It is often said that prevention is better than cure and there is little doubt that this applies to selfinjurious behaviour and any other behaviour problems. The most important thing that carers can do in order to try to prevent self-injury from developing is to be aware of the possible causes of self-injurious behaviour and the theories of why self-injurious behaviour can develop. It is important therefore, to be aware of the information and models that we have presented in Chapter 2 and Chapter 5.

More specifically there are some things that we would recommend that parents of children with Cornelia de Lange Syndrome attend to. The first is that a careful eye is kept on children with Cornelia de Lange Syndrome with regard to behaviours that might later turn into more severe self-injurious behaviour. This means trying to be alert to what can be a very mild behaviours such as gentle hand biting, soft head-banging and gentle scratching. These behaviours are sometimes referred to as proto-self-injurious behaviour in that they are thought to later develop into more severe selfinjurious behaviour. It is also important that others who are involved with the child are made aware of the potential for self-injury and they should be alert to the early signs of these behaviours. Communication is clearly important at this stage and using diaries that travel with the child between different environments will help to ensure that everyone is looking for the same thing and aware of how to respond should it occur.

If these early behaviours are identified then the most important thing to do is to work through the possible causes we have identified in Chapter 5 and check your response, and the responses of others who are in contact with the child, to these behaviours. By this we mean ensuring that your responses to these behaviours are not rewarding and thus the social reinforcement that we have described in Chapter 5 does not become operative. It is important to remember that your natural response to any self-injurious behaviour that is shown by your child will be to comfort and protect your child. However, it is also important to remember that this kind of natural response can be experienced by the child as a reward and consequently the behaviour will increase in the future. Understanding how and why this happens will be important in trying to prevent the behaviour from increasing over time. The future development of self-injurious behaviour in children and adults with Cornelia de Lange Syndrome, and in fact in any children with intellectual disability, is not inevitable. By checking early responses to the behaviour it is possible to decrease the chances that the behaviour will increase over time.

As we noted in Chapters 2 and 5 we believe that pain and discomfort are related to self-injurious behaviour in Cornelia de Lange Syndrome and anyone who has an intellectual disability. It is important therefore that any pain or discomfort that is experienced by the individual is dealt with as quickly as possible in order to reduce the chance that self-injurious behaviour may occur and may then become socially rewarded and thus increase over time. Being aware of the types of health problems that children and adults with Cornelia de Lange Syndrome experience, and the types of intervention that need to be brought to the attention of health professionals are important aspects of preventing self-injurious behaviour from developing. It is important that when pain and discomfort are present and that you believe mild self-injurious behaviour is occurring in response to pain and discomfort (Chapters 2, 5 and 6) that you act quickly, inform health professionals and seek effective intervention. Further information on health problems associated with Cornelia de Lange Syndrome can be found on the Cornelia de Lange Syndrome website.

Should self-injurious behaviour begin to develop then there is no harm in seeking early advice from a clinical psychologist or behaviour analysts. In the early stages intensive intervention may not be necessary and some advice from professionals who are familiar with self-injurious behaviour may go a long way. It made help to have advice from someone on how to respond to self-injurious behaviour when it occurs in the early stages and to be able to plan for the future.

The final aspect of prevention that we believe is extremely important in preventing self-injurious behaviour from developing into a socially reinforced and thus functional way of interacting with others, is the development of an effective and reliable communication system for the child. We cannot emphasise enough how important we believe it is that every child, regardless of the risk of developing self-injury, is able to make their needs known to others. There is very good evidence that children with all degrees of intellectual disability can develop basic but effective communication systems, and there are a variety of ways in which this can be achieved. We are also aware that it is difficult to find external help to teach effective communication systems and our experience is that those parents who lobby hard for this are those who are more successful in finding help. For children with a greater degree of intellectual disability, signing systems such as Makaton are effective and there is increasing evidence that PECS (Picture Exchange Communication System) is useful in reducing behaviour problems generally.

Assessing and intervening

Finally, there are some general points that we would make about assessing and intervening when self-injurious behaviour is occurring. 

  First, it is hard to take on self-injurious behaviour on your own. One role for carers is being aware of the various parts of an intervention that are necessary and trying to bring together people who have contact with the child to work towards the same goal. Additionally, it is important to include in the team outside help from clinical psychologists, behaviour analysts, speech and language therapists and others who can make an important contribution. As we have said at various points throughout the book, our experience is that parents who lobby are more successful in bringing together these groups of people than those who do not.

We have emphasised the importance of building a model of the causes of self-injurious behaviour for each person and then implementing an intervention that is based on this model. We believe that this is the most effective way of approaching the problem and putting together an intervention that is likely to be successful in different environments and over time. However, we have acknowledged that it is not always possible for assessments to show us the causes of self-injurious behaviour and then we fall back on a systematic trial-and-error process in which we may try various forms of behavioural intervention in order to find that which keeps the behaviour at a low level. There is nothing wrong with a trialand- error approach. Whatever intervention is employed the most important thing is that accurate records of the self-injurious behaviour are kept in order to evaluate whether or not the intervention is reducing the behaviour. These records will help decision making in the short term but will also help in the longer term in being able to look back at what was effective and when it is necessary to return to an intervention.

Finally, there are three things which we believe are important in trying to get interventions to work. The first is that there is agreement amongst all the carers who are involved with a child or adult showing self-injurious behaviour about the intervention that will be conducted and how and when it will take place. This is, of course, easier said than done and it may well mean that some degree of compromise will be necessary in order to achieve agreement. However, if there is no agreement then the evaluation of an intervention will not be possible as we cannot be sure precisely what is being done. Second, consistency in an intervention is critical in order to ensure that the intervention has been given a good trial. This means people who are involved with a child or adult agreeing to run an intervention in the same way for whatever time period has been agreed. Third, help with running an intervention is extremely important. Any behavioural programme is unlikely to show an immediate effect and commonly the interventions take time to show a reduction in self-injurious behaviour. It is important to persist both with individual interventions but also to be prepared to try another intervention if one simply does not work.

When interventions fail

It is worth thinking about why interventions fail if they do. This is important both because there may be things about the intervention that we can change but also because failures can often tell us something about why the self-injurious behaviour is occurring. Any failed intervention (which means that when we review the records there appears to be no decrease in self-injurious behaviour or there has been an increase) should be reviewed to try to uncover the reasons for failure.

There may be a number of reasons why interventions are failing or appear to fail. The first is that the assessment has not identified the right cause of the self-injurious behaviour and consequently the wrong type of intervention was put into place. (See Box 7.21). When this happens it is important not to dismiss behavioural interventions as not working, rather it is important to understand that getting the assessment right and identifying the cause will help with putting together the right intervention. The second is that the extinction burst referred to in box 7.13 may be taking place. You will remember that this means that when we first stop presenting a reward for a behaviour, the behaviour can temporarily increase in frequency and intensity prior to decreasing. This means that if we see this early increase in intensity and frequency, it may not be that the intervention is failing rather that it is the right intervention but that we are seeing an extinction burst.

A third reason is that the intervention may simply not be implemented across the board. There are two ways in which this might be apparent. The first is that the records may show that the intervention is working in one environment but not elsewhere. Under these circumstances it is important to look at the two environments and try to work out whether the intervention is being implemented in exactly the same way in both environments. The second is that an intervention that has worked in the past does not seem to work now. We noted above that it is difficult to sustain interventions over time and that when self-injury starts to decrease there is a temptation to take a bit of a breather. It is important to keep programmes under review and ensure that they are being carried out with persistence.

In addition to these specific reasons we do believe that the causes of self-injurious behaviour can differ over time and that what is causing selfinjurious behaviour at one point in time may not necessarily be important later on. If interventions stop working therefore, it is important go back to re-assess the potential causes of self-injurious behaviour as another cause may have emerged over time. Similarly, just because an intervention does not work at a given time does not necessarily mean that it will never work. If the intervention was not correctly matched to a given cause then that would be the reason why it did not work. If the cause does become influential later on then the intervention that previously failed may now be effective. The message is do not throw out interventions completely, they may work at a different point in time. Again, record keeping will help us to decide.

The goal

The final thought we leave you with is that it is possible to decrease self-injurious behaviour. The key to doing so is to adopt a systematic approach to assessment and intervention and to be guided by the records that you keep of the results of intervention. The first intervention you try may not work, the important thing is to keep trying and to persist with different kinds of intervention based on what you believe the causes are, keeping records to tell you when you are beginning to succeed. We firmly believe that with persistence, resources and time it is possible to reduce self-injury in all children and adults with Cornelia de Lange Syndrome to a level that ensures safety and does not significantly impinge on quality of life. That is the goal.

Chris Oliver

by Chris Oliver Jo Moss Jane Petty Kate Arron Jenny Sloneem Scott Hall

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