Behavioral Treatment of Obsessive-Compulsive Symptoms in Youth With Prader-Willi Syndrome: A Pilot Project
Behavioral Treatment of Obsessive-Compulsive Symptoms in Youth With Prader-Willi Syndrome: A Pilot Project
Obsessive-compulsive (OC) symptoms are often present among youth with Prader-Willi Syndrome
 (PWS). They are also associated with considerable problems in the daily functioning of the
 child and his/her family. Although medication and behavioral treatments exist that target OC
 symptoms among youth without PWS, these treatments have not been thoroughly adapted for this
 population nor scientifically tested. Although medication has been helpful in addressing OC
 symptoms in several published case reports, the associated efficacy is modest and the
 potential for side effects is a realistic concern. Given that behavioral treatment for OC
 symptoms has superior efficacy to pharmacotherapy in youth without PWS without the
 accompanying risk for adverse side effects, it follows that an adapted version of behavioral
 therapy may hold promise in treating clinically problematic OC symptoms in youth with PWS.
 Thus, the purpose of the proposed grant is to develop and pilot-test a behavioral treatment
 for OC symptoms for use in youth with PWS. This study will allow us to develop and test a
 treatment protocol aimed at reducing OC symptoms that are clinically problematic and
 negatively impact functioning and quality of life in the child and his/her family.
Background: Obsessive-compulsive (OC) symptoms in youth with Prader-Willi Syndrome (PWS) are
 commonly present and associated with considerable functional impairment to the child and
 his/her family. Although pharmacological and behavioral treatments exist that target OC
 symptoms among youth without PWS, these treatments have not been systematically adapted for
 this population nor rigorously tested. Furthermore, although pharmacological interventions
 have shown promise in PWS in case reports, the efficacy is modest and the potential for side
 effects has been documented in youth with and without PWS. Given that behavioral treatment
 for OC symptoms has superior efficacy to pharmacotherapy in youth without PWS, without the
 accompanying risk for adverse side effects, it follows that an adapted version of this
 modality may hold promise in treating clinically problematic OC symptoms in youth with PWS.

 Objectives: There are two primary study objectives: First, to develop a well-specified
 behaviorally oriented psychotherapy protocol that addresses OC symptoms, both food and
 non-food related, in youth with PWS. Second, to conduct a randomized wait-list controlled
 trial of the protocol in 12 youth with PWS.

 Study Design: There will be two phases to this study: Phase I - Therapy development/manual
 writing and a small open trial; and Phase II - a small wait-list controlled pilot trial.
 Briefly, Phase I will concentrate on developing the treatment protocol through a process that
 draws on expert opinion coupled with focused interviews with parents. Based on this, a
 preliminary manual developed by the investigators will be piloted in 6 youth with PWS. Phase
 II will involve a preliminary test of the protocol in a sample of 12 families. This trial
 will focus primarily on feasibility issues (versus efficacy) in order to refine the manual,
 develop process measures to evaluate therapist's competence and adherence to manualized
 guidelines, train evaluators in assessment tasks, and develop data collection and coding
 systems. Six youth will be randomized to receive the treatment protocol, while 6 will
 participate in a wait-list of an equivalent time period. Diagnostic and symptom severity
 assessments will be conducted at appropriate time points (e.g., Screening, Baseline,
 Post-treatment) by a blinded, trained independent evaluator.
Inclusion Criteria:

 1. The child must have a genetically confirmed diagnosis of PWS;

 2. CY-BOCS Compulsion Score ≥ 8. The Compulsion Scale Score is being used as youth with
 PWS may have difficulty understanding or identifying the connection between
 obsessional thoughts and subsequent rituals. Therefore, use of the Total Score may
 provide an inaccurate depiction of symptoms (Storch et al., 2005);

 3. Stable on any psychotropic medications for 8 weeks prior to study entry;

 4. Between the ages of 6 and 17 years as the present measures are valid for use in this
 age span;

 5. At least one parent available to accompany the child to all sessions; and

 6. English speaking.

 Exclusion Criteria:

 1. Current suicidal intent or risk;

 2. A positive diagnosis in the caregiver of mental retardation, psychosis, or other
 psychiatric conditions that would limit their ability to understand psychotherapy.