Intellectual Abilities and AAC Devices in Rett Syndrome
For decades, it was believed that individuals who suffer from Rett Syndrome have severe intellectual disability. As a matter of fact, most scientific literature to this day defines Rett Syndrome as one of the leading causes of “mental retardation” in girls.
As a parent, I remember that this was the first thing I researched when my daughter got her diagnosis at the age of 2. It was confusing as I knew that its not the case. My daughter was meeting all the key developmental milestones, she was climbing stairs, learning to say words, interacting with us and everyone she met, eating and drinking independently, using utensils, she was even able to play and type on the keyboard. She was a “typical” 18-month baby until the monster of Rett Syndrome fully invaded her body and locked it down.
Before being a scientist or a medical professional, we are mothers, and we know our daughters best. I know that my daughter is intellectually able, she is smart, capable and competent. She is just temporarily locked.
But why is the scientific literature so outdated? and is the ignorance of Rett Syndrome researchers justified?
A recent poll conducted by a parent on the Rett Syndrome Family Support group on social media, asked fellow parents on their perception when it comes to their daughters' intellectual abilities. A total of 82 families participated and 76% of the participants believed that their children with Rett Syndrome are competent and intellectually able as per their perception. The rest believe that their girls became intellectually disabled upon the onset of seizures, and 1% of the votes think that they are intellectually disabled.
If we asked parents 20 years ago, most definitely the numbers would have been different. Parents would have asserted that the girls (and RS boys) are intellectually disabled or “mentally retarded” as labeled. However, the narrative is starting to change. In the recent years, more articles and studies started to show the other side.
An excellent read is an article published in 2018 by Glasgow Caledonian University under the title “Clinicians make mistakes about intellectual impairments – as new Rett Syndrome findings show”
How and when did the narrative start to change?
With the introduction of AAC communication devices parents are no longer just relying on the “presume competence” notion but also on solid proof. The girls are “there” – they are competent, and they are for sure intellectually able.
Advances in Augmentative and Alternative Communication (AAC) have brought about significant improvements in the communication abilities and therefore quality of life for individuals living with Rett Syndrome.
One of the most notable advancements in AAC for Rett Syndrome is the development of high-tech communication devices. These devices utilize sophisticated technology, such as eye-gaze tracking or switch access, to enable individuals with limited motor control to communicate effectively. By tracking eye movements or responding to simple switches, individuals can select symbols, words, or phrases displayed on a screen, which are then synthesized into speech or displayed as text.
In addition to high-tech solutions, AAC for Rett Syndrome also includes low-tech and mid-tech options, such as communication boards, picture exchange systems, or sign language. These tools provide alternative means of communication for individuals who may not benefit from or have access to high-tech devices. They are often customizable to suit individual needs and preferences, allowing for flexibility and adaptability in various environments and situations.
Furthermore, advancements in AAC software and applications have expanded the possibilities for customization and personalization. Many AAC apps now offer customizable vocabularies, symbol libraries, and interface layouts, allowing users to tailor their communication systems to their specific needs and abilities. Additionally, these apps often integrate features for remote programming, data tracking, and cloud synchronization, enabling caregivers and therapists to support individuals in using AAC more effectively and efficiently.
Ongoing research and development in the field of AAC continues to drive innovation and improve accessibility for individuals with neurological conditions such as Rett Syndrome. Emerging technologies, such as artificial intelligence and natural language processing, hold promise for further enhancing the capabilities and usability of AAC systems. By leveraging these technologies, AAC solutions can become more intuitive, responsive, and personalized, ultimately empowering individuals to communicate more effectively and participate more fully in various aspects of life.
Advances in AAC have revolutionized communication for individuals with neurological conditions, providing them with powerful tools and strategies to overcome communication barriers and engage more fully with the world around them. As a result, the intellectual ability of Rett Syndrome individuals can now be better assessed.
A study conducted by Ahonniska-Assa et al. (2018) assessed the receptive vocabulary of girls with Rett Syndrome using AAC eye tracking device. The results demonstrated that AAC devices are indeed a useful tool in measuring the communication abilities and understanding of Rett Syndrome individuals. The study also confirmed that the age of the girls for when they start using the device is a key contributing factor; younger girls with access to an AAC device performed better compared to the older ones.
The main challenge that many parents face is the limited access to such devices, high costs and the lack of experienced professionals and parent training. Currently, in Ontario, Canada, the wait time for an appointment to be assessed for the eye gaze technology is over a year after a confirmed diagnosis. After the first appointments, there are a series of tests that need to be done to match the girl with the suitable device. Without going into many details, the long wait times and the complicated procedure makes girls such as my daughter who were diagnosed a bit later have to wait until after the age of 3.5 or 4 to complete their first appointment. All this wait time is time lost without practicing and developing alternative learning and communication skills.
Another challenge that many face is the difficulty of use and lack of accessible and affordable training. The use of AAC devices is difficult for the average parent. Most speech therapists are not properly trained to use them, or at least not at the moment. As a result, many parents will have to spend hours trying to learn, troubleshoot and figure out the simplest tasks. This impedes the girls' abilities to quickly learn and start integrating the new technology into their day-to-day activities.
We recently had a discussion with a Canada-based mom who is parent to a 5-year-old girl with Rett Syndrome. They received the eye gaze technology exactly one years ago (May 2023), and to date she had spent on average 6 to 7 hours a day learning. She had received minimal training and so far, their learning experience had been based on trial and error.
Her daughter, Mollie, started with low tech AAC and then E-tran when she was 2 years old. She received her eye gaze technology at the age of 3.5 years. At the moment, Mollie is showing great improvements in terms of communication. She can answer simple questions, thing from a list of options, she even uses it to play. Something that many Rett Syndrome individuals lose in their first regression. While Mollie is not the typical 5-year-old, she is more advanced than other individuals sharing her condition, especially those who do not use or have access to the eye gaze technology or other AAC devices.
We have to keep in mind that these devices not only facilitate communication but also empower individuals with Rett Syndrome to express their thoughts, needs, and desires more independently. By harnessing the latest technologies and ongoing research, we can continue to advance AAC solutions and enhance the individuals' quality of life.
That is why more speech therapists should integrate eye gaze into their education training. The government should provide individuals with complex medical needs with more funding and earlier access to communication devices as this can push those girls out of the “intellectually disabled” category to the able one. Our girls just need to have the means to prove it, and as a parent, I believe that my daughter and many others will change the outdated beliefs. Our girls can. We don't need to just presume competence because they are competent.
Ahonniska-Assa, J., Polack, O., Saraf, E., Wine, J., Silberg, T., Nissenkorn, A. and Ben-Zeev, B., 2018. Assessing cognitive functioning in females with Rett syndrome by eye-tracking methodology. European Journal of Pediatric Neurology, 22(1), pp.39-45.