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If at first you don’t succeed:
AAC and Rett Syndrome
Jane Farrall, Speech Pathologist
Leah Picking, Occupational Therapist
What is Rett Syndrome?
Rett Syndrome is a developmental disorder that begins in early infancy. It primarily affects females, but some sources also say that it can affect males. Children with Rett Syndrome are born healthy and develop normally until about 6 – 18 months of life. Four common stages then follow, as shown in the table below (taken from the International Rett Syndrome Website at www.rettsyndrome.org)
| Stage |
Approximate age of onset | Characteristics |
| I |
6 – 18 months |
Disinterest in play; hypotonia. |
| II |
1 – 3 years | Rapid regression; irritability; “autistic-like” symptoms. |
| III |
2 – 10 years |
Severe seizures; "intellectual disability"*; hand-wringing; hyperventilation; bruxism. |
| IV |
10
years+ |
Scoliosis; muscle wasting; rigidity; improved eye contact. |
*Many people who feel that the level of ID cannot be accurately assessed because of issues surrounding communication.
There are many diagnostic criteria for Rett Syndrome. These include:
(a) a period of apparently normal development until 6 – 18 months;
(b) normal head circumference at birth followed by the slowing of rate of head growth with age (3 months – 4 years);
(c) severely impaired expressive language; (d) loss of purposeful hand skills; (e) repetitive hand movements, including hand washing, hand wringing, hand clapping, hand mouthing;
(e) shakiness of the torso; and
(f) if able to walk, unsteady, wide-based, stiff-legged gait and/or toe walking.
AAC and Rett Syndrome
The term Augmentative and Alternative Communication (AAC) includes the complete range of communication strategies used to augment or replace speech. A great deal of change has occurred in the use of AAC with girls with Rett Syndrome in the last few years. Instead of an assumption that the girls have little understanding of language there are now many questions about whether, in fact, girls with Rett Syndrome do continue to develop receptive language skills but are unable to show it (Garrett, 2003). Many families believe that this is the case, and more professionals in the area are reporting girls being able to use much more complex communication systems than would ever have been tried in the past. Therefore, one of the primary questions is "How can we set up systems which can best access the receptive language and give the girls as powerful a mode of expressive communication as is possible?" The main factors that impact upon access to methods of expressive communication are problems with initiating movement and purposeful hand control. The control of facial expression also seems to be greatly affected by Rett Syndrome, so we need to think laterally about how these girls are going to communicate.
Eye pointing
Eye pointing is most commonly described as the communication aid access strategy used by girls with Rett Syndrome as they often have limited use of their hands. Eye pointing communication options can range from very simple to quite complex. A simple form of eye pointing may be done by holding up two objects and asking the person to look at the one they want. In a more complex version, multiple items or even the whole alphabet can be put on a page. The user again looks at the one they want and their communication partner checks that they have interpreted correctly by asking a yes/no question.
Body gestures/ facial expression
Many of the parents of girls with Rett Syndrome talk about the body gestures that their daughters use – a whole body movement can mean “no” or “yes” or “I’m hungry”. Or walking to a particular spot in the kitchen can mean “I’m thirsty”. Some girls also use facial expression – although sometimes it may be limited to one or two particular expressions when they feel strongly about an issue.
Headpointers
Some people with Rett Syndrome have sufficient head control to point to items on a communication board or even hit keys on a keyboard with a headpointer.
Facilitated communication
This is frequently mentioned at websites as an option tried with girls with Rett Syndrome. For some personal accounts of this go to the International Rett Syndrome Association website – www.rettsyndrome.org
Switching and scanning to use high technology
Many people report that high technology has not been particularly successful with girls with Rett Syndrome because of difficulties in accessing. The timing requirements in initiating the movement to operate a switch for scanning are very frequently mentioned as the primary problem. The Step-by-Step communicator is one option which does not have timing requirements as it has a sequence of messages recorded under one button. Many people have found it successful. The Cheap Talk also gets a lot of positive press at various websites. However, using scanning to access a greater range of items gets very mixed reports!
Providing services at DEAL to people with Rett Syndrome
DEAL currently provides services to a number of girls and women with Rett Syndrome on a regular basis. This article discusses four of DEAL clients with Rett Syndrome, highlighting issues related to access methods. Obviously the clients; real names are not used.
Client 1 – Annabel
Annabel is currently five years of age, and this year has been integrated full time into a mainstream primary school. Annabel’s family has always used devices coactively with her. She has trialled a Cheap Talk 4 and can move her hand to the button she would like to press but is unable to exert sufficient pressure to press the button independently – her family has helped out by pressing her hand to activate the button. Her main access difficulties lie in her ability to apply enough pressure and coordinate upper limb movements. She spends a long time looking at the item she wants to move her hand to before she is actually able to move her hand there. Her ability to exert pressure is improved if the device (keyboard or switch) is positioned at exactly the right angle. The angle on a Step-by-Step or a Jellybean booster works really well for Annabel.
Currently Annabel is using a multi-page eye point book, which has only recently been introduced, a yes/no eye pointing card, lots of facial expression, use of gaze and vocalizing. She did not find eye pointing easy – initially she only briefly glanced at the item she wanted. She is now able to look at her item of choice for longer and is learning to move from two items to four items. She finds it much easier to eye point if items are arranged vertically rather than horizontally but with practice she is getting much more accurate.
One of the challenges with Annabel is finding a balance between finding cognitively challenging “work” for her that is motivating but not too difficult physically. She is very interested and motivated by text. At the beginning of every session she walks straight to the computer and wants to use the Intellikeys keyboard.
Client 2 – Brigette
Brigette is currently 6 years old and attends a Special School full time. She has a very supportive and knowledgeable team at her school.
Brigette walks short distances. Before she began attending DEAL last year, her team had established a Yes/No response (she looks at someone for yes and looks away for no) and was using a BIGmack. In addition to the BIGmack she uses a talking dice and other switch toys and games at school. Brigette uses a range of different communication options in different situations. The BIGmack is used for single messages in the classroom and for sending messages between home and school.
In addition Brigette has recently begun using a Four Compartment Communicator (below) which speaks when an option is selected. This has worked well as Brigette is able to press the cells independently and make clear choices. The dividers guide her forearm and allow her to move her wrist with her forearm supported. Brigette has been able to isolate her index finger independently but not consistently, so she uses the Four Compartment Communicator with a gross wrist movement with an open hand. The staff at school change the objects at meal times and for classroom activities such as maths, concepts and colours.
Brigette always looks at the item before she selects it with her right hand. There is usually a delay of between 20 seconds to 2 minutes. Her left arm is sometimes restrained to reduce repetitive hand movements. Some eye pointing is being introduced with Brigette but at the moment she would rather walk or point to the item she wants even if her gaze gets there a lot faster!
Client 3 – Felicity
Felicity is current 17 years of age and has received services intermittently from DEAL over the past four years. Last year she began attending more regularly. She is positioned in a wheelchair or a recliner throughout the day. Until recently, no formal communication system had been used but she was able to communicate with her family and other familiar people using yes/ no responses (she is still able to shake and nod her head, but the responses are delayed by about 20 seconds), eye pointing to desired objects and body gestures/body language and some facial expression. Felicity was very enthusiastic about using a system that would give her more options for communication. She loves having books read to her and watching TV.
In the past Felicity had tried to use a head pointer to access equipment. This was not very successful. When the family commenced attending at DEAL again we talked primarily about eye pointing. Felicity had good control over both horizontal and vertical eye pointing and a range of communication displays were trialled with an eye point array with 8 items being the most successful. A small multi-level eye point book was initially established and Felicity was able to use this easily and successfully. This was enlarged upon over future appointments.
Felicity and her family were very keen to look at high technology communication options. Felicity has limited control over her hands, even when one hand was splinted to stop the repetitive hand movements. An access assessment was completed to look at possible switch sites. Felicity seemed to have good control over a gross movement of her right leg which she is able to use for scanning as she can move both on and off the switch reliably. Felicity is still learning a lot about switching at this stage and during this training period has benefited from using a switching module that eliminates quick double hits by ignoring the second hit, thereby reducing her error rate.
A number of electronic communication devices were trialled and Felicity found a device that also had auditory scanning the most successful. At the end of the trial phase a Dynamo electronic communication device, a custom device mount, a commercially available switch mount (the Universal Switch Mount) and a pillow speaker were obtained from the Aids and Equipment Program. Felicity loves her high technology system so much she won’t even look at her eye pointing book unless there is a good reason her high tech system can’t be used (hasn’t been charged, going swimming, etc.)
Client 4 – Hannah
Hannah has had many health issues such as seizures that have affected her ability to communicate as she is often in pain or extremely tired following seizure activity. Hannah was integrated full time at primary school and for part of her secondary education. She now attends an adult day program.
Hannah uses different communication strategies with different people. Since she was 11 Hannah has been using a yes/no strip to eye point to yes or no. Before the age of 11 eye pointing was trialled several times without success. She has been using a Macaw electronic communication device for several years. Different access methods have been trialled. Some of these have been successful for a period of time and then stopped being successful due to physical changes. Initially, the Macaw was accessed with a head pointer and with facilitated communication (hand). The head pointer was used at school and her mother facilitated her at home. Hannah then had a period of illness and following this was unable to use her head pointer. At this time elbow switching became the next access method. This was still being used at the beginning of last year when we began working with Hannah, but it was very inconsistent and so other access methods were investigated. In addition, time was spent developing a new low technology communication book. Hannah had previously had a low technology book of written words and sentences. She accessed it using a grid of colours and numbers that told her communication partner which item she wanted. Her day center staff found this too difficult and so a multi-level eye-pointing folder was developed, using very similar vocabulary.
The multi-level eye-pointing folder has been a huge success. Hannah is able to use it with her family, with staff at her residence and with staff at her day centre. She is able to use it when she is unwell, which is a first for an AAC system with Hannah. Staff have been able to use it to clarify what is wrong when she is distressed and are beginning to see how it can be used for news and chatting – Hannah and the rest of her team are absolutely delighted with how successful it has been.
On the other hand, the Macaw is rarely being used. Hannah is able to use a head switch to control it when she is well and not tired – but unfortunately this does not happen frequently. Hannah and her mother, who initially were absolutely focused on the electronic system, now see her low technology system as the primary and most important one and come along constantly with suggestions, additions and updates.
Some comments on communication aid access
Direct access seems to decrease with age as movements become less co-ordinated and contractures may occur. This means that the access method needs constant revision. Don’t assume that it will be appropriate forever. Also, some girls and women with Rett Syndrome develop spasticity and orthopaedic deformities, which obviously have an effect on access.
Difficulties with timing mean scanning and switching is not usually an option for most people with Rett syndrome – but as you have seen with Felicity, it can be!
Angled switches and supports for forearm and shoulders have made access easier for some of our clients. We feel that one of the reasons so many people on the net, as well as ourselves, report success with the CheapTalks and Step-by-Steps is due to the angle on these devices – possibly combined with the movement of the keys and the auditory click as the key is pressed.
With some of the girls we work with, supporting or restraining (e.g. with a splint) the non-dominant hand can reduce stereotypical “hand wringing” behaviour. This seems to have a two-fold effect – firstly it allows the dominant hand freedom to participate in other tasks and secondly it seems to improve concentration on the task at hand. This doesn’t just apply to communication – it can be useful for a range of activities.
As with all AAC users, you need to make sure a low technology system is in place as well as a high technology system. For many of the girls we see their low technology system is far more effective for day-to-day purposes than their high technology system.
One of the important things we have found is that motivation and heightened interest in an activity have a positive impact on intentional movement. Try making the activities a little harder and see what response you get!
Eye-pointing is often an effective access system, however it does not seem to come naturally. Often work needs to be done practicing and developing the eye pointing – we have developed a list of suggestions in stages to help with this. Also, it is important to note that movements in the peripheral visual field can be very distracting and can prevent eye pointing from being effective in lots of situations. For example, one of our clients cannot eye point successfully if she can see the peripheral movement of trees outside the window being blown by the wind.
Literacy
Many of the girls we work with are very motivated by text. If you provide them with a choice between a text-oriented activity (e.g. reading a book and participating in the reading by looking at a word on an eye point frame each time it occurs) and a non-text orientated activity (e.g. dressing Barbie with clothing chosen from an eye point display) they will choose the text activity every time. David Koppenhaver and Karen Ericksson have been doing a study in the US looking at text and girls with Rett Syndrome which has also shown how motivated they are by text-based activities.
Other
Try not to be too impatient – give an access method or a communication system a reasonable period of time to allow skills to develop before moving on to something else.
And finally, it is important to realise that just because something hasn’t worked one year, that doesn’t mean it won’t work the following year. In other words:
If at first you don’t succeed – try, try, try, and try again.
Ph. (61-3) 9509 6324
Fax. (61-3) 9386 0761
e-mail: dealcc@vicnet.net.au
Autism/ASD, Cerebral Palsy, Down Syndrome, Intellectual Impairment, Learning Disability, Fragile X Syndrome, Rett Syndrome, Stroke/CVA,
Persistent/Permanent Vegetative State, Acquired Brain Damage,
Motor Neurone Disease/ALS, and Huntington's Disease.
DEAL has been able to help people with all of these diagnoses to communicate.