Some Rett Syndrome Tips

Working with girls and women who have Rett Syndrome can be a unique and wonderful experience.  Rett Syndrome is a neurological condition that effects only girls (with a few, rare exceptions).  Although it varies in presentation from girl to girl, in general, those with Rett Syndrome are primarily alternative communicators, they present with hand wringing or similar movements and they tend to have amazing eye contact and eye pointing skills.  Most of the girls are very motivated by music and music can be an ideal teaching modality.  About half use wheelchairs for all mobility.  Most have seizures, have swallowing difficulties and have issues with unintentional breath holding.  Sleep disorders are common as are certain lung and heart issues.  Rett Syndrome used to be considered a form of autism, but it is, in fact, a specific genetic condition. 

Girls with Rett Syndrome used to be automatically assumed to have very severe to profound developmental and intellectual disabilities.  However, as more and more people are willing to practice the least dangerous assumption and presume competence and as technology has evolved to include somewhat affordable eye tracking based alternative communication systems we are finding that many, if not most, girls with Rett Syndrome have been dramatically underestimated and, sometimes, educationally neglected.

Here are just a few tips for working with girls and women who have Rett Syndrome in our classrooms and in other educational settings.

• Bringing the hands to mid-line (the center of the body) is one of the key diagnostic features of Rett Syndrome.  This can be hand washing movements, hand clenching, hand mouthing or clapping.  The girls cannot control this movement, in fact, an attempt to control this movement may be distressing and thus increase it.  Therefore, rethink hand use goals.
• hand wringing and related stereotypies are a hallmark of Rett Syndrome functional hand use is occasionally possible for some girls with Rett Syndrome, however, as a general rule goals that work towards hand use should be rethought.  
• such goals are likely to be lead to frustration for the girls and for staff.  Whatever energy or effort the girls must put forth to meet such goals will be a heroic effort in battling a symptom of Rett Syndrome, it is unfair to ask her to do this while doing other important things - like learning
• look through your IEP.  If any of the goals for communication, life skills or academics call for the girl to use her hands especially using her hands in a very specific way look for other ways to meet the goals.  Consider eye gaze from communication, switches with a head or foot movement for activating adapted items and generally adapting the environment to be accessed without hand us
• it is ok to have her use her hands, to encourage her to use her hands, but having it be the goal may not be best overall
• some girls do have more hand use than others, a few can hold a cup or spoon or even activate a device, but consider the effort required overall as you decide what to spend energy on

• Remember what apraxia (a key component of Rett) is:
  
• a inability to perform a task, especially speech even though:
• the request is understood
• there is willingness to do the task
• the muscles work properly
• the task may have already been learned
• This means
• you can't assume a Rett girl doesn't understand, is "acting out" or "refusing", is physically incapable or that she has forgotten what she has learned
• you can assume that the more the girl wants to do or say something the harder it will be and she is inevitably more frustrated than you are
• the girl with Rett will be consistently inconsistent, not because of intelligence or behavior but because of apraxia
• a burst of strong emotion may override apraxia 
• Also since you can't measure her knowledge fully because of the combination hand stereoypies and apraxia of speech, even using high technology, you must assume that she is competent to avoid causing an developmental disability through educational neglect

• Anxiety severely affects girls with Rett

•  Anxiety is a massive issue in Rett Syndrome.  The girls are often times unable to communicate their anxiety or ask questions to clarify situations which may be causing anxiety.  
• Anxiety can present as increased stereotypies like tooth grinding, hand mouthing, breath holding and other behaviors; it can also present as shutting down (closing eyes, appearing sleepy) or with crying or yelling.  Very rarely, some girls may hit their heads, bite their hands or otherwise hurt themselves.
• Teachers and therapists should seek to create a low stress, low anxiety enviroment by
• explaining changes in the environment, in staffing, in anything that can cause stress
• teaching about emotions and how to communicate about them
• ensuring the girls have the words to talk about anxiety
• using social stories to teach about how to decrease anxiety and to prepare the girls for difficult situations
• be aware of what your students particular anxiety markers are and intervene at the first sign
• know what music, sensory experiences and people your student finds calming and have them available, give the girl a way to ask for these things 

• Eyes are usually the best means of communication
  
• Although a few girls can speak some words and a few others can use their hands or switches to activate augmentative communication devices most girls do best when using eye gaze as a way to communicate
• Start evaluation for high technology eye gaze communication system early, at diagnosis or between nine months of age and two years, if possible
• there are no prerequisites for high technology eye gaze based communication, girls do not need to use low technology eye gazed interventions first, they do not have to master PECS or picture symbols first. 
• all of the best practices of Augmentative and Alternative Communication apply to eye gaze such as: implementation of a robust vocabulary, intensive and ongoing aided language stimulation and implementation across all environments

• Trying a high technology eye gaze system
• Be sure you work with a company representative and/or AAC specialist who is knowledgeable about both Rett Syndrome and eye gaze tracking technologies, but if there is no one available still proceed with trying high technology eye gaze systems
• Explain to the girl well ahead of time and more than once what a high technology eye gaze system is and what it is for, show videos of other girls using such devices
• Schedule the demo and trial of the system for when the girl is usually most alert, for most girls this is the morning
• Do not have the girl present for the boring set up or "how this works" session with the company representative, do not bring her in until you are ready for her to start using the system!
• Do NOT waste time trying to calibrate the device when the girl arrives, calibration has no built in rewards and is hard to understand why is it useful from the girls perspective.  It tends to cause the girls anxiety.  Have the rep turn on a fun sensory activity for the girl to try with her eyes.  She will likely "get it" right away.  Move from there into a communication activity with a large targets and a big pay off (something exciting happens when the buttons are activated even if that something exciting is you doing what the girl tells you - sing, dance, jump).  At this point the girl may be tired.  Take her cues.  Stop before she has any sense of failure.  At the next visit move through the same sequence more quickly and then try calibrating.  Rarely after two or three sessions when the girl is awake and feeling well is there then not enough evidence to get a month long trial of a system for further evaluation. 

•  Literacy is a human right!  Rett Girls can read!
  
• start teaching girls with Rett Syndrome letters, letters sounds, letter blending and word segmenting along the same time line as typical peers but,
• if the girl is older it isn't too late, start now,
• reading and spelling is being accomplished by girls and women with Rett Syndrome everyday, be a part of this reading revolution and teaching reading and spelling now

• Music as motivation
• girls with Rett Syndrome can be very motivated by music and may be more able to learn when music is involved
• consider music therapy and work to have it consist of more than cause and effect activities and listening to music, many girls are able to contribute to writing lyrics to music using there speech devices and many find music a way to relieve anxiety and express emotions
• use music and music videos to teach key concepts, you can find a music video for just about any educational concept on YouTube!

• Connect with resources
•  Rett Syndrome Educators Network
• RettSyndrome.Org
• Rett University
• Rett Syndrome Research Trust
• GirlPower2Cure
• Facebook IRSF Communication and Device Exchange