Quality Metric: Invited, Peer-Reviewed Chapter

Dozier, C. L., Dracobly, J. D., & Payne, S. P.  (2013).  Unique Considerations of Prader-Willi Syndrome. In D. Reed, F. DiGennaro Reed, & J. Luiselli (Eds.), Handbook of crisis intervention for individuals with developmental disabilities.  Springer. https://doi.org/10.1007/978-1-4614-6531-7

Prader-Willi syndrome (PWS) is a genetic, neurodevelopmental disorder with many unique characteristics. In this chapter, we discuss the (a) physical clinical features of the syndrome, (b) behavioral characteristics of the syndrome, (c) various assessment and treatment procedures for behavior disorders in PWS, and (d) unique considerations for assessment and treatment. The most serious clinical feature in PWS is hyperphagia (i.e., insatiable appetite), which is associated with food-related problem behavior such as overeating and food stealing. Hyperphagia coupled with low levels of activity contributes to numerous health concerns. In addition, individuals with PWS are reported to engage in self-injurious behavior (e.g., skin picking) and other problem behaviors (e.g., aggression, property destruction, obsessive-compulsive behavior) that interfere with learning, independence, and quality of life. We review behavioral assessment and treatment procedures for these behavior disorders with a particular focus on weight management and the use of functional analysis methodology and function-based intervention. Finally, we discuss special considerations and modified procedures for the assessment and treatment of problem behavior in PWS.