By Jackie Kearns, CCC-SLP, and Douglas Henry, MD
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Cleveland Clinic Children’s experts in pediatric speech and language pathology are using cutting-edge technology to improve communication skills for children with Angelman syndrome and other neurological disorders. Through the use of augmentative and alternative communication strategies, speech-language pathologists work to shorten the gap between a child’s receptive and expressive language skills.
An approach well suited to Angelman syndrome
Augmentative and alternative communication (AAC) refers to a set of procedures aimed at improving, temporarily or permanently, the communication skills of those who have minimal to no functional speech and/or writing. AAC can use both unaided (manual signs, gestures, finger spelling, eye gaze) and aided (picture communication symbols, voice output communication aids) forms of communication. While all speech-language pathologists generally have some cursory knowledge of AAC, not all therapists specialize in this area of communication.
At Cleveland Clinic Children’s, AAC has been especially rewarding for children with Angelman syndrome. This disorder is caused by a disruption in chromosome 15, with the most common disruption caused by a large deletion of the maternal chromosome. Despite several differences in gene mutation, some characteristics are present in all cases of Angelman syndrome. In addition to severe developmental delays, Angelman syndrome is characterized by a movement or balance disorder, ataxia, which can vary from mild to severe. Affected children demonstrate an apparent happy demeanor and hypermotoric behavior.
Children with Angelman syndrome have severe to profound communication impairments. Receptive language is typically more advanced than expressive language. Verbal speech is extremely limited, so all children with Angelman syndrome are excellent candidates for AAC.
Early intervention is critical
Children with Angelman syndrome vary greatly in the types of AAC they use, often employing more than one mode of communication. It has been observed that early communication milestones include the use of eye gaze, facial expression and body posturing.
While these behaviors are unintentional initially, it is crucial for early intervention services to train caregivers to interpret these unintentional actions. Only then will these unintentional acts be shaped into more conventional forms of communication.
A common example of language shaping can be seen with infants, who go through a period of reduplicated babbling. A typical child may spontaneously babble an approximation of “mama” or “dada.” This is not intentional at first. A caregiver, on hearing the child use that approximation, will say, “That’s right! You said ‘mama/dada’ … that’s me!” Shortly after receiving this consistent praise and attention, the child begins to use “mama” and “dada” purposefully.
Turning the unintentional into the deliberate
While the child initially demonstrates early, unintentional communication acts, it is really the caregiver who provides the necessary supports and encouragement to shape those unintentional acts into true communication. Similarly, this strategy is often used for children with complex communication needs.
Gestural communication is another strong skill in children with Angelman syndrome. Initially, these gestures may begin by physical contact. For example, a child may push away an unwanted object or take a caregiver’s hand to lead him or her toward a desired object. The 2002 article by Stephen Calculator (see Suggested Reading) provides an excellent therapeutic technique for creating enhanced natural gestures for children with severe communication impairments, including Angelman syndrome.
A role for communication aids too
In addition to nonaided forms of AAC, aided AAC techniques have proved quite successful in children with Angelman syndrome. These methods may include use of single pictures, communication books/boards or voice output communication aids (VOCAs). A wide range of VOCAs are available on the market today, from simple single-message communicators to dynamic display communication aids (see Figures 1 to 3). iPad® tablets with apps such as Proloquo2Go®, TouchChat™ or LAMP Words for Life™ also may be suitable communication aids for children with Angelman syndrome.
Figure 1. Pragmatic Organization Dynamic Display (PODD) communication books.
Figure 2. DynaVox Xpress™ voice output communication aid.
Figure 3. iPad with Proloquo2Go application software.
A team-based approach to care
The Technology Resource Center at Cleveland Clinic Children’s Hospital for Rehabilitation helps all children whose ability to communicate has been hindered by injury, chronic illness or congenital issues. The therapists at the Technology Resource Center help children interact with others by teaching AAC.
Every child is first seen by a speech-language pathologist who specializes in AAC. The therapist may consult with occupational therapists, physical therapists and physicians to help evaluate a child’s strengths and barriers. Parents, teachers and other professionals already involved with the child are important members of the team and participate in planning and decisions.
Based on team recommendations, a communication plan is developed. If a child needs specialized equipment, Technology Resource Center staff will help families find it. The staff not only prescribes communication devices but also teaches patients, family members and other members of a child’s care team how to use them.
About the Authors
Ms. Kearns is a senior speech-language pathologist and Coordinator of the Technology Resource Center in Cleveland Clinic Children’s Hospital for Rehabilitation. She works with children with a variety of complex communication needs. She can be reached at 216.448.6157 or firstname.lastname@example.org.
Dr. Henry is Director of Developmental Pediatrics and Physical Medicine and Rehabilitation. He can be reached at 216.448.6179 or email@example.com.
Angelman H. Puppet children. Dev Med Child Neurol. 1965;7:681-688.
Calculator SN. Use of enhanced natural gestures to foster interactions between children with
Angelman syndrome and their parents. Am J Speech Lang Pathol. 2002;11:340-355.
Calculator SN, Black T. Parents’ priorities for AAC and related instruction for their children with Angelman
syndrome. Augment Altern Commun. 2010;26:30-40.
Williams C, Frias J. The Angelman (“happy puppet”) syndrome. Am J Med Genet. 1982;11:453-460.