Cri Du Chat Syndrome Support Group

Common Behavioural Difficulties

Several surveys have discovered an excess of behavioural problems in CDCS children. These include hyperactivity, aggressive and oppositional behaviour as well as sleep problems (see above/below). Why this should be is a complex question; however, the degree of learning disability, communication difficulties and aspects of temperament such as high levels of irritability are important factors. What can you do if your child is behaving badly? Well, the first thing to do is to keep a record of what’s happening in the form of a diary. To do this systematically, it helps to have 4 columns entitled “what happened”, “what led up to it”; “what happened as a result” and, usually most importantly “what response to the behaviour did I make”. Surprisingly, by doing this you may easily detect what is happening and by altering your response to the particular behaviour, either stop it or change it for the better. For example, a regular tantrum at the checkout of the supermarket may result in the child being given a sweet “to shut him up”, thus paradoxically “rewarding” the behaviour.

Tantrums can be alarming especially in larger and stronger children. They are upsetting for everyone involved. Here are a few tips to handle them:

• nip them in the bud by distracting the child if they are “brewing up” by an activity that they usually enjoy
• avoid situations where they are more likely to occur (e.g. supermarket checkouts)
• don’t respond: full-blown tantrums thrive with an audience
• don’t attempt to “talk them down”: this rarely works and becomes stressful when parents “lose their cool” and make a bad situation worse
• walk away or, without any fuss, put the child into a safe unstimulating place until they calm down
• expect an apology if the child can understand cause and effect

It is also important that children know what is expected of them in terms of acceptable behaviour: they will not magically “discover” this. Communicate clearly, concisely, and with conviction using words or symbols that the child readily understands. Parents must agree to back one another up in matters of discipline: inconsistency can paradoxically increase bad behaviour. Parents should remember that their own behaviour is the most important example for their children: well-behaved adults encourage the same in children. Finally, rather than focussing on bad behaviour, promote good behaviour with lots of praise; furthermore, actively encourage children to do praiseworthy things such as tidying up their toys, playing nicely with their siblings or friends, etc. An ounce of praise is worth a ton of punishment in these circumstances.

Special Needs Teachers, Health Visitors and Child Psychologists are also good sources of sensible advice for families with badly behaved children. Some CDCS children have developmental problems such as ADHD that may need to be treated before behavioural measures can work effectively. Your GP will be able to refer your child to specialist paediatric or mental health services for this sort of help.

Some common behavioural problems are cited below:

(i) Concentration difficulties and hyperactivity.

About 90% of CDCS children will experience some problems with poor concentration, impulsiveness and overactivity. It might be that some CDCS children will be diagnosed with Attention Deficit Hyperactivity Disorder (ADHD). The extent to which these behaviours develop into adulthood is not yet known, although overactivity may become less of a problem in late adolescence and adulthood but they may still remain very distractible and have poor attention spans. Before weconsider some straightforward means which may promote attentional skills in children it is important to highlight some other reasons why children may be overactive and demonstrating poor attention: Firstly, the importance of children to get a regularly refreshing night's sleep needs to be emphasised. All children who are sleepy are likely to be both inattentive and overactive. By promoting healthy sleep habits in your child in the manner previously described this may well have a beneficial effect. Secondly, children may show attention difficulties if they are notprovided with the necessary limits and boundaries for their behaviour by their parents. A firm but fair parenting approach usually reaps rewards in these circumstances. Lastly, children who are generally either under or over stimulated through either neglect or overindulgence may show these features.

A prerequisite for promoting optimal attention is effective communication with your child. Both verbal and non-verbal (body language and expression) methods need to be employed in a manner which your child understands and demonstrably responds to. Generally speaking, in order for a child with a short attention span to understand what is expected of them communication has to be clear and concise. Strong eye contact is also important. Once one is sure that they are receiving the message loud and clear the tasks required should be quite simple and well within your child's general ability to perform. As soon as it is performed satisfactorily, praise should be given. Simple tasks requiring brief attention and concentration are very slowly over weeks and months replaced by progressively more complex and time consuming tasks. Certain constructional toys and educational video games may also promote attentional skills. One word of warning, expecting your child with a mental age of two years to learn to pay attention for an hour is unrealistic. The normal (non-novelty) attention span increases developmentally by a minute or two for every year of mental age. This means that a child with a mental age of two is unlikely to be able to be trained to pay attention for longer than three or four minutes. While your child is being trained it is important that extraneous sources of noise and other distractions (especially televisions and music centres) are avoided.

In addition to the therapeutic techniques described above, there is now increasing evidence to suggest that some forms of medication may also prove beneficial in reducing attention problems in some children.

Medication.

For children who are pervasively and chronically overactive and demonstrate very poor attention and impulse control which handicaps them in addition to their other problems the diagnosis of clinical hyperactivity or attention deficit hyperactivity disorder (ADHD) is made. When this condition presents to a severe degree, which often appears to be the case in CDCS, interventions based around behaviour modification, parenting skills and special educational provision may not help these core symptoms which cause tremendous problems for affected children's parents and teachers. In such circumstances medication may prove beneficial. In the past, tranquillisers would have been used but we now know that these produced no immediate or lasting gain. Indeed, sedatives have been shown to make some children much worse! It may seem strange but a class of drug called the stimulants have proved beneficial in this condition. Most of the clinical evidence for thesedrugs' efficacy has been obtained from work with non-learning disabled ADHD children. However, children with learning disabilities are now benefiting from this treatment. Child psychiatrists and paediatricians are the specialists who diagnose this condition and prescribe the medication.

(ii) Self-injurious and compulsive behaviour:

In recent years we have attempted to understand the nature of the self-injurious behaviour in CDCS children. From parental reports, we know that this behaviour causes demonstrable damage to the body and is particularly distressing for parents to witness. In our recent survey of the UK families, we found the main problems to be related to self-biting, hitting head with hand, and hitting head against objects. Indeed, 92% of parents surveyed reported some incidence of self-injurious behaviour with a third reporting that it occurred on a regular basis either weekly or daily. In a more detailed study we observed CDCS children’s behaviour during a typical school day and looked at both the “intervals/frequency” of a specific behaviour and the “duration” of an episode of the behaviour. We also interviewed their teachers to ascertain the range of self-injurious behaviours displayed within the classroom. We found that hand biting and skin picking were the two most common behaviours and were performed more frequently in children with CDCS compared to a comparison group of children who did not have CDCS.

Why do children with CDCS display these specific self-injurious behaviours?

Self injurious behaviour occurs in approximately 4 to 10 per cent of people who have intellectual disability. The most common forms of SIB are picking and scratching, biting and head banging and hitting. There are some individual characteristics that are related to self-injurious behaviour such as degree of intellectual disability (self-injury is approximately four times more common in those who have severe or profound intellectual disability) some genetic syndromes, such as Lesch-Nyhan and Prader-Willi syndromes, autism and poor expressive communication. Generally, the prevalence of self injurious behaviour rises with age up until the mid-twenties. There are three main theories that have been proposed to account for self injurious behaviour. The first focuses on neurotransmitters. These are chemicals in the nervous system that form links between the ends of different nerves. There are different types of chemicals and each has a different function. It has been suggested that a disorder of any of three neurotransmitters might be involved in self injurious behaviour. These are dopamine, serotonin and the endorphins. However, the evidence to support this theory is not very strong and at present there are no specific medications that have been shown to be effective. The second theory focuses on self injurious behaviour in response to pain or discomfort. Whilst there is little research literature to support this theory this is most likely due to the difficulty in establishing this association. This is because pain and discomfort can be rather transient and for people who are non-verbal it is hard to confirm the presence of pain and discomfort. The third theory has the strongest evidence and there is a research literature spanning over 40 years demonstrating various aspects of the theory and interventions that can be successful. This theory views self injurious behaviour as a learned behaviour that can occur because of the rewards that follow an act of self-injury. These rewards may be invisible to an observer as they comprise stimulation that follows the self injurious act. The best example of this is eye pressing which results in bright stimulatory flashes in the eye. Other rewards are more observable and are socially mediated. The most commonly cited are attention from carers and escape from difficult task demands. The evidence in the research literature suggests that for approximately 70 per cent of people with intellectual disability who show self injurious behaviour this theory is the most applicable.

When beginning the process of intervention for self injurious behaviour the importance of thorough assessment cannot be emphasised enough. The causes of self injurious behaviour will differ between individuals and there is no single intervention that can be applied to everyone. Generally the strategy that can be adopted is to first rule out any medical causes that can be giving rise to pain and discomfort. There is little to guide carers on how to approach this apart from their knowledge of the person they care for and how that person responds when in discomfort. It is important to be aware of common conditions that can give rise to local pain and discomfort such as tooth infection, middle ear infection and to note whether the self injurious behaviour is targeted at those parts of the body where pain and discomfort might be localised. In addition, when self injurious behaviour is associated to pain and discomfort it will be unlikely that the behaviour will vary with environmental events. It is critical that a careful examination of the association between self injurious behaviour and environmental events is conducted in order to see whether this is the case. Clinical experience suggests that addressing pain and discomfort as a potential cause of self injurious behaviour should be pursued vigorously in the first instance before psychological interventions are considered.

There is considerable evidence that self injurious behaviour can occur because it is rewarded either by social contact or other more tangible rewards, such as food, drinks and activities, or it is rewarded by escape from unpleasant tasks or other difficult situations. In order to evaluate whether this is the case it is essential that an assessment of the behaviour and the response of others to the behaviour is conducted. Once it is established that rewards might be influential then interventions based on behavioural principles can be constructed that comprise behaviour management strategies in response to the behaviour and increasing behaviours that can replace the function of self injurious behaviour. This often means increasing the functional communication of individuals who shows selfinjurious behaviour and there is a strong emerging literature that this is an effective strategy. Finally, when self injurious behaviour is rewarded by the sensory stimulation that follows the self-injury the general strategy is to try to increase alternative forms of stimulation and minimise the stimulation that results from the behaviour. This brief summary of the potential causes and interventions for self-injury gives an idea of the sorts of strategies that can be adopted in response to his behaviour. It must be emphasised that assessment is the key to successful intervention and that at present there is no evidence that these sorts of intervention that are effective for all people with intellectual disabilities will not be effective for individuals who have CDCS. Help in putting together effective assessments and interventions can come from clinical psychologists and professionals who had been trained in applied behaviour analysis and it is important to seek professional advice on the assessment and treatment of self injurious behaviour. Finally, there are three important things to remember when confronting the problem of self injurious behaviour. First, self injurious behaviour very rarely occurs at a stable rate all the time and in every situation. Because this is the case there is a cause. This is why assessment is critical. Second, whatever intervention strategy is adopted it is important that a careful record of the rate or frequency of self injurious behaviour is kept in order to evaluate whether the intervention is working. Sometimes, we do not know what is causing self injurious behaviour and the theories described above may not appear applicable. However, that does not mean that a trial-and-error process cannot be adopted and it is the records that will indicate when the intervention is working. Third, it is important to persist with any intervention over a reasonable period of time to see if the intervention is working. Self-injurious behaviour can take some time to decrease and it is important that any intervention is given a good trial.

(iii) Stereotyped behaviour:

Sterotypies are repeated body movements or postures that are not part of a goal direction act, such as hand flapping, finger twirling and rocking. These can typically occur when a child is agitated or upset and even when they are bored or when totally engrossed in a particular activity. The intensity of stereotyped behaviour does appear to decrease with age although it does not completely disappear. Instead, it may take on a more subtle appearance and only become very intense during periods of stress and excitement. You may find that your child responds to gentle encouragement to stop their hand flapping or rocking when you ask them to. Providing some token of a reward might also be effective and may well speed up the desired effect! With all these behaviours a Child Psychiatrist or Community Paediatrician might be able to provide some useful advice. In severe cases medication can sometimes be beneficial but this always has to be prescribed by and under the supervision of a specialist.