A mother’s advocacy results in answers

Aevary Kiernan, left, with her mother Jill Kiernan.
Photo by Jill Kiernan
Aevary Kiernan, left, with her mother Jill Kiernan.
Correction: An earlier version of this story misstated Aevary Kiernan's need for a knee replacement, and did not specify that a majority of cases of Tatton Brown Rahmann Syndrome are caused by random genetic mutations that were not inherited from parents.
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 autosomal dominant disorder affecting children who, by chance, inherit one copy of a mutated gene from either parent — but most cases occur because of a random mutation not present in the parent's DNA. 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 knee 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.
The LED sign in front of Amenia's Town Hall on Route 22 warns passing motorists of the current temporary burn ban, in effect from Oct. 2 to Oct. 15.
A statewide burn ban is in effect as of Thursday, Oct. 2, the New York State Governor's office announced in a press release.
The temporary ban is in effect until at least Oct. 15, and the statement released by the governor's office said the restrictions will be re-evaluated prior to the Oct. 15 deadline.
Lighting fires for brush or debris disposal and large, uncontained fires for cooking or other purposes are banned until at least Oct. 15 under the statewide order. Backyard fire pits, contained camp fires no larger than 3 feet in height and 4 feet in diameter, and small, contained cooking fires are still permitted under the burn ban.
New York State has faced dry conditions all autumn, triggering drought watches and warnings across most of the state.
Dutchess County, along with the other counties in the Catskills region, are under a drought watch according to the Department of Environmental Conservation's drought condition map.
Dutchess County is currently under a "high" fire danger rating, according to the DEC.
LAKEVILLE, Conn. — Barbara Meyers DelPrete, 84, passed away Tuesday, September 30, 2025.
A Funeral Mass will be celebrated Saturday, October 4, 2025, at 11:00a.m. at St. Mary’s Church, 76 Sharon Rd., Lakeville.
A complete obituary will appear in next week’s Lakeville Journal.
To offer an online condolence, please visit ryanfhct.com.
Volunteers were hard at work putting the finishing touches on the crucial creepy decorations for the Haunted Fortress of Stanford on Sunday, Sept. 28.
STANFORD — Greg Arent led a devoted team of volunteers on Sunday, Sept. 28, in a final push to prepare Stanford’s Haunted Fortress for opening day.
Final touches included cleaning the bottomless pit, scrubbing the pirate ship, raking the graveyard and dressing the dolls. By 2 p.m., about 20 volunteers had assembled to creepify the beloved local landmark.
Many of the volunteers have been coming back for years, Arent said, dedicating time and valuable skills to the town-owned haunted house that was constructed by the artist Peter Wing.
Arent has been building sets in his free time for forty years, but he started out volunteering with the Haunted Fortress in 2014 when his children wanted to get involved, he said. At that time he would help out one or two days a year. That grew into a leadership position over the course of a decade.
On Sunday, Arent was touring the decrepit grounds checking light bulbs, soundtracks and other set details to contribute to that perfect spooky ambience.
Nathan Miller
When it’s all said and done, the Fortress will be ready to welcome visitors starting on Friday, Oct. 3, with shows running from 6:30 to 9:30 p.m. on Fridays and Saturdays and 6:30 to 8:30 p.m. on Sundays. The Haunted Fortress is entirely staffed by volunteers, Arent said, including the actors that don costumes to scare visitors. Anybody aged 6 and up can volunteer to act in the horror show, and families often will volunteer to take over a scene.
“I love it because every scene is different every night,” Arent said. “Sure there are going to be vampires in this scene, but this family is going to do it different from these three kids from Bard, who are going to do it different from these three middle school kids.”
The sets themselves were all built from recycled material to be as creepy and decrepit as possible, Arent said. Concrete culverts adorn the sides of a bridge over a stream, stacked vertically with a cone on top to elicit a fortress’s spires. Reclaimed lumber bolted to a fence evokes a dark and scary forest blocking any escape. A rusting tractor with a steel box welded above the seat sits on a set of rails with two cars attached to it, mimicking a wrecked train with it’s inner mechanisms exposed and blowing steam on passersby.
This year the Haunted Fortress is following a haunted circus theme featuring clowns and other carnival staples alongside the classic settings of the Fortress.
The team of volunteers is dedicated and numerous, and the Stanford Highway Department lends a hand too, Arent said, but there’s always a need for skilled labor at the local attraction.
And the group appreciates all the help they can get. “Whatever you’re interested in and capable of doing,” Arent said.
The haunted pirate ship marks the spot where groups of visitors are paired with their ghastly guides for the remainder of the Haunted Fortress tour in Stanford.Nathan Miller
From left, Jim Milton, Spencer Parks and William J. Clark stand with Joseph Olenik on their first day working under him as the head of Public Works on Monday, Sept. 29.
MILLERTON — The Village announced a leadership change at the Highway Department on Friday following the resignation of Superintendent Peter Dellaghelfa. Police Chief Joseph Olenik will step into the role, effective immediately, officials said.
Olenik will continue to serve as Police Chief.
The decision was made Friday, Sept. 26, during an emergency meeting of the Board of Trustees following Dellaghelfa’s resignation.
Mayor Jenn Najdek said the “amicable split” with Dellaghelfa was part of an ongoing annual employee review process, adding that turnover is not uncommon in small municipalities. She expressed her gratitude for Dellaghelfa’s service over the last four years.
The Board went into Executive Session on Aug. 26 and Sept. 9 for “matters leading to the appointment, employment, promotion, demotion, discipline, suspension, dismissal or removal of a particular person,” but village officials could not comment on the specifics of those discussions.
The leadership shift will allow the Village to “better respond to the changing needs of our residents and community,” announced the Board in a press release shared with The News. “The Mayor and Board are excited to see Joe expand his impact on Millerton and we’re confident this change will bring lasting improvements to operations, communications and community engagement.”
Police Chief Olenik, who will continue his leadership role within the Millerton Police Department, said he is looking forward to the added responsibilities.
“I am excited to help move the department forward and rebuild it after the devastating fire,” Olenik said. “I am also looking forward to working with the dedicated staff and helping the residents of Millerton by making needed changes and repairs.”
Though Olenik will assume additional responsibilities as he leads the Highway Department, the Millerton Police Department will continue to operate separately — but with the same shared goals of supporting the village community.
William J. Clark installs the leaf container he and his colleagues built. Leaves from the Village will be delivered to McEnroe’s Organic Farm for composting.Aly Morrissey
Mayor Najdek added that Olenik has extensive supervisory and administrative experience that, given the imminent need to rebuild the Highway Department building, will be critical. In addition to managing the upcoming construction, his first orders of business will include making sure the village is on track for leaf pick-up and snow removal, working to ensure the right equipment and personnel are in place.
Olenik held his first meeting with the Village Highway Department on Monday, Sept. 29 and discussed a new leaf container — built by Millerton’s Highway Department — that will support efforts to collect leaves. The leaves will then be turned over to McEnroe’s Organic Farm for composting. While the team configuration — which includes Jim Milton, William J. Clark, and Spencer Parks — will be new, Olenik said they are not strangers. “They’ve always helped me out and I’m looking forward to working with them more closely.”
The Board said in a statement the Village is in good hands moving forward. “Joe’s leadership style — hands-on, structured, and community-focused — makes him well suited to guide this department through the transition.”