ed Downey, town historian, at eddowney12@gmail.com.
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.
MILLERTON — Calling the ongoing revitalization of Eddie Collins Memorial Park “the largest project the village has taken on,” Mayor Jenn Najdek has disclosed additional funding has come through in support of its upgrade.
This past December, the New York State Office of Parks, Recreation, and Historic Preservation awarded the village a matching grant of up to $675,000 in support of the Eddie Collins Memorial Park Swimming Pool Project. With these monies, funding for Phase II of the project, which stems largely from a NY SWIMS capital grant, is now at $7.56 million.
This most recent grant, which Najdek explained was the final one for which the village had applied, will go toward planned improvements for the park which include a new swimming pool and bathhouse, a community room and an on-site septic system. Additionally, all sections of the park will be accessible, making the venue ADA-compliant. The hope is to have complete and final designs of the project submitted over the next month or so. Completion of the project is expected by spring of 2026.
“It’s going to be great when it’s all done,” said Najdek, who acknowledged the need for quality outdoor recreation in Millerton, and who fondly recalled the swimming pool which was previously in place. “I’m excited,” she added.
The revitalization of Eddie Collins Memorial Park has been a work in progress for just under a decade, but strides have been made in recent years with the implementation of a soccer field, the recent installation of donated lights for the baseball field, and the paving of the parking lot as well as other revisions.
