Many of the ordinary problems that children have can be dealt with by a paediatrician who has some developmental or behavioural experience. These would be minor problems with eating or sleeping, tantrums, or even hyperactivity. Many paediatricians are quite skilled in the first and second line drugs for impulsive and hyperactive behaviours.
Persistent behavioural difficulties including hyperactivity, severe impulsive behaviour, oppositional behaviour, aggression, or self injury, is the kind of behaviour that will require the attention of a specialist in behavioural psychology or a child psychiatrist who has experience with the developmentally disabled. Sometimes, individuals may need referral to an epilepsy specialist first, if there is suspicion of seizures underlying the behavioural difficulties. But the long-term treatment of serious behaviour or emotional problems in individuals should almost always be the responsibility of a specialist in child and adolescent psychology/psychiatry, who has the appropriate background.
Documenting patterns of behaviour is the best way to provide help to those involved in making treatment decisions. If the behaviour follows a pattern – if it is always at the same time, same place or with a particular person – treatment will begin with examining whether the situation can be changed. If the environment does not provide enough structure or there is no appropriate educational or recreational program, this can be addressed. Learning the reasons for the behaviour is the most difficult part of treatment. Since anecdotal information (impression) usually portrays an inaccurate picture of what is really occurring, treatment decisions should only be based on consistent information that is gathered over a period of time. More detailed information about how to assess challenging behaviour can be found in the DVD: Understanding and Changing Challenging Behaviour in Cornelia de Lange Syndrome and the Book on Self-injurious behaviour. Both of these resources were written by Professor Chris Oliver and his research team for parents, carers and professionals caring for/working with people with CdLS.
Behavioural management techniques ranging from redirecting the person to other activities to the use of protective clothing have met with some success.
A common (but not the only) underlying factor associated with behaviour disorder in CdLS, is pain and discomfort. There are many health and medical problems which are characteristic of CdLS. GI discomfort, muscle pain, ear infections, dental decay and even seizure activity can lead to irritability, self-injurious behaviour or aggression. Even people who are mildly affected and communicate well may not recognise the source of their discomfort. Undiagnosed GERD and dental decay are two of the most common reasons for changes in behaviour. Possible sources of pain and discomfort should always be considered when changes in behaviour occur.