Judy Lariviere, M.Ed., OTR/L
Assistive Technology Specialist/
Occupational Therapist
judy.jlariviere@gmail.com

Introduction

The potential of girls with Rett Syndrome (RTT) to communicate and learn is frequently underestimated.  Factors that typically present challenges for girls with RTT include apraxia, delayed responses, inconsistency of performance in accessing and using technology, and apparent lack of interest over time (Hunter, 2007).  In addition, educators and clinicians are often unaware of effective strategies and approaches to use with girls with RTT as they have had limited experience in working with these girls.  All of these challenges are common for girls with Rett syndrome and their multidisciplinary teams and present barriers to moving forward with augmentative and alternative communication (AAC) on which these girls rely (Hunter, 2007; Sinner, 2007).

Various strategies can be implemented that enable girls with RTT to enhance their communication in a relatively short period of time. These include:

• Recognizing when a girl’s sensory system is in a “regulated state” and she is ready to communicate and participate in a classroom activity.
• Identifying when other access options to technology, beyond hand use, need to be explored with a girl to improve her communication skills.
• Improving a girl’s use of eye-controlled AAC devices during trials and daily interactions.

Sensory Regulation or Processing of Sensory Input for Girls with Rett Syndrome
Often the impact of a girl’s response to the sensory stimulation of her environment on her communication abilities is overlooked, particularly in a classroom environment.  When a girl with Rett Syndrome’s sensory system is out of regulation, her behavior can be misinterpreted as lack of interest or motivation or inability to interact or learn.  As a result, it is essential that a multidisciplinary team recognize when a girl’s sensory system is in a “regulated state” and she is ready to communicate or participate in an activity. 

To assist with identifying when a girl with Rett Syndrome (RTT) is “self-regulated” and her sensory system is in a functional state of arousal (i.e., she is calm, focused, and able to participate in back and forth, “reciprocal” interactions), Lariviere (2009) developed a 3-point scale for girls who walk independently and those who require assistance to walk/stand.

• The scale is designed to function as a countdown 3-2-1 (like a rocket blasting off into space). 
• When a girl’s sensory system is at level 3, her sensory system is out of regulation and she needs strong sensory input, or in some cases, a nap, to move her back to Level 1 where she is able to communicate and participate. 
• When a girl’s sensory system is at Level 2, she needs some sensory input to help her return to regulated state.  (Refer to Resources for additional details about Levels of Sensory Regulation Chart).
• Video of Catalina asking for a break and needing to rest her eyes (Level 2 – just woke up from nap; not ready to participate and she communicated this using her Tobii)

Improving Access
Girls with Rett Syndrome often experience delays in their communication development when their access to technology is not appropriate.  Often, the overreliance on hand use interferes with the progress that a girl makes in her communication development over the years such that they get stuck at making choices from two or three presented items and using a simple voice output device with a limited number of messages to which they have immediate access.

Significant improvements in communication abilities have been observed and documented for many girls over a relatively short period of time when access options beyond hand use have been explored and implemented (Lariviere, 2007).  The challenge is that although girls with RTT share common characteristics, each girl is unique in their motor abilities and the movements which they spontaneously use and repeat over the course of a day. 

Access to Communication

• Determine access first to a light tech communication system, simple voice output for building reciprocal communication skills, and high tech communication system
• Must represent an easy motor plan; build on natural spontaneous movements
• Avoid holding back communication development while hand use or other motor skills improve
• Common limiting factor – exclusive use a girl’s hands with limited progress
• Videos of Kate – using switch with Penguin toy; looking at switch before selecting with her hand; does not look at switch and activate with her hand (typical pattern see with girls with RTT). When presented with two photos on Talking Notepads and asked “Where’s Mom?” she indicates correctly with her eyes but not with her hand (this is not communication – it is identification).  However, when photos presented one at a time, eyes and hand selections match.
• While exploring access options, even single switch placement, it is incredibly important to avoid testing or target practice vs. engaging in highly motivating, meaningful exploration and participation in “real” activities that are open-ended (not expecting a specific response)
• Ultimate goal - to identify the “best means of independent access” to a sophisticated AAC device; once this is identified, a girl takes off in her communication.
• Don’t stop exploring options until a girl has a light tech communication system that they can use effectively and a dynamic display communication device that they can access and navigate independently.

Balancing Motor Demands with Communication/Cognitive Demands

• It is essential to balance the motor demands/load of an activity with the cognitive/communication demands/load.
• Recipe for success:
• With increased motor demands, lower cognitive/communication demands (i.e., when evaluating access) <OR>

With increased communication demands (i.e., time dependent nature; increased focus on reciprocal interaction or question/response format) or cognitive demands (evaluating knowledge of particular topic), lower motor demands

Access to a Light Tech Communication System

• Build on natural gestures for giving a “yes” response with partner-assisted scanning
• Accept more than one form of affirmative response at any one time otherwise miss communication responses – leads to shutdown
• Apraxia interferes with always giving a specific required response
• Examples of “yes” response – looking at communication partner, reaching out and touching flip ‘n talk or initiate movement; head nod, smile with looking at me, activation of a recorded message “yes, that one”
• Video of Catalina – immediately looks to me when I restated her selection “you need to rest your eyes?” after she initiated a request for a break. When I didn’t respond fast enough, she selected “I need to rest my eyes” again and then looks at me.

Considerations for the Design and Layout of Light Tech Systems
Layout

• Girls with Rett Syndrome naturally move their eyes more easily to the left and the right (i.e., horizontal eye movements) rather than up and down. 
• Typically girls can easily look up or down but not both directions without additional training. 
• We often present choices to girls, we hold up one option in our right hand and the other in our left.  This pattern of presentation works for girls. 
• Automatically adding more items in a four quadrant layout increases the motor demands on a girl’s use of her eyes and does not always work for every girl. 
• When you have to teach a girl a new motor pattern that is not part of her natural repertoire that she uses spontaneously, it is more likely that apraxia will interfere with her ability to consistently access items in this arrangement.  The result is inconsistent performance throughout the day or from day-to-day.
• Layout must also be considered with items presented through partner-assisted scanning.  Often 12 items presented and rows and columns can be visually overwhelming for a girl with Rett Syndrome. 
• A layout that is proving to be very successful given it is easier for girls to follow visually is five to six items displayed horizontally as shown below and used in Mayer Johnson’s large, yellow Flip n Talk.  

• Pati King-DeBaun’s The Light Tech Conversation Book CD and The Light Tech Communication Book CD are now available in the six location horizontal arrangement that can be set up in Mayer Johnson’s large, yellow Flip n Talk as these items are now available in this horizontal arrangement. (Refer to Resources section for additional information).

Visual Perception

• In terms of visual access, it is also easier for girls with Rett Syndrome to see white text on black background.  It helps to increase a girl’s attention and focus on the text (Damiao, 2010).
• This represents an important consideration for giving girls access to letters of the alphabet through a light tech and high tech system.  Girls are demonstrating great success with spelling when using light tech flip books with white letters on black backgrounds for spelling.
• I created an alphabet page for Tobii Communicator that builds on this layout and design as I wanted to have transfer of motor patterns between light tech and high tech systems.  Below is a screenshot of the a-f alphabet page with word prediction buttons at the bottom of the screen.

Dry Erase Boards

• Recently found that the use of a white dry erase board does not produce the same results with girls with Rett Syndrome as compared to a new dry erase board, Expo Neon that is black with colored dry erase markers of orange, yellow, or pink.
• The results with using the dry erase board with black background as compared to a white dry erase board have been incredible.  When using this board with girls in question-response format during literacy activities to determine what they are learning, girls have demonstrated extremely good eye gaze to choices and partner-assisting scanning to presented choices.  Their visual attention to the task and the duration with which the girls have been able to participate has been longer than with the white board or black marker on yellow paper or on white.

Access through “Eyes”

• Eye gaze/eye tracking technology builds on a girl’s incredible use of their eyes
• Direct selection is a more efficient means for communicating – seeing dramatic progress
• Provides access to communication when other methods and tools have met with limited success at best
• It is often assumed a girl has limited communication skills but really she has not had access or the opportunity; always important to presume competence and figure out how to tap into a girl’s potential.

Which Eye Gaze/Eye Tracking System to Trial?

• Size of the Trackbox of eye gaze/eye tracking systems is important relative to the girl’s positioning and natural movement patterns.
• Consider the amount of side to side movement or sitting position in chair (typically low tone in trunk)

• Eyegaze Edge Tablet - represents a less expensive system that is built on a Tablet PC computer with Grid software

Goal of Trial with Eye Gaze System

• Exploring a new means of access to technology
• Remember this involves a lot of motor planning and takes time to learn
• Given increased motor demands, lower the cognitive and communication demands
• It is not target practice – “find the _____” or “where’s the_____?
• Allow for natural and spontaneous interaction
• Respond to selections appropriately
• Assist with navigation to page (Model)

Judy’s Page Sets

• In Fall 2009, finalized design of my eye gaze page sets
• Set up based on girl’s natural use of horizontal eye movements in the middle of the page – looking left to right
• Navigation between pages is integrated into these middle buttons
• Typically girls have ease in looking up or down not both directions
• Tested and used across over 70 eye gaze trials with girls and teenagers with Rett Syndrome over the past year.
• Able to identify girl’s preferences very clearly as well as communication of immediate wants and needs.
• Videos of Katie – incredibly quick and independent navigation away from Potato Head page to return to one of her favorite music pages, Dora the Explorer and then a second time to indicate she was “all done” with playing Potato Head and looking at me and using her body gesture to tell me she wanted to go back to her music and dance.

What Settings?

• On Tobii-based eye gaze trials (i.e., Tobii devices and ECOPoint), use red dial/clock rather than the shrinking dot up to 1 inch in diameter
• Gives visual information about how long need to maintain eyes on location (i.e., dwell) before selecting a button
• Allows for acknowledgement of motor approximations
• EyeMax use fill button feature with red
• Dwell always set to under one second – 880 milliseconds (ms); even as low as 400 ms initially for girls who have more fleeting eye movements.

Calibrating during an Initial Trial Session?

• Don’t have the girl calibrate during an initial eye gaze trial unless accessing 20 or more buttons
• Use the sales representative’s calibration
• With my page sets, never have needed a girl to calibrate during a trial session
• Apraxia interferes with getting a good calibration
• Tires out a girl’s eyes before she gets started using the system
• Position device and locate eyes in the Track Status box, then ready to go
• For an extended trial (i.e., one to four weeks), use keyboard step-through option when calibrating. This option is available on all eye gaze systems.

Collect Data

• Videotape the sessions
• Want to capture “moments of brilliance”
• Document how integrating eye gaze system into other modes of communication in order to interact
• ECOPoint has Language Activity Monitor (LAM) which labels button selected and time of selection
• EyeMax has usage counts for each button

Qualitative Data Collection Example (from Video of Catalina’s request for a break)

Date - On page with 8 choices

A final word about trialing Eye Gaze Systems

• Always complete a trial with at least two different eye gaze/eye tracking systems; each one is different
• Don’t just go with the first one eye gaze system that a girl tries or only do a trial with the system that the team is most comfortable
• Remember:  Best access dictates the device, especially for a girl with Rett Syndrome – the easier the better. 
• You can always end up customizing for supporting conversations, use of words for interaction.

Integrating Eye Gaze System into Communication Repertoire

• Move beyond choice making related to needs and wants and program for conversation
• Incorporate activity-specific pages that allow a girl to direct activities – art, reading, etc.
• Video of Katie – using a reading page to direct and interact with her AAC Specialist during this activity. At Level 1 in spite of background noise in classroom; looking between book, reading page on Tobii, and her therapist.
• Include “About Me” pages that enable girls to talk about their favorites and music pages that they can navigate to on their own (very helpful for self-regulation)
• “Hot News” pages that enable a girl to share stories about her life experiences
• Video of Lauren during eye gaze trial – talking with Tobii rep about Haunted Houses

References

Damiao, J. (2010).  Visual perception: It’s not a matter of black and white…or is it? OT Practice, 7-8.

Hunter, K. (2007). The Rett syndrome handbook (2nd ed.). Clinton, MD: International Rett Syndrome Association.

Lariviere, J. (2007, December). Exploring options for access: Enhancing
communication and learning for girls with Rett syndrome. Technology
Special Interest Section Quarterly, 17(4), 1–4.

Sinner, C. (2007, May). Communication in the classroom. Paper presented at the annual conference of the International Rett Syndrome Association, Atlanta, GA. 

Resources

Expo Black Dry Erase Board with 3 Expo Neon Markers

• Available at Office Depot; online through Amazon.com
• Cost: ranging from $10.00 to $12.00

Judy’s Eye Gaze Page Set

• Judy’s Eye Gaze Page Set for conducting eye gaze trials with Tobii systems will be available in December 2010. 
• In January 2011, a teen version of Judy’s Eye Gaze Page Set will be available for Tobii systems with age-appropriate comments and content for teens with Rett Syndrome.
• A version for DynaVox EyeMax will also be available soon.
• These Eye Gaze Page Sets will be available for purchase and download through www.jlariviere.com

Levels of Sensory Regulation Chart

• Levels of Sensory Regulation or Processing of Sensory Input for Girls with Rett Syndrome – charting package for tracking/monitoring.  Available December 1, 2010. Can order through www.jlariviere.com after December 1, 2010.

Mayer Johnson’s Flip n Talk

• Mayer Johnson’s Flip n Talk (Large yellow) (Item M411) is available through www.mayerjohnson.com 
• Cost:  $29.95 (plus tax and shipping/handling)

Pati King-DeBaun’s –  The Light Tech Communication Book

• The Light Tech Conversation Book CD -  $75.00 Conversation Version Only with 6 location option
• The Light Tech Communication Book CD - $125.00 Full version with all 6 location option – includes conversation, words, and literacy (core words, alphabet and numbers)
• To order, go to Pati King-DeBaun’s website www.creativecommunicating.com  and go to the catalog page and print out the purchase order form and fill in the above information.

Books
Is it Sensory or Is It Behavior? Behavior Problem Identification, Assessment, and Intervention. 

• By Carolyn Murray-Slutsky & Betty A. Paris (2005). 
• Publisher: Austin, TX: Hammill Institute on Disabilities.
• Therapists can purchase for $58.00 through http://www.proedinc.com/customer/productView.aspx?ID=3790

Raising a Sensory Smart Child: The Definitive Handbook for Helping your Child with Sensory Processing Issues

• By Lindsey Biel, M.A., OTR/L and Nancy Peske
• 2009 edition

Link to download of pdf of Aleksandra Djukic, MD, PhD presentation at the International Rett Syndrome Foundation’s 2010 Conference, What’s Going Right in the Brain http://www.rettsyndrome.org/past-irsf-annual-conference.html

NOTE About DynaVox’s EyeMax.
DynaVox just released an updated computer base that will work with its EyeMax eye tracking system.  It is called the Vmax +.  Additional information is available at http://www.dynavoxtech.com/products/vmaxplus/

© Lariviere 2010