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!
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