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Coping with behavioural disturbances in Huntington’s Disease
Pete Ellis
Department of Psychological Medicine
Wellington School of Medicine

I would like to start by pointing out that the greatest expertise in how to cope with behavioural problems in the home lies in the collective experience of those who are doing this every day, both sufferers and their supporters. The Huntington’s Association plays a crucial role in passing on effective strategies to others, as do other professionals, but we all need your help in having new ways of coping brought to our attention.

In general, an ounce of prevention is worth a pound of cure, but even with the most favourable circumstances in the world, prevention is not always possible.

In this talk. I will start off by defining what I mean by behavioural disturbances and how they may arise. I will then describe some of these briefly, and outline some ways of coping with them. To start with some definitions,

Behavioural disturbance is a vague term and can cover a range of different problems. These problems can be due to a number of different causes, and it is important to identify these so that treatment of management is appropriate. The cause include the effects of psychiatric illness, the effects of Huntington’s disease on the brain, the effects of personality, both the person’s long standing personality and changes in this due to disease, effects of circumstances, such as relationship problems, money problems, tragedies in the family or community, and also the effects of clear decisions which others may not agree with. For example, most parents do not agree with everything their adolescent sons do, but not all of it would be behavioural disturbance - I hope!

I have already referred to the effects of Huntington’s Disease.

It is interesting that although there is a Clear relationship between the severity of movement disorder and memory problems, there is No clear relationship between these and the severity of emotional problems. There are two ways of looking at this. The first is that perhaps some of the emotional distress associated with the disease is not inevitably part of the condition, and that with less stigma, more information and more support we might be able to make some difference to this. On the other hand we must remember that The more clear cut psychiatric problems are now being associated with changes in particular areas of the brain, so that some of the problems may well be part of the genetic condition. Even so, they don’t affect everyone.

Now, when thinking about emotional problems I think it is useful to bear in mind both general problems that can affect everyone with a serious illness before looking at the specific issues related to Huntington’s.

I think that anyone with a serious illness is likely to experience Distress at the diagnosis of their condition and what it means to them. This includes fear of future losses of health, relationships and financial security, fears of incapacity, the need to come to terms with the need to give up some ambitions and hopefully find others, and changes in responsibilities both in the family and at work. The exact nature of these will depend on where the person is in their own family life cycle.

Many people will have a role as a spouse or partner, also as a parent, most as a child and a sibling. The issues facing those early in a new relationship are obviously different to those who have been together many years; just as the challenges for those with young children differ from those whose children have left home and have established their own independence.

Obviously, there are specific issues related to Huntington’s disease as well. I won’t dwell on these as they are all too familiar to people here.

They include the genetic aspects and implications for siblings, children and partners, and the core features of the disorder, the chorea, the memory problems and the emotional changes.

While the emotional aspects were recognised early on, it is surprising that the figures for the rate of these problems vary widely. I would also like to note that the impact of the condition on both spouses and children is largely unresearched, although Alison Gray’s research is a major exception to this.

I would also like to stress again that there is no link between other aspects of illness and emotional problems.

the figures on this slide may seem a bit foolish to start with. You might well ask if psychiatrists can count. However, they come from a range of studies of different groups, some based on all the people known to very good services for people with Huntington’s Disease where one would expect that a fully representative range of people with the disorder would be surveyed, and some from services where only those brought to their attention, presumably berceuse of some problems would be included. I hope this provided some explanation for the statement here that psychiatric symptoms are the presenting feature of the disorder in a quarter to three quarters of sufferers and that one third to three quarters of sufferers have some psychiatric symptoms at any time. The influence of the setting is perhaps most obvious in the finding that in one US residential facility, one third had aggressive behaviour. That, of course, may have been why they were admitted, so it isn’t that useful a figure. It is rather like saying that the average level of football skills of those playing sport in the winter is high.

Personality problems are the most common and least studied issue. Now I should say what I mean by personality. Usually, we mean those aspects of a persons usual style of relating to others and to themselves that are enduring and characteristic of the particular individual. We also tend to regard people as responsible for any actions based on their own character. However, as a result of brain injury of any type people may suffer a change in their character. We refer to this as organic personality change. This can be very difficult to change and the fact that it is due to a disease process or the result of injury is very important, I think - such changes are not within people’s ability to reverse, although their efforts may limit the effects. Perhaps I can make an analogy with a broken leg. If I break my leg, and have to keep it off the ground for a period, there is no way I can walk unaided. In the summer, I walk to work over Mount Victoria. Now, I could say that I could no longer go to work, or I could ask my wife to drive me there, use crutches around the hospital and so on. Like the person with an organic personality change, I cannot reverse the original problem; provided with some support and understanding, I may be able to partially compensate for some activities, but not others. For example, helping with the school camp would be impossible.

The conditions on this slide are difficult. Apathy may be part of depression; a result of excessive sedating medication; an unstimulating environment; or the result of decreased functioning of the frontal lobe of the brain due to disease processes. Before saying that it is the latter, and accepting the need that it cannot be reversed, we need to be sure that it is not due to another reason which is easier to completely reverse. Intermittent explosive disorder is a condition where an individual is able to maintain a reasonable level of functioning most of the time but is prone to a major catastrophic outburst over an apparently trivial matter, for reasons which are not always clear, although often a careful consideration of what has been happening recently will reveal an accumulation of stresses.

I think it is also important that professionals acknowledge that irritability is more commonly expressed at home, away from clinics and relative strangers. I think we all tend to feel freer to express our feelings of irritation more openly with those who know us best.

This slide is to repeat my earlier comment that there are some suggestions from PET scans that personality changes in HD are associated with changes in the limbic system of the brain, which is the part of the brain which controls our emotions.

Aggression is a problem that concerns many people. The first priority in the face of aggression is to ensure the safety of all involved. This is more easily said that done, but in the face of physical aggression it is important that carers are aware that in emergencies the police can assist, as can the various psychiatric emergency teams around the country. I am mentioning this first to make it clear that this is sometime necessary, but I hope that other measures at an earlier stage of things would reduce the need for this.

It is vital to consider what may be causing the aggression. It may reflect May reflect personality change, or sometimes previous personality. It may be due to persecutory delusions, where a person believes that they are being persecuted and have to fight back to protect themselves, or to the irritability that may be part of a depressive illness. It is said to be more common early in the disease than in the middle or late stages.

Management consist of treating the underlying cause and of course ensuring safety.

antipsychotics are indicated for delusions, antidepressants for depression, and perhaps propanolol for frustration and impatience

Anxiety is generally agreed to be very common, although the surveys I reviewed for this talk did not quote any specific figures. Anxiety is a common feature of many other conditions, such as depression, and when the underlying condition is treated, it usually improves at the same time. However, when anxiety is the main issue, it usually responds to appropriate anti-anxiety medication

Depressive illnesses affect some 30% of those with HD. This is not periods or unhappiness and dissatisfaction, but persistent periods of low mood, all day every day for at least a fortnight, which also affects appetite, sleep, concentration and makes the person feel helpless, hopeless and worthless. It is more common in those with a later onset of HD. This is a serious condition that requires treatment, both to relieve suffering and because there is an 4-6 fold increased risk of suicide, especially in the elderly.

In HD it may be due to involvement of caudate nucleus in brain.

It does responds to treatment with antidepressants.

Mania is a condition where the person has a markedly elevated mood, boundless energy, a lack of need for sleep, pressured speech so that you can’t interrupt them, increased appetites for food, social relationships, sex, and possessions, and a tendency to rapidly get into all sorts of difficulties.

It affects some 1% of the general population but 10% of those with HD. Episodes can be brief or more enduring, and tend to be recurrent. Hypomania is a less severe form of the same condition, which tend to persist for weeks or months, while mania last for days or weeks.

Both conditions respond to treatment with mood stabilising medication such as carbamazepine

Psychosis is a term used to refer to those conditions in which people are out of touch with reality and suffering either hallucinations, where they are hearing or seeing things which are not actually there, or delusions, where they have beliefs that are not shared by others of their social and cultural background. It affects some 6% of those with HD. It is more common in early onset illness. Treatment should include antipsychotic medication. However, there are some problems with the older antipsychotic drugs and the recently available newer drugs may be more effective.

Before going on to look at coping strategies, I would like to go back to the beginning, as it were. I would like to say a few words about predictive testing.

When this first became available, there were Initial concerns about the impact of bad news on those in the programmes around the world. Although there were some early reports suggesting that this fear was appropriate, currently, it seems that the level of serious psychiatric problems following testing is low. However, this doesn’t really answer the question of whether the existing support structures and particularly the psychological assessment process is the reason for this, or not. Personally, I think it is mainly a tribute to robustness of those choosing testing, but also to the level of support they receive form family and friends, the Association and the genetic services.

Some recent studies have shown that Most of those who did have problems had mild depression at time of testing and something else went wrong in their life soon after, usually some unpredictable event.

So, how do people cope at different stages?

At the time of initial diagnosis, there is often a period of denial. Those who have been through the predictive testing programme will be familiar with this as the period in the morning of waking up and questioning whether the result was only a bad dream, but others will recall family members who had clear signs of disease but adamantly insisted that they did not need to see a doctor. In some way, not being told that they had now developed the disease allowed them to continue to cling to the hope that their symptoms were not of any significance.

The other phase of the response to grief are now familiar to many people through the work of Elizabeth Kubler Ross. The anger at fate, the "what if" and "if only" questioning of one’s own and others past behaviour, and the depression. The only point I would like to stress is that while denial is usually the first stage, the other stages occur in any order and recur over a period of time until the person reaches some acceptance of the situation. However, if things deteriorate, confronting the person again forcefully with the effects of their illness, there can be a further period of grief and adjustment. For example, one can become used to early symptoms of te disease and adjust to these, but having to give up work may provoke considerable distress and further grief.

There is a need for diagnosis, as the start of working through the implications for the sufferer and the family, but there is also a need for timeliness in this. Early symptoms evolve slowly, and insistence by family on immediately seeking confirmation of the diagnosis may not be welcomed - although it shouldn’t be put off for too long, obviously. Choosing the best time is difficult, and relies on knowing the person involved well.

The person facing probable diagnosis of HD is likely to be confronting various fears, including abandonment by family and friends, the fear of losing their job in the near future if their employer is unsympathetic, angry and frustrated at the loss of what they had hoped for in the future and facing grieving for their children’s risk of the same condition, and perhaps not being able to see them grow up as they had planned.

Of course, the spouse and the family will be facing their own fears around similar issues.

So, what is available to help? This slide outlines some key issues in the area of practical support. These are essential resources for people and can ease some of te worries about the condition. However, it is important to acknowledge that acceptance of these forms of help can be difficult. It means that one is giving up some independence, and accepting that the illness is indeed advancing. This can increase fears of further incapacity, and concerns about how these will be faced. There is also the issue of accepting others into the privacy of your own home, doing tasks that you considered to be yours, often to a standard less than you expected yourself.

I would also like to mention stigma. I have just been at the College of Psychiatrists meeting in Christchurch which is running back to back with the Schizophrenia Fellowship meeting. It is interesting to reflect on how well the Huntington’s Association has been able to put over a sympathetic view towards HD in a range of media, and begin to change public opinion and acceptance.

The families and sufferers of those with HD face challenges in responding to changing feelings and capabilities of those affected. It is important to remember that these feeling may fluctuate from day to day and hour to hour in unpredictable ways. Over time it is necessary to repeatedly restructure expectations of the affected person, and accept increasing limitations of what they can do. The disease tends to lead to the affected person becoming more self centred and dogmatic, and there is a need to accommodate this as far as possible. If you can, avoid confrontations if they are over minor issues. Sometimes it is possible to put things in a different way, to get around some of these difficulties. For example, rather than saying: "You have to eat lunch now!" it might be possible to put this as "Would you like to have lunch in the kitchen or the dining room?"

People with advancing HD tend to become attached to regular routines and to become anxious when these are altered. It is useful to keep to a schedule of regular meals, exercise and social contact.

Social contact can be difficult to maintain and tend to come to rely on family and particularly good friends. It can be helpful to set up a regular weekly schedule of visits rather than unexpected dropping in when possible, and to cherish the friends who do maintain this contact. Inevitably there will be extra events, such as clinic visits or distant relatives visiting. These need warning for the person, but it is generally suggested that this should be only a day or two ahead so that the person does not become too anxious about the event.

Looking after the physical environment is also important, particularly to ensure safety and to prevent falls.

Sometimes the person with HD will accept some necessary limitation on their activity, such as giving you driving, or giving u p cooking, more readily from the doctor than from family.

confronting HD as a spouse is always difficult. How difficult will be affected by the spouses own personality, how much information they have had about the condition and when they got this information, and the age of onset of the condition, among other factors.

It requires changes in thinking and behaviour in relation to their partner, changes in financial plans, change in marital relationship and has implications for any children

Probably the biggest change as the disease advances is in roles

Gradually one often sees a conflict between the role of carer or nurse, and that of lover and partner.

The affected person may become much more irritable or depressed than before, or have other psychological problems.

Sexual relationships also tend to change, sometime becoming more active, sometime less, and these changes are not always desired by the spouse.

Ways of coping with all these changes include Support from other spouses, time out for days or weekend or longer with supportive friends, professional advice and involvement of other members of the extended family.

It is important not to forget the effect of all of this on the children. Obviously this varies with their age. I will not expand on this at this stage but we might discuss it more later.

The key principles though all this are to:
retain respect for the individual with HD;
to help establish routines;
to maintain sympathy for the sufferer’s situation;
and to protect the carer’s and children’s health

I will now say a few words about some specific problems. Irritability is common, and can sometimes be reduced by Decreasing the complexity of the environment. By this I mean perhaps reducing the number of tasks that the person is expected to do. Perhaps they are finding it difficult to deal with the time management issues around preparing a meal, but still want to contribute to cooking. Perhaps they could be given the task of preparing the vegetables and someone else can take over ensuring they go on the stove at the relevant times. Some people benefit from medications to reduce irritability, but this is not a consistent finding with everyone.

Many people get anxious when they feel that they can’t do all that is being asked of them. I know I do. Strategies to help the person with HD in this situation include decreasing the demands, such as by stopping work perhaps; and developing and sticking to predictable routines. Anti-anxiety medication can also be helpful.

Other problems include depression. Exploring the reasons behind the depression, and trying to work these through, is essential, and there are also a range of antidepressants which are effective in treating the physical aspects of Depressive illnesses.

Hallucinations and delusions are less common, but do respond to antipsychotic medication.

So, in conclusion, the main aims are to help the sufferer to

Maintain as much independence and autonomy as possible,

to foster Respect for the sufferer and the carers

to ensure that there is Support available as and when needed and wanted

and to accept the need for Continuing adjustments

Finally, going back to some original comments, HD has come a long way out of the closet, from being an unmentionable family disgrace to an acknowledged physical illness. I believe that there is hope that research is going to make a significant impact on management of this condition in the foreseeable future and that in any event the changed attitudes about the condition will lead to some decrease in the level of psychiatric and psychological disability which has been associated with the illness in the past.