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Huntington’s disease (HD) is a progressive neurological condition caused by the inheritance of a defective gene. The death of brain cells in particular parts of the brain results in a loss of cognitive (thought-related), physical and emotional functions.
Physical symptoms include lack of coordination, jerky movements and problems with eating and swallowing. Behavioural problems are thought to be caused by a combination of events, including damage to the brain as the disease progresses and the understandable frustration and depression that people feel when challenged by chronic illness.
Not all people with HD will experience the same behavioural problems, since HD affects people differently. The severity of behavioural changes can range from mild and barely noticeable to enormously disruptive. It is important for family, friends and carers to appreciate that the person’s behavioural changes are part of the disease and are not under their conscious control.
Common behavioural changes
Some of the changes commonly associated with HD include:
- Loss of motivation
- Depression
- Loss of task sequencing
- Inability to block out distractions
- Drop in performance
- Inappropriate social behaviour
- Irritability and aggression
- Difficulties with communication.
Loss of motivation
The sections of the brain that help us to plan, organise and initiate action are affected by HD. The person may appear lazy, because they will do nothing (except, for example, lie in bed or watch television) if left to their own devices. Suggestions for family members, friends and carers include:
- Realise that yelling or arguing can’t motivate the person.
- The person may respond well to doing things with others, so take the lead and encourage them to follow.
- Helping the person to participate boosts their sense of worth, which is vitally important to reduce the risks of depression.
Depression
It is thought that around one-third of people with HD experience depression. Symptoms of depression, such as lack of drive, should be medically investigated and not simply assumed to be part of the disease process. Suggestions for family members, friends and carers include:
- See your doctor for diagnosis. Medications are available to treat depression.
- Psychotherapy may be an option.
- Regular exercise and moderate sunlight exposure can help ease depression.
- Try to incorporate more activities that the person particularly enjoys into their daily schedule.
Loss of task sequencing
Tasks have to be performed in a certain order. For example, washing the dishes requires filling the sink with hot water and detergent, cleaning the dishes, drying them and putting them away. A person with HD can remember the components of the task, but not the correct order. They might fill the sink with hot water, but then put the dirty plates away without washing them. Suggestions for family members, friends and carers include:
- Supervise and help the person to perform their task in its proper sequence.
- Encourage the habit of doing one thing at a time.
Inability to block out distractions
Eating a meal while watching television or listening to music can be very difficult for a person with HD because they can’t concentrate on both events at once. That’s why it is recommended that meals should be eaten in a quiet environment. Suggestions for family members, friends and carers include:
- Encourage the habit of doing one thing at a time.
- Remember that activities we take for granted, such as walking, can require concentration for the person with HD. They may not be able to carry on a conversation at the same time.
Drop in performance
A person with HD may seem more careless. For example, they may not clean the house properly or may fail to maintain their usual standards of personal hygiene. Suggestions for family members, friends and carers include:
- Appreciate that the person is trying their very best. It is the disease that is affecting their performance, not laziness.
- The person may not even realise they have made errors. Make it a rule to check on their behalf.
- Try to establish strict routines for bathing.
- Don’t deny them tasks simply because it is easier to do things yourself – continuing to make a contribution is important to the person’s sense of worth.
Inappropriate social behaviour
The awareness of social conventions may wane, resulting in (for example) lewd or rude comments to others. Some people with HD no longer have the ‘inhibiting’ emotions of shame, embarrassment and fear that help keep social behaviour in check. Suggestions for family members, friends and carers include:
- Explaining the inappropriateness of their behaviour may be lost on them. Yelling and arguing probably won’t work.
- The person may not appreciate the inappropriateness of their behaviour, but they may adhere to rules if you set them.
- You may need to consider limiting your social events.
Irritability and aggression
Some people with HD become easily irritated or angered. This can be caused, in part, by an inability to see things from another person’s point of view. Some people with HD appear to be self-centred and selfish. Suggestions for family members, friends and carers include:
- Remember that the disease prevents the person from thinking in a flexible manner. They may be more comfortable and easy-going in familiar environments and situations.
- Make sure they have enough control over their options. For example, a person may become stressed and irritable if they aren’t allowed to choose their own clothes for the day.
- Look at what the behaviour may represent. For example, the person may be spitting out their food because they have too much in their mouth, not because they are deliberately trying to annoy.
- Remember the general rule of one thing at a time. Trying to do two things at once can cause agitation.
- Focus on positive behaviours and try as much as you can to ignore the rest.
- The give-and-take of a loving relationship is disrupted by HD, since the person may have lost crucial emotions to the disease.
Difficulties with communication
As the disease progresses, the parts of the brain that help control the muscles of the face, throat and tongue are increasingly affected. This can cause the person considerable speech difficulties. The person may not initiate conversations either, as the sections of brain responsible for this are also impaired. Suggestions for family members, friends and carers include:
- Don’t assume they can’t understand what you’re saying, just because they can’t articulate an answer. Comprehension is usually unaffected by the disease.
- Don’t rush them. Allow plenty of time for their answer.
- If you see the person is having trouble expressing themselves, ask if they want help rather than doing it automatically. For example, the person may not appreciate you finishing their words or sentences without their permission.
- Whenever you can, offer them choices instead of open-ended questions. For example, ‘Would you like pasta or fish for dinner?’ is easier to answer than ‘What do you want to eat?’
- Flash cards with common responses such as ‘yes’ and ‘no’ may be useful.
- If the person progresses to the point of losing speech, continue talking to them as normal. Otherwise, you risk making them feel isolated and invisible.
Where to get help
- Your doctor
- The Australian Huntington’s Disease Association (Vic) Tel. (03) 9563 3922
Things to remember
- Huntington’s disease (HD) is a progressive neurological condition caused by the inheritance of a defective gene.
- Behavioural problems are thought to be caused by a combination of events, including damage to the brain as the disease progresses and the understandable frustration and depression that people feel when challenged by chronic illness.
- The severity of behavioural changes can range from mild and barely noticeable to enormously disruptive.
You might also be interested in:
Huntington's disease - diet issues.
Huntington's disease - genetics.
Huntington's disease explained.
Nervous system.
Neuromuscular system.
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