Huntington's disease (HD), formerly known as Huntington's chorea (popularly called St.Vitus's dance) is an inherited brain disorder whose most famous exemplar is the Oklahoma singer and songwriter Woody Guthrie (1912-1967). It is characterized by involuntary movements (chorea), personality changes, decline in cognitive abilities, and emotional disturbances such as apathy, irritability and depression. Once it begins--typically in the thirties or forties though it can be much earlier or later--it inevitably advances over ten to twenty years, with no means yet available of stopping or ameliorating its course. Huntington's chorea attracted the attention of early twentieth century eugenics leaders on account of its onset in mid-life and the fact that most of those at risk for the disease had their children before any symptoms appeared. Moreover the autosomal (not sex-linked) dominant inheritance pattern meant that each child of an affected parent had a 50-50 chance of inheriting the illness and passing it on to future generations. (Only those inheriting the genetic abnormality are in danger of passing it on but those who do inherit it will inevitably develop the disease).
For eugenicists in many countries, then, Huntington's was an example of a severe hereditary mental and physical disease that they believed could be prevented only through non-procreation and sterilization. In 1942, the Canadian province of Alberta added an amendment to the 1928 Sexual Sterilization Act allowing involuntary sterilization in the case of persons with Huntington’s who were deemed “psychotic” although how many persons may actually have been sterilized remains unclear.
Huntington's and Heredity
The East Hampton, Long Island (New York) physician George Huntington (1840-1916) is often credited with discovering the hereditary transmission of this disease, which he described in his classic 1872 paper "On Chorea." But earlier medical writers also described this characteristic. Even in the early nineteenth century, if not before, members of affected families and their neighbors recognized a hereditary element. In some communities as early as the 1840s, parents from unaffected families threatened their children with disinheritance and ostracism if they married into one of the affected clans. Prominent early 20th century eugenicists such as the biologist Charles B. Davenport became interested in Huntington's as a case study to investigate patterns of human heredity and promote immigration restriction. As Davenport wrote in an influential 1916 paper he co-authored with the physician Elizabeth B. Muncey, "Huntington's Chorea in Relation to Heredity and Eugenics": "All these evils in our study trace back to some half-dozen individuals...who migrated to this country during the 17th century. Had these half-dozen individuals been kept out of this country much of misery might have been saved" (Davenport, 1916).
Davenport was even more exercised about the need for sterilization. "It would be a work of far-reaching philanthropy to sterilize all those in which [sic] chronic chorea has already developed and to secure that such of their offspring as show prematurely its symptoms shall not reproduce," he wrote.. "A state that knows who are its choreics and knows that half of the children of everyone of such will (on the average) become choreic and does not do the obvious thing to prevent the spread of this dire inheritable disease is impotent, stupid and blind and invites disaster" (Davenport, 215).
Still, none of the eugenic legislation passed in the United States between 1907 and 1956 nor in Canada prior to 1942 specifically mentioned Huntington's chorea. It is unclear how many members of HD families in North America may have been sterilized without their knowledge or consent. In Germany, however, approximately 3000 to 3500 persons with Huntington's in German psychiatric hospitals were sterilized under the 1933 Nazi Law for the Prevention of Genetically Diseased Offspring, which specifically included Huntington's chorea as grounds for involuntary sterilization. Later an unknown number of persons with Huntington's, along with other psychiatric patients and persons with disabilities, were murdered in Germany during the Third Reich.
Sterilization and Stigmatization
Even after the horrors of the Nazi era, testimony before the United States Congressional Commission for the Control of Huntington's Disease and its Consequences, in 1976 and 1977, suggests that North American physicians, neurologists, and pastors continued to counsel persons at risk for HD not to have children and, in some cases, to get sterilized. Many family members experienced this advice as traumatic, an intrusion into their intimate life and an assault on their freedom of choice. For women especially such advice could be devastating. The idea that authoritative figures counselled against having a family communicated the idea that they were damaged goods and that this illness was a mark of shame. It was also an incentive to keep Huntington's secret, thereby perpetuating ignorance about the disease.
Advocacy and Advances
Eugenic assumptions also shaped early biomedical research on Huntington's, in Canada as elsewhere, giving a high priority to developing a predictive test. Many clinicians assumed that once such a test became available, individuals at risk would submit to it without question and that those who learned they carried the genetic abnormality would necessarily refrain from procreation or agree to sterilization. In fact, a presymptomatic genetic HD test did become available following the 1983 localization of the abnormal gene. However the lack of a way to prevent or moderate the illness, the psychological burden of a positive genetic test result, and the risks of insurance loss and further stigmatization meant that few persons at 50% risk have chosen to get tested as of 2014. On the other hand, the 1993 discovery of the genetic aberration causing Huntington’s (on chromosome 4, an expanded stretch of DNA involving CAG repeats that forms part of the normal gene--HTT--for a protein called huntingtin) opened up many new research strategies. It also made possible a controversial (and expensive) form of in vitro fertilization called PGD or Presymptomatic Genetic Diagnosis, permitting those who do not wish to pass on Huntington’s the possibility of having children and grandchildren free of the disease.
The stigmatization of HD families did not begin with eugenics. However twentieth century eugenic ideas and practices intensified HD’s shame and secrecy, casting a long shadow on families, care, and research. In the late 1960s the emergence of a grassroots advocacy community (Huntington Society of Canada; Huntington’s Disease Society of America; Hereditary Disease Foundation) started by HD family members such as Marjorie Guthrie and Milton and Nancy Wexler in the USA and Ralph and Ariel Walker in Canada challenged many eugenic practices while also greatly expanding research to find effective treatments for the disease. The Canadian scientists Michael Hayden and Blair Levitt at the University of British Columbia in Vancouver have also been at the forefront of research and advances in clinical treatment and care for people living with Huntington’s disease.
Davenport CB, Muncey EB. (1916). Huntington's Chorea in Relation to Heredity and Eugenics. American J. Insanity, 73, 195-222.
Wexler A. (2008). The Woman who Walked into the Sea: Huntington's and the Making of a Genetic Disease. New Haven: Yale University Press.
Bates G, Tabrizi S., & Jones L. (2014). Huntington’s Disease. 4th Edition. Oxford: Oxford University Press.
United States. Commission for the Control of Huntington’s Disease and it’s Consequences. (1977) Public Testimony. In Report: Commission for the Control of Huntington's Disease and its Consequences (vol. 4, Pt. 5). Bethsda, Md: United States National Institutes of Health.
Wexler, NS. (2012) Huntington’s disease: advocacy driving science. Annual Review of Medicine, 63, 1-22.
Huntington Society of Canada (n.d.). Retrieved from: http://huntingtonsociety.ca.
Huntington’s Disease Society of America. (n.d.). Retrieved from: http://hdsa.org.
HOPES (n.d.). Retrieved from http://hopes.stanford.edu.
Institut de coproduction de savoir sur la maladie, France.