Children with Fragile X may present with a number of behaviours which you may find challenging. These may include aggression and atypical behaviours such as hand flapping and hand biting. These behaviours may arise out of sensory integration difficulties or because they serve a variety of purposes (e.g. to gain attention, to escape from a task demand, provide self-stimulation, reduce anxiety). Before attempting to change behaviour of concern, it is important to gather as much information as possible about the behaviour. A holistic assessment of the behaviour you wish to modify, the target behaviour, may be useful to determine the possible function of the behaviour for the child, and what might be triggering and maintaining it. Usually this involves gathering information about the ABCs (Antecedent, Behaviour, Consequence) of the target behaviour and may be carried out with the support of a clinical or educational psychologist. ABC Chart

The A stands for the Antecedent of the behaviour: the ‘where, when, with whom’ and it is increasingly recognised that tackling the antecedents or what comes before the actual behaviour is the most efficacious way to deal with challenging behaviour.

The B stands for Behaviour: what the child actually did. It is important to be as detailed as possible.

The C stands for Consequence: what happened as a result of the behaviour.

By collating data about behaviours of concern, a picture of the pattern of the presentation of the behaviours can be developed and it may be possible to identify possible causes and start implementing a programme to modify particular behaviours.

Focusing on the antecedents to the behaviour or concern

In recent years there has been an acknowledgement of the importance of focusing on the antecedents to a behaviour that is perceived as challenging. Some have termed this positive behaviour support (PBS). Positive behaviour support is an applied science that uses educational and systems change methods to redesign an individual’s living environment to achieve an enhanced quality of life and to minimize challenging behaviour. In the case of children with FXS much challenging behaviour can be reduced and/or eliminated by making environmental accommodations. Rearranging of the physical and/or social antecedent conditions associated with problem behaviour is imperative when examining behaviour displayed by a child with FXS that is perceived as positive. Pertinent examples here might include changing seating arrangements in a classroom, changing the physical environment (e.g. noise, crowding, temperature), adjusting activities (making a task easier to complete) and/or enrichment of the environment through social or sensory stimulation (e.g. implementing a ‘sensory diet’ with a child). Many children with FXS may have language and/or cognitive impairment and therefore adaptations may need to be made to take these factors into account. In supporting children with FXS in changing their behaviour, one should bear the following principles in mind:

  • Individuals do not exist in a vacuum. Considering parents, siblings, friends, teachers and others who interact with the child is important when examining challenging behaviour.
  • The child’s level of ability and understanding needs to be taken into account. Behaviour that is challenging may be due to the distress caused by an overestimation/underestimation of abilities. Children may feel overwhelmed by tasks that are too difficult or frustrated and bored by tasks that are too easy.
  • The child’s language and communicative abilities must be taken into account. Failure to comply with instructions may be due to lack of communicative abilities. Similarly difficulties with social interactions may lead to outbursts. The teaching of an effective means of communication and effective social skills can lessen the chances of outbursts and tantrums.
  • Rewards and incentives must be feasible and implementable.
  • Programmes should be personalised by taking into account the child’s likes and dislikes.
  • The person must get more out of behaving appropriately than inappropriately. The prevention of challenging behaviour in children with FXS should particularly focus on:
    • Following a consistent routine,
    • Using visual and written schedules to outline the day’s activities,
    • Keeping the amount of noise and visual distractions to minimum,
    • Keeping the children engaged in some type of activity.

Teaching children with fragile X prosocial behaviour works best using real life photographs and video vignettes that demonstrate appropriate social interactions using the private and public context. Widely used behaviour modification techniques may need slight modifications and adaptations in order to work with children with fragile X. A technique such as ‘Time-Out’ can be useful with children with fragile X provided it is adapted and modified. It is very important to decide on as simple and consistent phrase (e.g. “No hitting, time-out”) when implementing timeout with children with fragile X. Children with fragile X typically have language delays, so more language may lead to less, as opposed to more, understanding. For ‘Time-out’ to be effective, need a clear relationship between it and the undesirable act. This is particularly important for children with fragile X, who have short attention spans and difficulties with sequencing and cause and effect. The shorter the time between the behaviour and ‘Time-out’, the more easily the child is able to understand the relationship between the two and the more effective time-out will be in decreasing the unwanted behaviour. The rule of thumb for time spent in ‘Time-out’ is one minute for every year, so a three-year-old child would spend three minutes in time-out. This works fine for typically developing children, but for children with fragile X this time should be cut in half as they have attention difficulties, and keeping children in time out for too long will result in frustration for parents, teacher and children (Riley, 2006). As with all children time out should be used in a consistent manner.

It is important that cognisance is taken of cognitive, behavioural and linguistic factors all of which may impact on behavioural interventions. It is critical for therapists and educators working with individuals with fragile X syndrome to understand that many behavioural problems result from sensory-integrative deficits and are not intentional acts on the part of the child. A strict behaviour modification programme may not be appropriate if behaviours are resulting from sensory integration deficits. Therefore, it is vital that professionals and parents collaborate to get as full as picture as possible of behaviours of concern that are expressed by children with fragile X. While common behaviour modification techniques and approaches may well work with children with fragile X, it is vital that data gathering includes input from as many individuals who work with the child as possible, to ensure that behaviour modification programmes reflect the individual characteristics of the child with fragile X. A good knowledge of the common characteristics of the syndrome can help inform this process.

Focusing on the consequences of behaviour

As well as focusing on the antecedents to a behaviour that is perceived as challenging another approach changing behaviour is by focusing on the consequences of behaviour. There are four ways of modifying behaviour by focusing on the consequences once a functional assessment has been competed. Adding a positive consequence is known as ‘rewarding’ or ‘positive reinforcement’. Giving praise, a star or allowing a child to watch a DVD every time the child behaves appropriately for certain period of time is likely to increase the frequency of an appropriate behaviour and this may lessen the occurrence of the inappropriate behaviour. Subtracting or taking away a positive consequence is known as ‘extinction’. Ignoring a child’s cries or screams, although difficult for parents, is likely to diminish these behaviours. Adding a negative consequence is known as punishment or aversion. Punishment may work in the short term but in the long term may foster resentment and unhappiness. Taking a way a negative consequence is known as ‘negative reinforcement’. If a child is allowed watch a favourite DVD by a parent in order to placate a tantrum or aggressive behaviour, this is negatively reinforcing the parent’s behaviour by removing the aversive stimulus and the child is more likely to display aggressive behaviour in the future. It is important to recognise that children with fragile X may lack the skills to express appropriate behaviours and thus may need to be taught such behaviours.
If a behaviour plan is implemented, it is important that realistic rewards are used if the plan is focusing on positive reinforcement. In the initial stages of a plan, every occurrence of a desired behaviour is rewarded. The next stage is to ‘stretch the contingency’ by only rewarding every second, third or fourth occurrence of a desired behaviour. The third stage should involve a ‘random interval;’ reinforcement programme where the occurrence of a desired behaviour is rewarded at random intervals. The final stage should involve the desired behaviour no longer being rewarded by external but by internal rewards, such as the satisfaction of behaving appropriately.