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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

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.

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References

  1. Angelman, H. (1965). “Puppet” children: A report of three cases. Developmental Medicine & Child Neurology, 7, 681–688.

    Article  Google Scholar 

  2. Barlow, D. H., Nock, M., and Hersen, M. (2009). Single-case experimental designs: Strategies for studying behavior change (3rd ed.). New York: Allyn & Bacon.

  3. Boot, F. H., Owuor, J., Dinsmore, J., & Maclachlan, M. (2018). Access to assistive technology for people with intellectual disabilities: A systematic review to identify barriers and facilitators. Journal of Intellectual Disability Research, 62, 900–921.

    PubMed  Article  Google Scholar 

  4. Buiting, K., Williams, C., & Horsthemke, B. (2016). Angelman syndrome-insights into a rare neurogenetic disorder. Nature Reviews Neurology, 12, 584–593.

    PubMed  Article  Google Scholar 

  5. Catania, A. C. (2012). Learning (5th ed.). New York: Sloan.

    Google Scholar 

  6. Chadha, S., Moussy, F., & Friede, M. H. (2014). Understanding history, philanthropy and the role of WHO in provision of assistive technologies for hearing loss. Disability and Rehabilitation: Assistive Technology, 9, 365–367.

    PubMed  Google Scholar 

  7. Chadwick, D. D., & Platt, T. (2018). Investigating humor in social interaction in people with intellectual disabilities: A systematic review of the literature. Frontiers in Psychology, 9(SEP). DOI: https://doi.org/10.3389/fpsyg.2018.01745.

  8. Crawford, M. R., & Schuster, J. W. (1993). Using microswitches to teach toy use. Journal of Developmental and Physical Disabilities, 5, 349–368.

    Article  Google Scholar 

  9. Egan, M., Farrell, K., Hoey, E., McGuire, B. E., & Lydon, H. K. (2020). Interventions to improve sleep for individuals with angelman syndrome: A systematic review. Research in Developmental Disabilities, 97, 103554. https://doi.org/10.1016/j.ridd.2019.103554.

    Article  PubMed  Google Scholar 

  10. Falcomata, T. S., & Lang, R. (2013). Introduction to special issue: Challenging behavior and individuals with developmental and physical disabilities. Journal of Developmental and Physical Disabilities, 25, 1–4. https://doi.org/10.1007/s10882-012-9328-7.

  11. Fisher, K., Keng, J., & Ziegler, J. (2020). Nutrition assessment and intervention in a pediatric patient with Angelman syndrome: A case presentation highlighting clinical challenges and evidence-based solutions. Lifestyle Genomics, 13, 43–52.

    PubMed  Article  Google Scholar 

  12. Goswami, J. N., Sahu, J. K., & Singhi, P. (2018). Angelman syndrome due to UBE3A gene mutation. Indian Journal of Pediatrics, 85, 390–391.

    PubMed  Article  Google Scholar 

  13. Hamrick, L. R., & Tonnsen, B. L. (2019). Validating and applying the CSBS-ITC in neurogenetic syndromes. American Journal on Intellectual and Developmental Disabilities, 124, 263–285.

    PubMed  Article  Google Scholar 

  14. Heald, M., Allen, D., Villa, D., & Oliver, C. (2013). Discrimination training reduces high rate social approach behaviors in Angelman syndrome: Proof of principle. Research in Developmental Disabilities, 34, 1794–1803.

    PubMed  Article  Google Scholar 

  15. Hoppenbrouwers, G., Stewart, H., & Kernot, J. (2014). Assistive technology assessment tools for assessing switch use of children: A systematic review and descriptive analysis. Technology and Disability, 26, 171–185.

    Article  Google Scholar 

  16. Kaskowitz, A. P., Dendrinos, M., Murray, P. J., Quint, E. H., & Ernst, S. (2016). The effect of menstrual issues on young women with Angelman syndrome. Journal of Pediatric and Adolescent Gynecology, 29, 348–352.

    PubMed  Article  Google Scholar 

  17. Kazdin, A. E. (2001). Behavior modification in applied settings (6th ed.). New York: Wadsworth.

    Google Scholar 

  18. Kennedy, K. (2005). Single case designs for educational research. New York: Allyn & Bacon.

    Google Scholar 

  19. Lancioni, G. E., & Singh, N. N. (2014). Assistive technologies for persons with diverse abilities. New York: Springer.

    Book  Google Scholar 

  20. Lancioni, G. E., O'Reilly, M. F., Singh, N. N., Stasolla, F., Manfredi, F., & Oliva, D. (2004). Adapting a grid into a microswitch to suit simple hand movements of a child with profound multiple disabilities. Perceptual and Motor Skills, 99, 724–728.

    PubMed  Article  Google Scholar 

  21. Lancioni, G. E., Comes, M. L., Stasolla, F., Manfredi, F., O'Reilly, M. F., & Singh, N. N. (2005a). A microswitch cluster to enhance arm-lifting responses without dystonic head tilting by a child with multiple disabilities. Perceptual and Motor Skills, 100, 892–894.

    PubMed  Article  Google Scholar 

  22. Lancioni, G. E., O’Reilly, M. F., Singh, N. N., Oliva, D., Scalini, L., Vigo, C. M., & Groeneweg, J. (2005b). Further evaluation of microswitch clusters to enhance hand response and head control in persons with multiple disabilities. Perceptual and Motor Skills, 100, 689–694.

    PubMed  Article  Google Scholar 

  23. Lancioni, G. E., O'Reilly, M. F., Singh, N. N., Sigafoos, J., Oliva, D., Baccani, S., & Groeneweg, J. (2006). Microswitch clusters promote adaptive responses and reduce finger mouthing in a boy with multiple disabilities. Behavior Modification, 30, 892–900.

    PubMed  Article  Google Scholar 

  24. Lancioni, G. E., Singh, N. N., O'Reilly, M. F., Sigafoos, J., Chiapparino, C., Stasolla, F., & Oliva, D. (2007a). Using an optic sensor and a scanning keyboard emulator to facilitate writing by persons with pervasive motor disabilities. Journal of Developmental and Physical Disabilities, 19, 593–603.

    Article  Google Scholar 

  25. Lancioni, G. E., Singh, N. N., O'Reilly, M. F., Sigafoos, J., Oliva, D., Pidala, S., Piazzolla, G., & Bosco, A. (2007b). Promoting adaptive foot movements and reducing hand mouthing and eye poking in a boy with multiple disabilities through microswitch technology. Cognitive Behavior Therapy, 36, 85–90.

    Article  Google Scholar 

  26. Lancioni, G. E., Smaldone, A., O'Reilly, M. F., Singh, N. N., Sigafoos, J., Oliva, D., & Bosco, A. (2007c). Promoting adaptive hand responding and reducing face hiding in a woman with profound developmental disabilities using microswitch technology. Behavioral and Cognitive Psychotherapy, 35, 225–230.

    Article  Google Scholar 

  27. Lancioni, G. E., Singh, N. N., O'Reilly, M. F., Sigafoos, J., Didden, R., Smaldone, A., & Oliva, D. (2008). Helping a man with multiple disabilities increase object-contact responses and reduce hand stereotypy via a microswitch cluster program. Journal of Intellectual and Developmental Disability, 33, 349–353.

    PubMed  Article  Google Scholar 

  28. Lancioni, G. E., Singh, N. N., O'Reilly, M. F., & Sigafoos, J. (2009a). An overview of behavioral strategies for reducing hand-related stereotypies of persons with severe to profound intellectual and multiple disabilities: 1995-2007. Research in Developmental Disabilities, 30, 20–43.

    PubMed  Article  Google Scholar 

  29. Lancioni, G. E., Singh, N. N., O'Reilly, M. F., Sigafoos, J., Didden, R., & Oliva, D. (2009b). Two boys with multiple disabilities increasing adaptive responding and curbing dystonic/spastic behavior via a microswitch-based program. Research in Developmental Disabilities, 30, 378–385.

    PubMed  Article  Google Scholar 

  30. Lancioni, G., O'Reilly, M., Singh, N., D'Amico, F., Ricci, I., & Buonocunto, F. (2011a). Microswitchcluster technology to enhance adaptive engagement and head upright by a post-coma man with multiple disabilities. Developmental Neurorehabilitation, 14, 60–64.

    PubMed  Article  Google Scholar 

  31. Lancioni, G. E., Singh, N. N., O’Reilly, M. F., & Sigafoos, J. (2011b). Assistive technology for behavioral interventions for persons with severe/profound multiple disabilities: A selective overview. European Journal of Behavior Analysis, 12, 7–26.

    Article  Google Scholar 

  32. Lancioni, G. E., Sigafoos, J., O’Reilly, M. F., & Singh, N. N. (2012). Assistive technology: Interventions for individuals with severe/profound and multiple disabilities. New York: Springer.

    Google Scholar 

  33. Lancioni, G. E., O'Reilly, M. F., Singh, N. N., Sigafoos, J., Oliva, D., Alberti, G., et al. (2013). Technology-based programs to support adaptive responding and reduce hand mouthing in two persons with multiple disabilities. Journal of Developmental and Physical Disabilities, 25, 65–77.

    Article  Google Scholar 

  34. Lancioni, G. E., Singh, N. N., O’Reilly, M. F., Sigafoos, J., & Oliva, D. (2014). Intervention programs based on microswitch technology for persons with multiple disabilities: An overview. Current Developmental Disorders Reports, 1, 67–73.

    Article  Google Scholar 

  35. Micheletti, S., Palestra, F., Martelli, P., Accorsi, P., Galli, J., Giordano, L., Trebeschi, V., & Fazzi, E. (2016). Neurodevelopmental profile in angelman syndrome: More than low intelligence quotient. Italian Journal of Pediatrics, 42, 91. https://doi.org/10.1186/s13052-016-0301-4.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Parker, R. I., Vannest, K. J., Davis, J. L., & Sauber, S. B. (2011). Combining nonoverlap and trend for single-case research: Tau-U. Behavior Therapy, 42, 284–299. https://doi.org/10.1016/j.beth.2010.08.006.

    Article  PubMed  Google Scholar 

  37. Perilli, V., Stasolla, F., Caffò, A. O., Albano, V., & D’Amico, F. (2019). Microswitch-cluster technology for promoting occupation and reducing hand biting of six adolescents with fragile X syndrome: New evidence and social rating. Journal of Developmental and Physical Disabilities, 31, 115–133.

    Article  Google Scholar 

  38. Squires, L. A., Williams, N., & Morrison, V. L. (2019). Matching and accepting assistive technology in multiple sclerosis: A focus group study with people with multiple sclerosis, careers and occupational therapists. Journal of Health Psychology, 24, 480–494.

    PubMed  Article  Google Scholar 

  39. Stasolla, F., & Perilli, V. (2015). Microswitch-based programs (MBP) to promote communication, occupation, and leisure skills for children with multiple disabilities: A literature overview. In N. R. Silton (Ed.), Recent advances in assistive technologies to support children with developmental disorders (pp. 195–216). Hershey (PA): IGI Global.

    Chapter  Google Scholar 

  40. Stasolla, F., & De Pace, C. (2014). Assistive technology to promote leisure and constructive engagement by two boys emerged from a minimal conscious state. NeuroRehabilitation, 35, 253–259.

    PubMed  Article  Google Scholar 

  41. Stasolla, F., Boccasini, A., Perilli, V., Caffò, A. O., Damiani, R., & Albano, V. (2014a). A selective overview of microswitch-based programs for promoting adaptive behaviors of children with developmental disabilities. International Journal of Ambient Computing and Intelligence, 6, 56–74.

    Article  Google Scholar 

  42. Stasolla, F., Damiani, R., Perilli, V., Di Leone, A., Albano, V., Stella, A., & Damato, C. (2014b). Technological supports to promote choice opportunities by two children with fragile X syndrome and severe to profound developmental disabilities. Research in Developmental Disabilities, 35, 2993–3000.

    PubMed  Article  Google Scholar 

  43. Stasolla, F., Perilli, V., Damiani, R., Caffò, A. O., Di Leone, A., Albano, V., et al. (2014c). A microswitch-cluster program to enhance object manipulation and to reduce hand mouthing by three boys with autism spectrum disorders and intellectual disabilities. Research in Autism Spectrum Disorders, 8, 1071–1078.

    Article  Google Scholar 

  44. Stasolla, F., Damiani, R., Perilli, V., D'Amico, F., Caffò, A. O., Stella, A., et al. (2015). Computer and microswitch-based programs to improve academic activities by six children with cerebral palsy. Research in Developmental Disabilities, 45-46, 1–13.

    PubMed  Article  Google Scholar 

  45. Stasolla, F., Perilli, V., & Boccasini, A. (2016). Assistive technologies for persons with severe-profound intellectual and developmental disabilities. In J. K. Luiselli & A. J. Fisher (Eds.), Computer-assisted and web-based innovations in psychology, special education, and health (pp. 287–310). Amsterdam: Elsevier.

    Chapter  Google Scholar 

  46. Stasolla, F., Caffò, A. O., Perilli, V., Boccasini, A., Stella, A., Damiani, R., Albano, V., & Damato, C. (2017a). A microswitch-based program for promoting initial ambulation responses: An evaluation with two girls with multiple disabilities. Journal of Applied Behavior Analysis, 50, 345–356.

    PubMed  Article  Google Scholar 

  47. Stasolla, F., Perilli, V., Caffò, A. O., Boccasini, A., Stella, A., Damiani, R., & Albano, A. (2017b). Extending microswitch-cluster programs to promote occupation activities and reduce mouthing by six children with autism spectrum disorders and intellectual disabilities. Journal of Developmental and Physical Disabilities, 29, 307–324.

    Article  Google Scholar 

  48. Stasolla, F., Caffò, A. O., Perilli, V., & Albano, V. (2019a). Experimental examination and social validation of a microswitch intervention to improve choice-making and activity engagement for six girls with Rett syndrome. Developmental Neurorehabilitation, 22, 527–541.

    PubMed  Article  Google Scholar 

  49. Stasolla, F., Caffò, A. O., Perilli, V., & Albano, V. (2019b). Supporting locomotion fluency of six children with Cornelia de Lange syndrome: Awareness of microswitch responding and social validation. Technology and Disability, 30(4), 209–220.

    Article  Google Scholar 

  50. Stasolla, F., Caffò, A. O., Perilli, V., Boccasini, A., Damiani, R., & D'Amico, F. (2019c). Assistive technology for promoting adaptive skills of children with cerebral palsy: Ten cases evaluation. Disability and Rehabilitation: Assistive Technology, 14, 489–502.

    PubMed  Google Scholar 

  51. Trickett, J., Oliver, C., Heald, M., Denyer, H., Surtees, A., Clarkson, E., Gringras P. Richards, C. (2019). Multi-method assessment of sleep in children with Angelman syndrome: A Case–Controlled study. Frontiers in Psychiatry, 10. DOI: https://doi.org/10.3389/fpsyt.2019.00874, Multi-Method Assessment of Sleep in Children With Angelman Syndrome: A Case–Controlled Study, 10.

  52. Wheeler, A. C., Sacco, P., & Cabo, R. (2017). Unmet clinical needs and burden in angelman syndrome: A review of the literature. Orphanet Journal of Rare Diseases, 12(1), 164. https://doi.org/10.1186/s13023-017-0716-z.

    Article  PubMed  PubMed Central  Google Scholar 

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Correspondence to Fabrizio Stasolla.

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Stasolla, F., Caffò, A.O., Ciarmoli, D. et al. Promoting Object Manipulation and Reducing Tongue Protrusion in Seven Children with Angelman Syndrome and Developmental Disabilities through Microswitch-Cluster Technology: a Research Extension. J Dev Phys Disabil 33, 799–817 (2021). https://doi.org/10.1007/s10882-020-09774-6

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Keywords

  • Angelman syndrome
  • Microswitch-cluster technology
  • Positive participation
  • Social validation
  • Quality of life
  • Challenging behavior