CHARGE Syndrome Canada
Newsletter
Fall 2006
Vol. 1 No.12
Identification and analysis of behaviour in CHARGE syndrome
Research Studies need participation
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Past edition of the Newsletter
Identification and analysis of “Behaviour” in CHARGE syndrome
Ann Gloyn, Education Specialist, Canada
Many requests for information have been related to behaviour in CHARGE syndrome. Answers are always as simple as they are complex. With any population of individuals who have special needs, six steps can be utilized when looking at behaviour: identification, analysis, develop strategies, evaluation of the strategies, development of any new strategies, and then a re-evaluation process should take place. (Bridgett, 1987)
During the identification process, behaviour that is well described is much easier to begin to analyze. For example, instead of an observer listing “Paul got frustrated on the bus”, a better description could be “on the bus just as we passed the school, Paul began to bang his head against a window and kick his feet in the air”. Specific information will assist the analysis, as well as reference to when the behaviour starts; how long it lasts, or when it seems to have ended. (Gloyn, 1987)
As well, all possible variations the behaviour may take should be included. Instead of “he had a tantrum”, more specific information is needed, “He began to bite the Intervenor’s arm; then proceeded to bang his head backwards against the wall behind him. He reached for a picture on the wall and started to tear it down, when he found a chair with his feet, and picked it up and threw it.”
Chart and record specifics of the exact behaviour, and the amount of time it lasts. This charting should include the frequency, the intensity, duration, the events prior to the behaviour and the consequences, and also events after the behaviour. You baseline will then be established before any other discussions take place about strategy.
In CHARGE syndrome, there has recently been significant research around behaviour issues, so there are some known parameters to solutions. Research now shows there may be genetic factors to the behaviour. (Smith, et al 2004) Knowing more about the phenotype of individuals with CHARGE, the unique behaviour itself becomes a clinical feature of the syndrome. (Blake, Prasad 2006)
We know that in CHARGE the sensory system is usually underdeveloped. This includes the structures utilized for vision, hearing, and vestibular information. (Blake, Prasad 2006) The implication is that there is going to be distorted, missing, or incomplete information.
The involvement of combined sensory loss of differing degree and variety in the expression of clinical features make each child unique. The underdeveloped vestibular system (Verlos 2005) brings a whole new element of possible reasons for certain behaviour, while we continue to research the role and interaction of this system in development. This system functioning efficiently will tell you where you are in space, give feedback of what body parts are doing, will help establish equilibrium, and will take messages from the information given by vision and interweave it through a series of reflex reactions, especially when moving or spinning.
While unique vestibular development creates issues of concern, some children will demonstrate behaviour that is typical to the population of those who are congenitally deafblind, because of the sensory loss in the areas of vision and hearing. Harthshorne (2001) points to inefficient communication as not only typical of the population of those who are congenitally deafblind, but helps provide an explanation for some of the behaviour which is considered problematic. While he considers “behaviour is communication”, there are some further neurological aspects to the behaviour in CHARGE syndrome, some of which appears more autonomic. There has also been reference to it being "autistic-like" (Hartshorne, et al 2005)
Areas of the brain in some children are underdeveloped (Nicholas, 2005) and these children express features of behaviour which appear to be neurologically based. Many children have problems with self-regulation and impulse control. Brain dismorphology combined with the underdevelopment of the cranial nerves (Lawand, 2003) and possible inefficient recticular activating systems and underdeveloped autonomic nervous systems (van Dijk, 2005) make sensory integration therapy (Brown 2005) and nuerodevelopmenal techniques (Bluestone 2006) very useful strategies.
Van Dijk (2005) also describes the problems these children have during transitions, or moving place to place or just simply changing the activity. They need time to process and discover the implications of where they are in space; what the sensations are around them; what the distored picture and sounds may mean. Time taken during transitions and “neuro-breaks” are really useful. It is also important not to “overload” the senses with too much information from a number of different modalities at once.
We know many children with CHARGE syndrome have tic disorders and/or obsessive compulsive tendencies. (Bertstein 2005). Knowing what this behaviour could look like is important. The thing that the children may obsess about is often useful to develop educational strategy around. For example, if a child obsesses about “parades”, then using learning centered around this theme –making a toy parade out of boxes, reading a story on a parade, visiting a float being constructed--all will be very motivating for the child and so attention on task may increase while learning can take place.
Some children have incomplete development of the optic nerve and/or have experienced sleep apnea and other breathing problems which mean many could share the characteristics of those with cortical vision impairment. (Gloyn, Lauger 2004) Combine this with auditory processing delay because of cranial nerve involvement, (Lawand, et al 2004) and some a-typical “behaviour” can explained and resolved using specialized methods and techniques for CVI developed by Dr. Christine Roman (Roman 2005).
Finally there are other possibilities for the explaination of behaviour in CHARGE, like early attachment disorders (Williams, 2004); post-traumatic stress from the numerous surgery and hospitalization (Gloyn and Chinsen 2004); and finally many behaviours which could relate simply to pain. Gastro-intestinal reflux and problems with digestion and elimination are areas that need more research. Dental problems in early adolescence is another potential source of pain, possibly because of ongoing years of reflux. (CHARGE listserv)
There are also some key areas in common which can explain many of the reasons for behaviour problems in populations of children with special needs. During the analysis stage, these common reasons as well as those reasons more specific to CHARGE need to be kept in mind and evaluated before developing any strategy for each child. Here is a behaviour analysis summary that may be useful during the analysis stage.
Remember that once strategy is developed, it needs a "testing period" and then a re-evaluation. Record keeping is very important during this whole process, so specific success indicators and specific techniques can be developed, passed along, and also varified when questions arrise.
References <click here
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