This is our daughter Tess. She just turned 10. For her whole life, she's been almost entirely nonverbal.
In her early days she'd sometimes say the words "more" and "Mom," but these gradually faded away. We her parents and her older brother have modeled the heck out of language for her, talking to her constantly for years, but no go.
If Tess were typical in all other ways and her only issue was that she couldn't get the words out, she could simply use workarounds, such as writing things down or using sign language. But it's not that simple.
Aside from her problems expressing herself, she also has issues in receiving language from other people. If we give her straightforward directions, like "come here" or "sit down," she doesn't reliably follow them, or seem to grasp them. In fact, her receptive language issues are such that she doesn't respond to her own name.
This combo--deficits in both expressing herself and receiving language--has a name: Mixed receptive-expressive language disorder (DSM-IV 315.32).
That disorder isn't her only issue, when it comes to communication, though. Unfortunately, there are multiple additional layers standing in her way. Tess has what could best be termed a multilayer cake of language challenges. It’s part of her ultra-rare genetic disorder of USP7.
For one thing, she has autism. Doesn't initiate, engage in, or maintain social contact. Has trouble engaging in activities to socialize with her classmates. Emotional response to another person in a social situation? Nope.
On top of her autism, we think she might also have an auditory processing disorder. (The layer cake makes this extremely difficult to confirm.) Tess takes time to register sounds. Slam a door, and though everyone else in the room jumps at the sound, she'll take a good 5-count before reacting.
Another layer: major cognitive delays. We calculate they put her at roughly the level of a toddler, even though she's 10. Example: she doesn't understand danger. She doesn't avoid moving cars in a parking lot because she doesn't know any better. She'll grab a sharp object, like a pen or a knife, and put it in her mouth.
With all these layers--her language disorder, autism, auditory processing disorder, and major cognitive delays--you'd think she would just sit there, not even trying to make her wishes known. But she doesn't do that at all. In spite of all those layers, she does the opposite.
Tess deeply wants to communicate. She does communicate with us--constantly. She's deliberate in her movements, in letting us know what she wants. She reaches for things. She also gets upset and yells or cries when things aren't going her way.
As a result of this nonverbal communication, we know the things she loves.
She loves eating. She lives to eat, and her appetite is huge. We've only discovered two foods she doesn't like: celery and spinach. She lets us know what she wants to eat by going to the places in our house where we keep food. And if she's hungry and we aren't feeding her, she makes sounds, like the bleating of a goat.
She also loves swimming. When she's in the water, she laughs a lot and makes noises of contentment, like sighs and soft hums.
Another thing she likes is music and dancing. When a song comes on, she'll reach up, asking to dance.
But there are times when she clearly gets frustrated that she doesn't have words. In those frustrating times, her attitude is this: "Why don't you people give me what I want?!"
One of the first times we were able to unlock her language potential was during an appointment with Alycia Berg, a speech-language pathologist at Boston Children's Hospital in Waltham, MA.
Alycia specializes in augmentative and alternative communication, also known as AAC. These are methods designed to help people communicate when they have language impairments. AAC can include communication boards or books, speech generating devices, or voice output communication aids.
Alycia's first AAC successes with Tess weren't with any of these, though. They were with an electronic switch--a gigantic button the size of your hand. At our appointment in Waltham, Alycia picked something that motivates Tess: bubbles, the kind you blow with soap and a wand. Alycia used the switch to record her own voice saying "bubbles." She blew some bubbles for Tess, then stopped. To get more bubbles, Tess had to hit the switch. Soon, Tess got the picture about cause and effect. She figured out that hitting the switch and making it say "bubbles" would get her more of what she wanted (the bubbles.)
This was the beginning of a long journey for Tess with AAC. She's since graduated to a speech-generating device, like an iPad, with software called Novachat. Novachat is a grid of large tiles, each with one word. We pushed for years to get this device and software for Tess, imagining that it would enable her to say anything she wanted--anywhere, anytime.
The reality is this: she can navigate to the things she loves, like EAT and BUBBLES and MORE MUSIC PLEASE, but we can't tell if she's really distinguishing among them. For example, maybe she wants to say EAT, but she doesn't really look at the EAT button, and instead she hits BUBBLES. And since she likes bubbles too, she accepts bubbles, even though it isn't what she intended to say. In other words, it could be that she's just blindly stabbing at buttons, without intention, and since all three are things that she likes, we can't tell what she meant to say.
The same thing happens with another system she uses at school called PECS, which stands for Picture Exchange Communication System. PECS is a 6-phase protocol that's teaching Tess to give a single picture of a desired item or action to a "communicative partner" who immediately honors the exchange as a request. Tess has reached the phase in which she's supposed to distinguish between two preferred items and request one. Again, though, it's hard to tell what she means when she selects one of those PECS pictures, since she likes both things. Which one did she intend to ask for?
With both PECS and her device, she's been stuck at this phase for months now. Ideally, she'd proceed to skills like forming sentences with adjectives. Or even commenting or answering questions. But we aren't there yet.
Meanwhile, we haven't given up on her speaking. We've been experimenting with a system of online videos called Gemiini. Gemiini had instant credibility with me, because it was started by a mom for her twins, who have autism. It uses discrete video modeling, breaking language down into small parts with repetition, so Tess can retain the information. We've had some success with it. One video says "arm" a whole bunch of times, and one day Tess repeated it: "Arrrrrrrm!"
There have been other flashes of typical language lately. Example: at school the other day, a teacher said "Hi Tess" to her in the hall. Without any other prompting, Tess responded: "Haaaaaai!" (Side note: this is one of many examples in which Tess does amazing stuff at school or with her various therapists that she never does for us at home.) She also sometimes says "momomomom" and "dadadadad" in the morning when we come to get her out of bed.
These are exciting moments, for sure. But we still don't know what makes her come through with words on some days and in some settings but not others. She has her complex layer cake of issues, plus fatigue and gastrointestinal issues sometimes cause her to shut down.
We want to help her with language, but sometimes we also just want to be together as a family, without feeling like we need to be working on something all the time.
Those who know her best can understand her well. We refer to this as being able to "speak Tess." It's our dream that someday she'll be able to communicate in a meaningful way with a complete stranger. Tess is doing great things. She is known and loved by her classmates and her family. She has a distinct identity. And we want to help her develop her words even more, so she can better express her wishes. We're always open to new ideas. We're going to keep trying.