Behavioral Intervention Applications in Rare Disease: A Clinical Model of Care in Batten Disease (126805)
Charles Albright
1
,
Jessica Scherr
1
,
Emily de los Reyes
1
- Nationwide Children's Hospital, Columbus, OH, United States
- Batten disease is a rare, neurodegenerative disease that is fatal. Children with Batten disease are at increased risk for behavioral challenges that further reduce the quality of life for that individual, as well as their family. Evidence-based behavioral interventions, such as parent training programs and Applied Behavior Analysis (ABA), should be considered in the clinical model of care for children and families with Batten disease that are experiencing co-occurring behavioral challenges. Behavioral parent training is a well-known, evidence-based intervention for treating disruptive behavior in children, including those with neurodevelopmental disabilities. Goals of behavior intervention are often individualized to the child and family’s needs but often target decreasing behavioral challenges and increasing adaptive behavior, developmental functioning, and communication skills. The current presentation will focus on reviewing (1) symptom presentation, co-occurring behavioral challenges, and model of clinical care within Batten disease; (2) types of evidence-based behavior intervention including ABA therapy and parent training models; (3) content areas within behavioral intervention, including functions of behavior, antecedent management, consequence-based strategies, etc.; and (4) strategies on how to adapt behavioral intervention within Batten disease to promote skill acquisition and maintenance in functional communication, adaptive behavior, and developmental functioning. Future directions will be discussed on how behavioral intervention can help improve quality of life, enhance developmental, adaptive, and behavioral functioning, and inform a clinical model of care in individuals and families Batten disease and other forms of rare disease.