Adult Interpretations of Communicative Behavior in Learners with Rett Syndrome

Transcription

1 DOI /s z ORIGINAL ARTICLE Adult Interpretations of Communicative Behavior in Learners with Rett Syndrome Hannah M. Julien & Quannah Parker-McGowan & Breanne J. Byiers & Joe Reichle # Springer Science+Business Media New York 2014 Abstract This study examined adults interpretations of the communicative functions of potential communicative acts as defined by Sigafoos, et al. (2009) produced by individuals with Rett Syndrome (RTT). Video recordings of three learners with RTT engaged in daily routines were parsed into 5-second clips. Each clip demonstrated a potential communicative act. The primary caregivers and educational professionals who served each of the learners viewed the video clips and rated the communicative function of each behavior, if any. Analyses examined adults judgments as a function of their familiarity with the learner as well as their relative familiarity and experience in interpreting limited communicative repertoires (i.e., parents compared to special education professionals). The majority of special education professionals and parents interpret potential communicative behavior to represent communication with a clear intention. The three functions most commonly identified by raters were requesting object, commenting and protesting. There was modest variability in these ratings across participants and between professionals and parents. Variability in the interpretation of potential communicative acts between raters highlights the need for future studies with this population. Implications for future applied research along with educational implications are discussed. Keywords Rett syndrome. Communication Introduction Individuals with developmental disabilities may demonstrate delays in developing intentional communication (Johnston, Reichle, Feeley, and Jones 2012). Bates, H. M. Julien (*): Q. Parker-McGowan : B. J. Byiers : J. Reichle University of Minnesota, Twin Cities, 115 Shevlin Hall, 164 Pillsbury Drive SE, Minneapolis, MN 55455, USA julie006@umn.edu

2 Camaioni, and Volterra (1975) described the gradual progression of a typically developing child from unintentional communication to intentional communication. From birth until approximately 9 months of age, a typically developing child demonstrates preintentional communication, also known as the perlocutionary stage, in which the child s vocalizations have an unintended impact on the listener. Around 9 months of age, a child begins the transition to the locutionary stage by purposefully using vocalizations and gestures in order to impact the listener. By 13 months of age, a child communicates intentionally through the use of referential words, vocalizations and gestures (Wetherby, Cain, Yonclas, and Walker 1988). Intentional communication is present when an individual develops awareness a priori of the effect that a signal will have on [the] listener, and [develops persistence] in that behavior until the effect is obtained or failure is clearly indicated (Bates et al. 1979, p. 36). Bates description of intentional communication mentions two important indices: 1) the child sawarenessof the communicative signal s effect and 2) the child s ability to persist in signaling the communicative behavior. For persons with delayed intentional communication skills, having a wide range of social partners who differently interpret (and respond to) pre-intentional communicative behaviors may place them at a disadvantage in receiving consistent reinforcement for their spoken, or gestural behavior (Meadan, Halle, and Kelly 2012). In a previous study, Meadan et al. (2012) examined adults interpretations of the requesting, rejecting and unclear behavior of three preschool age children with limited expressive language and Autism Spectrum Disorder (ASD). Adult participants were categorized according to two primary criteria: familiar/unfamiliar and expert/non-expert. The participants were presented with short video clips that had been extracted from longer recordings made during a structured assessment designed to elicit different communicative functions. The participants judged three specific components of each clip: (1) whether or not the child was communicating, (2) whether or not the child s intention was clear, (3) what the child was communicating. The participants also indicated how confident they were in their judgments. Meadan et al. (2012) reported that familiar expert (defined as having formal knowledge about communication and language development, [p. 441]) adults were more accurate in their ratings than non-expert and unfamiliar adults. They also found evidence of greater variability in adults interpretations of rejecting behavior compared to requesting behavior. These findings underscore the importance of collaboration among social partners of individuals with limited expressive communication to provide consistent responses to (potentially) unconventional bids for communication, thus creating a more advantageous communicative interaction for the learner (Meadan et al. 2012). Rett syndrome (RTT) is a neurodevelopmental disorder and is one of the most common causes of profound disability in girls (Ellaway and Christodoulou 2001), as recent estimates place the prevalence between 0.5 and 2 per 10,000 females (Fehr, Bebbington, Nassar, Downs, Ronen, De Klerk, and Leonard 2011; Laurvick et al. 2006). Mutation in the methyl CpG binding protein 2 (MECP2) accounts for % of individuals diagnosed with typical, or classic RTT (Neul et al. 2008). In atypical RTT cases, % have been found to have mutations in MECP2. Necessary criteria for a diagnosis of RTT include regression, loss of purposeful hand motions, and spoken language beginning between 6 and 18 months of age, and the emergence of hand stereotypies and gait abnormalities (Neul et al. 2010).

3 Hagberg (1995; 2002) described the four progressive stages of RTT development. Typically, the first stage, known as the stagnation stage, begins between 6 and 18 months of age and is marked developmental delay and deceleration of head growth. Marschik et al. (2013) found that in terms of communication development, individuals with RTT exhibited atypical speech development as early as cooing and babbling milestones. Stage II, the developmental regression stage, typically begins between 1 and 3 years of age and lasts between a few weeks to a year. This stage is characterized by regression in communication, language, and fine motor skills. Individuals with RTT will, in this stage, lose any spoken language previously acquired. Some individuals may retain some words while others will have never acquired any spoken language. The variability in the communication abilities of these individuals depends upon the specific genetic mutations, use of communication device(s), and the implementation of communication devices (Urbanowicz, Leonard, Girdler, Ciccone, and Downs 2014). The psuedostationary stage, stage III, occurs when some communicative behaviors reappear while the characteristic stereotypical hand movements begin. The final stage, the later motor deterioration period, most often begins around age 10 and is characterized by the further deterioration of mobility. It is in this stage that most individuals become dependent on wheelchairs. The majority of individuals with both typical and atypical RTT demonstrate very limited expressive communication skills as well as extremely limited functional hand use (Hagberg et al. 2002; Hagberg and Witt-Engerstrom 1986). Much of the evidence related to communication skills in this population is based on both naturalistic and structured observations (see Sigafoos, et al. 2011, for a review) given that individuals with RTT are often unable to participate in the standardized protocols used to evaluate communicative comprehension and production (Demeter 2000). Sigafoos et al. (2011) systematically reviewed studies that examined the potential communicative behaviors of individuals with RTT. All of the eight studies that were identified and included in their review utilized direct observations of behavior as a means of assessment; half of these studies also included standardized assessments and structured interviews as a means to determine communicative form and function. The authors cautioned that the validity and reliability of these three forms of assessment (direct observation, standardized assessment, and interviews) has not yet been well established for this population. Given this, Sigafoos et al. (2011) concluded that based on the available evidence (e.g. Baptista et al. 2006; Brady and Halle 1997; Dahlgren Sandberg et al. 2000; Garber and Veydt 1990; Hetzroni and Rubin 2006; Ryan et al. 2004; Sigafoos et al. 2000a; Woodyatt and Ozanne 1997) individuals with RTT demonstrate a range of behaviors (e.g., shifting eye gaze, vocalizations, stereotyped hand movements, facial expressions) that may be either intentionally communicative, may be sensitive to changes in environmental consequences, or may be responses to changes in internal physiological states. Much of the evidence related to productive communication skills in individuals with RTT focuses on establishing the function of specific behaviors and the presence of intentionality. However, there remains very little information related to comprehension skills. In some reports, some parents indicate that their children with RTT can understand words or short sentences (von Tetzchner 1997) and may follow simple commands (Demeter 2000). On the other hand, Woodyatt and Ozanne (1992) described the comprehension skills of a small cohort of girls with RTT as severely affected (p.

4 165). They went on to elaborate that at least one of the individuals in their cohort did not respond to any environmental or speech sounds. These investigators reported inconsistent responses to phrases like no and the learner s first name being spoken. The skill heterogeneity found across studies could represent the wide range of skills across the population, limitations in assessment valid with this population, or a combination of both of these explanations. In summary, there remain many unanswered empirical questions related to both expressive and receptive communication skills of individuals with RTT. Given the limited spoken and gestural skills, along with the potential production of somewhat idiosyncratic communicative acts among individuals with RTT, it seems plausible to hypothesize that their social partners may differ in their interpretations of whether a child s actions serve as intentional communicative acts. With communicative acts that are intentional, a second area of interpretation is determining the intended purpose (i.e., communicative function) of the communicative act. There is some evidence that parents and caregivers of individuals with RTT are able to consistently identify potential communicative forms but show inter-observer variation in their interpretation of the function (Sigafoos et al. 2000b). This finding is consistent with evidence suggesting that social partners demonstrate inter-observer variation in their interpretations of communicative behavior in both children with (e.g., Yoder 1986) and without disabilities (e.g., Adamson, Bakerman, Smith, and Walters 1987). The extent to which parents attribute intentionality to communicative behavior does not seem to be affected by the severity of their child s disability (Yoder and Feagans 1988). However, parents may identify more communicative behaviors as intentional compared to non-parents (Adamson et al. 1987). Moreover, investigators have observed variation in the interpretations made between members of allied intervention disciplines (e.g., special education professionals versus speech and language pathologists; Wilcox et al. 1990) as well as within each of those groups (Carter and Iacono 2002). This study examined adult interpretations of the communicative intent of potential communicative behaviors of learners with RTT, a population for whom this type of evidence does not presently exist in the literature. A better understanding of how different social partners interpret potential communicative behavior furthers our ability to design and reliably implement communication interventions for individuals with RTT, a task that may be more difficult given their unconventional and limited communication skills (Sigafoos et al. 2009). Meadan et al. (2012) noted the importance of replicating their study with additional children, different communication functions (i.e., commenting) and particularly with samples of communicative behavior drawn from natural communication settings instead of structured elicitation contexts. As such, this study aims to partially replicate Meadan et al. (2012) with a particular focus on potential communicative acts produced by individuals with RTT in natural communication contexts. The two research questions are: (a) How do different adult social partners, specifically, special education professionals and parents, interpret the behavior of learners with RTT? (b) Are the adults interpretations related to their familiarity with learner or level of expertise? Similar to Meadan et al. (2012), we also examined the raters confidence in their interpretations.

5 Method Procedural Overview All procedures were approved by the University s Institutional Review Board prior to the beginning of the study. After obtaining informed consent from the learner s guardian, parents independently completed portions of the MacArthur-Bates Communicative Development Inventories (M-CDI) (Fenson et al. 1993), and the researchers administered sections of the Vineland Adaptive Behavior Scales Second Edition (Vineland-II) (Sparrow, Cicchetti, and Balla 2005). Because it was expected that all or most of the participants would score at or near the floor level on these standardized instruments, standard scores were not calculated. Rather, the measures were used to gather descriptive information about each participant s adaptive and communicative behaviors. Finally, parents completed a semi-structured interview regarding the learner s current communication skills and participation in daily routines, based, in part, on the Inventory of Potential Communicative Acts (Sigafoos et al. 2006). During the second visit, the researchers recorded the leaner engaging in several daily routines (details in subsequent section). The recordings were analyzed to identify short clips of potential communicative behavior produced by the learner (detailed below). Then, the recordings were spliced into short (5 8 s) segments such that a naïve observer would be able to see the learner engaged in the potential communicative behavior but would not be able to observe how the learner s social partner responded to her. The adult participants (i.e., raters ) were informed that they would be participating in a study that examined the communication of learners with RTT. After providing informed consent, they met with one of the researchers and participated in a structured interview and rating session in which they interpreted the short video clips. Participants This study included three females with RTT with uncertain receptive language skills and less than five expressive words, as reported by primary caregiver. Fourteen adult participants also participated. Jane Age 8, lived with her mother, father and three typically developing siblings. She had a diagnosis of classic/typical RTT and a confirmed MECP2 mutation (c.391dupa). She was able to walk with assistance. Jane consistently was able to eat solid food but was unable to drink liquids independently, however, she did not use a gastric feeding tube (g-tube). Her mother reported that her medical history was positive for seizure activity but that it was currently controlled with medication. Her mother reported that at the time of the study, Jane responded to her spoken name and understood short phrases such as Are you tired? but did not use any formal communication. Her mother reported that she reacted to loud noises and turned her head towards environmental sounds. Additionally, her mother had observed that Jane would listen to a story for at least 5 min. Jane s mother also reported that she almost always vocalized pleasure and smiled when spoken to by a caregiver. It was unclear whether this behavior was also present with less familiar social partners.

6 Sarah Age 14, lived with her mother and father. She had a diagnosis of classic/typical RTT, although genetic testing did not identify specific type of MECP2 mutation. Her medical history was positive for seizure activity, although during the video recording, seizures were not observed. Sarah consistently ate solid foods but relied on a bottle and g-tube tube to ingest liquids and additional nutrition. Her mother reported that at home, Sarah often reacted to loud noises and sometimes turned her head toward a sound. Additionally, she sometimes smiled when she heard the spoken word yes. She understood simple phrases that include Are you hungry? but did not use any spoken words, sign approximations, or graphic symbols. Judy Age 7, lived with her mother and father and two typically developing siblings. She had a diagnosis of classic/typical RTT and a confirmed MECP2 mutation (R168X). Her mother reported the absence of seizure activity at the time of the study. Judy retained the ability to eat solid food and consistently drink from a straw. She did not use a g-tube. Her mother reported Judy responded when her name was called, produced no, no, and produced some sound effects such as baa-baa for a sheep when shown pictures and asked to make the sound. Her mother also reported that when asked, Judy could point to common objects and body parts. Judy s mother reported that she inconsistently followed instructions involving two contingent steps (e.g., When you are done chewing your food, look at me ). She had access to a speech-generating device (Tobii C12 Communication Device with Eye Control) for approximately 2.5 years prior to the start of the study. The Tobii allowed Judy to directly select buttons using eye gaze from a single home screen that displays 20 symbols. The symbols included line drawings, real photos, and short videos. Adult Participants Fourteen adults ( raters ) also participated in the study. At least one parent of each of each of the learners participated. In addition, parents were asked to identify educators or other professionals who had worked closely with their child in the past year. All of the adult participants completed a 15-min structured interview designed to obtain general demographic information, information about the rater s contact time with the learner, and information about their knowledge of communication. Table 5 in Appendix provides the structured interview questions. Subsequently, the participants viewed video clips of each of the learners and completed forms indicating their interpretations of the potential communicative acts depicted. Table 1 outlines the professional designations of the adult participants and shows their relationship to the individuals with RTT. Inclusion criteria for educators/professionals included at least 2 years of experience working as a special education professional and provision of direct services to one of the learners with RTT for at least 2 months during the preceding school year. All of the special education professionals were female. The majority had worked in their respective fields for more than 12 years (M =17.22, SD =12.01) and displayed a wide range of experience with individuals with significant developmental disabilities. All of the educators (including assistants and therapy disciplines) reported experience with learners who used both high and low-tech augmentative and alternative communication (AAC) systems. The caregivers that participated included: Jane s mother, Sarah s

7 Table 1 Adult participants and relationship to individual with RTT Individuals with RTT Caregiver (n =4) Special Education Professionals (n =10) Jane mother Special Education Teacher (1) Paraprofessionals (2) Speech-Language Pathologist (1) Sarah mother Special Education Teacher (1) Paraprofessional (1) Speech-Language Pathologist (1) Occupational Therapist (1) Physical Therapist (1) Judy mother Paraprofessional (1) father mother and Judy s mother and father. They identified themselves as primary caregivers for their daughters. The raters were categorized by four variables: as familiar if they indicated they knew the learner whose behaviors they were rating, as unfamiliar if they indicated they did not know the learner, as professional if they were a special education professional and as parent if they were a caregiver to an individual with RTT. This created four subgroups: familiar professional, unfamiliar professional, familiar parent, unfamiliar parent. Procedures Preparing video samples of potential communicative acts During the first visit to the participant s home, the researchers identified daily routines in which the learner was reported by the consenting caregiver to engage in potential communicative acts. Jane s mother reported an afterschool mealtime, a transition to a TV activity in another room, and watching a preferred TV program as viable daily routines. Jane s mother was a potential communicative partner during these routines as she was in close proximity to Jane and was engaged in the routines with her. Sarah s mother identified an afterschool mealtime and dialogic book reading while rocking Sarah in a chair as familiar routines in which her daughter may demonstrate communicative behavior. Sarah s mother was the potential communicative partner during these routines. Judy s parents reported that watching a preferred TV program and eating a snack on the couch were daily routines in which she was likely to engage in potential communicative acts. During these routines, Judy s preschool-age brother and mother were potential communicative partners. During a second visit, the researcher observed and videotaped the learner and her communicative partners engaging in the routines, as described above. These sessions lasted approximately 75 min, with approximately 80 % of that time devoted to recording the learner and her communicative partner(s). From the recordings, five instances of the learner producing potential communicative acts were selected. These behaviors represented discrete behaviors that the learner

8 had independently produced. The behaviors were initially identified according to examples of communicative potential communicative acts outlined by Sigafoos et al. (2000a). The researcher operating the video recorder identified potential communicative functions of learner s behaviors (five for each learner) that had been selected. For each learner, the instances of potential communicative behavior represented at least one potential comment, one potential request, and one potential protest. For example, a potential request was captured as Jane s mother is cutting pieces of a preferred food item and feeding them to her. Jane s mother pauses to say something to the researcher who is also in the room. Jane directs her gaze toward her mother, then toward the food, then back toward her mother. An instance of a potential request was captured when Sarah s mother is feeding her. Sarah sits across from her mother who is giving her a spoonful of food. Her mother pauses, shifting her attention away from Sarah. Sarah turns her head and shifts her gaze toward the plate of food. A potential comment was captured when Judy is watching television. Judy is sitting on the couch, an audible change occurs on the television (outside of the camera shot). Judy smiles, starts to rock quickly, move her arms, and looks toward the camera (and the researcher off-camera). Reliability The behaviors that were identified were partitioned into 5 8 s clips, which limited the contextual information that could bias the raters, most importantly, the verbal and nonverbal behavior of the adult following the child s potential communicative behavior. The context that was shown provided information related to the daily routine in which the child was engaged (e.g., audible television noise, visible food and utensils, visible book), but did not show the communicative partner s response to any of the potential communicative behaviors. To establish reliability, a second researcher (a doctoral student and speech-language pathologist) viewed the video clips and identified potential functions of the learners behaviors. Agreement on the potential function, calculated by (number of instances of agreement total opportunities 100) was 100 %. Obtaining video ratings from social partners One of three researchers met individually with each rater at a convenient location (e.g., school, home) and time. The meeting locations provided privacy in order to protect the learners and raters identities. After the structured interview (see above for more detail), the researcher administered the video clip rating instructions (adapted from Meadan et al. 2012): You will be viewing short video clips of an individual with RTT. These clips were extracted from longer recordings of the learner engaging in routine activities in her home setting. You may be familiar with one of the learners. I will play each video clip once. After each clip, I will ask you a series of questions. The questions are related to the child s behavior shown in the clip. The rater sat facing a MacBook Pro 13 laptop computer. The five potential communicative behaviors for each learner were presented as a set; the sets were presented in a counterbalanced order across raters. The researcher reminded the rater that he/she would watch each video clip and then respond to the series of questions. The researcher

9 recorded their responses and verified with the rater regarding his/her response. The raters responded to these questions: (a) Was the child communicating? (b) Was the child s intention clear? (c) What was the child communicating? (choices available were: Request object, Request action, Protest or Reject, Request Comfort, Greeting, Comment, Other) (d) For each of the preceding questions informants were asked, How confident are you with your response? This was indexed on a scale from 1 to 6 with 0 anchored with the words, not confident, 3 4 anchored with confident and 6 representing very confident. All 14 raters responded to five video clips for each of the three learners (yielding 210 total ratings). That is, they rated the communicative behavior of both learners with whom they had contact time and learners with whom they had no contact time. For the purposes of data analysis, some of the options regarding communicative function were collapsed into broader categories. This resulted in four general categories: rejections/ protests, requests (for action, object, or comfort), comments (comment or greeting), and unclear (not communicating or communication but with unclear intent). Results There were no clear, qualitative differences with respect to special education professionals and parents definitions of communication. Generally, raters defined communication as a critical ability that involves an exchange of verbal and/or nonverbal symbols and occurs between at least two people. When judging the video clips, the raters indicated that the learners were communicating in 89 % (187/210) of the clips. For these potential communicative behaviors, the raters indicated that the learners intent was clear in 85 % (159/187) of the opportunities. A variety of communicative functions were reported for each participant, with requesting object, commenting and protesting most frequently observed. Table 2 represents the percent of total rating opportunities that were assigned to each of the general communicative function categories across the different adult rater groups. On average, familiar parents rated more of the video clips as unclear - meaning that there was no communicative act, or that the function was unclear - compared to the other rater groups. Both familiar and unfamiliar professionals rated more clips as representing comments than either parent group. Familiar raters (parents and professionals) rated more clips as representing requests compared to unfamiliar raters. The percentage of clips identified as representing protests did not differ much across rater groups. Table 3 shows the frequency of agreement regarding the identified function within and across adult rater groups for each of the children. On average, agreement was relatively poor, ranging from 5 to 87 %. With the exception of unfamiliar parents rating Sarah s behaviors, agreement within groups of similar raters was, overall, no better than between groups. These results cannot be extended to ratings within familiar parents, however, as only one pair of familiar parents participated in the study. Comparisons between the functions identified by the adult rater groups and the research team suggest similarly limited agreement, for the functions of commenting and protesting. Table 4 shows the percent of agreement between adult raters and the research team. Interestingly, familiar professionals and unfamiliar parents showed strong agreement with the research team regarding request behaviors, but familiar

10 Table 2 Percent of total ratings assigned to communicative functions across adult raters subgroups Communicative function Familiar Unfamiliar Total Professional Parent Professional Parent Comment Jane 40 % (8/20) 20 % (1/5) 27 % (8/30) 20 % (3/15) 29 % (20/70) Sarah 12 % (3/25) 0 % (0/5) 16 % (4/25) 0 % (0/15) 10 % (7/70) Judy 20 % (1/5) 10 % (1/10) 33 % (15/45) 10 % (1/10) 26 % (18/70) Total 24 % (12/50) 10 % (2/20) 17 % (17/100) 10 % (4/40) 17 % (35/210) Reject/protest Jane 15 % (3/20) 0 % (0/5) 30 % (6/30) 13 % (2/15) 16 % (11/70) Sarah 16 % (4/25) 20 % (1/5) 12 % (3/25) 13 % (2/15) 14 % (10/70) Judy 20 %(1/5) 20 % (2/10) 29 % (13/45) 0 % (0/10) 23 % (16/70) Total 16 % (8/50) 15 % (3/20) 22 % (22/100) 10 % (4/40) 18 % (37/210) Request Jane 40 % (8/20) 40 % (2/5) 33 % (10/30) 33 % (5/15) 36 % (25/70) Sarah 32 % (8/25) 40 % (2/5) 36 % (9/25) 40 % (6/15) 36 % (25/70) Judy 60 % (3/5) 40 % (4/10) 20 % (9/45) 10 % (1/10) 24 % (17/70) Total 38 % (19/50) 40 % (8/20) 28 % (28/100) 30 % (12/40) 32 % (67/210) Unclear Jane 15 % (3/20) 40 % (2/5) 10 % (3/30) 20 % (3/15) 16 % (11/70) Sarah 28 % (7/25) 40 % (2/5) 32 % (8/25) 20 % (3/15) 29 % (20/70) Judy 0 % (0/5) 30 % (3/10) 13 % (6/45) 60 % (6/10) 21 % (15/70) Total 20 % (10/50) 35 % (7/20) 17 % (17/100) 30 % (12/40) 21 % (46/210) Functions identified as other not counted towards in any of the categories listed parents did not. Familiar professionals also showed the highest agreement for commenting behaviors, with familiar parents again showing the lowest agreement. Finally, familiar and unfamiliar professionals showed slightly higher agreement regarding rejecting/protesting behavior compared to familiar and unfamiliar parents. Figure 1 provides average confidence ratings across each of the adult rater groups for all three learners. Overall, average confidence was relatively high and there were no consistent differences between rater groups. Discussion This study examined adults interpretations of potential communicative acts produced by three individuals with RTT. The findings indicate that despite limited behavioral repertoires and often, vague communicative signals, special education professionals and parents interpret potential communicative acts as having clear intentions and representing a range of communicative functions. There was modest variability in how familiar and unfamiliar professionals and parents judged the communicative functions of potential communicative acts when viewing short clips of learners

11 Table 3 Matrix of inter-rater agreement (agreements /[agreements+disagreements]) of communicative functions within and across adult rater groups Participant/Rater group Familiar parent Familiar professional Unfamiliar parent Jane Familiar parent N/A Familiar professional 35 % (7/20) 47 % (14/30) Unfamiliar parent 40 % (6/15) 47 % (28/60) 47 % (7/15) Unfamiliar professional 30 % (9/30) 51 % (69/120) 59 % (53/90) Sarah Familiar parent N/A Familiar professional 32 % (8/25) 30 % (15/50) Unfamiliar parent 53 % (8/15) 44 % (23/75) 87 % (13/15) Unfamiliar professional 48 % (12/25) 33 % (41/125) 63 % (47/75) Judy Familiar parent 60 % (3/5) Familiar professional 20 % (2/10) N/A Unfamiliar parent 5 % (1/20) 20 % (2/10) 40 % (2/5) Unfamiliar professional 24 % (22/90) 49 % (22/45) 20 % (18/90) Total across participants Familiar parent N/A Familiar professional 31 % (17/55) 36 % (29/80) Unfamiliar parent 30 % (15/50) 37 % (53/145) 49 % (22/35) Unfamiliar professional 30 % (43/145) 46 % (132/290) 46 % (118/225) Numbers in parentheses indicate agreements/total opportunities. N/A denotes agreement was not calculated due to only 1 rater in the group behaviors during daily routines, despite overall high levels of confidence reported by most groups. Overall, these results support the hypothesis that different communicative partners are likely to interpret and respond to the potential communicative behaviors exhibited individuals with RTT in different ways. This present study partially supports the findings of Meadan et al. (2012). First, Meadan et al. (2012) found no qualitative differences across the four groups of adult raters, either in how they defined communication or how they described how children communicate. Similarly, raters in our Table 4 Percent of agreement in identified communication functions between adult raters and research team Function Familiar Unfamiliar Parent Professional Parent Professional Comments 25 % (2/8) 42 % (8/19) 31 % (5/16) 32 % (13/41) Protests 40 % (2/5) 47 % (7/15) 36 % (4/11) 48 % (12/25) Requests 43 % (3/7) 75 % (12/16) 86 % (11/13) 56 % (19/34) Numbers in parentheses indicate agreements/total opportunities

12 Parent Familiar Expert Familiar Expert Unfamiliar Parent Unfamiliar 1 0 Jane Sarah Judy Fig. 1 Average confidence levels across potential communicative acts for each learner; not confident =0 1, confident =3 4, very confident =5 6 study did not show qualitative differences in their definition of communication and description of how children communicate. Second, Meadan et al. (2012) demonstrated that raters who were both familiar and expert were more confident and accurate in their ratings than those who were unfamiliar and non-expert. In the present study, there were no clear differences in confidence between groups. Findings reported by Meadan et al. (2012) suggested greater variability in accuracy across raters in their judgments of rejecting behavior compared to requesting behavior. The present study was less clear regarding accuracy of judgments of the function of behaviors, but found that familiar professionals and unfamiliar parents showed strong agreement with the research team regarding request behaviors. Familiar professionals also showed the highest agreement with the research team for commenting behaviors. Finally, familiar and unfamiliar professionals showed slightly higher agreement regarding rejecting/protesting behavior compared to familiar and unfamiliar parents. Implications There are important implications from the findings reported in this study. First, a better understanding of how adults understand and interpret potential communicative acts could lead to the development of more appropriate assessments and interventions for individuals with RTT. The variability in interpretation of communicative function across participants combined with our limited evidence base for assessing communicative intent in individuals with RTT (and other individuals with atypical communication development) highlights the critical need for systematic coordination between home and school environments to ensure accurate assessment of communication status. Second, if there are differences in how special education professionals and parents understand and interpret potential communicative acts, then feedback to the learner could vary, which could compromise the acquisition of new communicative skills (Meadan et al. 2012). For example, if special education professionals and parents

13 reinforce a learner inconsistently for a communicative act, she may be less likely to acquire and maintain that emerging skill/behavior. It seems important for social partners, across settings, to establish reliable responses for specific (and perhaps unclear or unconventional) topographies of potential communicative behavior. Responses that are delivered by various social partners with high fidelity would potentially increase the learner s propensity to use the forms that are reinforced consistently, thus increasing her overall communicative competence. Limitations There are limitations to our findings. This study reports the interpretations of a small number of raters who interpreted a small number of behaviors from individuals with RTT. Given the relatively small number of participants in the current investigation, a replication with another group of individuals with RTT and their social partners, including special education professionals is warranted. This would provide more evidence related to how adults interpret the potential communicative behavior of individuals with RTT and the inter-observer reliability of their interpretations. The small sample also precluded the use of statistical analyses to facilitate interpretation of the findings. Moreover, it is possible that the communicative behaviors that were selected and shown to raters served multiple functions. As the function of the behaviors shown in the video clips was not elicited in a structured protocol, or confirmed via functional analysis, these findings do not provide evidence regarding the accuracy with which special education professionals and parents interpret the function of the potential communicative acts produced by individuals with RTT, only their reliability with each other and with the research team. A structured protocol would provide evidence related to how different individuals with RTT perform during tasks designed to elicit specific communicative behaviors. A functional analysis would provide evidence to guide professionals and parents as they plan how to best respond to potential communicative behavior and increase consistency in their responses across contexts. Finally, the protocol was not designed to establish an exhaustive list of the learners potential communicative behaviors, but rather to identify those that were most salient to the primary caregivers. The methods we used were designed to answer a question related to reliable consensus among social partners, including the interpretations made by the research team. Future Directions Future research may incorporate functional analyses to verify the functions of potential communicative acts produced by individuals with RTT and compare these functions with the interpretations made by social partners. Knowledge about discrepancies between form, function and response, could aid interventionists as they seek to establish consistent patterns of reinforcement to bids for communication and thus increase communicative behaviors in individuals with RTT. As we continue to understand how potential communicative acts may be interpreted by the social partners of individuals with RTT, as well as describe the forms and functions of communicative behavior within this population, we will be better able to provide continuity of intervention across settings.

14 Appendix Table 5 Structured interview questions 1. Do you know this child? (Examiner will circle yes/no) 2. How do you know this child? (Examiner will note the relationship between rater and learner.) 3. How many months have you worked with his learner? 4. How many minutes each day are you in close proximity with the child? This means you are likely able to observe and/or interact with the child during these minutes. 5. Approximately how many days per week do you spend with this child? 6. From your knowledge and experience, how would you define communication? 7. Do you observe this learner to: a. make vocalizations? b. shake or turn her head? c. use gestures? d. use signs? References Adamson, L. B., Bakerman, R., Smith, C. B., & Walters, A. S. (1987). Adults interpretation of infants acts. Developmental Psychology, 23(3), Baptista, P. M., Mercadante, M. T., Macedo, E. C., & Schwartzman, J. S. (2006). Cognitive performance in Rett syndrome: a pilot study using eyetracking technology. Journal of Intellectual Disability Research, 50, Bates, E., Camaioni, L., & Volterra, V. (1975). The acquisition of performatives prior to speech. Merrill- Palmer Quarterly, 21(3), Bates, E., Benigni, T., Bretherton, I., Camaioni, L., & Volterra, V., (Eds.). (1979). The emergence of symbols: Cognition and communication in infancy. New York: Academic. Brady, N. C., & Halle, J. W. (1997). Functional analysis of communicative behaviors. Focus on Autism and Other Developmental Disorders, 12, Carter, M., & Iacono, T. (2002). Professional judgments of the intentionality of communicative acts. Augmentative and Alternative Communication, 18, Dahlgren Sandberg, A., Ehlers, S., Hagberg, B., & Gillberg, C. (2000). The Rett syndrome complex: communication functions in relation to developmental level and autistic features. Autism, 4, Demeter, K. (2000). Assessing the developmental level in Rett syndrome: an alternative approach. European Child and Adolescent Psychiatry, 9, Ellaway, C., & Christodoulou, J. (2001). Rett syndrome: clinical characteristics and recent genetic advances. Disability and Rehabilitation, 23, Fehr, S., Bebbington, A., Nassar, N., Downs, J., Ronen, G. M., De Klerk, N., & Leonard, H. (2011). Trends in the diagnosis of Rett syndrome in Austrailia. Pediatric Research, 70, Fenson, L., Dale, P. S., Reznick, J. S., Thal, D., Bates, E., Hartung, J. P., Pethick, S., & Reilly, J. S. (1993). The MacArthur communicative development inventories: User s guide and technical manual. SanDiego: Singular Publishing Group. Garber, N., & Veydt, N. (1990). Rett syndrome: a longitudinal developmental case report. Journal of Communication Disorders, 23, Hagberg, B. (1995). Rett syndrome: clinical peculiarities and biological mysteries. Acta Paediatrica, 84, Hagberg, B. (2002). Clinical manifestations and states of Rett syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 8,

15 Hagberg, B., & Witt-Engerstrom, I. (1986). Rett syndrome: a suggested staging system for describing impairment profile with increasing age towards adolescence. American Journal of Medical Genetics, 24, Hagberg, B., Hanefeld, F., Percy, A., & Skjeldal, O. (2002). An update on clinically applicable diagnostic criteria in Rett syndrome: comments to the Rett syndrome clinical criteria consensus panel satellite to european paediatric neurology society meeting Baden baden, German, 11 september European Journal of Paediatric Neurology, 6, Hetzroni, O. E., & Rubin, C. (2006). Identifying patterns of communicative behaviors in girls with Rett syndrome. Augmentative and Alternative Communication, 22, Johnston, S., Reichle, J., Feeley, K., & Jones, E. (2012). Augmentative and alternative communication strategies for individuals with severe disabilities. Baltimore: Paul H. Brookes. Laurvick, C. L., De Klerk, N., Bower, C., Christodoulou, J., Ravine, D., Ellaway, C., & Leonard, H. (2006). Rett syndrome in Australia: a review of the epidemiology. The Journal of Pediatrics, 148(3), Marschik,P.B.,Kaufmann,W.E.,Sigafoos,J.,Wolin,T.,Zhang,D.,Bartl-Pokorny,K.D.,&Johnston,M.V. (2013). Changing the perspective on early development of Rett syndrome. Research in Developmental Disabilities, 34(4), Meadan, H., Halle, J. W., & Kelly, S. M. (2012). Intentional communication of young children with autism spectrum disorder: judgments of different communication partners. Journal of Developmental Disabilities, 24, Neul, J. L., Fang, P., Barrish, J., Lane, J., Caeg, E. B., Smith, E. O., & Glaze, D. G. (2008). Specific mutations in methyl-cpg-binding protein 2 confer different severity in Rett syndrome. Neurology, 70(16), Neul, J. L., Kaufmann, W. E., Glaze, D. G., Christodoulou, J., Clarke, A. J., Bahi-Buisson, N., & Percy, A. K. (2010). Rett Syndrome: revised diagnostic criteria and nomenclature. Annals of Neurology, 68(6), Ryan, D., McGregor, F., Akermanis, M., Southwell, K., Ramke, M., & Woodyatt, G. (2004). Facilitating communication in children with multiple disabilities: three cases of girls with Rett syndrome. Disability and Rehabilitation, 26, Sigafoos, J., Woodyatt, G., Keen, D., Tait, K., Tucker, M., Roberts-Pennell, D., & Pittendreigh, N. (2000a). Identifying potential communicative acts in children with developmental and physical disabilities. Communication Disorders Quarterly, 21(2), Sigafoos, J., Woodyatt, G., Tucker, M., Roberts-Pennel, D., & Pittendreigh, N. (2000b). Assessment of potential communicative acts in three individuals with Rett syndrome. Journal of Developmental and Physical Disabilities, 12, Sigafoos, J., Woodyatt, G., Keen, D., Tait, K., Tucker, M., & Roberts-Pennel, D. (2006). The inventory of potential communicative acts. In J. Sigafoos, M. Arthur-Kelly, & N. Butterfield (Eds.), Enhancing everyday communication for children with disabilities (pp ). Baltimore: P. H. Brookes. Sigafoos, J., Green, V. A., Scholosser, R., O Reilly, M. F., Lancioni, G. E., Rispoli, M., & Lang, R. (2009). Communicative intervention in Rett syndrome: a systematic review. Research in Autism Spectrum Disorders, 3, Sigafoos, J., Kagohara, D., van der Meer, L., Green, V. A., O Reilly, M. F., & Lancioni, G. E. (2011). Communication assessment for individuals with Rett syndrome: a systematic review. Research in Autism Spectrum Disorders, 5, Sparrow, S. S., Cicchetti, D. V., & Balla, D. A. (2005). Vineland-II adaptive behavior scales: Survey forms manual. Circle Pines: AGS Publishing. Urbanowicz, A., Leonard, H., Girdler, S., Ciccone, N., & Downs, J. (2014). Parental perspectives on the communication abilities of their daughters with Rett syndrome. Developmental neurorehabilitation, (0), 1 9. von Tetzchner, S. (1997). Communication skills among females with Rett syndrome. European Child and Adolescent Psychiatry, 6, Wetherby, A. M., Cain, D. H., Yonclas, D. G., & Walker, V. G. (1988). Analysis of intentional communication of normal children from the prelinguistic to the multiword stage. Journal of Speech and Hearing Research, 31(2), Wilcox, M. J., Kouri, T. A., & Caswell, S. (1990). Partner sensitivity to communication behavior of young children with developmental disabilities. Journal of Speech and Hearing Disorders, 55, Woodyatt, G., & Ozanne, A. (1992). Communication abilities and Rett syndrome. Journal of Autism and Developmental Disorders, 22, Woodyatt, G., & Ozanne, A. (1997). Rett syndrome (RS) and profound intellectual disability: cognitive and communication similarities and differences. European Child and Adolescent Psychiatry, 6,

16 Yoder, P. (1986). Clarifying the relationship between degree of infant handicap and mental responsivity to infant communicative cues: measurement issues. Infant Mental Health Journal, 7(4), Yoder, P., & Feagans, L. (1988). Mothers attributions of communication to prelinguistic behavior of developmentally delayed and mentally retarded infants. American Journal on Mental Retardation, 93(1),