There will be many historical commemorations in the year ahead in the United States — the signing of the Declaration of Independence foremost among them. One of local note and of interest to medicine marks the day at the end of June 1806 when Phebe Hedges of East Hampton drowned herself by wading out into the ocean.
Phebe Hedges is the nominal center of a book I only recently began reading by Alice Wexler, “The Woman Who Walked Into the Sea: Huntington’s and the Making of a Genetic Disease.” It is fascinating to see how a tragic event 200 years ago helped advance science. As Ms. Wexler observed in the book’s introduction, “. . . these traces of the past speak to a historical moment when families and physicians in this town invented new knowledge of heredity and illness, . . .”
Hedges was 42 when she ended her life, suffering from what was then known as St. Vitus’s dance, a curse passed down within families. It was not until the 1870s that Dr. George Huntington defined the disease, an invariably fatal neurological disorder. Children of people with Huntington’s have a 50-50 chance of developing symptoms at some point during their lives.
The specific gene that causes Huntington’s was not discovered until 1993. A possible breakthrough for its treatment is in clinical trials. In a small three-year study, an experimental gene-therapy drug implanted in the brain appeared to slow the disease’s otherwise inexorable progress. Other research is ongoing. The developer of the new approach hopes to get accelerated approval from the Food and Drug Administration as early as this year.
Interesting for me at a personal level is that there are Hedges family members in my own family line. In fact, Phebe, a Tillinghast who married into that family, is a fourth great-grandmother on my father’s mother’s side. Small town, indeed.