Have you ever worked with a girl with Rett Syndrome? I can still remember seeing my first girls with Rett and feeling a little lost. This was quite a long time ago, and neither the technology nor our understanding of Rett was what it is today. As a matter of fact, this was only a few years after the second article about the disorder was published in 1983.
I had ideas, and we certainly tried a lot of different things. But knowing what I know now, I sure wish I’d tried some more. Now, decades later, as an AAC evaluator in a large urban AT setting, I can safely say I’ve seen my share of girls with Rett. And each one of them is unique. I’m learning all over again each time.
Rett syndrome is largely a disorder of Apraxia. Many SLPs are familiar with Apraxia (or Dyspraxia, if you prefer - the difference is the degree of severity) as a speech disorder. We see many kids, including those with Autism, who have difficulty with making the intentional movements of their speech mechanisms necessary to form the sounds of words and with getting from sounds in words to the connected words of continuous speech. ASHA has defined this as CAS; Childhood Apraxia of Speech.
In girls with Rett there is a widespread neurological disorder that appears after a period of typical development, showing first as a slowing or stagnating of development, followed by a period of regression. The Apraxia displays as a global inability to reliably make her body do what she wants it to do. Dyspraxia is less consistently unable; sometimes the message gets from the brain to the body - albeit slowly.
[Rett only rarely strikes boys. Because males have a different chromosome combination from females, boys who have the genetic mutation that causes Rett syndrome are affected more severely. Most of them die before birth or in early infancy. (Mayo Clinic, https://www.mayoclinic.org/diseases-conditions/rett-syndrome/symptoms-causes/syc-20377227)
However, Rett is not a degenerative disorder, and girls with Rett can and do make progress! (Mayo Clinic; Stages of Rett syndrome) The Apraxia - that inability to connect deliberate thought with deliberate motor movement - is the most debilitating and marked feature of the disorder. It interferes with all body movement, including speech and even eye gaze. It is consistently inconsistent; varying throughout the day, the week, and over time.
The impact on eye gaze is interesting to me. Because, in spite of difficulty with eye gaze, many girls with Rett do well with eye-gaze based AAC systems. I’ve seen girls blossom with the ability to be social that AAC gives them. One high school aged girl I’ve known for many years loves to be with her peers, enjoys whatever interactions are going on around her, and loves it when she can contribute using her eye gaze AAC system.
For other girls with Ret, however, eye gaze is not as successful. Many use Partner Assisted Scanning as a mode of access to AAC. Thanks to the tireless efforts of Linda Burkhart, (http://www.lburkhart.com/handouts.htm ) many successfully use PODD communication books (Pragmatically Organized Dynamic Display) with partner assisted scanning (PAS).
(On a side note - if you ever get the chance to see Linda in action, don’t miss it! No matter how often I hear her or talk with her, I’m always learning something.)
What can you do when communicating with a child with Rett?
Relax and wait. The more demands you place, the harder it will be for her to respond. Increasing tension, anxiety, and demands make it more difficult for her to make a response
Sometimes it can help to provide a slight physical assist to get the body towards initiation.
Sometimes it helps to hold one hand/arm, to interrupt stereotype movements.
Don’t keep repeating yourself. This actually makes it harder, and makes the wait time longer. (This is true for many other disabilities, as well, so remember this.) Think of it like refreshing a web page. You know how impatient you get, so you refresh? Then it takes even longer because the download has to start over again? Yep, that’s the thing.
Relax and wait - I can’t say this enough. No pressure.
Provide an easy way for the child to respond to PAS by teaching simple movements for yes and no, as a way to accept/reject items as scanned.
Apraxia impacts movement; not cognition. Never underestimate a girl with Rett. Communication is a beautiful thing.
Susan Berkowitz, M.S. CCC-SLP, M.Ed., has been a speech-language pathologist for 40 years. She has worked mostly with children and adults with Autism, Cerebral Palsy, and other developmental disabilities, as well as 8 years in the language-based classrooms in a school district. Susan has worked in public and non-public schools, residential settings, and nonprofit community agencies. She has written for peer-reviewed professional journals and presented at international conferences. Visit her website and blog for additional resources. For her complete resume, click here.
Burkhart, L., & Seligman-Wise, J. (n.d.). Getting past the barrier of Apraxia to interactive communication for children with Rett Syndrome. ISAAC, Lisbon, 2014. Retrieved January, 2017, from http://lindaburkhart.com/wp-content/uploads/2016/06/Rett_ISAAC_Lisboon_7-_14_handout.pdf