Sunlight and Nightshade
People with SMS are often described as very friendly and outgoing, and as finding social attention particularly rewarding. Many develop strong social relationships at home, school/college and in the wider community. This has benefits for the well-being and happiness of the person with SMS and can also be useful when planning programmes that aim to develop skills and behaviours, where social attention may be used as a reward.
In common with many people who have an intellectual disability, people with SMS can often lack understanding of the underlying, ‘unwritten’, rules governing social interactions (e.g. social/personal space) and may not be aware of the social constraints that are apparent to others. As such, they may approach strangers in an over-friendly manner. Understandably, this can be a major concern for caregivers, who fear that their child is too trusting and may be taken advantage of if not supervised all the time.
People with SMS may also communicate with others by interrupting their conversations and by using repetitive questioning. They also have a tendency to talk about repetitive themes (see the ‘Repetitive Behaviour and Restricted Interests’ section in our SMS Guidelines Booklet).
Having an intellectual disability, as well as often having an endearing personality, may mean that adult friends and relations may unintentionally ‘baby or ‘overindulge’ people with SMS. It is important, therefore, to explain to these adults how to behave appropriately towards the individual with SMS; while being as tolerant and patient as possible in relation to any difficulties he/she may have. Being able to explain clearly what a person with SMS is capable of (and what they might struggle with) can help this.
Specific difficulties with social behaviour in SMS are found in two main areas, ‘attention-seeking’ behaviour and features of autism spectrum disorder (ASD).
‘Attention-seeking’ behaviour is commonly described in people with SMS. This seems to be best characterised as a strong drive for the attention of particular people. It does not appear to be a more generalised, indiscriminate high level of sociability.
People with SMS are described as often wanting to contact or interact with particular ‘favourite people’. This behaviour has been reported by caregivers and teachers, suggesting it is shown both at home and in other settings.
While ’attention-seeking’ is often shown towards caregivers, it can also be siblings, teachers or even the bus driver who become the object of this behaviour. In children, this seems to be directed more towards adults than towards peers of their own age.
It is likely that this attention-seeking behaviour is more striking in SMS because of some of the other behaviours that are commonly shown; for example there are often reports of temper outbursts, aggression or self-injurious behaviour (all of which are more common in SMS) being shown when attention is unavailable. This will make the behaviour stand out more and can make it more difficult to manage. For caregivers, being the focus of persistent demands for attention may become stressful.
Repetitive Behaviour and Restricted Interests
Nearly every person with SMS will show some kind of repetitive or stereotyped behaviour and often several different types of behaviour will be shown.
Common behaviours: The most common behaviours seen are teeth grinding and inserting hands or objects into the mouth (mouthing), which are also common in other people with an intellectual disability. These behaviours are described by caregivers as interfering at least moderately with the lives of those who show them. Other frequently described behaviours include rocking, spinning and fiddling. In some people with SMS these behaviours are self-stimulatory, providing sensory input (see the ‘Sensory Issues’ section).
‘Unique’ behaviours: Other behaviours that have been described in people with SMS are found less often in others with an intellectual disability. These include an unusual ‘self-hugging’ behaviour which often happens when the person is happy or excited. It can appear as the person either crossing their arms tightly across their chests and tensing their upper bodies or them clasping their hands together in front of them and squeezing their arms to their sides. A second unusual repetitive behaviour is repetitive page-turning (‘lick and flip’ behaviour, where people lick their hand and then use it to rapidly flip over pages).
Preference for routine: People with SMS may have difficulties making transitions from one activity to another and strongly desire for consistency in their daily routine. Unexpected change can make some people with SMS anxious and may result in a temper outburst.
Repetitive questioning and restricted conversation: In individuals with sufficient expressive language, repetitive questioning or conversation is also described. This may involve talking excessively about particular favourite themes or topics, which may be inappropriate or not well integrated into the conversation. Where preference for routine is an issue, repetitive questions may involve plans and routines. This could also be related to short-term memory difficulties. One type of repetitive questioning which is particularly common in SMS is repetitively asking to see, speak to or contact particular favourite people (sometimes described as the person having a strong ‘attachment’ to specific people; see the ‘Social Relationships and Autism Spectrum Disorder’ section).
What might help?
Repetitive behaviours – if a repetitive behaviour seems to have a sensory element, then having appropriate alternative activities or sensory items available (that provide a similar sensory input to the behaviour), which can be accessed independently by the person, may increase available stimulation and reduce the self-stimulatory behaviour (see the ‘Sensory Issues’ section).
Preference for routine – Where a child has not yet established routines, it may be useful to ‘mix up’ routines to teach flexibility. If routines are already established then, where possible, try to keep schedules as predictable as possible. If a routine has to change or if a person is about to experience something new, it is important to explain this well ahead of time, and on a number of occasions, if possible. It might be helpful to use ‘Social Stories’ as an aid. These are made up stories about social situations, usually with pictures.
Repetitive questioning/conversation – Try to limit responses to repetitive questions. It might be advisable to respond verbally only once when answering the question, and then to provide a visual cue to refer to.
Behaviours that Challenge
Compared to people with an intellectual disability with a different cause, people with SMS show more behaviours which may be described as ‘challenging’. These include temper outbursts, aggression and self-injury.
‘Behaviours that challenge’ is currently a commonly used term for such behaviours, or sometimes ‘challenging behaviour’ is used; however some people feel this suggests that the person showing the behaviour intends the behaviour to be difficult.
Importantly when these behaviours are shown, the person themselves is not being challenging, rather the behaviour may be distressing for those around them and may possibly cause harm. It is important to recognise that these behaviours may act as a form of communication or to express distress or discomfort where alternative means of communication are not available.
Furthermore although these behaviours are more common in people with SMS than would be expected by chance, this does not mean that they are inevitable. Not all people with SMS will show these behaviours and for those that do develop them there are strategies than can be effective in reducing the behaviours.
A Guide to SMS Booklet
Our booklet ‘Smith-Magenis Syndrome: Guidelines for Parents and Teachers’ provides a lot of practical and helpful advice about coping with all aspects of SMS. It covers sleep, social relationships, behaviours, feeding difficulties, toilet training, dressing, school concerns, behaviour in adulthood, siblings and sources of further help.