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The Woman Who Walked into the Sea: Huntington’s and the Making of a Genetic Disease by Alice Wexler

imagesThe Woman Who Walked Into the Sea, published in 2008, is a historical account of Huntington’s disease in America. Alice Wexler, author of Mapping Fate and sister to geneticist Nancy Wexler, painstakingly documents the disease in East Hampton, Long Island families from its earliest historical appearance to the present. The book is rich with documents, and Alice Wexler grounds the history of Huntington’s disease (HD) through individual narratives and discussion of broader social movements.

Wexler argues that early perceptions of HD differed a great deal across communities. Although some individuals with HD were vilified, records of social standing and wealth indicate that others held prominent positions in their communities. She argues that the illness gained clinical interest in the 19th century because of a changing understanding of heredity(1). Heredity became central to practical breeding, conversations about race, and in tracing causation for disease and social ills. George Huntington’s paper on “hereditary chorea” appeared in 1872(2), and by the mid-1880’s, clinical reports of “hereditary chorea” began to appear in many countries.

By the 20th century, heredity became a legitimate focus of scientific inquiry and Huntington’s was the topic of many eugenic studies. Davenport, a prominent biologist based out of the Eugenics Record Office in Cold Spring Harbor, theorized that Huntington’s presence in the United States could be traced back to a few Connecticut colonists. Relying on tenuous diagnoses and hearsay accounts of family histories, this was the first origin theory of HD in the U.S. Another researcher, Vessie, connected the disease to individuals accused of witchcraft, criminal acts, and sociopathic behaviors. His work would not be fully discredited until the 1970’s, and helped legitimate prejudicial associations in the medical literature for decades.

Like many diseases, Huntington’s had been linked to social class in the earliest reports of the disease. Davenport tied HD to other nervous disorders and ‘feeble-mindedness,’ and held that a small number of individuals resulted in an expansion of disease across the US. This strengthened the call for eugenic legislation. The 1920’s through 1940’s were a dark point for people with Huntington’s and other genetic disabilities and diseases(3). For unknown reasons, American publications on Huntington’s disease decreased in the 1920’s, and articles on treatment and therapeutics disappeared. In 1927, the Supreme Court upheld a Virginian program of sterilizing the mentally disabled. In 1935, a committee of the American Neurological Association defended the practice of sterilization with the consent of a patient or their caretakers, and called for restrictions on marriage for conditions like Huntington’s.

Negative portrayals of Huntington’s did not begin with eugenics, but the movement increased these portrayals in medical writings and occasionally in the popular media. However, by the 1970’s, with the backdrop of civil rights, informed consent, and feminism, the cultural landscape around HD had changed. In 1976 and 1977, public Congressional hearings were held to respond to demands of families affected by HD. Some two thousand people, mostly family members, caregivers, and health professionals, came forward to discuss their experiences. Family members placed emphasis on the need for social services and affordable facilities for long-term care. They called for more informed and sympathetic physicians, more research for a treatment and a cure, and the need to end the stigma of talking about the disease.

Wexler’s very thorough book emphasizes how HD can be understood as part of “larger cultural narratives.” She emphasizes that rather than simply dismissing the history as an example of bad science, we need to recognize that substituting scientific fact for superstition does not necessarily reduce stigma and prejudice. For example, knowledge of the Mendelian dominant inheritance of HD and vague notions of a familial curse coexist just as easily with arguments for eugenic sterilization. Wexler writes, “science is always a complex construction that involves arguments about meaning and value.” As we continue to search for treatment and a cure, constructing scientific knowledge in ways that benefit the HD community is tightly linked to our politics and priorities.

Key Clarifications
(1) Medical literature on HD was considerable by the early 1800’s.

(2) Although HD would be named after him, George Huntington was not the first to describe symptoms of HD. Several 19th century Americans and at least one Norwegian described the illness before him. Medical historians have speculated that early modern European portrayals of chorea and St. Vitus’ dance may have also included cases that would be diagnosed as HD today.

(3) The eugenic movement began in the late 19th and early 20th century, but the most hostile representation of HD in American medical literature emerged in the decades following World War II.