CompAS
The effect of a high intensive training in augmentative and alternative communication on communicative possibilities in patients with Angelman Syndrome

Children with AS have a strong will to communicate, but live in a world in
which they are surrounded by a language that they will never fully master:
speech. Research shows that the ModelER approach (modelling, encourage and
respond/recast) is successful to increase the use of Augmentative and
Alternative Communication (AAC) in children with an intellectual disability
(Sennott, Lighty, & McNaughton, 2015; Sennott & Mason, 2016). The child first
has to be immersed with AAC, all day long, in all natural environments. We
start talking to typically developing children on the day they are born. We
presume competence; we expect that someday they will understand and use speech
themselves. Children with a developmental disability such as AS, should be even
more immersed with communication than typically developing children, as they
need more repetition before mastery can be expected. In our study, we presume
competence in children with AS. Together with parents and their children, we
will create Communication Passports, which will show how the children already
communicate, what their interests are and how to help them develop their
skills. Parents will then be coached how to apply the ModelER approach while
doing a range of activities with their child (e.g. reading a personalized book,
in- and outdoor activities) to promote independent AAC use.

The aim of the study is to investigate the effect of an intensive high frequent
parental coaching intervention on communicative abilities in patients with AS.
Patients with AS who receive standard care, will serve as controls.
Parents/caretakers in the intervention group will be taught to use the ModelER
approach in combination with the making of communication passports, activity
language displays (ALD*s) and personal storybooks (PEB*s). Our goal is to
teach and coach parents/caretakers in how to immerse their child with AAC in
everyday activities.

The study contains two phases. In the first phase we will study the
Communication Matrix in a cohort of (N=50) of children with AS with two or more
completed matrices, to be able to study natural course.
In the second phase we will focus on 17 patients with AS while their parents
receive a high-frequent coaching intervention. It is a block-randomized
controlled study with follow up design. (ABA-Follow up) A stands for baseline
measurements, B for high intensive parental coaching intervention.
The primary outcome measure (Communication Matrix) and secondary outcome
measure VAS-scale, will be compared between the interventiongroup and
controlgroup.

The study is conducted through the ABA-follow-up-design. All AS patients
included in the intervention group will have a communication passport made.
Following structured observation and consideration of parental goals, the most
optimal AAC is made for each patient as well as ALD*s (this entire process is
called AAC assessment). Baseline measurements (=A) include a) Communication
Matrix (primary outcome) and b) C-BiLLT and ABC (Aberrant Behavior Checklist)
and other secondary outcome measures (see paragraph 5.1.2.) This baseline is
set up to assess how well parents/caretakers and children are able to use these
communication means before the start of intervention. After this a parental
coaching intervention based on the ModelER approach is started (=B) with
parents/caretakers, after which baseline measurements are repeated. Ten months
after the start of the intervention, follow-up assessment will take place
(including primary and secondary outcome measures). Outcome measures will be
collected online from October 2020, due to Covid- 19 pandemic.
Block-randomization takes place in a 1:2 ratio (1 intervention versus 2 in
controlgroup), stratified for age.
In the controlgroup the communication matrix will be filled out at baseline and
after 10 months, also the VAS-score will be asked on satisfaction on the type
of AAC that is being used and of the communication in general.

Former research has repeatedly and consistently proven that receptive language
skills of patients with AS outperform their expressive language skills. In
multiple case studies progress is seen after AAC intervention. However, further
implementation, maintenance, and development of AAC skills often fails because
of difficulties parents experience with implementing the intervention in
everyday situations. They need coaching of a skilled professional. In children
with special needs (not AS), the ModelER approach is already proven effective
and was experienced to be easy to implement by parents/caretakers. We expect
that the efforts of patients and their parents/caretakers does not weigh
against the advantage they can achieve by stimulating communicative functions
in the most optimal way. Behavioral problems often (if not always, or at least
partly) occur, maintain and worsen because of problems in the communication to
and from patients with AS. With optimal use of the communicative possibilities
of the patients with AS and by correct AAC implementation, there would almost
certainly be decrease of behavioral problems and improve of quality of life.
During the AAC assessment, the goals and capabilities of parents and children
are closely considered. Prior experience has taught us that intervention should
be a direct fit to the wishes and possibilities of parents and their children.
This will also lessen the burden we place on both parents and children: we
follow their goals.