Communication in People With Angelman Syndrome Stephen N. Calculator, Ph.D., Professor Dept. of Communication Sciences and Disorders University of New Hampshire Durham, NH Communication in People With Angelman Syndrome Stephen N. Calculator, Ph.D., Professor Dept. of Communication Sciences and Disorders University of New Hampshire Durham, NH

Defining Characteristics of AS (Deletion Positive) Severe to profound mental retardation None or minimal use of words Receptive language exceeds expressive language Ataxic gait Tremulous movement of limbs Easily excitable Hypermotoric Short attention span Apparent happy demeanor and frequent laughing/smiling

Other Characteristics Significant to Communication Protruding tongue Frequent drooling and excessive chewing/mouthing Sleep disturbance

Communication Profile of AS Problems first noted in early infancy. Fail to develop any functional speech. One group identified on REEL with receptive language skills in 9-22 month range and expressive skills in 9-14 month range (Joleff & Ryan, 1993). May use one or more words indiscriminately early on. Often drop out of lexicon.

Communication Profile of AS (cont.) Typically rely on multimodal communication (Calculator, 1997). –Marginal success with VOCAs in terms of spontaneous use. –All required prompting to use AAC systems. –Minimal initiations. –Self-selected simple means of communication: (natural gestures – actions on people and objects, eye gaze, and reaching). *Majority of gestures required physical contact with referent or interactant – contact gestures. *Majority of gestures required physical contact with referent or interactant – contact gestures.

Use of Gestures by Children with AS Frequent reliance on gestures (Alvares & Downing, 1998; Clayton-Smith, 1993). Those using manual sign (about 20% of population) use signs difficult to understand – motor problems (Clayton-Smith, 1993). Manual communication is preferred modality for most individuals (Alvares & Downing, 1998). Difficulty imitating gestures, including those already in communication repertoire (Joleff & Ryan, 1992; Penner et al., 1993).

What Other Communicative Behaviors Should I Be Looking For? VocalizationsAAC Challenging Behavior: (socially unacceptable and perhaps harmful behavior). (socially unacceptable and perhaps harmful behavior).

Vocalizations Often limited – vowels, consonants and syllable shapes Goal – expand phonetic and phonemic inventories

AAC Principle of Zero Exclusion Moving away from cognitive prerequisites Cognitive skills that ARE important (Rowland & Schweigert, 2003) –Awareness (theory of mind, social awareness.

AAC –Communicative intent (importance of parents’ responsiveness and contingency). –World knowledge (general experience) –Memory (sensory, working, long-term) –Symbolic representation (levels of abstraction)

AAC Cognitive and Related Requisites to Use Different AAC Systems? Consider child’s language skills when introducing (immediate needs) and planning future uses of AAC systems. AAC to foster communication growth.

Challenging Behavior Functional alternatives to socially unacceptable behavior –Self-injury –Aggression Must identify function (impact on environment) of behavior. Functional communication training (Carr & Durand, 1985).

Challenging Behavior Functional communication training (FCT) 1.Identify function of the unacceptable behavior. (what are the reinforcers?) – ABC paradigm (escape, attention, gain item or activity, sensory. 2.Make certain the communicative response is as efficient or more efficient than the unacceptable behavior (effort, frequency of reinforcement, and delay). 3.Evaluate reinforcers provided for unacceptable and communicative responses.

Communication Stages Perlocutionary Stage –Lack communicative intent. –Not goal directed per se. –Adults ascribe meaning. –Include responses to internal states. –Might teach adults to read and interpret children’s behaviors, gaze patterns.

Communication Development Perlocutionary Stage – Intervention –Alter partners’ perceptions of children as communication partners. –Replace children’s unintentional behaviors with voluntary communicative behaviors (BIGmack) –Increase children’s responsiveness to others, as well as to objects and events. Means-end behavior. –Move toward functional uses of objects. ENGs

Communication Development Illocutionary Stage –Intentional communicative acts. –Usually gestures and vocalizations Often to request attention or request objects and actions (protodeclaratives and protoimperatives)

Communication Development Illocutionary Intervention –In late stage may be taught to use symbols to request, reject, share information, and engage in conversations. –Scripting & embedding communication within routines Partial participation Backward chaining –Communication dictionary?

Communication Development Locutionary Stage –Emergence of symbolic communication –Expansion of communicative functions, vocabulary, and conversational skills. –Reciprocal social exchanges

Communication Development Locutionary Stage Intervention –Stress conventional methods of communication. –Topic initiation and turn taking. Home-school coordination – VOCA journal –Expansion of vocabulary. General case instruction. Fast mapping (Rice, 1989; Wilson & McIlvane, 2002).

What do we mean by ‘Success’ of Communication’? Success – adult acknowledges (“okay”, “I see”, “mmhmm”), interprets (oh, you’re telling me…; you’re still thirsty, huh?), or complies with child’s message. Rejections are also successes since they are contingent replies. Non-success – adult ignores, fails to respond, requests clarification, or responds non-contingently. Circle of Conversational Partners