Behaviours and Anxiety in Angelman Syndrome

Quick Overview

The presentation at the 2024 ASF Family Conference focused on managing behaviours and anxiety in individuals with Angelman Syndrome. Dr. Christopher Keary and Dr. Cesar Ochoa-Lubinoff discussed the prevalence of behavioural challenges such as hyperactivity, attention issues, and anxiety, which are common across all ages in those with Angelman Syndrome. They emphasised the importance of understanding the antecedents and consequences of behaviours to develop effective treatment plans. The speakers highlighted the role of medical issues like sleep disturbances, constipation, and reflux in exacerbating behavioural problems. They also discussed the potential use of medications like Buspirone for anxiety and guanfacine for hyperactivity, while cautioning about the side effects of more potent drugs. The session underscored the need for a comprehensive approach, combining behavioural strategies, medical evaluations, and, when necessary, pharmacotherapy, to improve the quality of life for individuals with Angelman Syndrome and their families.

The 2024 ASF Family Conference featured an insightful talk by Dr. Christopher Keary and Dr. Cesar Ochoa-Lubinoff, focusing on the complex interplay of behaviours and anxiety in individuals with Angelman Syndrome. The session was introduced by Courtney Swafford, a parent of a child with the UPD subtype of Angelman Syndrome, who shared personal anecdotes about the behavioural challenges faced by her son, Aaron.

Understanding Behavioural Challenges

Dr. Keary, a child and adolescent psychiatrist, began by discussing the prevalence of behavioural issues in individuals with Angelman Syndrome. He highlighted that these challenges often manifest early in life and can become more pronounced as individuals age. Common behaviours include hyperactivity, short attention spans, and aggressive actions such as biting and hair-pulling. These behaviours can hinder participation in therapies and community activities, making it crucial to address them effectively.

Chain Analysis and Functional Behavioural Assessment

Dr. Keary emphasised the importance of understanding the antecedents, behaviours, and consequences through a process known as chain analysis or functional behavioural assessment. This approach helps caregivers and professionals identify the underlying causes of behaviours, which is essential for developing effective treatment plans. He provided examples, such as a child pulling hair to avoid sitting in a circle, illustrating how understanding the context can lead to better management strategies.

Hidden Medical Concerns

Dr. Ochoa-Lubinoff, Director of Behavioural Paediatrics at Mount Sinai, discussed the potential medical contributors to behavioural issues. Conditions like insufficient sleep, constipation, gastroesophageal reflux, and dental problems can exacerbate behavioural challenges. Identifying and addressing these medical issues is a critical first step before implementing behavioural interventions.

Anxiety in Angelman Syndrome

Anxiety is a common concern in individuals with Angelman Syndrome, often presenting as irritability, agitation, and aggressive behaviours. Dr. Ochoa-Lubinoff noted that separation anxiety is a frequent trigger, and managing it requires a combination of behavioural strategies and, in some cases, medication. He discussed the use of medications like Buspirone and the challenges of using traditional anxiety treatments due to sensitivity in Angelman patients.

Case Studies and Practical Approaches

The session included case studies illustrating various behavioural and anxiety-related challenges. For instance, a 20-year-old woman with difficulty separating from her mother exhibited aggressive behaviours when anxious. The doctors recommended a comprehensive approach, including medical evaluations, occupational therapy, and consistent routines to manage anxiety.

Pharmacological Interventions

While behavioural interventions are preferred, the speakers acknowledged that severe cases might require pharmacological solutions. They discussed the cautious use of medications like atypical antipsychotics and antiepileptic drugs, emphasising the need for careful monitoring due to potential side effects.

Conclusion

The talk concluded with a Q&A session, where parents shared their experiences and sought advice on managing specific behaviours. The speakers reiterated the importance of understanding the individual needs of each person with Angelman Syndrome and working closely with experienced professionals to find effective solutions.

This session provided valuable insights into the behavioural and anxiety challenges faced by individuals with Angelman Syndrome, offering hope and practical strategies for families navigating these complex issues.