Each day is a kaleidoscope of challenges, triumphs, learning and love for Lily, Bella and Rylee Heiss. And now that the Hendersonville, TN sisters have their Tobii Dynavox T10s with Compass communication software in place, their mom Danielle says they’re getting more out of life because each has found her voice.
Though some may see them as fragile, the Heiss sisters are strong in mind, spirit and heart. They’re excellent spellers, good with numbers, love swimming and horseback riding, and care about people. The girls were born with Joubert syndrome, a rare genetic malformation affecting a part of the brain that controls balance and coordination. Apraxia of movement, intellectual disabilities, visual impairment, and liver and kidney problems are also associated with the condition, along with oral motor skill deficits and apraxia of speech.
The Heiss girls are non-verbal. For most of their young lives, they were a puzzle in that their receptive and expressive language abilities didn’t seem to match up. But now, with technological support and human encouragement, they’re starting to bridge the gaps. “They’re highly observant girls, taking in everything in their environment,” said their speech-language pathologist, Sara Schneider, M.S. It was just a matter of waiting for the right tool to come along so they could convey to others what they knew.
Bella and Lily, nine-year-old twins, were introduced to augmentative and alternative communication (AAC) technology first. Sign language just carried them so far because of their motor control issues. “It was really very limiting and relied on familiar people to put together the rest of the message,” said Mrs. Schneider, who works at Sumner Station-Sumner Regional Medical Center. The girls also used an AAC app to make requests but needed more vocabulary to balance their expressive and receptive language. Mrs. Schneider had Bella and Lily try a device offering both eye and touch access. Their condition makes it difficult for their eyes to work together so the touch access option proved more successful. After learning some basics of AAC device use, the girls again reached a standstill. A more portable, language-boosting device was in order to help them move forward.
By this time, Mrs. Schneider was also seeing Rylee Heiss, a perceptive girl eager for the same freedom of self-expression her older sisters enjoyed. Knowing the potential benefits of AAC intervention and the planning it required, Danielle and her husband Ched decided to pursue it earlier for Rylee.
The T10, with its tablet design and flexible Compass content, seemed a promising solution for the three Heiss sisters. So far, it is. Through modeling, their parents are showing their daughters the mechanics of using the device while introducing new vocabulary. Sometimes the girls motion to mom or dad to hold their hands steady while they compose messages. The girls respond to questions in age-appropriate language, sometimes selecting a pre-programmed message. They love spelling and are learning to use the device keyboard to type complete thoughts, letter by letter.
For Danielle, adapting to the process is not so unusual. She had a brother with cerebral palsy who could not speak. The two of them communicated intuitively through facial expressions and laughter. As her girls grow used to their AAC devices, things they talk about give their parents insight into their unique personalities.
Bella, a quiet thinker, has expressed frustration at her inability to speak. Combining single words with short phrases, she used the device to say she wanted to be able to talk to her friends, but her brain did not let the words come out. A favorite memory is when Bella, who likes to be outside, said she wanted to celebrate her and Lily’s birthday with a picnic and a Hello Kitty®* backpack as a present.
“I was in tears that for the first time my six-year-old was able to tell me exactly what she wanted for her birthday,” Danielle said.
Bella once asked her parents for two goldfish—one for her and one that her sisters would share. She spelled N-E-M-O on her device when asked what she would name her fish.
Lily, who tends to be free with her emotions, turned to her T10 months after Danielle gave birth to a son, Elijah, who had symptoms of Joubert syndrome and lived for less than a day. “She said, ‘I miss my baby brother, I know he is in heaven and I want to go to heaven with him,’” Danielle said. “Without that device, she would have struggled to keep her feelings inside.”
Recently, Lily argued with her mom using her device. Danielle said they were going to the mall. Lily, eager to eat at the restaurant there, voiced her disappointment when Danielle told her they were shopping first. “I do not like that idea,” Lily said.
Rylee, 5, also has her silly and serious sides. She likes watching football with her dad and used her T10 to tell him, “I want a pink football.”
Mrs. Schneider particularly liked the way Rylee kept a conversation going independently one summer day.
“How are you?” Mrs. Schneider asked.
“Terrible,” Rylee said, “I have a bug bite on my face.” After a brief pause, she typed “Let’s ask my mom for some cream,” on her device. Mrs. Schneider said the bite was too close to Rylee’s eye for cream and they did not have the cream with them, anyway. So Rylee simply told Mrs. Schneider, “Thanks for your help anyway. I will be OK.”
Danielle and Ched look forward to interactions that will unfold as their daughters start to use the technology with unfamiliar people in broader settings. They’ve set the stage by encouraging them to use it at home for communication during activities like playing games together or helping mom try out a new recipe.
“The next frontier is to integrate their Tobii Dynavox devices into everyday life,” Ched Heiss said. “The more opportunities they have to use them socially, they better they are at using them.” They use their devices to order at restaurants, where Danielle finds reaction to the technology interesting.
“Some people are just really excited and think it’s the coolest thing in the world,” she said. “Sometimes you can tell they’re a little bit uncomfortable, but they’re usually kind.”
The positive change the technology brings is what matters most.
“Before, we didn’t even know their favorite colors, something parents might take for granted with their own children,” Mrs. Schneider said. The Heiss girls’ success, she said, begins at home. “They have an incredibly supportive and involved family. That never ceases to amaze me.”
The girls are homeschooled and it is not unusual for some of their lessons to occur in waiting rooms between therapy appointments. Special helpers keep the daily routines manageable. One is Jane Speyer, Rylee’s former physical therapist. Retired and wanting to do something meaningful, Mrs. Speyer volunteered to help the Heiss family at home while Danielle was pregnant with Elijah and stayed on board for about a year. Mrs. Speyer sees improvement in the girls’ communication skills each time she visits and enjoys receiving emails that the girls write and send from their T10s.
Another special helper is Danielle’s mom Lily Cloutier, who lives with the family. The girls know her as “Grand-Maman”—“grandmother” in French Canadian, Mrs. Cloutier’s native language. She likes that they can tell her things that are important to them, as Lily did when Danielle left with Rylee for speech therapy. Lily usually went with them, but didn’t have an appointment that day. She told her Grand-Maman she felt confused and upset that they were going without her.
“It was good for me to know what was bothering her in the moment because otherwise we would have had to play a guessing game,” Mrs. Cloutier said.
Through technology, Grand-Maman is finally getting to know her granddaughters better.
“It’s a relief to know how they feel about life.”
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*KerPlunk® is a registered trademark of MATTEL, Inc.