We have followed Charlie and his assistants for over a year as they worked on adapting to a dance activity. To support persons with CHARGE syndrome, for them to actively partake in new activities, we need knowledge on how to adapt both the physical and social environment.
Charlie is a smart and persistent 30-year-old with a strong personality. He has a great sense of humour, appreciates practical jokes, and likes to play around. For the past ten years, Charlie has lived in an apartment in a residential facility adapted for persons with congenital deafblindness. Most of Charlie's neighbours have known him since his school years.
”Charlie has a great sense of humour, appreciates practical jokes, and likes to play around.”
Charlie has assistants present both day and night to provide company or support when needed. He sleeps between 4-6 hours a night. Despite this, he remains alert and active during the day, but due to his sleeping pattern, he needs his assistants present during the night as well.
Charlie likes being occupied. He enjoys the outdoors, going on car trips and exploring his surroundings. He also enjoys turn-taking games and engaging in cooking and taking care of his apartment together with his assistants. He prefers close interaction in a 0ne-to-one setting, where the other person can focus exclusively on Charlie. In larger groups, he often observes, while in smaller groups, he interacts more directly.
Before taking part in an activity with another person, Charlie wants to sit and look into the other person's eyes, very close, for a long time. He holds the other person's hands and plays a rhythmic, swinging game to settle down and find a common tempo. After this he is ready for adventure.
Living with CHARGE
Charlie’s sensory integration and perception of the world around him are affected by his CHARGE syndrome. He has a narrowed visual field and possibly a cerebral vision impairment. He can observe the smallest changes in his environment one day, whilst the next he may walk straight into a parked car. He needs a guide-interpreter to be safe when he is in traffic, and, by choice, also when he is exploring new places. He visually understands simple, adapted, and concrete sign language but often prefers sign language in a tactile modality. Bright colours attract him, and pictures and photos are easier for him to see on a screen with high brightness.
Further, Charlie has functional deafness. He neither responds to sound nor use hearing as a channel of communication. When tested in childhood, he had a hearing loss of 60 decibel in his right ear and is considered totally deaf in the left ear. Charlie has cup ears, which are very common for CHARGE syndrome, making it difficult to try out hearing aids. After primary school, he has completely stopped using his hearing aids.
”When it comes to how clothes feel against his body, Charlie is very sensitive; they must fit tightly."
When it comes to how clothes feel against his body, Charlie is very sensitive; they must fit tightly. He also appreciates tactile stimulation such as massage, showers, and baths.
Charlie does not seem at all affected by temperature changes. He likes to wear many layers of clothing even if they make him very warm. This is most likely linked to a need for pressure on the body to enhance his body perception.
He enjoys pressure on joints and muscles. It can be very soothing for him if someone taps on his body rhythmically from top to bottom putting extra pressure on his joints. Charlie often sits with his legs folded beneath him. This increases his awareness of his body. Charlie also enjoys sitting in confined spaces. He appreciates pressure on his body, by means of a weighted blanket, a bean bag, or another person leaning on him.
In new places, he needs to lie on furniture or other things, on his back with his legs bent. Sometimes he puts one foot against the knee of the other leg and places his head lower than his body. This position puts pressure on large joints and helps him perceive his surroundings. This is his strategy for calming down, to self-regulate. Charlie appreciates if the assistant is imitating his position and gives him plenty of time to prepare for exploring the new environment. He needs to observe his surroundings in peace without demands. He also needs long time after activities to process what’s been happening.
Charlie has scoliosis, which, along with narrow airways, asthma, and allergies, is challenging. He needs to control his weight to avoid too much pressure on his lungs.
Self-regulation – ways to stay in control
Charlie stresses easily. He needs to know where he is going, and why, to be willing and able to participate in activities. Changes in physical or social environment is hard for him. The same is true when many people move around and worse if they are moving things around. Charlie then has an increased need, compared to when he is in his own home, to check that furniture and things are in order. He likes things to be in a straight line and persons around him can help make things easier for him by supporting his need for structure.
Charlie has certain behaviours that are his way of dealing with these situations. They are strategic and conscious ways to self-regulate, calm down, and maintain self-control:
First, he connects his hands and bite on one of them.
He then may need to stand up and spin around a few times, with one foot planted firmly in one place and the other making the circle.
If this doesn’t help, the next way to regulate is to run in small circles. Often Charlie is making sounds while running, it is easy to hear that he is worried.
Lastly, if none of these strategies help him, Charlie chooses to leave the activity and/or the room.
Different communication strategies
Charlie uses body language, tactile gestures, and signs to communicate. He also uses concrete objects to show what he wants to do.
Charlie understands adapted sign language through both visual and tactile means. This includes using short sentences, adjusting the pace of communication, and considering distance. The needed adaptation depends on Charlie's state of mind, motivation, and the conversation topic. Concrete objects combined with signs are used as augmentative and alternative communication (AAC). For example, to clarify what to do outdoors, the sign for car is used in combination with the actual car key.
Charlie's year with a dance activity
One winter a few years ago, Charlie was introduced to a new daily activity: a dance session focused on body awareness, body image, and social skills. This should suit his interests perfectly. Despite taking place in a group setting, it involved one-to-one interaction with an assistant close to Charlie throughout the session. Together they followed the dance instructor’s teachings.
The activity would enhance the rhythm of the music with joint movements such as rocking, back-to-back. In the dance, the assistant used fabrics, feathers, balls, pea bags, and hands to create breezes, beats, pressures, stretches, and strokes on the participant’s body.
To avoid a new environment, the activity began in a familiar setting, the residential facility’s common room.
Curiosity
Before the dance activity was started, the furniture in the common room was rearranged. Beanbags and yoga mats were laid out to mark the location of the dance area. Charlie was very curious about what was happening. After it was explained to him that the furniture would be placed differently for the duration of the activity, Charlie laid down on a beanbag but found it difficult to get fully settled. There were a lot of things he needed to keep track of. He wanted the furniture back in place and asked for this, several times. The assistant tried to help Charlie endure and calm down by, among other things, laying down next to him.
Charlie participated in the beginning of the session when rhythms were tapped on the floor near his body. He did bite his hand but remained lying down. In the next exercise, Charlie got up and tried to correct other participants and move them around. He wanted to have the assistant close to him but could not calm down. After an exercise, when a large piece of silk fabric was raised and lowered over Charlie, he got up and left the room.
When the dance activity was over and the furniture was back in its usual place, Charlie wanted to check several times that everything was in order and found it difficult to do other activities that day.
A new environment
For the rest of the term, Charlie could not settle into this activity. He joined in, rearranging the furniture before the dance activity, and participated at the beginning. But often, after ten minutes, went home to his apartment.
After the summer break, the dance activity moved to the daily activity centre. In a large room, the dance area was defined by curtains, screens, and a ring of yoga mats with the equipment in the middle. There were also some beanbags in different colours for those who wanted to use them instead of lying or sitting on the yoga mats. The room has good sound equipment, and the walls and floor are made of wood, which provides good vibration amplification of the music.
Charlie quickly chose an orange beanbag as his place and the assistant sat next to him. Again, he found it difficult to stay in the activity with the group. After a few exercises, he decided to move to a sofa behind the curtain at one end of the room and lay down with his head in the assistant’s lap. In this position he could continue to do some of the exercises, though separated from the group in a smaller, confined space. This repeated itself over the coming sessions.
Changes became a turning point
After two months, some further changes were made.
Charlie especially appreciated the pea bags as they provided weight and pressure on the body and could be stacked in many fun ways. The pea bags became the symbol of the dance activity and were used as AAC before, during and after the activity.
Charlie’s beanbag was moved towards one wall to reduce distractions and other colourful beanbag were placed around him.
”We made sure that Charlie always had a familiar assistant with him who is used to interpret his expressions and interacting with him.”
We made sure that Charlie always had a familiar assistant with him who is used to interpret his expressions and interacting with him. It was not possible to have the same assistant each time, but a small group was selected and rotated between sessions. They also received guidance in the dance itself with clarifying explanations on how the different exercises in the session focus on strengthening interaction and body perception.
Video was used to show the progress of the interaction between Charlie and his assistants, to help analyse which exercises and materials seemed to attract Charlie the most. Then, these were expanded and used more in the activity.
After these changes, Charlie became more resilient and partook longer in the activity.
Yet, another two months and further adaptations were made in the room, to reduce sensory impressions and create a cozy and safe atmosphere. Charlie’s place in the activity was moved to a corner marked with two large fabrics on the walls, one blue and one orange. His orange beanbag was placed in the corner and the ring of yoga mats was moved closer making it easier for Charlie to be part of the group.
Charlie fully participates
In the early spring, more than a year after the activity first was introduced, Charlie began to stay in the corner for the entire 40-minute session. Initially, he focused more on himself and his assistant, skipping some parts of the dance but Charlie now fully engages in the group.
Throughout the spring term, he actively participates in all elements of the dance, often smiling, laughing, and showing clear appreciation of the different parts of the activity.