Most children who come to see me have a combination of developmental delays, communication challenges and symptoms of anxiety, resulting in frequent tantrums. It is common for parents to share details about their daily struggles. The familiar patterns are that their children often have meltdowns during transitions, aggressive behaviors during tantrums or panic-like symptoms in new or unexpected situations.
Over time, parents begin to worry about how they will manage these behaviors or what others will say or think. I often see a pattern develop where parents can anticipate when difficult behaviors will emerge for their children, and they try many different strategies to support their children, but sometimes they feel that what they are doing is not helpful.
Why Do These Behaviors Occur in Fragile X Syndrome?
Let’s begin by talking about the neurobiology of fragile X syndrome (FXS). Based on brain studies, we now know that the brains are wired differently for individuals who have FXS, which leads to a situation of hyperarousal of the sympathetic nervous system. People with FXS tend to have trouble habituating (getting used to) to different sensory experiences.
For example, when they experience loud noises, crowded places, unexpected movement, visually distracting places or strong smells, it can be overwhelming. Denser neural connections within a part of the brain called the amygdala (the integrative center for emotions and emotional behavior) also increase the likelihood of hyperarousal and the probability of increased anxiety.
Children with FXS tend to have maladaptive behaviors (tantrums, screaming, trying to flee a situation or panic) when their brains and bodies move into fear/fight or flight following a triggering event. The hyperarousal leads to a disorganized state with decreased self-regulation, decreased access to language and communication and reduced attention. Once this process begins, it can be hard to avoid an explosive outburst.
Strategies to Manage Anxiety
We try to use strategies that focus on preventing the escalation of anxiety and maladaptive behaviors by planning ahead and using strategies to keep children (and parents) calm.
Structure and Predictability
We increase structure and predictability by creating practiced routines that are supported with visual supports. We often use visuals (pictures, visual schedules or check lists) to show a sequence of events. There are a variety of apps that work well for this. One I like is called “Choiceworks”. It allows one to set up a visual schedule using their pictures, or importing your own photos, so it is easy to individualize. Additionally, you can check pictures off as you go so that it functions like a checklist. It can be used to show a sequence of events or to show steps to a task.
One family I know is using it to help with anxiety around going to the dentist. They have set up a plan with their dentist to make repeated visits (one per month), where the child can follow the sequence while getting comfortable with the setting. For this child, the hardest part is the dental exam. Before we get to that step, we will use the checklist to practice all of the other steps, including a trip to the prize box. After he is more comfortable, we can add in steps like “Dentist will look into open mouth,” “Dentist will count teeth” and “Dentist will brush teeth” so that he can learn the longer sequence without experiencing panic. Similar pictures and steps can be used to practice brushing teeth or other self-help skills.
We decrease the length and complexity of the language we use with children with FXS. When children are feeling worried or experiencing anxiety, it is harder for them to understand complex language. Unfortunately, in moments of frustration, sometimes parents talk more and give more directions. This tends to add to overstimulation, which can intensify the likelihood of maladaptive behavior.
Instead, it is helpful to use short phrases that are calming and have clear directives imbedded with in them. By relying on short phrases, children can quickly understand their meaning. By using predictable, practiced phrases, children know what to expect.
We teach calming routines. When they are practiced ahead of time, calming routines can interrupt the escalation of anxiety and overstimulation. Examples might be taking deep breaths, counting to 5 or 10, listening to a calming song, looking at a favorite picture or book, using fidget toys or stress balls, doing mindfulness exercises, holding yoga poses, pouring beans or rice or playing in Kinetic Sand. These are all strategies that when practiced in non-stressful situations can be used to bring greater calmness during points of increasing anxiety. It can be very helpful to offer a familiar activity that has a calming influence and can interrupt perseverative focus that can develop as anxiety is heightened.
Once these strategies have been practiced in calm moments, they can be offered as a regular part of the overall daily schedule, and they can be used prior to a transition or a known triggering event. Your child may benefit from working with an occupational therapist at school, or in private consultation, to work on calming strategies as part of their overall sensory processing program.
Identifying and Expressing Feeling States
We teach identification and appropriate expression of feeling states. Children can point to pictures to show how they are feeling. They can learn short words to associate with feeling states (mad, sad, worried, scared). “The Incredible 5-Point Scale” by Kari Dunn Buron uses visuals and allows children to reference how they are feeling, regardless of delays in expressive communication skills. A child can point to show what they are experiencing on a feeling thermometer, and a parent can help the child to use strategies to return to a calmer state.
Here are some examples that were made for a child who moved into a tantrum each time he experienced too much frustration.