Positive Behavior Supports for a Young Adult with CHARGE Syndrome

Abstract

CHARGE is a multifaceted syndrome, so named for a collection of characteristics commonly exhibited by individuals with this genetic condition. The acronym CHARGE stands for coloboma, heart defects, atresia choanae, retarded growth and development, genital hypoplasia, and ear anomalies and deafness (Blake, Salem-Hartshorne, Daoud, & Gradstein, 2005). Although individuals with CHARGE may not exhibit all of these traits, most--between 80% and 90%--meet the qualifications for deaf-blindness even when the hearing or vision impairment is mild (Hartshorne, Hefner, Davenport, & Thelin, 2011). Intellectual disabilities, however, are not as common in this population as early research indicated (Graham, Rosner, Dykens, & Visootsak, 2005). Those with CHARGE syndrome have a wide range of cognitive functioning, and some contend that intelligence is often underestimated due to communication barriers or the use of inappropriate assessments (Hartshorne et al., 2011). Furthermore, CHARGE syndrome is often associated with conditions such as obsessive-compulsive disorder, autism spectrum disorder, attention deficits, tic disorders or Tourette syndrome, anxiety, and executive dysfunction (Blake et al., 2005; Hartshorne et al., 2011; Hartshorne, Nicholas, Grialou, & Russ, 2007). In addition to these concomitant conditions, communication is often a challenge related to CHARGE. Some children with CHARGE develop symbolic communication, whereas others do not (Thelin & Fussner, 2005). Most experts attribute delayed or lack of language development to the sensory impairments associated with CHARGE (Graham et al., 2005; van Dijk & de Kort, 2005). Those who do develop symbolic communication most likely use total communication (the pairing of spoken and signed languages; Thelin & Fussner, 2005) and are prone to idiosyncratic communication patterns (van Dijk & de Kort, 2005). Communication skills are imperative for social and educational outcomes as well as for self-regulation (Kennert, Ramirez, Hartshorne, Deuce, & Nicholas, 2015). Closely related to issues of communication are behavioral challenges that are prevalent with CHARGE syndrome. Problematic behaviors are often attributed to communication difficulties, and as Hartshorne and colleagues (2011) note, behaviors are communication. Behaviors exhibited by individuals with CHARGE are distinct and more frequent than expected when compared to others with deaf-blindness or similar syndromes (Graham et al., 2005; Hartshorne et al., 2011). Beyond communication difficulties, other causes cited for challenging behaviors include sensory impairments, responses to or lack of control over environments, balance and equilibrium problems, medical issues and discomfort, stress and anxiety, and executive dysfunction (Blake et al., 2005; Graham et al., 2005; Hartshorne et al., 2007, 2011; van Dijk & de Kort, 2005). Experts say that behavior interventions for individuals with CHARGE syndrome should incorporate three key features: recognizing the cause of behaviors; reducing stress, anxiety, and discomfort; and accounting for sensory impairments (Blake et al., 2005; Graham et al., 2005; van Dijk & de Kort, 2005). These priorities align well with the positive behavior support model. Recognizing that challenging behaviors lead to exclusion and isolation, positive behavior support aims to reduce problematic behaviors to improve quality of life (Horner, 2000). Positive behavior support utilizes functional behavior analysis to understand the cause of inappropriate behaviors and then addresses issues in an individual's environments because [t]he assumption is not that individuals are defective or broken, but that they experience the world around them in a way that is different from their peers (Horner, 2000, p. 99). The present study investigates the use of positive behavior support strategies with young adults who are deafblind, including those with CHARGE syndrome, to determine which are effective for preventing or stopping problem behaviors. …