Angelman Syndrome (AS) is a rare neuro-genetic disorder that affects approximately 1:15,000 live births. Characteristics include developmental disability, balance and coordination challenges, epilepsy, disrupted sleep, and complex communication needs. Individuals with AS typically have a profound expressive language disorder, however their receptive understanding far exceeds their expressive ability. Augmentative and Alternative Communication (AAC) is vitally important to bridge this gap. While individuals with AS require assistance throughout their life, with the right support they continuously learn and progress!
Individuals with Angelman Syndrome present with a variety of relative strengths. They are usually extremely social, enjoying interaction with others. Individuals with AS tend to be very attuned to social cues, constantly monitoring those around them to help be informed of changes that may happen. For example, a child with AS may wave goodbye as soon as a visitor reaches for his keys or her purse.
They tend to be aware of even informal repeated routines, noticing if something changes. Hyperacoustic hearing can be a feature of Angelman Syndrome, meaning the individual with AS hears and attends to sound which others may not hear or may hear only as part of the background noise. A child with AS may hear a car door open and close several houses down the street. In general, a child with AS needs the sounds they are attending to be labeled before they can refocus on other things. Similarly, those with AS tend to love music. Music can be used in a variety of ways to focus, calm or engage a child or adult with AS.
People with AS also tend to walk a tightrope between happy anticipation and anxiety. They may love anything that builds happy anticipation from simple things like a jack in the box toy or a knock at the door to knowing they are going to get in the car to more complex things like understanding they are going to go on vacation. However, it is easy for that happy anticipation to tip into anxiety and worry. A set of directly taught and reinforced communication and coping skills can help with anticipatory anxiety.
Angelman Syndrome may create challenges, but looking to the strengths of those with AS can help us find tools to address those challenges.
Individuals with Angelman Syndrome frequently display symptoms of apraxia and/or dyspraxia. Apraxia is the inability to perform a movement or task despite understanding the request, despite having the desire and physical capability to carry it out.
In Angelman Syndrome, apraxia is associated with absence of speech, as well as other motor planning challenges. According to the American Speech-Language-Hearing Association, "to speak, messages need to go from your brain to your mouth. These messages tell the muscles how and when to move to make sounds. If [an individual] has an apraxia of speech, the messages do not get through correctly". This same motor planning deficit also makes it very difficult to learn to use sign language and perform other motor tasks.
Individuals who do not speak may often be misjudged as having a lack of intelligence or decreased desire to communicate (communicative intent). It is extremely important to remember that a profound discrepancy between receptive language and expressive language is a hallmark of Angelman Syndrome. A common statement about individuals who are non-speaking is, "Just because I cannot speak doesn't mean I have nothing to say." Augmentative and Alternative Communication (AAC) can provide the tools to allow individuals with Angelman Syndrome who have apraxia a means to share what they have to say. When individuals with Angelman Syndrome are provided with access to language through robust AAC and appropriate interventions they have the potential to learn to become autonomous communicators.
OUR PHILOSOPHY: All individuals with Angelman Syndrome have a right to robust communication systems and interventions which can help them become autonomous communicators.
Angelman Academy believes the ultimate goal of every individual with complex communication challenges is autonomous communication.
It is the job of the communication partner to support each individual to find her or his own unique voice through the use of Augmentative and Alternative Communication. Each AAC user should be supported to express their own individual thoughts and intentions. We can do this by assessing the needs of the individual and providing appropriate access to a robust vocabulary.
There are absolutely NO prerequisites to learning to use Augmentative and Alternative Communication (AAC)!
TYPES OF COMMUNICATION
Individuals with Angelman Syndrome are multi-modal communicators.
They employ a variety of strategies to get their message across, such as facial expressions, gestures, sign language, vocalizations, and a variety of other non-electronic and electronic Augmentative and Alternative Communication systems, such as symbol communication boards and books, AAC apps or AAC dedicated communication devices. At Angelman Academy, our fundamental belief is that you need to have the words in order to say what you want to say! We believe all individuals with communication needs have the right to have all their communication methods acknowledged and to have unfettered access to a robust vocabulary. All individuals have the right to support and instruction consistent with best practice.
When you decide how many words you are going to give a kid you are deciding the size of their WORLDKATE AHERN
ROBUST LANGUAGE SYSTEMS
Autonomy is defined as being able to say to say whatever you want to say, in the way you want to say it, to whomever you want to say it, whenever you want to say it (Gayle Porter, 2012)
It is for this reason that Angelman Academy promotes access to a robust vocabulary of words. A robust vocabulary enables our individuals with AS to communicate across a range of communication functions (request, comment, ask questions, answer questions, greetings, and express emotions). Many educators and therapists feel the need to isolate the skill of choice making as a precursor to AAC use. However, evidence based practice and current research tells us that an AAC user should be provided with access to a large selection of words in order to find the motivation to communicate her or his intentions. This type of logic may seem very obvious but it is logic that has been wrongly applied to understanding language acquisition in children with complex communication needs. What we know and what the research such as Sennott, Light, and McNaughton (2016) tells us, is that we must put in exactly what we expect to get back, and we must be extremely patient in doing so when asking an individual to successfully overcome profound motor planning and other challenges Read Article: AAC Modeling Intervention Research Review
If YOU use my talker... I WILL LEARN HOW TO USE MY TALKER" KATE AHERN
AIDED LANGUAGE STIMULATION
A typical baby is spoken to for over a year before we have any expectation of even hearing a single word in response. And we certainly don't expect a response in a different language! It's important to point out here that we would never speak to a baby in English and expect to hear a response in German, right?
This type of logic may seem very obvious but it is logic that has been wrongly applied to understanding language acquisition in children with complex communication needs. What we know and what the research tells us, is that we must put in exactly what we expect to get back, and we must be extremely patient in doing so when asking an individual to successfully overcome profound motor planning and other challenges.
When teaching a child with complex communication needs we must speak to them in the language they will use to speak back to us. If they will be speaking to us using a symbol based communication system, then we speak to them using a symbol based communication system along with our verbal speech. This is called many things: aided language input (ALI), partner augmented input (PAI), aided language stimulation (ALgS) but most often it is called modeling. We model language by using the child's communication system as we talk to them. For beginning AAC learners (of any age) we may do this for 18 months or more before the child may be able to show they have learned both language and how to access their communication system. For early AAC learners we continue to model as we speak to teach more vocabulary, functions of language, and grammar. With a typical child using oral speech adults intervene to teach vocabulary and grammar until the child is about 7 or 8 years old, and often older. Additionally, language acquisition experts will tell you that the best way to learn a language is through immersion. Immersion, in this context, means being exposed to the language or language system being learned, in this case AAC, across all environments, including incidental environments such as overhearing conversations between others, at least 70% of the time. To maximize learning of AAC systems we must strive for consistent, ongoing and intensive aided language input across all environments and interactions as much of the time as possible.
AS & Anxiety, Sensory Needs & Behavior
Many individuals with Angelman Syndrome use behavior as a means of communication and to quell what can be significant anxiety. In Angelman Syndrome anxiety often arises from a variety of sources including separation from caregivers, changes in routines and schedules, unexpected or difficult to understand demands, difficulty understanding changing expectations, visitors and new people and other situations.
Individuals with Angelman Syndrome often times use a coping technique learned in infancy of establishing eye contact with a caregiver to create a secure base and feel safe. When they are unable to ask for this eye contact in a conventional manner, such as speech, signs or use of an AAC system they may resort to throwing, hitting or hair pulling. Hair pulling is a particularly common way people with AS seek eye contact and relief from anxiety.
Similar to asking for eye contact through aggressive behavior a child or adult with AS might use behavior to communicate for other reasons. For example, she may drop to the floor to refuse a request to move to a new space or change tasks or he may throw items to indicate he is excited or finished with a task. Teaching an appropriate means of communicating these messages can be an effective way to decrease these behaviors.
It is important to determine, through observation and analysis, what meanings various behaviors may have and then create a way for the child to communicate those needs. A button in a communication system which says, "look at me" can be taught to replace behaviors seeking eye contact. A button which says, "I need a break" or "I don't want to" can help with behaviors seeking to avoid or end an activity.
Those with Angelman Syndrome might also have difficulties modulating their sensory state. They often seek out deep pressure through pushing, kicking or biting on objects, or by jumping or crashing. They may also seek out vestibular stimulation through swinging or spinning. Having sensory items available and teaching and reinforcing their use can help decrease difficult sensory related behaviors. A therapy ball or mini-trampoline can help with deep pressure to the body and items designed for chewing can help with biting or chewing on clothing. Stress balls and therapy putty or clay can help with a desire to pinch or squeeze. Additionally, swings and spinning toys like a sit and spin might help with the need for vestibular stimulation.
Music can also be an effective means to help with anxiety and/or sensory needs. Matching the mood of music to the current state of the child and then gradually moving it towards a calm, 60 beats per minute song can be helpful. Some children and adults might also have calming or alerting response to various songs or styles of music. Careful observation can allow for the creation of calming, alerting or engaging playlists.
There are several conditions that can affect the vision of individuals with Angelman Syndrome. Cortical/Cerebral Vision Impairment, and Strabismus are two disorders that are seen in the AS population. Each requires specific accommodations for access to education and AAC. Additionally, individuals with Angelman Syndrome may present with certain vision differences such as frequently using peripheral vision or a difficulty sustaining visual attention. These differences, while not associated with specific visual impairments, should be recognized and accommodations provided, if needed.
CEREBRAL/cortical VISION IMPAIRMENT (CVI)
CVI is a neurological condition that impacts the visual part of the brain. A person with CVI often has healthy eyes but difficulty processing visual information. Characteristics of CVI include preferences for certain colors, familiar items and environments, and items that are closer.
Those with CVI may engage in light gazing behaviors and have a need for movement, uncluttered or simplified visual environments and for items to be in a particular visual field. Often times individuals with CVI have delayed visual latency and decreased or absent visual reflexes. Difficulty coordinating vision and hearing or vision and motor movements often occur in CVI resulting in, for example, a tendency to be unable to look at and reach for an item at the same time. Over time and through proper interventions like vision therapy and modifying environments, people with CVI can improve their functional vision.
Strabismus is a disorder, caused by neuromuscular difficulty, in which the eyes are misaligned and do not fixate on the same point simultaneously. Often one eye focuses on an object of interest while the other eye wanders outward or inward. Strabismus can result in double vision or difficulty with depth perception. It can be improved through therapy, eyeglasses and sometimes surgery.
Vision challenges should not serve as a deterrent for educational intervention, including literacy, or introduction and use of AAC. A teacher of the visually impaired, special educator and speech therapist can help modify educational materials, home and school environments and Augmentative and Alternative Communication(AAC) systems to meet the needs of an individual with vision impairment.
FOUNDER AND DIRECTOR
Shibvon Sargent is the founder and managing director of Angelman Academy, a website with educational content intended to help inform parents and caregivers about opportunities and strategies to enhance communication abilities for persons with Angelman Syndrome (AS). Shibvon is the Mom to Samantha, a 16 year old girl with deletion + AS. Shibvon has been a volunteer, organizer, and passionate advocate for the Angelman Community for over 10 years. Her primary focus has been to help promote educational awareness and opportunities for individuals with Angelman Syndrome. She has assisted in the planning of numerous events and workshops, including educational seminars held at the annual FAST-sponsored Global Angelman Summit in Chicago. Shibvon has also spoken about AS related challenges at Harvard University and at other venues with the intent of allowing individuals to find their own unique, autonomous voice. Shibvon is a proud and passionate supporter of the Angelman Communication Revolution. Outside of her work for the AS community, Shibvon has spent many years in the culinary industry having trained as a professional chef at Johnson and Wales University in Rhode Island.
Jessica lives outside Boston with her husband and their three sons, including Owen (pictured), who has Angelman Syndrome. She earned her B.A. in Sociology from the University of Massachusetts - Amherst. She currently serves on the Special Education Parent Advisory Council board for her school district. As a SEPAC leader and mom of three children with developmental disabilities, she is passionate about partnering with schools to optimize the educational experience for students with Angelman Syndrome and bringing best practices in communication, education, and inclusion to school teams.
Amy Girouard holds a Master’s degree in Social Work from The Ohio State University and is a Licensed Independent Clinical Social Worker in New Hampshire. She provides a systems focus in healthcare culture change and a clinical focus in social work supervision. She serves as Chair of Family Support Council for Community Bridges and is also on the Medical Care Advisory Committee for the NH Department of Health and Human Services. She has also volunteered for Lite the Nite; FAST. Amy is a NH Leadership Series Graduate; and a member of NH NASW. Amy has been invited to participate in strategic planning and implementation of Charting the Lifecourse for the State of NH. Amy resides in Concord, NH with her husband Chuck Crush and two children Joshua and Allison Girouard. She likes to travel and have adventures with her family.
Katharine Grugan, PhD
Katharine Grugan has more than 17 years of biomedical research experience focused on oncology, immunology and the development of antibody therapeutics. She received a doctorate in cell and molecular biology from Northwestern University in 2007. Since 2011, she has been a scientist at Johnson and Johnson in their pharmaceutical division. She became passionate about Angelman Syndrome upon the diagnosis of her 10 month old daughter in 2011 and is a member of the Angelman Syndrome Foundation Scientific Advisory Committee. In her spare time, she enjoys making PODD books and traveling to conferences to learn about best practices in AAC and literacy instruction for individuals with complex communication needs. Katharine resides outside of Philadelphia with her husband Kevin and three children
Sasha Siegelbaum has worked as an educator for over 10 years and is currently the Co-Director of Alamander Camp, a child-centered, progressive After School and Camp Program. This past summer, she began an Inclusion Program for children with disabilities and looks forward to growing this program. Sasha graduated from Barnard College with a BA in Art and Art History and holds a MS in Early Childhood and Childhood Education from Bank Street College of Education. In her free time Sasha loves spending time with her family, playing with her children Milo and Iris, and exploring creative ways to integrate AAC in their daily lives.
Nicole Meade is a mother to two amazing boys, one of whom is 6 years old and has Angelman syndrome (Imprinting Center Defect). She introduced AAC to her son only days after his diagnosis at the age of 2 after searching Google for something along the lines of "what in the world are you supposed to do when your kid has Angelman syndrome?" She continues her never-ending growth on this AAC journey with humility and amazement at the people and the rewards that AAC has brought to her son and her family.
When she is not stumbling through the joys and challenges of AAC she works as a nurse midwife at a busy academic medical center in New York City. She also proudly serves on the board of directors of INCLUDEnyc, a non-profit organization in New York City that for 35 years has provided services and resources in New York City that promote love, equity and access for youth with disabilities and their families. She is a parent co-founder of ACES-ICT (Academic, Career and Essential Skills-Integrative Co-teaching), public/private initiative in collaboration with the New York City Department of Education. This program is dedicated to inclusion of learners with complex needs, all of whom are AAC users, with general education peers in the classroom and school community at large. In its first year, and with two pilot programs in two NYC public schools, the goal is for this model to become the norm in the largest school district in the country.
Desiree de Los Angeles
Desiree de Los Angeles is the owner and primary seamstress at Angeles Designs. Both her grandmothers inspired her interest in sewing but Desiree’s greatest inspiration was her daughter Jasmine. Jasmine is diagnosed with Angelman Syndrome. She has complex communication needs and uses an iPad with a PODD app to communicate. After searching for a cute and functional way for her daughter to safely carry her iPad, Desiree decided to just create one. It became known as the Jabber Jas harness! The harness was specifically designed for AAC users to carry their device safely and securely. Desiree’s goal for every harness she creates is that it’s comfortable, functional, safe, and stylish.
In addition to being a maker of harnesses, Desiree loves all things crafty! She enjoys photography, sewing, DIY projects, and her family. With four kids and a big family Desiree is always being inspired to create.
Nidia holds a degree of Bachelor of Arts, with a double Major in Graphic Design and Media Advertising at the University of Texas at El Paso. She is Mexican, based in Argentina since 2004 with her husband and two girls, one of whom has Angelman Syndrome (Deletion+). She was co-founder and vicepresident for Angelman Argentina, an non-profit organization based in Argentina. There she helped to raise awareness about Angelman Syndrome. When she started learning with her girl about Angelman and AAC, she felt she needed also to raise awareness about Angelman and the best AAC practices, because of a lack of information available in Spanish. In 2016 she started the blog HabloconCAA, where she writes about her experiences on her AAC journey. There she translates useful and research-based resources (& creates her own) for the Spanish speaking community.
Tina Thompson is a mother and advocate. When her son, Finn, was diagnosed with Angelman Syndrome, Tina became passionate about issues surrounding disability, inclusion and supporting individuals with complex communication needs. She has attended numerous trainings and conferences in that regard and has participated in AAC camps with Finn. She is advanced PODD trained and has used PODD with Finn for the last 8 years. She has been an advocate for both inclusion and communication and has held various trainings for schools regarding AAC. She has also been a guest speaker at local colleges regarding both inclusion and AAC. She spent 3 years as a paraprofessional supporting students with disabilities at WISH Charter Community School, an all-inclusive school in Los Angeles. She is currently in the process of getting a graduate certificate in special education: complex communication needs from Edith Cowan University.
Kelly David, M.A., CCC-SLP, received her BS in Biology and Master of Arts degree in Communication Sciences and Disorders from the University of Central Florida. She has served populations from pediatric to geriatric, with developmental and/or acquired disorders. She currently works as a clinical instructor at the University of Central Florida's Communication Sciences and Disorders Clinic with individuals across the lifespan. She volunteers as co-Vice Chairperson for the Foundation for Angelman Syndrome Therapeutics (FAST), and has a passion for helping families access education and resources related to best practices in alternative and augmentative communication. Her most important role, however, is as mother to Colin and Madeline. Her son, Colin, lives with Angelman syndrome.