Management of behaviour can be a problem in Angelman Syndrome. Studies have shown that aggressive behaviour in Angelman Syndrome is more likely to occur when levels of adult attention are low, or conversely, too high, suggesting that individuals are engaging in the behaviour to instate or maintain attention that they find rewarding or enjoyable.
Behaviours that Challenge in Angelman Syndrome
‘Behaviours that challenge’ means any behaviour having a negative impact on a person’s well-being and/or leading to their exclusion from the community. These behaviours are those that most people would find difficult to watch, or cope with. They include self-injury, aggression, shouting, screaming and others. The term ‘behaviours that challenge’ does not mean that there is something about the individual themselves that is challenging. The degree to which behaviour is seen as challenging will depend on the environment and situation in which it occurs.
It is important to remember that behaviours that challenge are not inevitable in any child or adult who has Angelman Syndrome and there is now an increasing understanding of why these behaviours may occur and what can be done. Like any other behaviour type, they occur for good reasons and can be changed.
- Pain and discomfort Treatment of underlying health difficulties (eg reflux, constipation, problems with teeth, ears etc) followed by regular monitoring may help to reduce behaviour.
- Sensory reinforcement There are many strategies to help reduce behaviours maintained by sensory reinforcement. Understanding the kind of sensory reinforcement the person is receiving from the behaviour and trying to provide that via other means may be helpful. Increasing the general level of sensory experiences the person has throughout the day may also be helpful.
- External causes External factors, including access to social interaction, escape from a demand, access to a preferred object or activity, escape from social contact, and escape from an aversive sensory experience may increase the level of behaviours that challenge. Interventions for these behaviours usually involve teaching a person an alternative way to communicate what they want and changing their environment so that the thing they want is available as much as possible. This might involve teaching a sign, giving access to visual clues (such as photos or objects of reference), or helping the person to use a communication system. It is not always possible for a person to have the thing they want when they ask for it and at these times it is important to help the person discriminate when the thing they want is and is not available.
Types of Behaviours that Challenge
- Self-Injurious Behaviour in Angelman Syndrome Self-injurious behaviour means any non-accidental behaviour (i.e., accidentally bumping your head on the wall would not be classed as self-injury whereas intentionally hitting your head on the wall would) that has the potential to cause some kind of damage such as reddening, bruising, bleeding, hair loss, etc. There has been very little research conducted on self-injurious behaviour in Angelman Syndrome, although many parents and carers report it as being a problem. Recent research at the University of Birmingham revealed that around 4 out of 10 individuals with Angelman Syndrome display self-injurious behaviour but this was no more likely than a control group of individuals with an intellectual disability who did not have Angelman Syndrome. The types of self-injurious behaviour shown by individuals with Angelman Syndrome were quite variable, however, they included self-hitting (with body or object), self-biting and self-pulling, rubbing or scratching.
- Aggressive Behaviour in Angelman Syndrome Aggressive behaviour means any behaviour that has the potential to cause injury to another person and does not imply that an individual with Angelman Syndrome is intentionally trying to hurt another person. In a recent study, 7 out of 10 individuals with Angelman Syndrome showed aggressive behaviour. This research also revealed that an individual with Angelman Syndrome is more likely to display aggressive behaviour compared to individuals of the same age, gender and degree of disability who don’t have the syndrome. It was also found that individuals who displayed aggressive behaviour were more likely to score highly on measures of impulsivity and overactivity. Common forms of aggression seen in Angelman Syndrome are hair pulling and grabbing, although many other forms may occur such as hitting, kicking, scratching and biting.
When is aggressive behaviour most likely to occur?
Aggressive behaviour in Angelman Syndrome is more likely to occur when levels of adult attention are low, suggesting that individuals are engaging in the behaviour to reinstate attention that they find rewarding or enjoyable. Aggressive behaviour may also be exhibited when levels of adult contact are high (i.e. during one-to-one interaction), implying that aggressive behaviour may also serve to maintain social contact, although there are of course many other reasons for aggressive behaviour.
Many parents and carers report that aggressive behaviour is not malicious in any way and the individual has no intent to harm anyone else.
Coping with Behaviours that Challenge
At any point when parents and support workers are tackling behaviours that challenge it is important to seek advice. It is easy to feel overwhelmed by the behaviour if it is happening frequently and because when it happens you may feel helpless and unable to deal with the incidents.
Initially, it is important to notice that behaviours that challenge are there, that it is a cause of stress and that it is time to act. This does not need to be done straight away.
Start to develop a plan of what needs to be done and how you are going to do this. Building a team of people around you who you can trust and rely on and who have skills to help you take the right steps will be more helpful than trying to tackle these behaviours alone.
Management of Behaviours that Challenge
Successful management of behaviours that challenge depends on several key aspects. Firstly, the intervention should improve a person’s quality of life, rather than only suppressing an unwanted behaviour. Secondly, the intervention should always be based on anassessment of the behaviour- It is important to have a good idea about the cause of the behaviours before trying to reduce them. Behaviour can be caused by different things for different individuals, so trying to change behaviour without fully understanding it can lead to the behaviour becoming more ingrained over time. No intervention should ever be restrictive. It is also advisable to seek professional help before putting interventions in place, eg from a clinical psychologist.
When teaching a new form of communication, episodes of behaviours that challenge may be seen to increase for a short time. This is a natural part of behaviour change and is known as an ‘extinction burst’.For new forms of communication to be successful, the old form (the behaviours that challenge) need to be less successful. When families change the way they respond to behaviour, such as ignoring it, the person with Angelman Syndrome may show more behaviour as they try harder to make their needs known. Over time the behaviour will decrease.
It is very important thatnot to ignore behaviours that challenge without additionally teaching an alternative communication strategy,otherwise the person has no way to communicate their needs.
Further help and advice can be obtained from: