Stigma, or the devaluation of a person is a huge and pervasive problem in HD. In varying degrees, it burdens virtually all in families touched by this disease. Stigma is a two-edged sword: an outside cutting edge from public and intra-family sources, and an equally destructive inside cutting edge that is self-directed.
The factors that may contribute to stigma in HD have never been studied. But learning from studies of stigma in other diseases, it is likely due to a combination of several characteristics that define the disease: chorea, mental illness, cognitive loss and genetic basis of illness. Part 1 of this series will focus on chorea and how this symptom may contribute to stigma.
Chorea Chorea is HD's most visible symptom, and it likely contributes to stigma. Though not studied in HD, abnormal body movements and related stigma has been examined in Tourette Syndrome (TS), and to a lesser extent in Parkinson's disease (PD).
Tourettes: TS is a condition that has many similarities to HD. Though other symptoms can occur, TS is characterized by uncontrollable movements of the extremities or "tics". As in HD, the severity varies among individuals. Also like HD, the TS individual is not aware of the body movement although observers certainly are.
A recent study described how those with TS are perceived in public to address the question of why TS stigma is severe [Davis KK 2004]. Investigators showed that body movements are highly attention getting, and are psychologically disruptive to the public viewer. The TS individual is perceived as being out of control, which in turn can generate fear and anger among observers, particularly if the movement threatens to go into another's social space. And like HD, the body movements and abnormal gait are mistaken for, and automatically associated with alcohol or drug abuse -- that serves to further increase stigma. The degree of motor symptoms correlates with stigma, and in turn may result in frustration and anger directed at the observer.
As quoted in the Davis article, those with TS describe these reactions of people in public:"the staring, the strange looks, fear, the anger, hostility, or avoidance, or being stared at on the subway, edged away from at a bus stop or in line at the grocery store, pointed at outright by a whispering unthinking child. " These public reactions to TS are very consistent with those that occur in HD when chorea is prominent.
In TS, stigma is considered an important reason for drug treatment of tics. The "response of the environment is an important determinant of how disabling this symptommay be"[Sandor P 2003]. The author used "environment" to refer to both public and family situations.
Parkinson's: In PD certain parts of the movement disorder, the freezing of gait -- which disrupts walking -- is correlated with more stigma [Moore O 2007]. Younger age of onset in PD (less than 50) where more severe motor complications occur also correlates with stigma levels [Schrag A 2003]. Might a similar finding occur with a study in HD: more chorea, more stigma?
Summary: The mechanisms that cause public stigma to tics in TS are likely the same as those that chorea causes in HD: Like the tics of TS, chorea is highly visible, the HD individual similarly appears out of control with movement into others' physical space, and the gait is mistaken for alcohol abuse. Contrast this with Parkinson's disease (PD) for which there is lesser stigma than that of TS or HD: the movement disorder of PD is less visible and much more physically constrained in space and is not perceived as threatening by the public. But, even in this disease, stigma correlates with severity of visible motor symptoms.
Editor's comments: In a perfect world, we -- and society -- could separate the movement disorder from the person within, or we could all generate thicker skins so stigma wouldn't hurt -- and harm -- as much. But until the world is more perfect, might a greater effort to treat chorea decrease public stigma (at least that related to this symptom) for HD individuals and their families?
My opinion: Chorea is harmful. Just like tics, chorea harms by creating stigma -- and just like tics, when chorea is severe enough to contribute to stigma, this is reason enough for a treatment trial with care to balance benefit against the risks of drug treatment for each individual.
Davis KK, Davis JS, Dowler L. In motion, out of place: the public space(s) of Tourette Syndrome. Soc Sci Med. 2004 Jul;59(1):103-12. PubMed abstract
Sandor P. Pharmacological management of tics in patients with TS. J Psychosom Res. 2003 Jul;55(1):41-8. PubMed abstract
Moore O, Peretz C, Giladi N. Freezing of gait affects quality of life of peoples with Parkinson's disease beyond its relationships with mobility and gait. Mov Disord. 2007 Nov 15;22(15):2192-5. PubMed abstract
Schrag A, Hovris A, Morley D, Quinn N, Jahanshahi M. Young- versus older-onset Parkinson's disease: impact of disease and psychosocial consequences. Mov Disord. 2003 Nov;18(11):1250-6. PubMed abstract