Difficulties with communication have a profound impact on the lives of individuals with Rett syndrome and their caregivers. Globally, many families report difficulty accessing appropriate and timely information and services from professionals with expertise in augmentative and alternative communication (AAC) as it pertains to Rett syndrome. To address this need, international consensus-based guidelines for managing the communication of individuals with Rett syndrome were developed by combining available evidence and lived experience with expert opinion. A two-phase Delphi survey was built on statements and recommendations extracted from a review of over 300 pieces of literature combined with survey responses from communication professionals and caregivers. All statements that reached a predetermined threshold of 70% agreement were incorporated into guidelines that consist of 268 statements and recommendations relating to (a) rights of the individual; (b) beliefs and attitudes of communication partners; (c) professional knowledge and team work; (d) strategies to optimize engagement; (e) assessment; and (f) intervention (targets and goals, techniques), including the use of AAC. To date, this project is the largest of its kind, with 650 participants from 43 countries contributing to development of consensus-based guidelines for Rett syndrome.

Communication aids were reported to have a positive influence on communicative functions. Swedish SLP services followed best practice in several aspects, but there are areas with potential for development. Tools and best practice guidelines are needed to support SLPs in the AAC process for clients with Rett syndrome. [Box: see text].

Purpose: To examine the effect of a communication intervention package on expressive communication and visual attention in individuals with Rett syndrome. Materials and methods: A modified withdrawal (A-B1-A1-B2-A2) single case experimental design with a direct inter-subject replication across three participants was applied. Three women with Rett syndrome participated. The study took place over a six-week period and comprised 32 sessions with each participant. All sessions were video recorded. During the intervention the communication partner used aided language modelling on a gaze-controlled device in combination with using responsive partner strategies. Expressive communication was assessed as synthesised words per minute and unique synthesised words per minute. Visual attention was assessed as rate of focused gazes (1 s or longer) in interaction.Results: An intervention effect was found on the rate of unique words for all participants. The rate of words increased for two participants when the intervention was introduced but no withdrawal effect could be seen. An intervention effect on visual attention could be seen for one participant. The intervention appeared to have social validity as reported by caregivers. Conclusion: Aided language modelling (ALM), while using responsive partner strategies and a gaze-controlled device may be used with adult individuals with Rett syndrome to increase their rate of expressive communication. Detailed observational measures revealed individual learning patterns, which may provide clinically valuable insights. ä IMPLICATIONS FOR REHABILITATIONAdults with Rett syndrome may benefit from access to gaze-controlled devices in combination with responsive partner strategies. Responsive partner communication may be effective for some individuals with Rett syndrome to increase their rate of synthesised utterances. Rate of focused gazes may be considered as an outcome measure for individuals with oculomotor difficulties when introducing aided language modelling.

This was a two-phase study that aimed to (a) develop a tool for assessing visual attention in individuals with Rett syndrome using AAC with a communication partner during naturalistic interactions in clinical settings; and (b) explore aspects of the tool's reliability, validity, and utility. The Assessment of Visual Attention in Interaction (AVAI) tool was developed to assess visual attention operationalized as focused gazes (1 s or longer) at the communication partner, an object, and a symbol set. For the study, six video-recorded interactions with nine female participants diagnosed with Rett syndrome (range: 15-52-years-old) were used to calculate intra-and inter-rater agreement, and 18 recorded interactions were analyzed to examine sensitivity to change and acceptability. There was a significant difference in the AVAI results between two conditions (with and without aided-language modeling). Inter-rater agreement ranged from moderate and strong. There was a range in scores, indicating that the AVAI could differentiate between participants. The AVAI was found to be reliable, able to detect change, and acceptable to the participants. This tool could potentially be used for evaluating interventions that utilize aided AAC.

Assistive technology (AT) can be used as early intervention in order to reduce activity limitations in play and communication. This longitudinal case study examines eye-gaze control technology as early intervention for a young child with high spinal cord injury without the ability to make sounds. The young child was followed by repeated measures concerning performance and communication from baseline at 9 months to 26 months, and finalized at 36 months by field observations in the home setting. The results showed eye-gaze performance and frequency of use of eye-gaze control technology increased over time. Goals set at 15 months concerning learning and using the AT; naming objects and interactions with family was successfully completed at 26 months. Communicative functions regarding obtaining objects and social interaction increased from unintentional actions to purposeful choices and interactions. At 36 months, the toddler was partly independent in eye gazing, used all activities provided, and made independent choices. In conclusion, the results show that a 9-month-old child with profound motor disabilities can benefit from eye-gaze control technology in order to gradually perform activities, socially interact with family members, and make choices.