I like writing, as perhaps you do, and sometimes I stop caring about whether anyone’s ever going to read my stuff. But if you put your heart and soul into baking a cake then you hope someone’s going to eat it. Same with a book. Please do eat one of mine, or at least some of it, and tell me what you think.
Chris Ward - February 7, 2020
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One of my challenges as a specialist physician was Huntington’s disease. To give you a picture, imagine 'Diane' [i] slumped in a wheelchair, stirred into speaking by something her husband has said about their 7 year old son, Will. Diane’s strong feelings are obvious but what she wants to say is not, because her speech is unclear and her body’s jerking movements make the wheelchair shudder. Huntington’s is a genetic condition and there is a one-in-two chance that Will will eventually develop problems like his mum’s. She could expect disorders of cognition and mood as well as involuntary movements, the best known of which is the irregular jerking called chorea [ii].
One of my challenges as a specialist physician was Huntington’s disease. To give you a picture, imagine 'Diane' [i] slumped in a wheelchair, stirred into speaking by something her husband has said about their 7 year old son, Will. Diane’s strong feelings are obvious but what she wants to say is not, because her speech is unclear and her body’s jerking movements make the wheelchair shudder. Huntington’s is a genetic condition and there is a one-in-two chance that Will will eventually develop problems like his mum’s. He could expect disorders of cognition and mood as well as involuntary movements, the best known of which is the irregular jerking called chorea [ii].
I want to reflect here on the patients I worked with in the hope of capturing something of their experience. What I am after is experience, not experiences: not a 'then-I-did-this’ narrative, but the mental and bodily qualities, in other words the phenomenology, of Huntington’s. Medical language has little to do with experience, as I’ve argued in a recent book [iii]. What, then, can a doctor say about his or her patient’s consciousness? Not much, but not nothing. Our bodies constrain what and how we feel, and the particular constraints of Huntington’s disease are worth thinking about. The one I will consider first is disordered movement. In subsequent pieces I reflect on two others, slowness of thinking and obsessive traits, before coming finally to the larger question of what it might be like, subjectively, to have these difficulties.
My specialist nursing colleagues and I met people like Diane in their houses, and in nursing homes, and in our clinics. The 'patient’ would often be restlessly silent, as though preoccupied with something other than our conversation, so that we often found ourselves talking around, rather than directly to, the person. I wondered then, and I still wonder, what lay behind our patients’ apparent inscrutability and what made it difficult to establish some kind of presence within their mental worlds.
I think we can be sure that people with Huntington’s have the same desire to communicate as any of us. Above is a sample from a journal (I feel able to publish them here because the person is not identifiable, 30 years have elapsed, and there was nothing secret about them at the time). The lower is several months after the upper page. Over this period, the person’s efforts to communicate seem to be outstripping the capacity to write. The journal shows the effect of abnormal movements on handwriting, which might give us a picture of a faulty TV in which 'noise’ (jerkiness) interferes with a 'signal’ (thoughts).
This simple model has been rejected by theorists of human communication and says little about the conversational efforts of someone affected by Huntington’s, where noise frequently evolves into a signal. The ghost of some meaningful expression often flits across the person’s face, sometimes fading but at other times becomes unmistakeably expressive, and a movement of the head or of the arm may either subside or else turn into a recognisable gesture, all of which makes it difficult to separate the means of communication from the message. Facial grimaces, head-nodding, shoulder shrugs and movements of the spine can be part of anyone’s communicative repertoire. In Huntington’s, similar movements are hallmarks of brain dysfunction. Should we, for that reason, dismiss them as meaningless?
The body speaks to me, and also through me. A cough is a symptom but also an utterance. It tells everyone I have a cold, it suggests that I am someone to avoid (chorea has the same effect, socially), and it can also be a conversational gambit, transmitting private messages such as 'Stop talking about that!’. In communicating with each other we use pathologies such as coughing together with facial expressions, bodily postures and limb movements, and we also resort to whatever images or objects the environment happens to contain (the floor, say; or a spoon; or a part of the body). Together, these are among the affordances of communication. I learned to use the word affordance in this way from James Gibson, a psychologist who studied perception and action [iv]. We can only expect to understand someone with Huntington’s if we attend carefully to the person’s affordances. It is difficult to notice what someone is trying to say when the body’s involuntary movements and awkward postures are interpreted purely as signs of disease, or when a lack of verbal fluency is interpreted simply as dementia. Effective communication requires imagination, patience and, above all, curiosity about that person’s experiences, ideas and feelings.
Dementia, if we want to call it that, can expose affordances that were not available to the person before the onset of symptoms. Some of the modes of expression that people with Huntington’s resort to have a tragi-comic dimension. One angry man used a mower to write c*** on the lawn. He had his reasons.
Notes
[i] 'Diane' and other characters I mention here are not identifiable individuals, but nothing in their stories is invented.
[ii] For some of the physical challenges of Huntington’s, and more, see: https://www.youtube.com/watch?v=U4BSZ4FlGRg&feature=youtu.be.
[iii] See Between Sickness and Health. The Landscape pf Illness and Wellness. Routledge, 2020. .
[iv] Gibson, JJ. The Ecological Approach to Visual Perception. Houghton Mifflin, 1979.
I wrote this piece for a blog called dementia day to day
Ivan lived on his own [1]. He was unsteady on his feet, and scarcely able to care for himself despite a large amount of outside help. His isolation made him vulnerable but whenever we met him at home he seemed complacently, if not blissfully, unconcerned about the situation. On the other hand his mother, who was constantly appearing on his doorstep, was deeply alarmed, and so were we. Our anxieties came to a head when people in the town reported seeing Ivan staggering home from the convenience store with more packs of lager than anyone could safely carry, let alone a man with the swaying gait of Huntington’s disease. Ivan wasn’t eating enough, could scarcely feed himself, and shouldn’t logically have been subsisting on alcohol. Then there was an extraordinary change. Whether this was due to his mother’s persuasive efforts we never knew, but Ivan switched quite abruptly. Instead of lager, he started hoarding similar quantities of Coca Cola. The last time I visited him he was reclining on his couch, looking a little neglected and yet somehow content. The living room and the kitchen were lined with enough 2-litre bottles of Cola to last a lifetime. Â
Many of the people we met tormented their families (and us) in similar ways. One man, David, would disappear for hours on end, determinedly walking through the streets, often well into the night. A woman called Maeve heard that fish oil could stave off the effects of Huntington’s on the brain. When we visited her we found cans of sardines stacked in every corner, and every drawer was full of them.Â
Opinions differ about the best psychiatric description for people like Ivan, David and Maeve. Medical authorities are currently saying that Huntington’s produces OCD – obsession-compulsion disorder. An obsession is an idea that you can’t shake off and that makes you feel compelled to act in a certain way. But how can we distinguish obsession as a brain disorder from the urge to have a drink on a hot day? If you feel the desire to walk the streets all night, or to stockpile sardines, has something gone wrong with your brain? A possible answer to this question is that an obsession is pathological when it is unwanted.  Perhaps, as biologically-minded neurologists and psychiatrists imagine, a brain lesion is sending an uninvited push-notification into consciousness, carrying a message such as ‘sardines’. This is roughly what happens when certain kinds of epilepsy generate strange sensations and thoughts. Dreaming, delirium and psychosis might be thought of in a similar way. Patients with various diagnoses have demonstrated that damage to the frontal lobes of the brain can produce feelings of compulsion [2]. Among the adolescents diagnosed with OCD that I have worked with, many did seem to want to be rid of their compulsions as though they might have sprung up in their brains, unbidden. Some people with Huntington’s appear to have an OCD-like awareness that their compulsions are bad for them. I remember a woman – call her Ella - who began to alienate her children because she brushed their hair at least daily in an obsessional hunt for nits. She knew that this was hurting her family. Â
However, obsession-like thoughts and compulsion-like behaviours are not always unwanted, and nor need they always be seen as meaningless biological events. When they arise from a background of anxiety their meaning can sometimes be traced. As I observed in adolescents diagnosed with OCD, ritualistic behaviour is a strategy – although often a self-defeating one - to exert control in a dangerous world. The same might be true in Huntington’s. It is possible that Ella fixed on hygiene as a desperate expression of care for her children, so that at some level perhaps her routines were not imposed, or not entirely imposed, on her. As for Ivan, Maeve and David, I could just about conceive of their having chosen to do what they were doing. None of them appeared to be anxious, and while their habits were troublesome to others, they themselves were unconcerned. The transportation, storage and consumption of lager, and then of Coca Cola, had become part of Ivan’s raison d’être, just as canned sardines were part of Maeve’s. There was more than a grain of logic in Maeve’s determination to eat more fish, and quite possibly the actions of Ivan or of Dave made some sort of sense, at least to themselves.
Rituals and obsessions are part of the materials for a life with Huntington’s. They are affordances, channels through which actions and purposes must flow, just as choreic movements are affordances for communication. In medical language they are disorders of behaviour, but they are cut from the same neurological cloth as admirable qualities such as determination and perseverance. Calling them behaviours has a distancing, depersonalising effect. A worried parent or an exasperated teacher or a frustrated clinician might puzzle over your ‘behaviour’, but would you ever use that word about yourself? Clinical language is not a good way to describe the feelings and purposes of someone lumbering home with a crate of coke or a stack of sardines.
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References and further reading
[1] Ivan and the other characters I mention here are not identifiable individuals, but nothing in their stories is invented.
[2] See Ward, CD. Transient feelings of compulsion. Journal of Neurology, Neurosurgery and Psychiatry, 1988; 51: 266‑268