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A big part of parenting Greta has been finding effective ways to communicate with her. As a young child, Greta had a severe speech delay – a common issue in people with Prader Willi Syndrome (PWS). It stems from a combination of the developmental delays, thick saliva, and low muscle tone all associated with PWS. Greta didn’t really start using words until she was about four years old and she was difficult to understand, especially if you weren’t familiar with her unique speech patterns and didn’t know which sounds meant what.
Eventually, in addition to her PWS-related speech issues, she was diagnosed with childhood apraxia of speech. “Childhood” refers to the fact that it was present from birth; “apraxia of speech” is a neurological disorder affecting the brain pathways that produce speech. The brain knows what it wants to say, but cannot properly plan and sequence the required speech sound movements.
Over the years we used several different strategies, often at the same time, to help Greta communicate:
PECS (picture exchange communication system)
We started by teaching Greta to give us (or the speech therapist) a picture of a desired item, and she would immediately receive that item. It progressed with teaching her how to put the pictures together in simple sentences.
Her PECS, as we called each small, square, laminated picture, were attached with Velcro onto sheets categorized in a binder. As new words were required to talk about new experiences – flying on a plane, a new routine, going on vacation, a school field trip – new pages were added to her ever-growing binder. This method was most useful with people who didn’t know sign language or, because visual processing was easier for her than auditory processing, to help her understand a schedule or sequence of events.
Speech Therapy
We saw a number of speech language pathologists (SLPs) over the years, but had the most success working with a talented and dedicated SLP named Susan Rafaat. She used a combination of the Kaufman Method and PROMPT therapy approaches, and I became proficient in both.
The Kaufman Method teaches sounds using a specific hierarchy. Greta worked on word approximations first and, once the basic patterns were mastered, more complex consonants and syllables were introduced. Click here to watch a short video about the Kaufman Method.
PROMPT is an acronym for Prompts for Restructuring Oral Muscular Phonetic Targets. The technique uses touch cues to a patient’s articulators (jaw, throat, lips) to manually guide them through a targeted sound or word. PROMPT is an intense form of therapy that requires daily practice and, as such, I was trained to practice with her. This video is a good example of how I would work with her at home.
I remember we had been working on the sound “ee” for about four months, and while she occasionally achieved that sound, it seemed like she would never be able to produce it consistently. Then suddenly one day in session, “Ee. Ee. Ee. Ee! Ee! Eeeee!” I actually had tears in my eyes as I clapped and cheered her on! Greta was five and a half years old at the time.
Augmentative Communication (AugComm) Device
We also worked with an SLP at the Alberta Children’s Hospital to secure funding and training in a computerized augmentative speech device. It was a device about the size of a small lunch box with a screen and a generic, computerized voice. It was pre-loaded with PECS that she could use the touch screen to access.
Greta’s AugComm device never became a preferred method of communication – for Greta or for us. It was cumbersome, slow, and a pain in the butt to lug around. Her preference was always sign language: immediate, efficient, no equipment, no searching through a book or reading a screen.
American Sign Language (ASL)
This was our go-to method of communication for many years. We introduced some basic signs around six months of age – “help”, “more”, “yes”, “no”, “milk”, “please”, “thank you” – but Greta didn’t really begin using ASL regularly until she was about a year old.
Once she got the hang of it, there was no looking back! Auntie Cristina (my sister), Lindsay (Greta’s developmental aide at the time), and I completed three levels of ASL through Continuing Education, and we still couldn’t keep up with Greta!
Because she has small hands (a characteristic of PWS) and didn’t have the greatest dexterity when she was very young, we had to modify many of the more commonly used signs for the first couple of years... we called it “signing with a Newfie accent”.
Chatty Greta
Now, at 14 years old, Greta communicates verbally most of the time. It can still be difficult to understand her sometimes, even for me, but if I ask a few questions to gather some context about what she’s trying to say, we usually figure it out together.
Greta loves to meet new people, and she’ll happily strike up a conversation with a stranger (as long as she gets to ask most of the questions, which inevitably revolve around pets and children). People are overwhelmingly generous with their time when she tries to engage them, and many a time I have walked away from an exchange between Greta and a total stranger with a lump in my throat because they’ve been so kind and patient with her.
One of my greatest pleasures is to keep a record of the cute and funny things she says; I jot them down in “My Quotable Kid” journal, and there are some real keepers from both my girls. Here are some of my Greta favourites:
9 years old, begging Kelly (a respite provider) to stay a little longer and give her a back rub. When Kelly asked why, Greta responded, “You’re a weely good wubber.”
10 years old, tucked into bed for the night and waiting for her bedtime story – [giggle, giggle…] “I just so happy today!”
And one of my all-time favourites…
11 years old, feeling sleepy while sitting in her comfy chair in the family room – “Mommy, my eyelids keep falling down.”
I often take for granted my ability to communicate my feelings, needs, wants. For Greta that has never come easily, so I continue to fill up that book with her sometimes cute, sometimes funny, sometimes touching, always hard-earned quotes.