Behavioral Assessment and Treatment of Problem Behavior in Children With Cornelia de Lange Syndrome
Hugo W. Moser Research Institute at Kennedy Krieger, Inc.
Summary
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.
Description
The presence of problem behavior such as self-injury and aggression is a prominent and challenging feature of Cornelia de Lange syndrome (CdLS). Despite the growing body of research on behavior problems associated with behavioral phenotypes in genetic disorders, problem behavior in this population is not well understood. For example, individuals with CdLS are at high risk for developing self-jury, however it is not known why some do not develop this problem behavior. Additionally, few studies have examined environmental influences on problem behavior in CdLS. The field of applied behavior anal…
Eligibility
- Age range
- 3–15 years
- Sex
- All
- Healthy volunteers
- Yes
Inclusion Criteria for treatment group: 1. Child with CdLS and problem behavior: 1. clinical diagnosis of CdLS confirmed; 2. age 3 to 15 years; 3. is English-speaking; 4. exhibits SIB, aggression, and/or disruptive behavior; and 5. is able to participate in several hours of assessment/treatment. 2. Parent of child with CdLS is English-speaking and is willing to: 1. attend 2 full-day and one half-day clinic visits; 2. complete and return study measures; and 3. implement a behavior plan. Inclusion Criteria for control group: 1. Child with CdLS without problem behavio…
Interventions
- BehavioralABA-based functional analysis and treatment
Functional analysis to identify environmental variables maintaining child problem behavior and function-based treatment
Location
- Kennedy Krieger InstituteBaltimore, Maryland