The goals of this clinical trial are to identify factors associated with the development of problem behavior in Cornelia de Lange syndrome (CdLS) and to develop an effective behavioral assessment and treatment model for problem behavior in children with CdLS.
The hypotheses are as follows:
1. Based on pilot data, the investigators hypothesize that individuals with CdLS will exhibit preferences for auditory stimuli relative to other categories (e.g., visual, tactile) of stimuli. 2. Based on pilot data, the investigators hypothesize that individuals with CdLS will exhibit problem behavior to obtain adult attention or to escape task demands relative to tangible and control conditions, as measured by functional analysis results. 3. Function-based behavioral treatments will reduce problem behavior in individuals with CdLS by 80% or greater relative to baseline rates. 4. Individuals with CdLS and problem behavior will exhibit more impaired communication, demonstrate increased emotion dysregulation, and exhibit more severe symptoms of Autism Spectrum Disorder (ASD) relative to those with CdLS and no problem behavior.
Participants in the intervention group (families of children with CdLS and problem behaviors) will be asked to complete study measures and attend 2 full days and one half-day of clinic services at Kennedy Krieger Institute so that the study team can provide assessment and treatment of child problem behaviors, and then train parents to apply the intervention. Participants in the control group (families of children with CdLS and no problem behavior) will be asked to complete study measures once every 3 months for a 2-year period to monitor the children.
This study will improve the ability to effectively treat problem behavior is CdLS, as well as identify key variables associated with problem behavior in CdLS which may be examined in future studies and clinical practice to foster early intervention and prevention efforts.
clinical diagnosis of CdLS confirmed;
age 3 to 15 years;
is English-speaking;
exhibits SIB, aggression, and/or disruptive behavior; and
does not exhibit SIB, aggression, or disruptive behavior;
child is younger than 3 years or older than 15 years;
is not English-speaking;
has severe SIB that produces significant tissue damage or requires protective equipment;
Parallel
ABA-based functional analysis and treatment
BehavioralFunctional analysis to identify environmental variables maintaining child problem behavior and function-based treatment
Change in Behavior Problems Inventory (BPI) scores
The investigators will also quantify changes in problem behavior following the intervention relative to baseline with the normalized scores on the Behavior Problems Inventory (BPI). This scale has two sections: scores on Frequency of problem behavior (with a maximum score of 208 and a minimum score of zero) and scores on the Severity of problem behavior (with a maximum score of 156 and a minimum score of zero). Higher scores on these sections indicate worse outcomes, so decreases in the scores from pre-intervention to post-intervention would be indicative of a therapeutic outcome.
Change in Aberrant Behavior Checklist (ABC) scores
The investigators will also quantify changes in problem behavior following the intervention relative to baseline with the normalized scores on the Aberrant Behavior Checklist (ABC). The ABC has 5 subscales: Irritability, Social Withdrawal, Stereotypic Behavior, Hyperactivity/Noncompliance, and Inappropriate Speech. Scores on the Irritability subscale (maximum of 45 and a minimum of zero), in particular, would be indicative of problem behavior. Higher scores on this subscale indicate worse outcomes, so decreases in this scale scores from pre-intervention to post-intervention would be indicative of a therapeutic outcome.
direct observation of problem behavior reduction
The goal of this clinical trial is to decrease problem behavior (e.g., aggression, tantrums) in children and adolescents with CdLS. The investigators will measure behavioral changes using a multidimensional approach. One measure is derived from direct observation of problem behavior during baseline (i.e., before the intervention) and at the conclusion of the treatment (i.e., after the intervention). The mean rate of problem behavior at the conclusion of treatment will be subtracted from the mean rate of problem behavior during baseline. This quantity will be divided by the mean rate of problem behavior during baseline, then multiplied times 100 to convert to a percentage. A common clinical benchmark using this design is an 80% reduction in problem behavior for successful treatments. Thus, treatments that reduce problem behavior by less than 80% are said to be ineffective.
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