Boy with Fragile X at the 2018 NFXF ConferenceMost 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. (Read another NFXF article on visiting dentists)

Simpler Language

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 embedded within them. By relying on short phrases, children can quickly understand their meaning. By using predictable, practiced phrases, children know what to expect.

Calming Routines

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.

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The Calm Down Book

At past International Fragile X Conferences, I have demonstrated the use of a flip book (the Calm Down Book) that teaches a sequence of calming exercises, which use deep breathing and modified progressive relaxation with pictures from Board Maker. If you would like a copy of it, please feel free to email me.

calm 3

Where to Begin

These ideas need to be individualized for your child. It is important to keep in mind what you already know about your child. Try to work with a friend to make a list of what are likely triggers for panic and anxiety that result in tantrums.

  • Pay attention to patterns when your child starts to perseverate or repeat ideas in a way that makes you feel he or she is anxious rather than excited.
  • Pay attention to their body language. Kids will often try to hide, chew more, cover their ears or leave a situation that is overwhelming. Flushing is a common reaction when feeling anxious.
  • Pay attention to what you already know about your child’s likes, what helps them calm down and what objects and activities provide good distractions.
  • Model use of feeling words and begin to introduce feeling pictures.
  • Practice taking a big breath before major transitions, counting or using positive self-talk so that your child sees you using the same strategies you want your child to use.
  • Consider making a video of one of your child’s older siblings, or a peer, using the strategy you want your child to use.
  • Pay attention to the words you use. Help your child to focus on something they like that is happening in the present moment rather than offering reassurance about a future event.
  • By adding one piece at a time, it is possible to build skills in the areas of self-regulation and calming strategies.

Additional Resources

Pincus, Donna. Growing Up Brave, (2012). Little, Brown, and Company.

Buron, Kari Dunn and Mitzi Curtis, The Incredible 5 Point Scale, 2nd Edition, AAPC publishing, (2015).

Downloadable forms that go with the incredible 5 point scale:

Read more NFXF articles about Life Strategies.

Jennifer H. Epstein, PsyDJennifer H. Epstein, PsyD is a licensed clinical psychologist in private practice in Hingham, MA, where she works with children and families affected by autism, anxiety and other developmental concerns. She is a certified therapist and certified trainer in the Early Start Denver Model of autism intervention for toddlers. She worked at the Fragile X Treatment & Research Center at Children’s Hospital in Denver, Colorado from 1996 until 2010.