Challenging Behaviour and Huntington's Disease
Doing two tasks at once
Many everyday situations involve people doing more than one activity at a time, e.g. answering children's questions while cooking or watching the television whilst doing the ironing.
Doing two things at once requires the mental flexibility to switch attention rapidly between tasks. However engrossed in the TV programme one may be, it is essential to keep switching attention back to the ironing to avoid burning the clothes.
Huntington's Disease can impair rapid switching of attention, making it difficult for people to carry out two tasks at once effectively. In contrast many people with HD are very good at sustaining attention on a single task, provided they are not distracted. "One thing at a time" is a good general rule.
Overloading what the person can cope with at one time maybe one source of irritability and disruptive behaviour. It is worth remembering that a physical activity such as walking requires much more conscious attention for the person with HD than for other people.
A person with HD may find it difficult to carry on a conversation at the same time as walking and may sometimes be noted to stop walking before answering a question. This is because both activities require conscious attention. To carry out both simultaneously would require rapid switching of attention from one to another.
The person with HD who has difficulty rapidly switching attention is obliged to stop one activity before embarking on another.
Quality of Performance
It is not uncommon for people affected by HD to carry out everyday tasks less efficiently than before. For example, in writing a letter, the person may miss out words; in washing dishes, plates may not be properly cleaned.
Indeed, for many people with HD, reduced efficiency is the precipitant of medical retirement from work. The poorer quality of performance on tasks may be a source of irritation to the person's family who may perceive the sufferer as being ‘slapdash' or ‘not bothered'. It is not the case that the person is simply not trying. In fact many people with HD put an enormous amount of effort into their activities.
The errors arise as a result of the changes that take place in the brain. People with HD do not forget how to do a task. What becomes impaired is the ability to self-monitor and check the results of one's own performance. The person with HD is often not aware of errors that are apparent to others. Encouraging the person to carry out tasks is a good thing. However, it is worth being aware of, and trying to accommodate, the person's possible limitations.
In the case of the individual who lacks initiative, it falls on other family members to act as a ‘stimulator to action'. So too, in the case of a person who carries out tasks inefficiently - ‘checking' procedures are dependent on others.
Hygiene and Self Care
It is quite common for people with Huntington's Disease to show less interest in their personal appearance and subsequently their standards of hygiene and self care decline. Loss of drive and initiative undoubtedly contribute to this change. Also of relevance is that Huntington Disease can impair personal and social awareness and blunt emotions.
The person with HD is likely to be unaware of the change in him/her and insensitive to the effect that an unkempt appearance has on others. Moreover, he or she may not experience the feelings of shame or embarrassment that under normal circumstances act as a strong motivator to self-care.
The person may need to be prompted to bathe or change clothes. A prompt often suffices. However, some people with HD still adamantly refuse to wash or change their clothes. It is worthwhile to try to establish bathing and clothing changes as part of a fixed routine - for example occurring at a specific time on specific days. It is also worth considering whether certain conditions influence the person's level of cooperation.
The manner in which the prompt is given may be relevant. A person may react badly to being told what to do, yet respond positively when he or she is encouraged to participate in making decisions.
People, who refuse, for example, to put on clean clothes given to them, may be willing to put on clothes that they themselves help to select. People who participate in making decisions are less likely to be behaviourally disruptive than those from whom all choices have been taken.
Some people with Huntington's Disease may act in a disinhibited way that is embarrassing to others.
Disinhibited behaviour may take a variety of forms. People with HD may act impulsively or rashly without thought, such as making a sudden purchase of a car that they cannot afford. They may make socially inappropriate remarks, for example, making personal comments about a person who is within earshot. They may behave in a sexually disinhibited way, such as making sexual advances to a partner in front of children. Such behaviour results from a breakdown in the individual's social awareness and ability to think through and appreciate the social consequences of actions. They do not see the repercussions of their own actions. People with HD may also no longer experience so acutely the feelings of embarrassment, guilt and shame that under normal circumstances place constraints on social behaviour.
People with Huntington's Disease may sometimes seem rather inflexible and mentally rigid.
They may adhere to set behaviour patterns or routines and appear unwilling to adapt to new situations or altered circumstances. They are not being deliberately obstinate. The brain changes that occur in HD can impair the ability to think flexibly and to adapt easily to novel situations.
People with HD generally feel most comfortable and confident in highly familiar situations, involving a fixed routine. If the person appears to need routine, then it is worthwhile trying to accommodate this. It does not mean that variety or a stimulating environment is inevitably sacrificed, it is simply that a structure is imposed on the person's day so that he or she knows what will happen and when.
Challenging Behaviour and Huntington's Disease
Behavioural changes are a characteristic feature of Huntington's Disease which although less obvious to the casual observer than the involuntary movements, represent for many families the most distressing aspect of the condition. Some alterations in the person with HD such as frustration, irritability and loss of confidence may occur to some extent as an understandable reaction to the presence of a disabling illness and to the loss of personal independence that it entails. However, behavioural changes also occur as an integral part of the disorder as a direct result of changes that take place in the brain.
Some of these changes arise as a direct result of alterations in the way a person thinks (intellectual changes); others represent alterations in a person's mood. Just as the nature and severity of the movement disorder varies from one person to another, so too do behavioural symptoms. In some people alterations in behaviour are mild without social impact. However, in others they can be profound, leading to disruption of family life and breakdown in social relationships, and placing a severe burden on carers. There are no easy solutions to difficult behaviour - precisely because the behaviour arises as part of the illness and is not under the person's control. Nevertheless, understanding the behaviour can help; it can lessen the burden and provide clues to the most appropriate methods of management.
Drive and Initiative
People with Huntington's Disease may show a loss of drive and initiative. If left to their own devices they may do nothing, stay in bed or spend the day watching television. This behaviour can be highly frustrating for family members, who may perceive the behaviour as "laziness" or the person as "not pulling his or her weight".
It can be a great source of family conflict when the person's partner is under stress from multiple responsibilities, for example, acting as breadwinner, caring for a young family and carrying out domestic chores. It is important to recognise that the patient's behaviour is not due to laziness.
Huntington's Disease affects the part of the brain (the deep part of the brain or sub cortex and its connections to the regions at the front of the brain), which are crucial for drive and initiative. These are the parts of the brain that allow us to think ahead, make plans, generate the actions appropriate to those plans, and persevere on tasks until goals are achieved. When those parts of the brain are damaged, the drive or foresight to self-initiate activity is severely compromised, even though the skill to carry out the activity is still retained. The person with HD may potentially be capable of carrying out a task, yet is unable to generate the motivation or initiative to embark on it. Arguing with the person will not overcome his or her difficulty. Encouraging joint participation in activities, e.g. helping with the washing up, may be helpful, and is generally more successful than assigning the person solitary pursuits.
This is because the other participants in an activity act as an external stimulator or motivator. The person with HD does not have to rely on his or her own drive and initiative. Participation is important. Not only does it help to keep the person active, it also helps to provide a sense of worth so that the person with HD is able to make a constructive contribution.
Denial of Illness
The onset of Huntington's Disease inevitably leads to life changes, which require accommodation and adjustment from both sufferers and their families. The process of adjustment is made difficult if the person diagnosed with HD refuses to accept that there is anything wrong. To an external observer, the involuntary movements may be obvious, and it may be evident that the person no longer carries out occupational and domestic tasks as efficiently as before. It is easy to assume therefore that these changes must be equally apparent to the sufferer. The conventional interpretation of lack of acceptance of illness is that the person is "in denial", that the patient at a subconscious level is aware of the reality that he or she refuses consciously to acknowledge. Such an interpretation may be an oversimplification.
Research suggests that people affected by Huntington's Disease may not have normal experience of their involuntary movements. If the person's own direct physical experience does not ‘match' with their perception of the illness in others, it is perhaps not surprising that they feel that they do not have that same illness. There are additional factors that may contribute to non-acceptance. The disease itself can impair the ability to self-monitor and to reflect on one's own performance. People with HD may genuinely be unaware of mistakes that are evident to others. It can impair too, the ability to draw inferences; a person may be aware of clumsiness and forgetfulness, yet fail to see the implications of these symptoms for Huntington's Disease.
Refusal to accept illness is not simply a result of obstinacy on the part of the person with HD; it is a feature that occurs in some (not all) people as a consequence of the disease process itself.
Carers and professionals may need to accept that confronting people with a diagnosis of HD will not always induce immediate acceptance. An approach that focuses on specific symptoms rather than diagnosis can sometimes be helpful (for example, a suggestion that the person is ‘a bit clumsy and coordination is not so good' may be accepted more readily as a reason for curtailing driving than ‘having Huntington's Disease'). Most people do come to accept the diagnosis in time.
Behavioural Problems: Action and Reaction
There is no doubt that the behavioural changes described above are an integral part of the condition, arising as a direct consequence of physical changes that take place in the brain. However, people affected by Huntington's Disease have to contend with a whole host of obstacles as a consequence of the disease; loss of job, reduced dependence, impoverished social life, impaired mobility, lack of understanding and tolerance from others. It is not surprising that people should feel frustrated and even angry with the apparent injustice.
The behaviour of people affected by Huntington's Disease often represents an interaction between the direct effects of the disease and the reaction to its consequences. A person with HD, for example, who assaults someone who falsely accuses them of being drunk, is showing an understandable feeling of anger at being victimised and falsely accused (reaction to consequence of illness), combined with the lack of emotional control resulting from HD itself (effect of disease).
People affected by HD cannot simply be ‘made to see' the consequences of their actions if the capacity to do so has been damaged by the disease process. Nor can the person be made to feel guilt, shame or embarrassment if the ability to experience these emotions have been taken from them by the disease.
Disinhibited behaviour may have the inevitable and unfortunate consequence that it leads to a restriction in a person's freedom; for example, a partner being obliged to take control of family finances. Some disinhibited behaviour, such as socially inappropriate sexual advances, are best managed by imposing limits, by letting the person know what is acceptable and what is not, and as far as possible adhering to those ‘rules'.
Behaviour and Disease Progression
Certain aspects of behavioural change become more pronounced with disease progression. For example, people with HD typically show less and less initiative over the course of the disease; they show progressively less concern over their own appearance; they become systematically less aware of the feelings of others. However, there is not an inevitable association between the length of time that the person has been ill and the severity of behavioural disturbance.
Indeed, some behaviour may become more manageable as the disease becomes more advanced; for example, irritability and aggression may gradually give way to apathy and unconcern. Similarly, disinhibited behaviour is most pronounced early in the disease when the person is most active, and diminishes and becomes less of a problem later as he or she loses drive and initiative. Mood disturbances such as depression tend to occur sporadically and are unrelated to the duration, severity or progression of the disease.
Sympathy and Empathy
People with Huntington's Disease may sometimes seem self centred, uncaring and thoughtless. The apparent disregard for the emotional needs of a partner can be particularly hurtful, and is especially poignant when it contrasts with a former loving and caring relationship. The natural tendency is for a partner to feel personally slighted. It is important to emphasise that the person with HD is not being deliberately awkward, wilful or unkind.
Apparent self-centredness is in part a consequence of the loss of mental flexibility associated with Huntington Disease. Individuals may no longer be able to put themselves in another person's shoes, to see another's point of view, to weigh up all sides of an argument. They may genuinely fail to see how their remarks or actions affect others.
Moreover, Huntington's Disease can impair the ability to experience the complex range of subtle emotions that contribute to interpersonal relationships, so that the person's emotions are more shallow or ‘blunted'.
The adverse effect of HD on an individual's capacity for sympathy and empathy with others is a major reason that Huntington's Disease can have such a devastating effect on families. Relationships that ought to be mutual may seem one sided.
There are no magical remedies; it is not possible to put back emotions and feelings that have been lost by disease. But remember it is the disease that is at fault. The person is not being deliberately uncaring. The emotional changes are not under his or her control.
Depression is a relatively common problem in HD, although by no means all people are affected. Its recognition is important because its effects can be profound, yet it can be effectively treated resulting in dramatic improvements in patients' well being and ability to function efficiently.
The loss of drive and initiative, which is an integral part of the mental changes that occur in Huntington's Disease, does not necessarily indicate a depressed mood. Nevertheless, the possibility that it represents a symptom of depression should always be considered, particularly the change in the individual's level of motivation and interest has occurred relatively rapidly.
Do not hesitate to seek medical advice if depression is suspected.
Irritability and Aggression
Although some people affected by Huntington's Disease may be even tempered throughout the disease course, it is not uncommon for sufferers to become emotionally volatile. They may ‘flare up' for no apparent reason, or over trivial issues.
People with HD may experience a feeling of internal agitation and be aware that they are easily ‘worked up', yet the sudden urge of anger comes without warning and is outside the person's control. In these circumstances it is best to avoid confrontation, which will tend only to add fuel to the fire. If necessary, leave the room, particularly, if there is a threat of physical aggression.
There is no foolproof solution to prevent emotional outbursts. However, it is worthwhile considering whether there are specific precipitating factors that can be avoided. Experience may suggest a range of everyday situations, which get the person ‘worked up'. These might appear quite trivial- for example, someone switching over the television channels while the person is watching a program.
Some people with HD may become irritable whenever they do not get their own way or their own views are opposed. Remember that people with HD, by virtue of the changes that take place in their thinking, may have difficulty seeing another's point of view. Continually arguing a point is unlikely to convince the person - it is more likely to increase his or her emotional agitation. Another point is worth reiterating; people with Huntington's Disease find it more difficult than other people to do two things at once. Overloading what the person can cope with at any one time may provoke in the individual feelings of agitation and potential loss of temper.
Avoid whenever possible placing multiple simultaneous demands on the person with HD. One thing at a time is best. Symptoms of irritability and aggression, like depression, can be treated medically.
Understanding Huntington's Disease
Huntington's Disease is a disabling condition that creates significant difficulties for the person diagnosed. Nevertheless, the traditional notion of the ‘mental' changes associated with HD as ‘global' are both misleading and inaccurate.
Huntington's Disease damages selective parts of the brain, leading to specific difficulties in thinking, which in turn give rise to specific and predictable changes in behaviour. Some aspects of a person's mental or ‘intellectual' functioning remain well preserved even when the condition is advanced.
People with HD can, for example, see and hear, and understand the meaning of what they see and hear. The task for carers and professionals working with people affected by HD is both to recognise the person's abilities so these can be harnessed most effectively, and to understand their limitations, so that these can be compensated for. Behavioural changes in Huntington's Disease represent the greatest challenge.
The profound impact of disordered behaviour on families is at last being recognised by professionals. Researchers investigating new treatments for Huntington's Disease recognise that improving mobility and reducing involuntary movements are not enough. Treatments must also be directed at improving behavioural aspects of the condition.
We gratefully acknowledge that this fact sheet is adapted with permission from the UK HAD Fact sheet series. (With thanks to Dr Julie Snowden)