Facing Up to the Behavior Challenge
by Dr. Philippa Hyman and Prof. Chris Oliver.
(reported by CdLS-UK Foundation)

Dr Philippa Hyman and Professor Chris Oliver from the School of Psychology, University of Birmingham conducted a study into the most common types of challenging behaviors shown by people with CdLS. It aimed at increasing understanding of the different behaviors but especially self-injurious behavior. Postal questionnaires were sent to over 200 parents and carers via the CdLS Foundation (UK).

In previous studies, self-injurious behavior (SIB) has been reported at high levels in CdLS and is considered to be strongly associated with syndrome. However, some other studies have reported no SIB in CdLS. Therefore, it is unclear to parents, carers and the medical professionals just common this behavior is.

Additionally, other behaviors that may be of concern to parents, such as physical aggression or destruction of property have not been examined in detail.

In relation to SIB, a number of theories have tried to explain why some people self-injure, but so far no theory has been widely accepted.

Some doctors believe that self-injury is a means of communicating to others (for example that the individual wants social interaction from a parent). The theory argues that the self-injury is adopted as behavior because the individual does not have adequate verbal communication skills to express needs. Thus, the individual learns that they can gain a response from a carer if they engage in self-injurious behavior. This theory suggests that the self-injurious behavior is under the person's control. Interventions aimed at altering other people's responses to the SIB, and helping the individual who self-injures to improve communication have proved successful for some people but a number of individuals continue to self-injure when these treatment strategies have been tried.

This failure of some treatments has led researchers to believe that there may be more complex causes of SIB and this might indicate that different treatment strategies are needed. A number of research papers have described the severe nature of SIB in CdLS, and have argued that the behavior is outside of the control of the individual and perhaps ‘compulsive'. If the SIB was found to be a ‘compulsive' behavior this would have clinical implications because it would suggest that for some individuals the SIB might require different treatments. Evidence for this theory is that some individuals who self-injure also attempt to stop themselves from doing so. This is called self-restraint and examples include wrapping arms in clothing and sitting on hands.

This became the second aim of our study - to examine whether individuals with CdLS showing SIB also engaged in self-restraint, which might suggest that the self-injurious behavior was not within their control.

We also looked at whether those showing SIB and self-restraint also showed more compulsive behaviors.

86 carers of individuals with CdLS recruited via the CdLS Foundation (UK), completed questionnaires regarding the occurrence of challenging behaviors in those they cared for. The main caregiver who responded was the mother for 62 (72.1%) people and the father for 9 (10.5%). Of the 86 individuals with CdLS that were identified, 45 (52.3%) were female and 41 (47.7%) were male. Individuals ranged in age from 1 to 38 years old, and the average age was 12˝. 79 (91.9%) individuals were living at home.

The most common behavior reported was self-injurious behavior (SIB) displayed by 54 (62.8%) individuals. The average age of those showing SIB was 14.2, which was higher than for individuals who did not display SIB. The prevalence of other challenging behaviors is shown in Table 1.

• Physical aggression, stereotyped behaviors and destructive and disruptive behaviors were reported at relatively high levels. These results suggest that individuals with CdLS may experience a wide range of behavioral difficulties and not just SIB. Many individuals in this sample displayed more than one type of challenging behavior. 19 (22.1%) individuals displayed all four behaviors (SIB, physical aggression, destructive behavior and stereotyped behaviors).
• Males and females were found to display these behaviors equally.
• Despite the high prevalence of problematic behaviors, 64 (74.4%) individuals were receiving no psychological input for these difficulties.
• Levels of SIB, physical aggression and destructiveness were occurring at higher rates than in other studies of individuals with learning disabilities who do not have CdLS. Consistent with other studies was the finding that SIB is most common in the 10-20 year old age group.
• Of the 86 participants, 47 (54.7%) individuals showed some form of self-restraint. An interesting finding was that 35 (64.8%) individuals displaying self-injurious behavior also engaged in self-restraint. Additionally a relationship was found between those displaying stereotyped behaviors, self-injurious behavior and self-restraint. This was the first study to find an association between these three behaviors.
• Compulsions were divided into five categories: ordering, checking, completeness, cleaning and grooming. 37 (43.5%) individuals displayed more than 5 compulsions with ordering compulsions being the most common. At least one type of compulsion was shown by 53 individuals (61.6%). Table 2 shows the most frequently reported types of compulsions.
• Those individuals who display stereotyped behaviors, self-injurious behavior and self-restraint showed more types of compulsions than those without these behaviors. This association lends tentative support to the notion that the purpose of self-restraint might be to attempt to resist compulsive, involuntary SIB.

Conclusions

• Thanks to the parents, carers, families and the CdLS Foundation, important information was obtained for 86 individuals with CdLS. This is the second largest survey to date focusing on the behavioral aspects of this syndrome (88 individuals were studied by Dr. Tom Gualtieri in 1990). Other large-scale studies have mainly examined the clinical, medical and development aspects of CdLS.
• This study revealed a high prevalence of SIB, physical aggression and destructive behaviors compared to past studies of individuals with severe learning disabilities who do not have CdLS. However a more direct comparison does need to be made and future research must focus on all the problematic behaviors that are of concern to carers.
• The association between SIB, stereotypy, compulsive behaviors and self-restraint provides tentative evidence that SIB may be a ‘compulsive' behavior in some people. However, further studies are required to explore these findings, so that more effective clinical interventions and strategies can be developed to manage behaviors that are understandably of concern to carers.
• Given that not all individuals with CdLS display challenging behaviors it is important for studies to explore what factors are predictive of, for example, SIB. This would require a study from early childhood through to adolescence to explore the complex way that these behaviors can develop. If these factors can be identified then this would indicate a possible role for early intervention programs that could reduce the risk of these behaviors occurring at all.
• Further studies need to look at younger children with CdLS to see if the origins of SIB can be identified.

The new research program should cover these points.

Table 1. The number and percentage people with CdLS showing each form of challenging behavior.

Table 2. Number (%) of people with CdLS showing the most frequently reported types of compulsion.