How should I approach the management of behavioral issues commonly associated with Fragile X Syndrome in my practice?

Approach to managing behavioral issues in Fragile X Syndrome (FXS) in clinical practice:

• Recognise that behavioral challenges in FXS often overlap with autism spectrum disorder (ASD) features; therefore, management strategies for ASD are applicable.

• Begin with a comprehensive assessment to identify factors that may trigger or maintain challenging behaviour, including communication difficulties, coexisting physical or mental health conditions (such as anxiety or ADHD), environmental factors, changes in routine, and developmental changes like puberty.

• Develop a personalised care plan with the child or young person and their family or carers that addresses identified triggers, including treatment of coexisting conditions, environmental adjustments to increase predictability and structure, and support for families and carers.

• Use psychosocial interventions as first-line treatment for behaviour that challenges, informed by a functional assessment of behaviour to identify triggers, patterns, needs met by the behaviour, and consequences reinforcing it.

• Ensure psychosocial interventions have clearly identified target behaviours, focus on quality of life outcomes, modify environmental factors, are developmentally appropriate, have specified timescales, use systematic measurement of behaviour, and are consistently applied across settings with agreement among all involved.

• Consider pharmacological interventions, such as antipsychotic medication, only if psychosocial or other interventions are insufficient or cannot be delivered due to severity of behaviour, and ensure prescribing is initiated and monitored by specialists with clear guidance for primary care.

• Regularly review interventions for effectiveness, side effects, and adherence, adjusting the care plan as needed.

This approach emphasises a multidisciplinary, personalised, and evidence-based management plan tailored to the individual’s developmental level and coexisting conditions, with a focus on non-pharmacological strategies first and cautious use of medication when necessary.