• Amrinder Babbra, M.S.

ABA and Angelman Syndrome

Amrinder Babbra, M.S. - Director of Behavioral Research


Photo by David Straight

B.F. Skinner envisioned a science of behavior that would solve the problems of humanity by effectively understanding how to deal with behavior. In Beyond Freedom and Dignity (1979) he referred to this science as a technology of behavior and discussed the theoretical applications to pervasive social issues such as disease, education, the environment, housing, nuclear holocaust, pollution, world famine, etc. In 2018, we have yet to live up to Skinner’s vision of a technology of behavior to solve the problems of the world. Instead, Skinner’s technology of behavior is limited to Autism Spectrum Disorder in which, admittedly, it has met undeniable success. That being said, a few questions that have continued to be of great concern to us have been, as a field, why do we limit the principles of behavior analysis to Autism Spectrum Disorder? Why are we not utilizing proven behavioral principles to other developmental disabilities and genetic syndromes? Why are we not using a technology of behavior to tackle pervasive issues that continue to weigh on humanity? Why are we not solving the world’s problems with the science of behavior? And perhaps, most importantly, why have we failed to translate post-Skinnerian theoretical advances and evidence-based technologies to society at large? Thus, it would seem appropriate to us to begin the expansion of Skinner’s vision to genetic syndromes similar to Autism Spectrum Disorder such as Angelman Syndrome.


In 1965, Dr. Harry Angelman published a case study about three unrelated children who had severe intellectual disability, seizure disorder, jerky movements, lack of speech development, and frequent bouts of laughter (Angelman, 1965). The criteria to evaluate and diagnose Angelman Syndrome would be established as functionally severe level of intellectual disability, motor dysfunction, absent or minimal expressive speech, excitable demeanor, with the possible presence of abnormal electroencephalography (EEG) patterns, delayed growth in head circumference, and seizures (Williams, Angelman, Clayton-Smith, & Driscoll, 1995).


Since the first case study by Dr. Harry Angelman in 1965, behavioral features of Angelman Syndrome have been widely noted. Some of those behavioral features consist of aggression, attraction to water, chewing or mouthing of hands or objects, hand flapping, non-compliance, pica (eating and drinking non-food items), self-injury, and temper tantrums (Horsler & Oliver, 2006). Many of these behavioral issues are consistent with behaviors of Autism Spectrum Disorder. It was further concluded that many of these behaviors are maintained by social contact and impulsivity. As students of behavior analysis, we understand that behavior often occurs within a broader context, therefore environmental and social factors could influence challenging behaviors. Similar to the interventions used with individuals with autism, challenging behaviors should be replaced with appropriate behaviors and skills that would allow the individual to achieve greater independence and improve their quality of life.


The literature in Applied Behavior Analysis (ABA) has extended to include children with Angelman Syndrome, although it is currently limited. ABA is an empirically validated treatment that utilizes interventions that provide reinforcement for appropriate behaviors and withholding reinforcement for inappropriate behaviors by establishing the function of said behaviors. As with any behavior, if we are able to understand why someone does something, we can effectively influence it. Summers and Szatmari (2009) found discrete trial teaching could be utilized to teach basic language and learning skills to children. The children received approximately two to three sessions per week for a year. The targeted skills coincided with most ABA programs as they pertain to Autism Spectrum Disorder – eye contact, manding, fine and gross motor imitation, one step instruction, and matching-to-sample.

It has widely been reported that problem behaviors in individuals with Angelman Syndrome are strongly linked to lack of communication skills (Didden, Sigafoos, Korzilius, Baas, Lancioni, O’Reilly, & Curfs, 2009). Without the ability to communicate children with Angelman Syndrome will seek other avenues to have their needs met. The logical solution would be to improve the functional communication skills to reduce or eliminate problem behaviors. Teaching appropriate communication skills would eliminate the need for the child to seek out attention in inappropriate ways. A point to consider throughout the intervention programming is the need for pragmatic goals. Currently, expressive speech skills are not realistic, therefore it would be beneficial to provide augmentative forms of communication – modified versions of Picture Exchange Communication System (PECS) or Sign Language.


As with individuals with Autism Spectrum Disorder, utilizing ABA-based interventions and strategies would allow individuals with Angelman Syndrome to learn basic skills that could provide the fundamentals to learn more complex skills. A key part in this strategy is to involve the parents in the treatment process. Training the child’s parents on how to correctly understand the principles of behavior and utilize ABA strategies to effectively be in a better position take an active and successful role in the ongoing development of their child.




This material is for general experimental purposes only and does not replace the work of a trained behavioral professional. All information suggested in this blog post should only be done within your own scope of understanding. If you need further assistance, please find a behavioral professional to consult with.




References:


Angelman, H. (1965). “Puppet” children: A report on three cases. Developmental Medicine and Child Neurology, 7, 681-688.


Didden, R., Sigafoos, J., Korzilius, H., Baas, A., Lancioni, G. E., O’Reilly, M. F., and Curfs, L. M. G. (2009). Form and function of communicative behaviors in individuals with Angelman Syndrome. Journal of Applied Research in Intellectual Disabilities, 22,526-537.


Horsler, K., and Oliver, C. (2006). The behavioral phenotype of Angelman Syndrome. Journal of Intellectual Disability Research, 50, 33-53.


Skinner, B. F. (1979). Freedom and Dignity. New York, NY: Knopf/Random House.


Summers, J. A., and Szatmari, P. (2009). Using discrete trial instruction to teach children with Angelman Syndrome. Focus on Autism and Other Developmental Disabilities, 24, 216-226.


Williams, C. A., Angelman, H., Clayton-Smith, J., and Driscoll, D. J. (1995). Angelman Syndrome: Consensus for diagnostic criteria. American Journal of Medical Genetics, 56, 237-238.

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