A Cruel East Hampton Legacy

Tracing a family history of Huntington’s disease
At the East Hampton Library Susan Gandolfo MacNeill discussed her self-published book about her grandparents and her family’s connection to Huntington’s disease. Judy D’Mello

“Driving with my father through a wooded road leading from East Hampton to Amagansett,” wrote Dr. George Huntington, who was then 8 years old, “we suddenly came upon two women, mother and daughter, both tall, thin, almost cadaverous, both bowing, twisting, grimacing. I stared in wonderment, almost in fear. What could it mean? My father paused to speak with them, and we passed on.”

What it meant was that Huntington’s disease became officially recognized in 1872, after young Dr. Huntington included the above excerpt in a paper called “On Chorea,” which he presented before an audience of physicians in Ohio. In the paper, he highlighted his father’s and grandfather’s — both doctors who practiced in East Hampton — personal accounts of patients stricken with “that disorder,” as it was then called.

The condition was known at first as Huntington’s chorea, from the Latin and Greek words meaning “group of dances,” which described the involuntary muscle jerks and flailing limbs, the “twisting” and “bowing.” East Hampton became inextricably linked to it, as documented in books and several articles in this newspaper.

As for its true origins, Susan Gandolfo MacNeill, a descendant of one of the first settlers of the town, maintains that this debilitating hereditary disorder can be traced all the way back to Henry VIII.

Ms. MacNeill recently self-published a book titled “Grover and Elizabeth: An Amagansett Tragedy.” On Oct. 28, in a reading at the East Hampton Library, she explained the “tragedy” in the title. In the early 1900s, her maternal grandparents, Grover and Elizabeth Payne, were living in Amagansett when Grover was diagnosed with Huntington’s, an invariably fatal genetic condition that causes the slow death of brain cells, jerky body movements, and the eventual loss of speech, among other effects. Grover was institutionalized in a Central Islip asylum, where he remained for almost 20 years until his death in 1946 at 61. His wife was left not only to cope with the stigma of the disease, at the time considered more of a curse, but to care for seven young children, with no means to earn an income. 

While researching family archives, Ms. MacNeill writes, she was able to trace her lineage back to Lady Jane Grey, the grandniece of Henry VIII and a first cousin once removed of Queen Elizabeth I. She became fascinated by the Tudor king’s recorded medical history, which famously cites his mental decline from a jovial, charismatic, and athletic young man into an increasingly paranoid, brutal tyrant. 

Indeed, the monarch’s transformation has attracted many medical diagnoses and theories, leading a pair of American bio-archaeologists in 2011 to start a movement to exhume his remains. They believe that through genetic studies they can prove he suffered from McLeod’s syndrome, a progressive neurological disorder causing behavioral changes and peripheral neuropathy. The current Queen Elizabeth’s permission for an exhumation is necessary, which, according to published reports, seems unlikely. 

And that, said Ms. MacNeill, is a shame, because she believes the king suffered not from McLeod’s but from Huntington’s, which he passed on to subsequent generations until a descendant carrying the mutant gene arrived in East Hampton circa 1650.

Today it is known that individuals with a parent affected by Huntington’s have a 50-50 chance of inheriting the gene, which, when inherited, never skips a generation. Symptoms usually manifest in adulthood, somewhere between 35 and 55, with death occurring from 10 to 25 years later. With no cure, it is not surprising that Huntington’s patients often suffer from severe depression. About 30,000 Americans have Huntington’s, most famously the folk singer Woody Guthrie, who died of it in 1967.

Two hundred years ago, people afflicted by the yet-to-be-identified disease were believed to be possessed by devils, and were stigmatized. Although largely unsubstantiated, there are claims that at least one of the alleged witches executed in Salem in the 1690s had Huntington’s.

Which brings us back to Ms. MacNeill, who is convinced that skeletons in her family’s closet include those of witches. The author, who grew up in East Hampton and Queens and now lives in the Catskills, where she taught elementary school for 25 years, believes that two of her direct ancestors were stricken with Huntington’s and fled Massachusetts to avoid the witch hunt. 

“With whispers of witchcraft, there were two families who couldn’t afford to stay in Salem. Those were the Mulfords and the Paynes, from whom I’m descended. These families knew they’d be targeted if they began to have symptoms of HD. It made East Hampton a safer place to be,” she writes in “Grover and Elizabeth.”

Whether a respite from persecution or simply an isolated place to live with the dreaded disease, East Hampton soon became a Petri dish for Huntington’s, due to the prevalence of intermarriage among prominent families. Ms. MacNeill is not only related to the Mulfords, who are known to have had Huntington’s, but to the Paynes, Bennetts, Millers, and Hedgeses.

“Basically, everyone in the cemetery is related to me,” she said. Her mother’s brother Edmund was diagnosed with Huntington’s and she believes her mother had it too, although she only suffered from hand tremors and was never officially diagnosed. Her brother, she believes, was also a victim, although he was diagnosed a schizophrenic and refused to be tested for Huntington’s.

Advancements have been made in HD treatment. In 2016, researchers announced that a “gene-silencing” drug had been found. In May, a worldwide campaign called HDdennomore (pronounced “hidden no more”) was launched, recruiting Pope Francis to help lift the stigma, particularly in poor communities, that still shrouds the disease. In 2015, a Huntington’s-blighted family in the United Kingdom had a genetically engineered baby born without the mutant gene.

In her book, Ms. MacNeill, who is almost 80, writes that her aim is “to draw attention to eliminating the dread of Huntington’s chorea, for all my remaining family and others around the world who also have it.” Like her brother, she has refused testing. Huntington’s is too terrifying a fate, she said

Ms. MacNeill at about 5, with her uncle Edmund Payne, home for the funeral of his father, who died of Huntington’s disease. Some years later, he proved to have the disease himself. Courtesy of Susan Gandolfo MacNeill