PLEASE SCROLL DOWN FOR ARTICLE

Transcription

1 This article was downloaded by:[johns Hopkins University] On: 30 July 2007 Access Details: [subscription number ] Publisher: Informa Healthcare Informa Ltd Registered in England and Wales Registered Number: Registered office: Mortimer House, Mortimer Street, London W1T 3JH, UK Augmentative and Alternative Communication Publication details, including instructions for authors and subscription information: Identifying patterns of communicative behaviors in girls with Rett syndrome Orit E. Hetzroni a ; Corinne Rubin a a School of Education, University of Haifa, Haifa, Israel Online Publication Date: 01 March 2006 To cite this Article: Hetzroni, Orit E. and Rubin, Corinne (2006) 'Identifying patterns of communicative behaviors in girls with Rett syndrome', Augmentative and Alternative Communication, 22:1, To link to this article: DOI: / URL: PLEASE SCROLL DOWN FOR ARTICLE Full terms and conditions of use: This article maybe used for research, teaching and private study purposes. Any substantial or systematic reproduction, re-distribution, re-selling, loan or sub-licensing, systematic supply or distribution in any form to anyone is expressly forbidden. The publisher does not give any warranty express or implied or make any representation that the contents will be complete or accurate or up to date. The accuracy of any instructions, formulae and drug doses should be independently verified with primary sources. The publisher shall not be liable for any loss, actions, claims, proceedings, demand or costs or damages whatsoever or howsoever caused arising directly or indirectly in connection with or arising out of the use of this material. Taylor and Francis 2007

2 Augmentative and Alternative Communication, March 2006 VOL. 22 (1), pp Identifying Patterns of Communicative Behaviors in Girls with Rett Syndrome INTRODUCTION ORIT E. HETZRONI* and CORINNE RUBIN School of Education, University of Haifa, Haifa, Israel The purpose of this study was to investigate behaviors of girls with Rett syndrome under different conditions and to determine if there were patterns of functional communicative behaviors. Six communication protocols portraying familiar and non-familiar activities were created to assist in identifying specified behaviors. The protocols incorporated activities and pre-scheduled interruptions designed to enhance communicative behaviors. Eight girls in the third and fourth stages of Rett syndrome were videotaped during the protocols. Results indicated that the girls alternating eye gazing increased and persistence decreased when activities were interrupted. Additional behaviors varied by participant across different protocols. Several patterns of effective and ineffective communication were observed. Implications for practice and future research are discussed. Keywords: Communicative behaviors; Rett syndrome Most of the research in the last decade that has addressed characteristics of Rett syndrome has indicated that it results in severe physical, cognitive, and communicative disabilities (Kerr & Witt Engerstrom, 2001; Perry, 1991; Sigafoos, Laurie, & Pennell, 1995, 1996). This neurodevelopmental, X-linked dominant disorder manifests itself by stereotypical hand movements, apraxia, loss of communication skills, and loss of motor skills. These clinical expressions occur approximately between the ages of 6 and 18 months of age (International Rett Syndrome Association, 1998; Schanen et al., 1998; Xiang et al., 1998), during which girls with Rett syndrome lose most of their acquired speech, leaving them with only residual communication behaviors such as vocalization, facial expressions and eye gaze. The results of several investigations into the abilities and communication behaviors of girls with Rett syndrome have been mixed; in particular, the patterns of behaviors associated with communication have varied across studies (e.g., Hetzroni, Rubin, & Konkol, 2002; Sigafoos et al., 1995, 1996; von Tetzchner, 1997; Woodyatt & Ozanne, 1992, 1993, 1994, 1997). Form and function have been assigned to several behaviors that were considered to be communicative, despite not meeting published criteria for intentionality (Hunter, 1999; Lewis, & Wilson, 1996). In more recent studies into specific communicative behaviors, there has been an attempt to comprehend the nature of those behaviors (e.g., Iacono, Carter, & Hook, 1998; Sigafoos, Woodyatt, Tucker, Roberts-Pennell, & Pittendreigh, 2000b). No conclusive results were obtained from those attempts, however. Thus, there is little evidence as to the nature of the communicative behaviors of girls with Rett syndrome. Communication Issues in Rett Syndrome Early studies on Rett syndrome and communication focused on exploring the sensorimotor status and level of communication behaviors of girls with Rett syndrome (Woodyatt & Ozanne, 1992, 1993, 1994). In those studies, the girls exhibited a preintentional level of communication. Woodyatt and Ozanne (1992, 1993, 1994), for example, observed girls with Rett syndrome in naturalistic settings and analyzed their communicative behaviors. The analyses indicated that the girls *Corresponding author. c/o P.O. Box 399, Herut Tel Mond 40691, Israel. Tel: hetzroni@construct.haifa.ac.il ISSN print/issn online Ó 2006 International Society for Augmentative and Alternative Communication DOI: /

3 COMMUNICATION BEHAVIORS IN GIRLS WITH RETT SYNDROME 49 cognitive status was consistent with their communicative level: They performed below the Piagetian Cognitive Sensorimotor Stage III and their communicative level was preintentional (Woodyatt & Ozanne, 1994). Significant motor difficulties, however, demonstrated by the girls during the assessment, prevented the authors from being able to rule out the potential for motor limitations to confound their results. von Tetzchner (1997) interviewed 42 families with daughters who had Rett syndrome in order to collect information about the girls language development and any communicative behaviors they may have exhibited. Of these families, 32 reported that the girls understood some words and simple sentences, whereas 10 families perceived that the girls had no language comprehension, and 21 families stated that the girls used eye gazing for expressing simple choices. Nearly half of the group reported that they received AAC intervention (von Tetzchner, 1997). Behaviors associated with choice making were observed during an investigation of seven girls with Rett syndrome by Sigafoos et al. (1995). The girls participated in a series of structured opportunities for choice making. Their responses were accepted if the child reached and looked toward an object within 30 s of it being presented. The results of the study suggested that most of the girls could make meaningful choices and that their responses were valid. In another study, Sigafoos et al. (1996) found that girls with Rett syndrome could utilize a want symbol in order to make a request. In a later study, Sigafoos and colleagues attempted to identify potentially communicative acts of individuals with Rett syndrome (Sigafoos et al., 2000b). Teachers and direct-care staff who were familiar with the girls were interviewed about their behaviors. The girls were then videotaped in contexts that provided opportunities for few and many social interactions during mealtime with regular staff in their residential facilities. Reviews of the videotapes by independent observers indicated that all of the girls demonstrated extremely limited behavioral repertoires, but that their behaviors were communicative in nature, having the functions of greeting, conversation, requesting an object, protesting, and rejecting. Although the staff identified the behaviors as communicative, there was not a consensus among them about the nature of the communicative attempt. Sigafoos et al. (2000b) interpreted the results as conditioned communicative behaviors, but could not determine if the actions were socially motivated. Thus, although the staff judged that the behaviors had functions, they could not agree on the nature and meaning of those functions. Intentional Communication Iacono et al. (1998) examined the communicative behaviors of children with cognitive and physical disabilities to determine whether they met the conventional criteria for intentionality. The study involved engaging the children in familiar routines, which were then interrupted to create opportunities for communication. Traditionally, intentional communication in prelinguistic children has been operationally defined according to the following behaviors: (a) alternating eye gaze between a target and a listener; (b) persistence in signaling until the goal is reached; (c) changing the quality of the signal until the goal is met; (d) using a signal that is ritualized or has a conventional form within a specific context; (e) awaiting a response from the receiver; (f) terminating the signal when the goal is achieved; and (g) indicating satisfaction if the goal is met or dissatisfaction if it is not met (Wetherby & Prizant, 1989). Iacono et al. (1998) found that the children in their study demonstrated an intentional goal directed behaviors but they rarely exhibited coordinated attention, which was crucial to meeting the published criteria for intentionality. The children did show communicative attempts, however. The authors suggested that the children s lack of clarity in signaling the intentionality of the communication was, potentially, a result of their multiple disabilities rather than a lack of intentional communication per se. The literature has shown that children with multiple disabilities, including Rett syndrome, use various and, often, unconventional modalities to communicate. Their behaviors are often interpreted to be communicative, and therefore able to be assigned a function (Iacono et al., 1998; Sigafoos et al., 2000a). It is still not clear, however, if those behaviors are consistent, or if any cluster of behaviors form into a particular pattern that could be identified with a particular communicative function. Furthermore, researchers have demonstrated that these potentially communicative behaviors differ across situations. Iacono et al. (1998), for example, argued that familiarity had an impact on the patterns of behaviors produced by the children in their study. Koppenhaver et al. (2001) also demonstrated that familiar and unfamiliar stories read by mothers of girls with Rett syndrome had varying effects on the girls symbolic communication. Similarly, Sigafoos et al. (2000a,b) found that some behaviors produced by the girls appeared

4 50 ORIT E. HETZRONI AND CORINNE RUBIN differentially sensitive to the type of communication opportunity provided. STUDY AIMS The purpose of the current study was to examine modality and form of behaviors in order to identify patterns among the participants that could be regarded as being functionally communicative, even if they failed to meet published criteria for intentionality. Both Iacono et al. (1998) and Sigafoos et al. (2000a) used interrupted procedures for creating communication opportunities. Those opportunities were designed to assist in engaging the children in meaningful communication behaviors and create contexts that helped to clarify the function of each behavior or group of behaviors. A detailed description of those behaviors (including the modalities used) was then used to develop criteria for investigating behavior patterns associated with intentional (or potentially intentional) communication acts. In light of these earlier investigations, a further aim of the current study was to determine how behaviors manifested by girls with Rett syndrome differed across familiar and non-familiar situations in which the children were engaged in activities, which were then interrupted. METHODOLOGY Participants Eight girls, aged 4 11 years, participated in the study. All the girls were identified at various hospital-affiliated developmental centers as having Classic Rett syndrome. Four of the girls clinical characteristics were consistent with the pseudo-stationary stage (Stage 3) and four of them were in the late motor deterioration stage (Stage 4B) because they had never been ambulatory (Percy, 2002). All of the girls were enrolled in a school for children with developmental disabilities. They lived at varying distances from school and were bussed daily from their homes. All participants received intensive physical, occupational, music, speech, and aquatic therapy as part of their school program. Assessments All girls were assessed using evaluations conducted by the school psychologist, speechlanguage pathologist, and teachers. The Adaptive Behavior Scale-School (ABS-S:2) test (Lambert, Nihira, & Leland, 1993), the Inventory of Potential Communication Acts (IPCA) assessment (Sigafoos et al., 2000a) and non-speech tests (Huer, 1983) were used for evaluating their behavior and communication abilities. Staff interviews were conducted to obtain information about the girls use of eye gaze and facial expression, vocalizations, bruxism and hyperventilation, and body and hand movements. Videotapes taken during regular daily activities in the classroom were analyzed for additional information relating to these areas. All eight of the girls had hearing and vision within normal limits (Edith had corrected vision). All the girls met the criteria of the American Association on Mental Retardation (AAMR) 2002 definition for intellectual disability (or, in that organization s terminology, mental retardation) because they exhibited pervasive needs in all main life domains. Age equivalent scores on the Nonspeech Test (Huer, 1983) ranged from 0 to 6 months on the expressive language section and from 6 to 13 months on the receptive language section of the test. On the ABS-S:2 (1993), the girls domain score ratings ranged from very poor to below average in comparison with other individuals with intellectual disabilities Based on the IPCA (Sigafoos et al., 2000a), the girls behaviors, such as eye gaze, crying, laughing, vocalization, tremors, hyperventilation, and physical contact, were used for the functions of obtaining attention, protesting, requesting an object, or to initiate or respond during a conversation. The eight girls had no speech, limited use of their hands, engaged in frequent stereotypical hand movements, and were dependent on others for their basic needs. They all had scoliosis. Table 1 presents a summary of each girl s level of functioning and specific characteristics. Communication Protocols Six protocols were created to enhance the opportunities for demonstrating communicative behaviors across different situations. Each communication protocol included an activity and an interruption of the activity, which was repeated twice during each session. The interruptions were structured into the protocols to obstruct ongoing activities and to provide opportunities for the girls to communicate (Sigafoos et al., 1995, Sigafoos, 1999). The protocols included familiar and unfamiliar activities (see Table 2). An example of a familiar protocol was an exposure to a short video-clip on television. The girl was seated in front of the television with the teacher seated in close proximity within the girl s field of vision. The video contained songs that were interrupted by a stopwatch every 90 s. Once the

5 COMMUNICATION BEHAVIORS IN GIRLS WITH RETT SYNDROME 51 TABLE 1 Description and Rett-related characteristics of the eight girls, including by age, clinical stage (CS), and non-speaking (NS) test Name Age CS ABS-S:2 NS test (in months) Hand skills Ambulation Communication skills Rett-related behaviors Ann 10 3 Average poor Expressive: 0 Receptive: 8 11 Mandy 10 3 Average poor Expressive: 3 6 Receptive: 7 10 Helen 11 3 Average very poor Tammy 11 4B Below average very poor Expressive: 3 5 Receptive: Expressive: 0 Receptive: 6 8 Eats with an adapted spoon with assistance. Activates a switch with an open hand when her other hand is restrained or in a splint. Eats with an adapted spoon with assistance. Activates a switch with an open hand. Eats with an adapted spoon. Activates a switch with an open hand. Activates a switch with an open hand. Walks independently with a wide gait. Can sit up and get up independently from a chair. Walks with a wide gait but tends to walk asymmetrically; often needs assistance. Walks, but needs assistance to initiate. Can get-up from a chair independently. Listens attentively to stories and can answer simple questions. Expresses needs and desires by eye gazing, or by pointing with nose or hand to one of three symbols placed on a communication board. Can identify graphic and orthographic symbols. Walks toward a desired item or person. Listens attentively to stories and can answer simple questions. Communicates by eye gazing or by pointing with her nose to request a specific item from a choice of three graphic representations. Listens attentively to stories and can answer simple questions. Can choose one of two graphic representations by (a) pointing with head or nose, and (b) sometimes with an open hand using a double message switch. Not ambulatory Sometimes chooses between two food items by activating one of two single switches. Very limited communicative repertoire. Behaviors include crying and discontent. Stereotypical hand and chin banging Tremors Stereotypical hand wringing Frequent seizures Irregular breathing, bruxism, and gait dyspraxia Stereotypical hand movements Epileptic seizures, bruxism, irregular breathing and immature sleep patterns (continued)

6 52 ORIT E. HETZRONI AND CORINNE RUBIN TABLE 1 (Continued) Name Age CS ABS-S:2 NS test (in months) Hand skills Ambulation Communication skills Rett-related behaviors Nell 6 4B Average poor Expressive: 3 6 Receptive: 5 7 Tess 6 4B Average poor Expressive: 3 6 Receptive: 5 7 Rene 7 4B Average below average Edith 9 3 Average very poor Expressive: 4 7 Receptive: 9 11 Expressive: 2 4 Receptive: 4 6 Activates a switch with an open hand. Activates a switch with an open hand when other hand is restrained or in a splint. Activates a switch with an open hand. Eats with an adapted spoon. Activates a switch with an open hand. Not ambulatory Teachers report that she can choose one of two items using eye gazing and can activate a switch with assistance. Not ambulatory Teachers report that she chooses a preferred food at meals using a switch with one hand or by eye gazing. Not ambulatory Teachers report that she can choose one of two items by eye gazing and by placing her face on the preferred item. Walks with assistance; can sit and get-up independently from a chair. Teachers report that she can choose one of two items by pointing with her head or with her face. Bruxism Stereotypical hand clasping Bruxism Stereotypical hand flapping and waving Stereotypical hand movements Irregular breathing, bloating, and gait dyspraxia Stereotypical hand twisting

7 COMMUNICATION BEHAVIORS IN GIRLS WITH RETT SYNDROME 53 clip stopped (after 90 s), the teacher waited for another 90 s before getting up and turning on the video for a second 90-s interval. This procedure was repeated with two interruptions and then resumed until the end of the protocol. Once finished, the teacher indicated a need to return to regular class activities by describing the activity occurring there at that time, assisting the girl towards the door, and escorting the girl back to her classroom. An example of an unfamiliar protocol was exposure to water and sand. The girl was placed between two plastic basins containing sand in one and water in the other and told of the contents of each; one hand was placed in the sand and the other in the water. After 90 s, the containers were removed and placed out of reach. The teacher told the girl that they were being temporarily removed. After 90 s, the basins were returned to the previous positions and the girl s hands again placed inside. This procedure was repeated twice. Each time the basins were removed and then placed again within reach, with the girls hands placed in the sand and water basins. The procedure finished after two activities and two interruptions. Once finished, the girl was escorted back to the classroom. Each communication protocol was implemented on two separate occasions, with the second protocol administered 1 month after the first one. General Procedures Prior to the implementation of the communication protocols, each girl was assessed for developmental and communication level. After assessment the girls were taken individually to a designated classroom equipped with materials prepared for each session. All protocols were conducted in the classroom by the girls classroom teacher. The classroom was arranged and equipped with materials according to each of the protocols (i.e., blocks, television, computer, audiocassette, and books). When the communication protocol required the use of a television, for example, a unit that included a television and a videocassette player was wheeled into the room. Sessions were conducted near a table unless specified differently in the protocol (see Table 2). An individually adapted chair was used for each of the girls, and a video camera was positioned to capture them and their teacher. An independent observer videotaped all sessions. The procedure was explained and each communication protocol was demonstrated to the teachers prior to intervention. The girls classroom teacher implemented each protocol as demonstrated using written instructions as guidelines (see Table 2). Sessions lasted for approximately 10 min. A stopwatch was used by an independent observer to ensure time management and appropriate implementation of the procedure. Rooms were set-up in advance according to the protocols used before bringing in each girl. As an example, when the block building protocol was implemented, the teacher brought a participant s adapted chair into the classroom, placed it by the table, and arranged the blocks on the table. Only once everything was arranged and set-up as pre-planned, including the video-camera and the stop-watch, would the girl enter the room. Once the intervention terminated, she was escorted back to her classroom and then another participant was brought in. The teacher was seated next to the girl for the duration of the session (see Table 2). The procedures were implemented again 1 month later to determine the consistency of the participants communication behaviors. Thus, 12 sessions were conducted for each of the eight participants. All 12 protocols (i.e., six protocols repeated twice) were videotaped. Thus, all girls were videotaped twice for each protocol. Four observers were trained to identify behaviors that could be considered as communicative. The tapes were observed using 30-s intervals, during which the presence of each specified behavior was monitored and coded using a coding inventory. The specified behaviors were coded under the two conditions: during the activity and during the interruption. The tapes were coded independently, with the specified behaviors analyzed for patterns of modality and form. Coding Each videotape was analyzed to record the presence or absence of specified behaviors (i.e., percentage of touches, alternating eye gaze, persisting eye gaze, stereotypical hand movement, body movements, vocalization, smiles, frowns, bruxism, and walking to a target). The list of specified behaviors was assembled from studies by Iacono et al. (1998) and Sigafoos et al. (2000a), and were coded based on the operational definitions of communicative behaviors used in those studies: eye gazing, head movement, hand movement, body movement, and vocalization. Those behaviors were considered to be communicative (although not necessarily intentional) if they included one or more of the following (a) alternating eye gaze or sequencing between the teacher and one or two objects for at least 3 s; (b) persistence or escalation with eye gazing toward the teacher or the desired object until goal was achieved; (c) touching a desired object; (d) hitting

8 54 ORIT E. HETZRONI AND CORINNE RUBIN TABLE 2 Protocols for identifying patterns of communicative behaviors Protocol Paradigm Materials Activity Interruption Block building Water and sand activity Reading activity Computer activity Audio-cassette activity Television activity Unfamiliar Repeat procedure 3 times Unfamiliar Repeat procedure 3 times Familiar Repeat procedure 3 times Familiar Repeat procedure 3 times Familiar Repeat procedure 3 times Familiar Repeat procedure 3 times Nine blocks Water and sand containers Two books PC computer with monitor, speakers, and interface with single switch Audio-cassette with interface and switch Television and video-tape recorder with familiar video-tape. Blocks are placed on table within the reach of the child; after 45 s child is assisted in knocking the blocks down. Blocks are built again, procedure is repeated. Containers are placed on both sides of the child, whose hands are placed in containers. Books are placed for selection near the child; once selection is made, the story is read. A computer game is activated and the switch is placed within reach of the child. Audio-cassette is placed near child. Television is turned on; child is seated within viewing range. After 90 s blocks are moved out of reach for 90 s. After 90 s containers are placed out of reach for 90 s. After 90 s the book is closed and placed out of reach for 90 s. After 90 s computer monitor is turned off and switch is put out of reach for 90 s. After 90 s audiocassette is turned off and switch is placed out of reach for 90 s. After 90 s television is turned off for 90 s. a switch in order to initiate an event; (e) body movement, such as wiggling or moving head or torso toward or away from an object or person; and (f) any vocalizations other than breathing. In addition, specific other behaviors were added, based on publications that determined those behaviors to be communicative (Hunter, 1999; Koppenhaver et al., 2001). These behaviors were (a) smiling and other facial expressions, such as frowning; (b) bruxism; (c) stereotypical hand movement; (d) rapid body movements that could be identified as tremors; and (e) hyperventilation, which was defined as rapid audible breathing (see Table 3). They were coded in an attempt to associate them with the activities and the interruptions and, thereby, determine if they could be considered communicative. For the purposes of the current study, modality was referred to as the channel through which the information would be transmitted, form was referred to as the way the information was transferred, and functionality was referred to as the ability to transfer the information. For example, if a girl used her forehead (modality) and bent (form) towards a book placed on the table to indicate her choice for book reading (functionality), the communication was coded as functional. The behaviors were coded for presence of a communicative act (form), the modality used, and the apparent functionality of the act as interpreted by the observer. Interobserver Agreement Four independent observers coded all videotapes, of which 25% were randomly selected for coding twice for each participant across all protocols. Agreement was calculated for all behaviors as the number of agreements divided by the number of agreements plus the number of disagreements multiplied by 100. Interobserver agreement ranged from 86 to 98%, with a mean of 93% for all of the girls across all protocols. RESULTS Overall Patterns Table 3 presents individual data for each girl (all names are pseudonyms) according to activities and interruptions (with data collapsed across all protocols). An inspection of Table 3 reveals that seven of the eight participants demonstrated an increase in alternating eye gaze when activities were interrupted. However, only half of the girls demonstrated a decrease in eye gaze persistence during interruption, while two of the girls demonstrated an increase in persisting eye gaze

9 COMMUNICATION BEHAVIORS IN GIRLS WITH RETT SYNDROME 55 TABLE 3 Number of behaviors occurring for each variable for each girl during activities and interruptions Mandy Edith Ann Helen Nell Tammy Rene Tess Behaviors Ac In Ac In Ac In Ac In Ac In Ac In Ac In Ac In Alternating eye gaze Body movements Bruxism Frown Hyperventilation Persistence Stereotypical hand movements Smile Switch Touch Tremors Vocalization Walking to object or person during interruption. Seven of the girls demonstrated varying levels of increase in their stereotypical hand movements when activities were interrupted. Seven girls decreased their use of switches when the activity was interrupted. Four girls increased their touches during interruptions, while the other four decreased their number of touches. Thus, there were differences across behaviors in terms of changes in occurrence when comparing activities and the interruptions that occurred repeatedly every 90 s (i.e., according to the protocols). Changes in behaviors also differed for each of the girls. Mandy, for example, showed an increase in alternating eye gaze when the activity was interrupted and an increase in persistence during activity. She activated the switch more often when the activity was interrupted. She did not demonstrate hyperventilation, and frowned during any activities. Edith demonstrated mainly an increase in alternating eye gaze, stereotypical hand movement, and touches when the activity was interrupted. The other behaviors did not change noticeably in percentage of occurrence between activity and the interruption. Ann used her hands to touch objects, switches, and people. Switch activation and persistent eye gaze increased during activities. She used little alternating eye gaze overall, but this behavior did increase noticeably during interruption. Stereotypical hand movements and body movements also increased during interruption. Unlike the other girls, little change was demonstrated in Helen s behaviors, apart from an increase in her alternating eye gaze and her stereotypical hand movements. Nell decreased her persistent eye gaze and her touches during interruption, but increased all other behaviors, with her alternating eye gaze being most noticeable. Tammy increased both alternating and persistent eye gaze during interruption. In addition, she increased her stereotypical hand movement, her vocalization, and her hyperventilation. Rene decreased her touches, her switch activation, and her persistent eye gaze, and increased her alternating eye gaze and her smiles during interruption. Tess decreased her number of touches and her vocalizations during interruptions. Almost all of her behaviors remained consistent during activities and interruption protocols. Behaviors According to Conditions Percentages for each modality were calculated by dividing the total frequency of that modality across all participants by the total number of behaviors across all modalities and participants, and multiplying by 100. Behaviors (i.e., modalities) coded during all activities and interruptions across all girls are presented in Figure 1 according to the percentage of alternating eye gaze, body movements, bruxism, frowns, hyperventilation, persisting eye gaze, stereotypical hand movement, smiles, switch use, touches, tremors, vocalization, and walking to a target during activities and interruptions of the procedures. Inspection of Figure 1 reveals noticeable differences between activities and interruption of the six protocols by the girls across some behaviors. The most noticeable behavior change that occurred once an activity was interrupted was alternating eye gaze, which increased during interruptions, while switch use, persistence, and touching decreased during interruptions. A noticeable increase was

10 56 ORIT E. HETZRONI AND CORINNE RUBIN Figure 1. Percentage of behaviors of all girls with Rett syndrome across all activities by activity and interruption. also evident in the percentage of stereotypical hand movement manifested by the girls during interruptions. A Wilcoxon two related sample test (two-tailed) was conducted to determine if differences across activities and interruptions evident in Figure 1 were significant. Significant differences were found for alternating eye gaze (W ¼ 2.38; p ¼ 0.017), stereotypical hand movement (W ¼ 2.24; p ¼ 0.025) and switch use (W ¼ 2.11; p ¼ 0.035). These results demonstrated that during interruption, alternating eye gaze and stereotypical hand movement increased and switch use decreased. No other comparisons were significant (p ). Behaviors According to Familiar and Unfamiliar Protocols Table 4 shows the frequency of behaviors manifested for all the girls across the different familiar versus unfamiliar protocols according to the activities and the interruption (i.e., with data collapsed across the girls). As demonstrated by each girl, alternating eye gaze and stereotypical hand movements were the most noticeable behaviors that increased across all activities during interruption. Differences in persistence varied across various activities. During the computer protocol a familiar procedure switch activation and touches increased greatly during the activity, while alternating eye gaze increased during interruptions. A similar pattern of behavior occurred during the audio-cassette protocol, also a familiar procedure. In addition, there was an increase in touches, body movement, and stereotypical hand movement when the music was interrupted in the audio-cassette protocol. In the television protocol, another familiar procedure, there was more persistence when the television was on. When interrupted, there was more alternating eye gaze, more touches, more stereotypical hand movement, vocalization, and more walking towards an object or a person. In book reading, there was more persistence and alternating eye gaze during the activity and very little variability across all other behaviors. The block building and water and sand protocols were unfamiliar protocols. Results demonstrated that, although differences between activity and interruption were not large during the water and sand procedure, there was an increase in most behaviors during interruption for all girls.

11 COMMUNICATION BEHAVIORS IN GIRLS WITH RETT SYNDROME 57 TABLE 4 Number of behaviors occurring by each variable for all girls during activities and interruptions across all protocols Blocks Sand and water Reading Computer Audio cassette TV Behaviors Ac In Ac In Ac In Ac In Ac In Ac In Alternating eye gaze Body movements Bruxism Frown Hyperventilation Persistence Stereotypical hand movements Smile Switch Touch Tremors Vocalization Walking to object or person During the block protocol, the girls demonstrated an increase in most behaviors when the activity was interrupted. Figure 2 depicts the differences between the percentage of behaviors across familiar and unfamiliar protocols, with data across the girls collapsed. When a protocol was familiar, the girls demonstrated, proportionately, more persistence during the activity. When the unfamiliar protocol was presented, the girls showed more alternating eye gaze and stereotypical hand movements when the activity was interrupted. When looking at the results of the familiar and unfamiliar protocols, the most visible difference was again for alternating eye gaze. Although the data in Figure 1 demonstrate very little difference between activity and interruption across variables, the differences between familiar and unfamiliar settings were large across behaviors. Although persistence and stereotypical hand movement varied between activity and interruptions, the most noticeable difference for both familiar and non-familiar protocols was alternating eye gaze. Thus, the most consistent and effective behavior across all activities by all the girls was alternating eye gaze. A Wilcoxon two related sample test (two-tailed) was conducted to determine if differences across activities and interruptions for familiar and unfamiliar protocols evident in Figure 2 were significant. Significant differences were found for alternating eye gaze in familiar protocols (W ¼ 2.19; p ¼ 0.028) and switch use in familiar protocols (W ¼ 2.11; p ¼ 0.035). These results demonstrated that during interruption in familiar protocols, alternating eye gaze increased and switch use decreased. No other comparisons were significant (p ). DISCUSSION In this study the variability of communicative behaviors of girls with Rett syndrome across activities and interruptions was investigated. The results indicate that there was a pattern of communicative behaviors manifested by the girls and that the pattern was expressed across all protocols, which were designed to promote communication through activities and interruptions. The results indicate that the girls manifested many different behaviors, particularly in alternating eye gaze. The girls alternating eye gazing increased significantly when a given activity was interrupted, as opposed to the level of alternating eye gaze that occurred when they were engaged in an activity. The girls also showed significantly more stereotypical hand movements when the activity was interrupted. Switch use decreased significantly when an activity was interrupted. Persistence and touches decreased slightly when an activity was interrupted; however, those results were not significantly different. The other behaviors did not change significantly between activity and interruption. The behaviors used for coding in the current study were designed according to a theoretical framework based on previous reports, teacher observations of the children s communicative behaviors, the IPCA (Sigafoos et al., 2000a) and the results from the Iacono et al. (1998) study. In addition, the coding form included some neurological characteristics of Rett syndrome, such as stereotypical hand movements, bruxism, and hyperventilation that have been described as communicative in nature (Lindberg, 1991; Sigafoos et al., 2000a).

12 58 ORIT E. HETZRONI AND CORINNE RUBIN Figure 2. Percentage of behaviors of all girls with Rett syndrome by activity and interruptions across familiar and unfamiliar activities. The coding decisions also were based on teachers reports that attached meaning to various behaviors of the girls in lieu of minimal functional hand use and lack of verbalization. The teachers attached meaning to stereotypical hand movement, hyperventilation, and tremors behaviors that have a neurological basis to them as well as to other behaviors such as touch and switch activation. Thus, because many teachers thought that those behaviors were functional, they were added to the inventory. Yet, results indicated that eye gaze was the most consistent behavior across all protocols, while other behaviors differed according to the nature of the activity and each girl manifesting them. This result confirms descriptions by many practitioners (Kerr & Witt Engerstrom, 2001; Lindberg, 1991). It appears then, that alternating eye gaze is the most significant modality used during interventions, with results of the current study consistent with previous research (e.g., von Tetzchner, 1997). Although the general manifestation of behaviors was consistent across all girls, some behaviors were specific and unique for each girl, based on her motor and cognitive level. For example, the most noticeable difference in Mandy s behavior was an increase in persistence when engaged in an activity. Mandy showed no hyperventilation or bruxism, and did not demonstrate any walking to an object, although she was physically capable of doing so. All other behaviors did occur at differing levels. Edith showed a substantial increase in alternating eye gaze and stereotypical hand movement when an activity was interrupted. When Ann was engaged in an activity, she showed persistence, which decreased when the activity was interrupted, while her alternating eye gaze and stereotypical hand movement increased. Helen showed a great difference in alternating eye gaze between engagement in an activity and its interruption. Although she did not use many of the observed behaviors, the ones she did use were used quite frequently (persistence, alternating eye gaze, stereotypical hand movement, and smiling). Nell showed a substantial difference in alternating eye gaze between engagement during an activity and interruption. She also showed more body movements, stereotypical hand movements, bruxism, and vocalization, when an activity was interrupted. Tammy showed few communicative behaviors. She showed more persistence when an activity was interrupted. She showed no body movement, tremors, or frowning. Rene persisted more when engaged in an activity, and she alternated more when the activity was interrupted. Tess showed very little difference in

13 COMMUNICATION BEHAVIORS IN GIRLS WITH RETT SYNDROME 59 communicative behaviors across all situations, with no differences between activities and interruptions. Several researchers have implemented familiar and unfamiliar protocols to investigate communicative behaviors (e.g., Iacono et al., 1998; Koppenhaver et al., 2001). In an attempt to investigate the communicative behaviors of girls with Rett syndrome, familiar and unfamiliar protocols were administered in this study. Results indicated that several behaviors changed across familiar and unfamiliar protocols. There were noticeably more touches during unfamiliar protocols. Results also demonstrated that stereotypical hand movements and bruxism, which are indicative for diagnosing Rett syndrome, varied across familiar and unfamiliar protocols. There was consistency within each girl s behavior across protocols. Yet, results of this study demonstrated that different children engaged in different types of behaviors. These results were consistent with a previous study by Koppenhaver et al. (2001), who investigated the use of storybook reading as a means of enhancing symbolic communication in girls with Rett syndrome. In that study, mothers read familiar and unfamiliar stories incorporating AAC symbols to their daughters in their home environment. Results indicated that storybook interactions provided a useful context for learning language and for communication (Koppenhaver et al., 2001). However, there was not a consistent pattern to indicate the situation that optimized the girls communication performances. Half of the girls exhibited their highest communication level during the reading of familiar stories, while the others exhibited their highest communication level during the reading of unfamiliar stories. Results of Koppenhaver et al. s (2001) study demonstrated differences across various behaviors between familiar and unfamiliar protocols. In the current study, a difference in the quantity of stereotypical hand movements was indicated when an activity was familiar. Stereotypical hand movements are mandatory criteria for clinical diagnosis of Rett syndrome (Percy, 2002). The girls showed an increase in stereotypical hand movements when an activity was interrupted. There was a minimal difference, however, in unfamiliar situations. Furthermore, when the girls were engaged in a familiar activity, there were fewer stereotypical hand movements. One explanation could be that the girls engaged in stereotypical hand movements when they knew an activity was interrupted and was about to resume, thus explaining the difference in the stereotypical hand movements between familiar and unfamiliar situations. Bruxism is another supportive criterion of Rett syndrome (Percy, 2002). Results of the current study demonstrated that there was less bruxism during unfamiliar activities. There was also little difference between when the girls were engaged in an activity and when that activity was interrupted. One explanation could be that the girls engaged in bruxism when they knew what to anticipate; hence, the bruxism may have been an indication of their excitement in anticipating a known, familiar activity. Irregular breathing is a neurological characteristic that is also a supportive criterion of the clinical diagnosis of Rett syndrome (Percy, 2002). However, often practitioners and researchers cite hyperventilation as a communicative act (Lindberg, 1991; Sigafoos et al., 2000b). Hyperventilation did not differ across protocol activities and interruptions regardless of whether or not the girls were familiar with the protocols. Thus, results of this study did not provide support for hyperventilation as being intentionally communicative. One of the aims of this study was to investigate differences between activities and the varying effects on the girls behaviors. Results demonstrated that the behaviors manifested by the girls varied according to each protocol. For example, while persistence increased during block activity, it decreased during television viewing and reading. Alternating eye gaze also decreased during interruption of reading, unlike most of the other protocols. Desrochers, Morissette, and Ricard (1995) noted that in some situations, such as book reading, the criterion for joint attention is different than most other joint attention activities. This criterion implies that a child may assume that the adult is already looking at the target, and therefore alternating eye gaze would not be necessary during reading activities. Possibly, this could be an explanation for the differences found in the eye gazing behaviors demonstrated during reading. Some of the activities elicited active participation, such as activities that had switches. For example, the girls had to activate a switch to play a computer game and activate the audio-cassette. However, the results demonstrated differences across the various behaviors. As an example, when the computer was turned off, the girls touched the switch less frequently; when the music was interrupted, the girls increased their switch activation to start the music again. This pattern could imply that the girls understood the varying role of the switch across different tasks. An alternative explanation was that the music had a motivational effect for these girls (Kerr, 2002; Merker & Wallin, 2001).

14 60 ORIT E. HETZRONI AND CORINNE RUBIN Further Research Stereotypical hand movements appeared significantly more during interruptions. These results are consistent with previous literature revealing the appearance of various and, often, unconventional modalities to communicate, which were often assigned a function (e.g., Iacono et al., 1998; von Tetzchner, 1997). Thus, in the current study, those behaviors were interpreted as communicative by the schoolteachers and parents. Further research is needed into the role of stereotypical hand movements within the communicative realm. Uniform protocols were designed to ensure consistency across all activities for all of the girls who participated in the research. The use of those protocols prevented any individualization tailored to the girls cognitive and communicative abilities or their preferences. Further research is needed into the variability across different situations, taking into account differing cognitive levels and the challenges of communicating in a stimulating environment tailored to their preferences. Results demonstrated that there are different types of communicators and attested to the functionality of the girls behaviors. However, it is still not clear if those behaviors were, in fact, intentional. By carrying out the protocols twice, the consistency of the behaviors could be demonstrated. However, intentionality could not be confirmed. These results are consistent with previous research demonstrating the complexity of assessing intentionality in individuals with multiple disabilities (Iacono et al., 1998; Sigafoos et al., 2000a,b). Further research is needed on building an assessment tool that will facilitate the analysis of intentional communication in individuals with severe physical disabilities and complex communication needs (e.g., girls with Rett syndrome) and the role of their communication partners within the communication environment. CONCLUSIONS AND CLINICAL IMPLICATIONS Several behaviors were observed as a means of gaining information regarding behaviors of girls with Rett syndrome. Results of this study confirmed that the most significant and consistent behaviors manifested by all of the participants was alternating eye gaze. Behaviors such as stereotypical hand movement and switch use were also consistent for most of the girls. Most of the other behaviors showed no consistency across protocols and across girls. Thus, teachers and practitioners may need to treat with caution specific behaviors such as stereotypic behaviors manifested by students with Rett syndrome before acknowledging them as communicative behaviors. Based on observations of the girls behaviors across activities and interruption during the protocol activities, there seemed to be four styles of communication: (a) effective communicators; (b) communicators; (c) ineffective communicators; and (d) non-communicators. Only one girl, Ann, fit the first style as an effective communicator who had a specific and limited array of behaviors that were noticeably different between activity and interruption. Four of the girls, who had a specific array of behaviors that were different between activity and interruption, appeared to be communicators; however, some of those behaviors were not as numerous, and the differences were not as noticeable. Ineffective communicators included one girl, Nell, who had noticeable differences between behaviors, but those behaviors were not specific and spread across many behaviors. Two of the girls fit the non-communicator group, demonstrating behaviors that were limited in number, range, and the difference between activity and interruption. The first group of effective communicators could, and usually did, manage to convey their messages. For the second and third groups of communicators, who comprised the majority of participants, it seems important to teach their communicative partners how to recognize potentially communicative behaviors. This could lead to their increased use during regular daily activities. Results of this study stress the importance of the girls use of eye gaze for functional communication. It is essential for teachers and practitioners to distinguish between behaviors that serve a functional purpose and specific Rett associated behaviors that are neurologically based. References Adamson, L., & Chance, S. (1998). Coordinating attention to people, objects, and language. In A. M. Wetherby, S. F. Warren, & J. Reichle (Eds), Communication and language intervention series: Vol. 7. Transitions in prelinguistic communication (pp ). Baltimore, MD: Paul H. Brookes. Desrochers, S., Morissette, P., & Ricard, M. (1995). Two perspectives on pointing in infancy. In C. Moore & P. J. Dunham (Eds), Joint attention: Its origins and role in development (pp ). Hillsdale, NJ: Lawrence Erlbaum. Hetzroni, O. E., Rubin, C., & Konkol, O. (2002). Use of a computer-based intervention for teaching girls with Rett syndrome to identify symbols. Journal of Intellectual and Developmental Disability, 27,