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2. Augmentative communication across the life span in AS

Two significant facts emerge from available descriptions of the clinical course of individuals with AS: (1) they rarely, if ever, develop functional speech, and (2) they seem to have a normal life expectancy. To date, we have limited information about what AAC strategies and devices are helpful, when these should be introduced, and how they should be taught. This article suggests a framework to address AAC intervention issues with persons who have AS. Suggestions are anecdotal and/or hypothetical. They are not based on research or extensive clinical experience. This article simply offers a place to start. Readers are invited to react, offer suggestions, agree and disagree.

Table I (not included) summarizes ages and stages of life that roughly parallel those described in the literature. A quick glance reveals that information about adulthood is sparse. Each category in Table I (not included), i.e., General Characteristics, Description of Communication, Desired Outcomes and Strategies for Supporting Families and Caregivers is discussed in more detail below.

The perinatal history of individuals with AS is often unremarkable. Genetic counseling for those who have children with AS is available, although there is no increased chance of recurrence in Types I and II. Prenatal identification of AS for Types III and IV is not yet available. Chances of giving birth to another affected child (in the same family) may be as low as 1 percent or as high as 50 percent.^{8, 9}

Birth to One Year: Infancy

Although the diagnosis of AS is rarely made until later, behavioral and physical problems emerge during this period. The head circumference begins to fall below normal ranges and infants may develop seizures. Hypotonia and delayed motor milestones are evident by 6 to 12 months. Some babies are thought to have cerebral palsy. Feeding problems include difficulties sucking and swallowing and frequent spitting up. Strabismus may be noted.^{6, 7,13}

Description of communication All development is delayed. Oral and speech motor problems underlie, to some extent, delays in cooing and babbling. Oral motor problems include feeding problems, abnormal tongue thrusting and excessive drooling. Early social smiling as well as giggling and unprovoked "chortling" may occur in some babies.^{6, 7,13} Even if a diagnosis of AS is made during the first year (and it rarely is), doctors are unlikely to refer babies to a communication specialist. Doctors and families may find these guidelines useful:

Desired outcomes for infants

1. Engage in soundplay; (b) Take turns and interact with people and objects; (c) Respond and initiate interaction using signals, i.e., vocalizations, gestures, facial expression, body language; (d) Develop a small repertoire of signals, recognizable to caregivers.

Supporting caregivers

1-3 Years: Toddler

Developmental delays in all areas are evident. A neurologic exam may reveal microcephaly, ataxia, seizures; an abnormal EEG and possible atrophy of the cortex on MRI or CT scans. Professionals identify a variety of behavioral concerns including hyperactivity, excessive laughter, mouthing, a short attention span, motor problems, general developmental delay and a lack of speech. Children rarely walk during this stage; fine motor skills also are delayed. Tactile hypersensitivity is noted in some. Play skills are restricted.^{6-8, 15}

Description of communication Significant oral and speech motor delays, as well as severe cognitive delays underlie the lack of speech. Immature babbling, periodic shrieks or screams and one or two apparent words may be used indiscriminately and inappropriately. Expressive language and speech delays are more severely affected than the child's understanding of language. Communication is made even more difficult because intonational patterns, facial expression and body gestures may not express clear communicative intent. Professionals and families should introduce AAC strategies (and simple technologies) to children with AS. Their prognosis for speech is quite guarded.^{3, 6-8,12,14-15}

Desired outcomes for toddlers

1. Engage in soundplay; (b) Use signals (gestures and vocalizations) to communicate; (c) Make choices and requests, initiate interaction, respond and reject, using signals recognizable to caregivers; (d) Engage in turntaking; (e) Demonstrate understanding of cause/effect; (f) Interact with some objects in a meaningful way; (g) Enjoy interaction with caregivers.

Supporting caregivers

Professionals should: (a) answer all questions honestly and provide information about neurogenic speech problems; (b) discuss how AAC techniques can help; (c) reassure the family that AAC interventions do not inhibit speech development or mean professionals are "giving up on speech;" (d) help primary caregivers recognize signals and establish a repertoire of signals, both vocal and gestural; (e) begin to introduce simple technology.

3-8 Years: Preschool/Early Elementary

Walking is a major theme during this period. However, ataxia may preclude independent ambulation until a later stage, and hyperactivity and instability place these children at risk for injury. Sleeping problems are frequently noted. As a result, families generally construct a special "safe" room for their child. Cognitive and fine motor problems interfere with play, so toy adaptations are almost always necessary. Seizures continue, and, in some children, management can be difficult.^5-6,15-16

Note: By 8 years of age most children who develop seizures will have done so.

Intervention generally occurs in the school setting, with self help skills, toilet training, communication and play the focus. A team approach is recommended. Patience and flexible, functional training programs are needed.^3-6,12,14-15

Description of communication

^{Some children (39%) are reported to use a few words (4 or less) starting at a mean age of 3.8 years; however, dysarthria continues to preclude functional speech. Protrusion of the jaw and the wide mouth may become more pronounced, but less tongue protrusion may occur. For many, fine motor and cognitive problems result in a limited use of intelligible signs/gestures. To access language, these children generally need to use AAC aids and devices.3-6,12,14-17}

^{Desired outcomes for children}

1. ^{Understand signals (gestures and vocalizations) and some symbols (real objects, photographs, adapted signs); (b) Use a calendar/schedule box; (c) Express a variety of communicative functions using signals and symbols (e.g., make choices, initiate interaction, request, express emotions, refuse); (d) Enjoy interacting; (e) Interact with peers; (f) Participate actively in school curricula that accommodate cognitive, motor, language, and cognitive disabilities.}

^{Supporting caregivers}

^{Professionals should help families: (a) encourage children to explore and interact with toys and people; (b) participate in activities at home and in school; (c) recognize idiosyncratic signs and body language as a primary means of expression; (d) establish the symbolic value of object symbols. (Note: Miniature objects are not recommended as long as child puts objects in his/her mouth; (e) begin to pair objects with photographs and pictures if child shows an interest.)}

^{Childhood: 8 - 21 Years}

^{Gradual improvement occurs and children continue to learn, but at their slower pace. Tremors may occur and be secondary to anti-epileptic drugs. During adolescence, sexual maturation may be somewhat delayed. Behavior and}

^{sleep problems are noted. 6,7,11,15-16}

^{Description of communication}

^{Bursts of laughter occur in approximately 72% of patients in certain situations (after convulsions, during menstruation, in new situations). Drooling, chewing and licking of objects are still noted. As speech does not develop, the use of AAC techniques grows ever more critical. A survey of 179 families (with approximately half of the children in this age group) reports that the "majority of these children communicate intimately using sounds, hand-over-hand, and gestures. In a school environment electronic communication is favored."6,11,15-17}

^{Desired outcomes for children and youth. (a) Participate in school and community activities using AAC techniques, strategies and devices; (b) Engage in interactive recreational activities for increasing lengths of time using adapted equipment; (c) Use a calendar/schedule box, conversation book and remnants; (d) Express needs, preferences and emotions using appropriate AAC techniques; (e) Have friends; (f) Develop skills increasing the likelihood of living in a community and doing meaningful work; (g) Learn about using the phone.}

^{Supporting caregivers. Professionals should: (a) expect continued improvement and increasing independence in communication; (b) interpret challenging behavior as communicative; (c) teach socially appropriate ways of expressing a range of communicative functions; (d) actively support the use of communication books, devices and computers. (Equipment must be rugged and protected as individuals can be "very rough on it.") (e) acknowledge person's efforts and frustrations; (f) deal with issues that affect all adolescents, i.e., sexuality, independence, confusion, peer relationships; (g) make every effort to provide age-appropriate materials and activities; (h) encourage child's independence.}

^{Adulthood: 22+ Years}

^{Individuals with AS tend to "calm down and have less bursts of laughter" as they grow older. EEG patterns also tend to improve. Major characteristics are a lack of speech, mental retardation, happy disposition and ataxia. Some older patients refuse to walk after discovering the comfort of a wheelchair. Adult patients studied were in excellent general health except for thoracic scoliosis reported in elderly patients (predominantly females), which can result in cardiorespiratory problems. The oldest patient described in the literature is 76 years old. Many adults with AS probably remain undiagnosed.6, 8}

^{Description of communication}

^{Functional communication continues to be the key issue throughout adulthood. Unfortunately, case reports that focus on adults with AS are lacking. However, the general trend is for adults with mental retardation to reside in group homes and work in their communities. To do so, they will require adequate support and independent, functional communication skills.6, 8,18,19}

^{Desired outcomes for adults}

1. ^{Work and live in the community with support; (b) Be supported in ways that enable person to choose activities, food, clothes, hobbies, friends, and so on; (c) Communicate feelings, thoughts, ideas; (d) Have friends; (e) Continue to learn new things; (f) Use the phone.}

^{Supporting caregivers}

^{Professionals should (a) support the use of AAC devices; (b) advocate for interventions and living situations that address functional communication skills; (c) expect individuals to communicate using language, as well as their well-developed idiosyncratic signals; (d) expect continued improvement and increasing independence in communication.}

This article appears in ACN Volume 8, # 3.

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