Individuals with Angelman Syndrome face many communication challenges. They have a good understanding of language but encounter significant difficulties in expressing themselves verbally due to speech apraxia. Speech apraxia is a motor disorder that makes speaking difficult and can significantly complicate the correct articulation of sounds and words.
In Angelman Syndrome, this motor disorder arises from a neurological issue related to the dysfunction of the maternal UBE3A gene expression. Although individuals are clear about what they want to communicate, they face a barrier: the brain cannot plan or coordinate the necessary movements of the muscles involved in speech, such as those of the jaw, lips, and tongue, which are essential in articulating syllables, words, and complete sentences.
Individuals who are nonverbal are often mistakenly perceived as less intelligent or having less communicative intent, which is a huge misconception. There is a famous phrase that clarifies:
..
..
..
..
People with this syndrome are generally very social and sensitive to social cues, constantly observing those around them to understand interactions and changes in the environment. This attention also extends to routines, noticing immediately any changes in usual patterns.
Hyperacusis may also be a characteristic of these individuals, making them particularly sensitive to sounds that others may not notice. Music, on the other hand, plays a special role, being used to calm, focus, or cheer these individuals.
Moreover, individuals with Angelman Syndrome can develop anxiety about future events, even if they are positive. Teaching and reinforcing communication and adaptation skills can be crucial in helping them manage this anxiety throughout life. (Angelman Academy, 2023)
Individuals with AS often exhibit good receptive language abilities but suffer from severe expressive language dysfunction. They are, therefore, people with Complex Communication Needs (CCN). Augmentative and Alternative Communication (AAC) is the tool used to overcome this barrier and give voice to these individuals who, with the right support, learn and continuously evolve.(Angelman Academy, 2023)
AAC is a set of tools and strategies that an individual uses to solve everyday communication challenges. Communication can take many forms beyond speech or verbalization, such as: shared gaze, text, gestures, facial expressions, touch, sign language, symbols, pictures, speech-generating devices, etc.
We all use various forms of communication depending on the context and our communication partner. Effective communication occurs when an individual's intention and meaning are understood by another person. The form is less important than the effective understanding of the message. (ISAAC, 2023)
The placement of words is organized in a consistent motor plan (that does not change), but allows for vocabulary expansion:
Essential Vocabulary: a lean and stable set of high-frequency words
Accessory Vocabulary: words related to the individual's interests and life experiences, and context-specific words
Provides space for predefined messages: sentences and questions that speed up communication, make it easier or less stressful to convey an idea
Offers keyboard/alphabet and word prediction (in high-tech systems) (ComunicaTEA, Practical AAC, 2023)
..
..
All individuals with Angelman Syndrome (as well as their families) should have access to robust communication systems and training to develop autonomous expressive communication.
..
There are apps that allow some robust AAC systems to be used on tablets. These features are part of the group of high-tech systems.
Because they contain many words, robust printed AAC systems result in communication books, which are part of the low-tech systems group.
In addition to robust AAC systems, thematic boards and general interaction boards greatly assist in modeling communication and use by individuals with Complex Communication Needs. Each board should follow the same motor plan and use the same symbols from the individual's robust communication system. The image below shows an example of a general interaction board with about 100 essential words.
Provided by the GAMAS Initiative for free download here.
Steping into AAC
A project by the ASF e PrAACticalAAC created to support the learning of AAC by individuals with AS and their families.
Angelman Syndrome Foundation video.