Behaviour can cover many aspects of Fragile X Syndrome (FXS). Some behaviours are charming and idiosyncratic, others can disrupt daily life. Some behaviours may be linked to co-occurring conditions such as autism, ADHD, anxiety and depression. Often there is a combination of the above, unique to the individual and with changes in behaviour over the life course. It is important to identify any co-occurring conditions by professional assessment and have professional support in assessing and managing behaviours which challenge.

In addressing behaviours, please identify the strengths of the person with FXS, and build on these strengths as one aspect of helping to approach behaviours which may challenge.

The information in this section gives an overview of what can be a complex and inter-linked topic, with these various aspects being seen in different combinations in different people. There is no one-size-fits all approach to therapies — what may help one individual may not help another. It is important to discuss which therapies may be beneficial to a person with the relevant professionals.

This section covers:

An image of a model of a brain made out of modelling compound in different colours for different regions of the brain

Autism

Individuals with Fragile X Syndrome (FXS) may also have autistic-like features or fit the criteria for autism. Some of those with autism view their characteristics as an identity, and do not wish to change as it is their personality and personhood. Please be aware of the positive, unique traits autism may bring.

Autism is a neurodevelopmental condition characterised by difficulties with social interaction and communication, and by restricted or repetitive patterns of thought and behaviour. Managing behaviours associated with autism can sometimes need specialist support. Autism specialists and behavioural therapists will be able to assess individuals to determine behavioural therapies which may be beneficial.

Repetitive behaviours can be common in FXS and in autism, However, in FXS the repetitive behaviours can be a distinct feature and not linked to an autism diagnosis as other autism criteria are not met. Some studies have shown repetitive behaviours may be linked to changes in routine and/or anxiety. More on this can be found in this resource on repetitive behaviours.

Applied Behavioural Analysis (ABA) therapy is the most common behavioural therapy for autism. There is an additional section which can be accessed for information on ABA therapy. ABA is not the only therapy available; others include Naturalistic Developmental Behavioural Interventions (NDBIs), speech and language therapies, Developmental Individual differences Relationship based (DIR) therapies, and adapted Cognitive Behavioural Therapy (CBT). There are also separate sections with more detail available for Occupational Therapy and Speech and Language Therapy.

NDBIs are play-based approaches to therapy which combine the principles of ABA therapy with developmental and social approaches. It is often useful for young children as it can help to use their choices of activities to build communication, cognitive and social skills. From this, NDBI therapy can then move towards developing motivation, independence and responding to multiple cues. This may be a good choice for young children with FXS as they tend to be very socially motivated individuals.

DIR therapies focus on emotional connections and shared experiences to build skills, similar to those of NDBIs, but may be more suitable for older children, teenagers and adults. For example, if an individual expresses an interest in baking, it can be used to teach skills such following instructions, reading a recipe and sequential tasks using an activity which is enjoyable for everyone involved.

CBT for individuals with autism is adapted from traditional CBT. Traditional CBT is a talking therapy used to help understand feelings, thoughts and behaviours which can assist in managing anxiety, rigid thinking and emotional regulation. Traditional CBT may not be appropriate for individuals with autism due to them processing their surroundings differently to those without autism. People with autism can have difficulty identifying emotions (known as alexithymia) or have black and white (literal) thinking which is not necessarily compatible with traditional CBT. Adapted CBT includes sensory and emotional regulation, cognitive proprioception exercises and mindfulness to increase one’s understanding of their emotions and how they interact with their surroundings. This can be done through using clear and literal language, visual aids, role play and structured sessions to ensure an individual gets the most out of CBT.

Further Inform Neurogenetic Disorders have more information available on the autism spectrum.

Attention Deficit Hyperactivity Disorder (ADHD)

Overactivity and impulsivity can be features of FXS. These are commonly diagnosed as Attention Deficit Hyperactivity Disorder (ADHD). ADHD is often treated using medication, however behavioural therapies can be used alongside or independently of medication. In FXS, behavioural therapies should be tried first, as they are often highly effective, with medication used only as needed.

Most behavioural therapies for ADHD focus on building executive function skills and emotional regulation. Types of therapy suited to people with ADHD include adapted Cognitive Behavioural Therapy (CBT), Organisational Skills Training (OST) and social skills training.

Adapted CBT for people with ADHD focuses on managing executive function challenges and emotional regulation. CBT is suitable for teenagers and adults. It has different focuses compared to traditional CBT used for anxiety and depression. CBT can be used to help with procrastination, time management, lack of focus, impulsivity and frustration. The approaches to managing these traits include breaking large tasks into smaller steps, using planners and digital reminders effectively, reframing “all or nothing” thinking and building emotional resilience to mistakes. The goal of CBT for people with ADHD is teaching strategies to manage symptoms thereby reducing frustration.

OST focuses on building skills to tackle executive functioning issues. ADHD often results in an individual becoming overwhelmed due to not being able to process or manage multiple factors in their environment. This includes being disorganised, late, unfocussed or struggling to prioritise tasks. OST aims to teach individuals how to use checklists and calendars, break down tasks into smaller steps, prioritise tasks and prepare for transitions between activities.

Some people with ADHD can struggle in social situations due to the level of focus needed, understanding turn taking, avoiding interrupting and reading social cues. Social skills training is suitable for older children, young people and adults, and uses small group settings and role play. Individuals with Fragile X Syndrome (FXS) often imitate social behaviour and are highly socially motivated, therefore modelled social behaviour will likely be beneficial.

Further Inform Neurogenetic Disorders have more information available for Overactivity and Impulsivity in Fragile X Syndrome.

Анксиозност

People with Fragile X Syndrome (FXS) often have anxiety. This can include a range of types such as social anxiety, generalised anxiety and separation anxiety. Social anxiety is particularly common in females with FXS compared to males. Addressing anxiety can help someone with FXS flourish and their engaging, helpful and kind personality can shine through.

While many individuals with FXS are socially motivated, they often struggle in social situations. Generalised anxiety can show itself in many day-to-day activities. Often changes in routine or unfamiliar environments are likely to trigger feelings of anxiety. Individuals experiencing anxiety can be supported through behavioural therapies such as Cognitive Behavioural Therapy (CBT), exposure therapy, mindfulness and keeping predictable and consistent routines. Traditional CBT is usually used for people experiencing anxiety, however if an individual has autism or ADHD they may benefit from adapted CBT. Separation anxiety is most commonly seen in young children, but this may not always be the case. Explaining to an individual in an age appropriate manner before transitions occur and building tolerance to separation may help. There is also the option of medication, for example Selective Serotonin Reuptake Inhibitors (SSRIs).

More information is available from the National Fragile X Foundation on managing anxiety for people with FXS.

Депресија

Depression and low mood can impact some people with Fragile X Syndrome (FXS). There are multiple factors that can contribute to this, including anxiety, social isolation, low self-esteem, communication difficulties and chronic stress. Depression in people with FXS may not look the same as depression in the rest of the population. It may present itself as irritability, shutdowns, withdrawal from activities they usually enjoy, decreased engagement in usual routines, increased self-stimulatory behaviour (stimming) or self-soothing behaviours, repeated frustration and reduced attention or motivation. Addressing depression can help the person with FXS lead a full and happy life, allowing them to participate in activities and engage with the wider world.

Traditional CBT is usually used for people experiencing depression, however if an individual has autism or ADHD they may benefit from adapted CBT. Additionally, environmental support to reduce stressors and maintain autonomy will help a person feel more at ease. There is also the option of medication, for example Selective Serotonin Reuptake Inhibitors (SSRIs).

An image of a girl sitting on a sofa holding a teddy bear talking to a woman on a chair opposite holding a clipboard

Behaviour Which Challenges

A behaviour is challenging if it causes harm to the person or others, or if it stops them from fulfilling their desires/potential. Often it is the impact that makes the behaviour challenging (for example, unable to stay in school due to aggression). Examples of challenging behaviours include: self-injury, biting, damage to property, hair pulling and sexual behaviour/staring in public. Please also remember to build on the strengths of the person with FXS, as this increases self-esteem and is a productive way of enabling the individual to flourish.

There is always a reason for challenging behaviour. Often it will be because the person feels out of control and the behaviour results in their needs being met. The behaviour is their way of communicating they need something.

This section includes:

Types of behaviour which may be challenging

Here are some of the behaviours which may be challenging:

Self-Injury

Self-injurious behaviour may be seen in individuals with an intellectual disability, and can be difficult to manage and understand. Self-injurious behaviour refers to non-accidental behaviours initiated by the person which have the potential to be harmful. This typically includes head banging, hand biting and scratching, though this list is not exhaustive. Identifying the cause of the self injurious behaviour will assist in managing and attempting to decrease the behaviour. The PEACE acronym can assist with this:

  • P: Pain
  • E: Environment
  • A: Anxiety
  • C: Control
  • E: Exhaustion

Identifying whether one of the above might be the cause of the behaviour can help to reduce the likelihood or severity of the self-injurious behaviour. Individuals with an intellectual disability are likely to struggle with communication, leading to self-injurious behaviour. By managing the cause of the behaviour, it is less likely to occur. Cerebra have a more extensive guide to managing self injury.

Self-Harm

Self-harm is not the same as self injurious behaviour but can also be difficult to manage. Self-harm includes behaviours such as cutting, burning, skin picking and hair pulling. Self-harm is usually as a result of overwhelming feelings or experiences. This is different from self-injurious behaviour, where the motivation is usually something other than causing physical pain. Self-harm is more frequently seen in those with a less severe intellectual disability and therefore may be more common in females with Fragile X Syndrome. Helping a loved one who self-harms is difficult, but getting to the root of the cause can help, as well as treating any underlying mental health difficulties. More information on mental health struggles can be found in the sections on anxiety and depression, and more information on self harm can be found from Young Minds’ website section on self-harm.

Biting and Chewing

Biting and chewing is common behaviour in individuals with Fragile X Syndrome (FXS). This can include chewing on objects and biting themselves. Biting and chewing is not necessarily a challenging behaviour in itself if it is redirected to something safe, such as a teething ring or object specifically designed for chewing. Biting and chewing may be caused by a number of different reasons. These include sensory-seeking needs, self stimulatory behaviour (stimming), anxiety and frustration. As well as using objects specifically designed for biting and chewing, using straws for drinking or offering crunchy snacks may help to replace challenging behaviours associated with biting and chewing.

Emotional Outbursts

People with FXS sometimes find it difficult to control their emotions and behaviours in a way others find acceptable. Strong feelings can lead to emotional outbursts. Struggling to handle emotions is known as emotional dysregulation. Dysregulation can make it hard for them to manage their feelings, pose challenges in their friendships/relationships and make it tricky to focus on learning.

People with FXS may be more prone to emotional dysregulation as they feel their emotions more strongly, or equally may struggle to recognise their emotions (alexithymia). This means that situations others may not find distressing may be difficult for them. There are medical reasons for this — it is not them being ‘naughty’ or trying to be difficult.

Some signs of emotional dysregulation include: meltdowns (explosive reactions like anger or distress), shutdowns (becoming very quiet and unreactive), restlessness (finding it hard to sit still) or withdrawn behaviour (avoiding others or activities).

These behaviours are not about the person ‘misbehaving’ but is the person showing they are overwhelmed or finding it difficult to deal with their emotions. People with FXS can struggle to communicate verbally so their actions may be them showing you how they feel. When this happens it is important to remain calm and be understanding. Creating a safe environment helps the child to regulate and manage their emotions better. Try to offer empathetic responses and avoid punishment.

Although it can be difficult to manage emotional dysregulation, here are some strategies that may be helpful. Firstly, maintain regular routines and quiet spaces where possible. Predictable environments will reduce likelihood of outbursts. If they are having an outburst try to find a calming environment. Secondly, use of sensory tools/toys can help calm them down. For example, use weighted blankets or headphones. Additionally, you can encourage movement break activities or teach them breathing techniques (for example blowing bubbles or pretending to blow on a hot chocolate). Additionally, give them a tactile activity, for example playing with sand or sorting out beads. This gives them something to focus on and allows them to release energy.

More information is available from this resource on Helping Children With Dysregulation.

Aggressive Behaviour/Hurting Others

We all make mistakes growing up, but for those with additional needs this can be harder to navigate and resolve. Aggressive behaviour is often a method of communicating something is wrong and can stem from frustration. Strong feelings and outbursts can sometimes result in a person being hurt or objects being broken.

For people with Fragile X Syndrome (FXS), upon calming down they are usually very apologetic and remorseful for the hurt or damage they have caused. Depending on the individual involved it may be appropriate to help them to resolve the outcome of their behaviour. For example, explain that they have hurt someone and that an apology is the correct next step, or have them assist in cleaning up any damage. Be clear on what is right and wrong, using the situation as a learning opportunity. Threats and punishment do not lead to a change of behaviour or de-escalation of the situation.

It can be difficult to identify what causes aggressive behaviour, but figuring out what is causing the behaviour can help to determine how to manage it. Reducing triggers for the behaviour can help to reduce the behaviour itself.

Another method which may work is to redirect the behaviour if it is safe to do so. For example, if an individual is prone to hitting, giving them soft pillows or a punching bag can help to reduce harm caused to objects or other people.

Sexually Inappropriate Behaviour

Please see the separate section on Сексуалност, which includes discussion on sexual behaviours which may be inappropriate or challenging. These may include repeating sexualised language, sexual staring, obsession with others’ body parts, self-stimulation in public and sexual touching of others without consent.

Figuring out what is causing a behaviour

Cerebra have a Behaviour Checklist which may be a useful tool.

Below are some potential causes of challenging behaviours. This is not an exhaustive list, but it can be useful to think about why your loved one is exhibiting these behaviours. Understanding the trigger for the behaviour can help with managing challenging behaviours and de-escalation. 

Pain

Pain is easy to overlook. Your loved one may not be able to tell you they are in pain and it may not be obvious (for example, constipation or ear infections), so it’s important to consider all signs. Signs to look out for include: facial expressions, crying, unable to be still, changes in eating or sleeping patterns. Pain can cause both physical and emotional stress so it is important to consider if it could be a relevant factor. There is more information available on pain in people with FXS.

Sensory Avoidance

People with Fragile X Syndrome (FXS) may get overwhelmed by excess sensory inputs (for example, loud noises) or specific senses they find disturbing (for example, a flashing light). This can result in them exhibiting anxious behaviours. Signs to look out for include: moving away from specific noises/lights/textures, seeking specific senses (for example, hugs), over-fidgeting or other signs of agitation. Please see the section on ‘Sensory Processing’.

Meeting Needs

Often challenging behaviours can be a method of the loved one getting what they need. For example, if the individual wants 1:1 attention, they may have learned that when they wet themselves someone helps them change their clothes, and so they use wetting themselves to get what they need, the 1:1 attention (please note that with FXS, wetting can also be caused by muscle laxity).

Another example of meeting their needs could be: the individual knows that if they hit someone they get put in the corridor, so they may use hitting someone as a means of escape from an overwhelming situation. Some behaviours are linked to a desire for certain items or things such as food or a toy. The behaviour is a sign of them wanting this item.

Анксиозност

Anxiety can affect your loved one’s sleep, cause low mood or make them feel uncomfortable. This can result in them exhibiting challenging behaviours. The behaviour may be triggered by a stressful event or situation. If you know the trigger it may be useful to address that; if not, general relaxation techniques may be helpful. Signs to look out for include: avoiding situations or events, clinging to another person, appearing tense, crying or showing other signs of distress. Please note general anxiety and social anxiety can be co-occurring with FXS. Your doctor can advise on treatment for anxiety. There is more info above in the Анксиозност section.

Low Mood

Low mood may cause behaviour which challenges. Low mood can have many causes, such as pain, discomfort and anxiety. If this is the case, you can try to change the environment or activity and do something the person enjoys to try to raise their mood. This may improve their behaviour.

Signs to look out for related to low mood include: avoiding an activity or people, low levels of interest in things, a lack of smiling or laughing, or crying. Depression can be a co-occurring condition of FXS, please seek advice from a professional if you suspect depression. There is more information above in the section on Депресија.

Sleep Difficulties

Sleep is crucial for us all. If your loved one is tired this may be the cause of their behaviour. There may be underlying reasons for the sleep difficulty, for example, anxiety, pain or discomfort (for more information, please look at our information on sleep). Signs to look out for include: waking during the night, not sleeping for very long, needing to sleep with parents, or being tired during the day.

Change

Change is tricky for us all, but particularly for people drawn to routine such as those with FXS. Consider if there have been any big life changes for the person with FXS. They may need support to work through this change. Try to talk with your loved one about any recent changes and encourage them to communicate their feelings about it.

More information is available on causes of challenging behaviour.

Ресурси

Managing behaviour which challenges

Overview

In managing behaviours which challenge, please consider these questions:

  • Could the behaviour be evidence of a health problem (for example, toothache or sight issues)?
  • What is the person trying to say through this behaviour?
  • Investigate the behaviour: if you change something, does this stop the behaviour?
  • Is there a pattern to the behaviour? (It may be useful to keep a record of events)

There is no quick fix, however it can be useful to figure out what is causing the behaviour (see figuring out what is causing a behaviour).

Try to develop communication skills by encouraging and teaching the person with FXS to show or tell you what they want. Behaviours can often stem from frustration and the inability to communicate a need or feeling. Working on communication skills is a positive way to address challenging behaviour.

Encourage your loved one to talk to you through the difficult situation so they develop the skill of communicating their feelings and needs. This is key as they will inevitably end up in situations where their behaviour is not understood and communicating what they need is required. Developing this skill will take time and patience, it will only come gradually, but all improvements help the individual. Be aware that communication can also be linked to shyness and not wishing to bother someone. Being clear about when it is appropriate to speak out about one’s needs and giving guidance on self-advocacy can be empowering.

After an incident, when things have calmed down, talk to your loved one about what happened before the behaviour, the behaviour itself and the result of the behaviour (Antecedent – Behaviour – Consequence). See if anything could explain why the behaviour occurred, and address these triggers to help reduce future issues. Please see the section on ABA Therapy for further information on this technique.

Preventing challenging behaviour can be difficult, but if you understand why the behaviour is occurring steps can be taken to try to prevent it. Think of when the behaviour is most likely to happen and the triggers, for example: at the supermarket, with bright lights. Then try to approach the situation calmly, slowly and avoid triggers when possible (for example, use sunglasses). You can talk your loved one through the situation and keep an eye out for when they start getting distressed. As they get more comfortable, you may try to encourage them to be more independent by not offering as much reassurance and letting them have time to take in the environment/situation. It is okay to lower your expectations and to let go of something or even choose unconventional options to prevent challenging behaviour, even though it is against your intuitive parenting.

If your loved one is showing serious challenging behaviour, please reach out for help. Challenging behaviours can be difficult to deal with, and you do not need to do so alone. Especially reach out for help if you think there could be an underlying health issue, there is a sudden decline in behaviour, they are hurting themselves or others, or you are finding it difficult to cope.

Please also see the Challenging Behaviour quick guide.

Positive Behaviour Support

One way to combat challenging behaviour is to focus on positive behaviour. The idea is that by teaching a more appropriate behaviour, the challenging behaviour will subside. To support better behaviours you need to accept there is nothing wrong with your loved one wanting attention or to escape a situation, but rather teach your loved one to use different strategies to get these. Positive behaviour support aims to understand the reasons behind the behaviour and give the individual skills to communicate more effectively while still meeting their needs.

A positive behaviour support plan can be used for all ages and means everyone consistently uses the same techniques and understands the individual’s goals. The plan can be used in all settings including home, school, day services, friend’s houses or in the community. Ideally, the plan will be based on a functional assessment which is carried out by a psychologist or behavioural specialist. A functional assessment aims to understand the behaviour and give ideas on how to reduce the challenging behaviour. This can take a while so it may be useful to try to start a plan yourself, below is a guide.

Creating a Positive Behaviour Plan

The Challenging Behaviour Foundation has come up with 8 steps to help create a plan, these are summarised below. They have their own resource on example plans.

  1. Think about the behaviour you want to focus on. Give it a name, describe what it looks like, record the frequency, severity and duration.
  2. Why is the behaviour occurring? Examples include: to gain attention, to escape something, to get an object or to fulfil sensory needs. Use this to teach the individual they can get what they want by other means. For example, if they need attention they can tap your arm.
  3. Be proactive. Keep calm and keep the person with FXS calm and content, therefore reducing the chances of challenging behaviours occurring. To do this you can try to make a list of things they enjoy and try incorporating these daily, for example: colouring, baking or talking about their interests. Other tips include: praising them and rewarding positive behaviour, changing the environment to suit them, making sure they have a good diet and exercise, and maintaining a routine.
  4. Write down warning signs. Despite behaviours seeming spontaneous there can often be subtle signs, for example: sweating, facial expressions, increased heartbeat or pacing. Once these have been identified, think of ways to help the individual become calm, for example: taking away the trigger, using humour, changing the situation or giving them sensory aids.
  5. Plan for when things get difficult. It is impossible to prevent all challenging behaviour so it is good to have a plan of what to do when things get tricky. Firstly, try to appear calm yourself and ideally get them into a calm environment (this isn’t always possible and that’s ok). Try to not overwhelm them — what works will be specific to them. For example, consider touch (hard touches or no contact?), noise (music or noise cancelling headphones?) and distraction (walking or talking?). It may take experimenting to see what works best for the individual.
  6. Rest and reset. After a challenging behaviour has subsided, it is important to give the individual space and time to reset. Allow them time, and don’t make demands of them. If you were not able to change the environment before, try to do so now. Check those around/involved are okay. Try to engage them in an activity that is enjoyable and comforting.
  7. Agree the Positive Behaviour Plan. Inform all those involved in the care of your loved one, whether care support, teachers, other family members or friends, so that they know what to do. Make sure they understand the Positive Behaviour Plan, and take into consideration any suggestions they have for improving the plan.
  8. Keep updating and adding to the plan. Behaviours and situations can constantly change. Keep the plan up to date and in alignment with the individual’s current coals.

Applied Behavioural Analysis (ABA) Therapy

Applied Behavioural Analysis (ABA) therapy is a therapeutic approach to changing behaviour. Its aim is to use consequences (positive or negative) to influence an increase in desirable behaviour or a decrease in undesirable behaviour by linking action and reaction.

ABA therapy works by breaking down skills into small, teachable steps and using reinforcement to encourage desired behaviours. For example a child is shown/told how to do something, upon the child doing it they are rewarded. This links the action and the response, with repetition used to reinforce the desired behaviour and increase the frequency of the desired behaviour occurring.

The key techniques used in ABA are:

  • Positive reinforcement (desired behaviour leads to reward)
  • Environmental based teaching (using real life situations to teach behaviours)
  • Prompting and fading (reducing the amount instructions are given over time to encourage independence)
  • Step by step (breaking behaviours into smaller steps to aid understanding)

ABA therapy is primarily an individualised programme adapted for every person. It is usually designed and supervised by behavioural analysts and delivered by trained therapists. This makes ABA a versatile form of therapy as it can cover many areas of life including communication skills, self care and life skills.

While ABA therapy has many benefits it has had a controversial history. Early forms of ABA often used punishment based methods rather than positive reinforcement. While modern ABA has predominantly moved on from this, many peoples’ opinions are still negative towards ABA associated therapies, particularly due to the previous focus on making an individual compliant rather than supporting them and allowing autonomy.

While modern ABA usually focuses on person based approaches and meeting the needs of an individual it is important to discuss any concerns with a professional.

Further information:
Cleveland Clinic Applied Behavior Analysis
Raising Children Applied Behaviour Analysis (ABA) and autistic children

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