Promoting Object Manipulation and Reducing Tongue Protrusion in Seven Children with Angelman Syndrome and Developmental
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Promoting Object Manipulation and Reducing Tongue Protrusion in Seven Children with Angelman Syndrome and Developmental Disabilities through Microswitch-Cluster Technology: a Research Extension Fabrizio Stasolla 1 Vincenza Albano 2
& Alessandro
O. Caffò 2 & Donatella Ciarmoli 1 &
Accepted: 5 October 2020/ # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract We further extended the use of a microswitch-cluster technology to promote object manipulation and to reduce tongue protrusion in seven children with Angelman syndrome. Study I included seven participants with severe to profound developmental disabilities. An ABB1AB1 experimental sequence was implemented. During the baselines (i.e., A phases) the technology was available but inactive. During the intervention (i.e., B phase) the adaptive responding was positively reinforced irrespective of the challenging behavior. During the cluster (i.e., B1 phases) the adaptive responding was contingently reinforced only if it occurred free of the challenging behavior. A long-term follow-up (i.e., 24 months) was conducted. Intervals with indices of positive participation as an outcome measure of the participants’ constructive engagement and favorable occupation were additionally recorded. Study II recruited 56 external raters (i.e., equally divided in 4 groups among caregivers, physiotherapists, psychologists, and teachers) in a social validation procedure. Results evidenced the effectiveness and the suitability of the technology to pursue the dual goal (i.e., increasing the adaptive responding and simultaneously decreasing the challenging behavior). All the participants consolidated their learning process and positively participated along the intervention phases. Social raters favorably scored the use of the technology. Educational, clinical, psychological, and rehabilitative implications of the findings were critically discussed. Some useful insights for future research and practice were emphasized. * Fabrizio Stasolla [email protected]; [email protected]
1
Università Giustino Fortunato Benevento, Benevento, Italy
2
Department of Educational Sciences, Psychology, Communication, University “Aldo Moro” of Bari, Bari, Italy
Journal of Developmental and Physical Disabilities
Keywords Angelman syndrome . Microswitch-cluster technology . Positive participation .
Social validation . Quality of life . Challenging behavior
Introduction Angelman syndrome (AS) is a genetic and neurological disorder mainly caused by a deletion in the long arm of the 15th maternal chromosome, first described by the British pediatrician Angelman (1965). Because three of his patients evidenced excessive laughing, jerky movements, severe to profound developmental delays, and flat head disorder associated with physical development abnormalities, they were called “puppet children”. Additionally, the E6-AP (i.e., E6 protein encoded by the UBE3A gene) seems to be responsible for the genetic AS (Fisher et al. 2020). The phenotype usually includes ataxia, seizures
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