Understanding Angelman Syndrome: A guide for parents
·

Understanding Angelman Syndrome: A guide for parents

ByLondon Speech and Feeding

Angelman Syndrome (AS) is a complex neurological disorder that affects development. It’s caused by a missing or functionally incorrect gene (UBE3A) on chromosome 15. While AS is rare, understanding its characteristics is crucial for parents and caregivers.

What are the key characteristics of Angelman Syndrome?

Children with AS typically exhibit a range of unique characteristics, which can include:

  • Developmental delay: Significant delays in reaching developmental milestones, such as sitting, crawling, and walking.
  • Speech impairment: Limited or absent speech. Individuals with AS may use few or no words.
  • Movement and balance issues: Difficulties with coordination, balance, and movement, sometimes causing a jerky or unsteady gait.
  • Happy demeanour: Frequent smiling, laughter, and a generally happy, excitable personality.
  • Intellectual disability: Varying degrees of intellectual disability.
  • Seizures: Seizures are common and often begin in early childhood.
  • Sleep difficulties: Disrupted sleep patterns and difficulty falling asleep.

The role of Speech and Language Therapy

Speech development and social communication is significantly affected in Angelman Syndrome, and therefore, Speech and Language Therapy plays a vital role in helping individuals with AS to communicate. As Speech and Language Therapists (SLT) we can work with the child and family support any of the following:

  • Assess communication skills: We evaluate the child’s current communication abilities, including any vocalisations, gestures, or signs they may use. With younger children we do this through play and playful social games as well as observation of a child playing and interacting with their siblings or caregivers.
  • Develop alternative communication strategies: Since spoken language may be limited, SLTs can help the child learn other ways to communicate, such as nonverbal communication, e.g
    • Gestures: use of pointing, waving and miming certain activities.
    • More formal sign language: Teaching basic signs to express needs and wants.
    • Core boards: Using pictures and symbols to represent everyday common words, actions, and feelings.
    • Augmentative and Alternative Communication (AAC) devices: Providing electronic devices that can produce speech.
  • Encourage vocalisations: When words don’t readily develop, SLTs can encourage the child to make vocalisations and sounds, as these can be a form of communication.
  • Support language development: SLTs can work on understanding of language, even if expressive language is limited.
  • Educate and support families: SLTs provide families with strategies and techniques to support their child’s communication at home.

The importance of a multidisciplinary approach

Caring for a child with Angelman Syndrome requires a team effort. A multidisciplinary approach, involving various healthcare professionals, is essential to address the diverse needs of the individual. This team may include:

  • Paediatrician: Provides overall medical care and monitors the child’s health.
  • Physiotherapist: Helps with movement, balance, and coordination.
  • Occupational therapist: Works on daily living skills, such as feeding, dressing, and self-care.
  • Speech and Language Therapist: Addresses communication and language needs.

By working together, we can provide comprehensive care, address the unique challenges of Angelman Syndrome, and help the child reach their full potential.

Conclusion

Angelman Syndrome presents unique challenges and opportunities. With early diagnosis, appropriate interventions, and a strong multidisciplinary team, children with AS can make good progress and live fulfilling, joyful lives. As Speech Therapists we delight in supporting parents in their role in advocating for their child.

Do get in touch via my contact form if you are concerned about your child’s development.

Sonja McGeachie

Highly Specialist Speech and Language Therapist

Owner of The London Speech and Feeding Practice.

Health Professions Council registered
Royal College of Speech & Language Therapists Member
Member of ASLTIP

Find a speech and language therapist for your child in London. Are you concerned about your child’s speech, feeding or communication skills and don’t know where to turn? Please contact me and we can discuss how I can help you or visit my services page.

1
Rethinking the PECS approach
·

Rethinking the PECS Approach

ByLondon Speech and Feeding

I want to talk about some concerns of SLTs, parents and increasingly autistic adults who explain to us how this communication method did not really work so well and why.

What is PECS in a nutshell:

PECS (Picture Exchange Communication System) is based on the idea of exchanging pictures in return for desired items. For more advanced users, it is used to communicate different functions such as emotions, comments, negations using the exchange of a sentence strip. It was founded on the principles of Applied Behavioural Analysis (ABA).

How does that look in practice?

In my experience, having been trained in the approach myself, the overall aim is eventually for the child to spontaneously go and get their picture book (PECS book), open it, look through a range of pages to select the correct picture of what they want to have or say, then go and find their communication partner, and finally place that picture onto the communication partner’s outstretched palm to be rewarded with an item or with a response of some sort. Or the child selects a range of pictures to create a little sentence, such as: ‘the blue fish swims in the sea’, ‘the red bird flies in the sky’ or ‘I see a red bird’ for example. This can be part of a structured table top activity.

The system follows a series of phases, starting from simple picture exchanges to eventually construction of sentences using symbols. PECS’s aim is to promote communication initiation and reduce frustration for those who struggle with speech.

So far so good one might say, why not? Before I go into the various concerns, I would want to add my own working experience with PECS, and whilst it is my opinion, I would say I have NEVER seen a working PECS book being used spontaneously!

My experience

I have seen attempts of stages 1 and 2 done quite well, in schools, and where people knew that I was coming in “to have a look at how PECS is working with child X”. Yes, in those instances an effort was made of course to try and show me how it worked. I must add that have never been very impressed. I cannot recall it used for any other items than: biscuits/quavers/crisps/ raisins and bubbles/puzzle pieces or spinners.

If we want to see a child trained to exchange for these items in a structured setting, i.e., the child sits at a little table with the adult sitting opposite enticing the child with one or other item, then yes that can be done successfully. I have seen children exchange 25 pictures with a crisp on it, for said crisp and they might have asked for another 25 of those crisps given half the chance. Yes. Good. But. I have yet to see a child go to their PECS book and go through all the motions that I mentioned above to get a crisp. In school they don’t need to: they know that crisps are only available when the PECS book is being practised. Otherwise, let’s be honest, it’s fruit at 10.30 am!

So, they don’t get a spontaneous opportunity to ask for highly motivating items as that is not how school works, is it? ‘SIR! Can I have a crisp?’ At 10.02am, in the middle of maths? Didn’t think so… So in reality this does not get practised in my experience.

A few concerns in no particular order:

Limited Generalisation

One issue often raised is the limited generalisation of skills learned through PECS. The structured nature of the program may result in a child only being able to communicate effectively within the specific contexts where they were taught to use the system (as I suggest above: crisps: yes, please let’s do the PECS for it). This limitation can pose challenges when trying to apply communication skills in new or unstructured/spontaneous situations.

Lack of Spontaneity

Critics suggest that PECS can sometimes lead to scripted and less spontaneous communication. This is also what I have observed. Since the method is designed to follow a structured progression, there is a concern that individuals might struggle to initiate communication outside of the established framework, potentially hindering their ability to engage in more natural interactions.

Narrow range of communication functions being practised

While PECS is quite successful in focusing on requesting and naming items, there are many other important communication functions, such as expressing emotions, asking questions, giving opinions or greetings for instance. We can argue that a communication core board where we have a whole range of different core words available lends itself much better to practising a range of communicative functions.

The Pictures are movable

They are attached to the book via Velcro. They are constantly being picked and exchanged and then returned to the book. This means that the pictures tend to be always in different places. This goes against the motor planning that takes place when one is learning a new skill: imagine you want to learn to touch type and the letters always move and are at different places? How can you be quick about finding a letter? You can never get to “automatic” with this type of approach.

Communication is not taught via behavioural means

Only if you say “banana” in the way that I dictate that you should will you get a piece of banana. Who does that? Nobody. Typically, child points to the counter where there is a banana and says: ‘ba’ or ‘ana’ and mother/carer will look over there and say ‘oh banana! You want a banana? Ok there you go have a piece.’ Or something like it. Mother will not say: ‘SAY BANANA or else you won’t get it.’ Child hears mum saying ‘Banana’ each time and with time will point and say ‘banana’ or ‘I want-a-nana’ or something. This is how communication is learned: through the adult modelling it cheerfully all day long and the child hearing it and then gradually copying it.

One other gripe I personally have but I am reliably informed by all my parents that they share this about PECS:

IT IS SO LABOUR INTENSIVE!

There are 10, 50, 100’s of little pictures that first of all need laminating… then velcroing, then finding and replacing. As I said above, it’s a constant moveable feast for one, but also you LOSE them. Yep. You want to find the picture for “trampoline”. ‘Where is it? I saw it yesterday… We had it outside when we practised you asking for the trampoline. I am sure we put it back? Where is it??? Ok. We need to print off a new one.’

It is also labour intensive for the first stage where you need to have TWO adults to ease the exchange (pick up and release of picture into the communication partner’s hand). Who has two adults available for what can be weeks until the child is able to pick up and release by themselves?

YEP. So it’s really not for me you can tell! I much prefer Core boards (see my previous post on using one) or electronic speech generating AAC devices like GRID, or LAMP or TOUCHCHAT. These are all great to use and there is good support out there for introducing these.

Finding a Balance

While the concerns surrounding the PECS approach are valid, it’s fair to note that the method also has some merits. There is anecdotal evidence of many individuals who have successfully improved their communication skills and quality of life through PECS. But, finding a balance between using PECS as a stepping stone and ensuring the development of more comprehensive and SPONTANEOUS communication is key.

As educators and therapists, we need to extend the focus beyond requesting and labelling by incorporating symbols that represent emotions, actions, and more complex ideas. This expansion encourages a broader range of communication functions. When the time is right, gradually transitioning from PECS to more advanced communication methods such as Core boards or electronic AAC tools and speech-generating devices is the way forward.

We want to value all communication equally and our approach ought to be playful and child-led and to focus on intrinsic motivation instead of extrinsic rewards and reinforcers.

If you have any questions or if you are looking for a therapist who endorses play-based and child-led therapy approaches, please do reach out.

Find a speech and language therapist for your child in London. Are you concerned about your child’s speech, feeding or communication skills and don’t know where to turn? Please contact me and we can discuss how I can help you or visit my services page.

0