This paper will provide a brief explanation of Fragile X Syndrome (FXS) and answer the questions: How can assistive instructional technologies help kids with FXS? What types of instructional technologies are available to help facilitate communication and are they effective? Some currently available hard and soft assistive instructional technologies will be discussed and shown how these technologies can help promote effective nonverbal and verbal communication in children with FXS. The specific types of technologies this paper will discuss are: the Picture Exchange Communication System (PECS), hardware such as Speech Generating Devices (SGD) or Voice Output Communication Aids (VOCAS) like DynaVox Maestro, Go Talk Pocket, digital picture boards such as MyVoice and software programs such as Picture This and Boardmaker by Mayer-Johnson.
Many children with special learning needs can greatly benefit from assistive instructional technologies, specifically in helping to promote communication. For children with Fragile X Syndrome (FXS) these technologies are especially important. Fragile X Syndrome is a genetic disorder caused by a change in mutation of the X chromosome. FXS is the most common form of intellectual disabilities occurring in both males and females. It occurs in approximately 1 in 3600 males and 1 in 4000 to 6000 females with about one-third of boys with FXS having some degree of autistic-behaviors (Abrams 2011; Abrams & Dixon Weber, 2009). FXS is a common cause of autism in male children and usually causes speech delays, which can range from a slow formation or progression of sounds, words and sentences, to no speech development at all. Presently, there is no known cure for Fragile X making it extremely important to provide the best available assistance possible to help give these children extra support to aid in their speech development. Early intervention therapies such as speech and language, occupational, physical and behavior therapies are helpful in addressing the speech delays and other cognitive issues caused by FXS. In addition, there are many available assistive instructional technology options that may be utilized. Children should be carefully matched to the right instructional technology that best suites their stage of development, cognitive level and best matches their strengths and weaknesses. This paper will look at some of these assistive instructional technologies that can help children with FXS make progress in verbal and nonverbal communication.
What is Fragile X Syndrome?
The history of FXS can be traced back to 1943 when Martin and Bell found that a particular form of mental retardation was linked to the X chromosome. However, wasn’t until 1969 that Herbert Lubs developed a chromosomal test for diagnosing Fragile X. This test was not widely used until the late 70s and 80s and even then, was not always accurate (Abrams, 2011). It was not until 1991 that the FMR1 gene was identified as the specific cause of Fragile X. This discovery resulted in the development of two, more diagnostic molecular DNA tests; the Southern Blot analysis and the polymerase change reaction (PCR) (Abrams & Dixon Weber, 2009). These tests were more accurate and are currently the main methods in which Fragile X is diagnosed.
For a (full mutation) diagnosis, an individual will show an abnormal amount of CGG repeats on their FMR1 gene. CGG is the name for the specific pattern of DNA located within the FMR1 gene on the X chromosome. If an individual has an abnormal amount of CGG repeats, the X chromosome appears damaged or broken under a microscope, resulting in the name “Fragile X”. This abnormality is significant because the FMR1 gene produces an important protein to the brain for normal functioning and development (Abrams & Dixon Weber, 2009). In a person with FXS this protein is not produced normally, resulting in a variety of developmental delays.
In the 20 years since the FMR1 gene was identified, there has been progress in understanding Fragile X’s impact on proper growth and development. It is now understood that it can affect many areas such as behavior, intellect, physical characteristics and health. For males, the range of intellectual disabilities varies from minor learning disabilities to severe mental retardation and autism; while females generally present milder symptoms (Abrams, 2011). In addition, FXS can cause attention deficit disorders, speech disturbances and other autistic behaviors.
For children with FXS, speech development is one of the most significant areas affected by the genetic disorder. According to Abbeduto, and Hagerman (1997); Dykens et al. (1994); Turner, Daniel and Frost (1980), and Wolf-Schein et al. (1987) “Delayed acquisition of functional speech, repetitive speech, dysfluency, misarticulations, and unusual prosodic features have been reported” (as cited in Philofsky, Hepburn, Hayes, Hagerman & Rogers, 2004). In contrast to the reported speech delays, Newell, Sanburn, and Hagerman (1983) reported “that relative strengths in receptive language abilities are often associated with X-linked retardation” (as cited in Philofsky et al., p 208, 2004). In sum, children with FXS generally show a significant delay in speech development but also show significant strength in their receptive abilities. These factors are significant because these high receptive abilities, when enhanced with assistive instructional technologies, can result in positive progress in the promotion verbal and nonverbal communication.
Assistive Instructional Technologies and Meaningful Communication
The National Fragile X website defines assistive technology as “equipment that helps the child improve his or her functional capabilities.” It goes on to say “For children with Fragile X who are not yet speaking, the assistive technology might be picture cards, a language board, or a computerized, talking device” (Harris-Schmidt, 2011, n.p.). Many types of assistive instructional technologies exist to help these children. Assistive instructional technologies can include simple devices such as picture cards and white boards to more advanced technology such as electronic devices that can help simulate speech and computers or computer software technologies. For parents, educators or administrators it is important to connect each child to the specific assistive technology that best suites their cognitive, receptive and overall stage of development. Authors Dykens and Hodapp (2001) and Hodapp and Fiddler (1999), suggest that different children with different behavioral and development diagnosis benefit from a variety of intervention approaches which need to be based on the particular child’s strengths and weaknesses (as cited in Philofsky, Hepburn, Hayes, Hagerman & Rogers, 2004, p.215). If children with FXS are successfully connected with technologies that suite each of their specific cases, then it will open important doors to promoting verbal and nonverbal communication. Philofsky et al. (2004) state “recognizing the relative strengths in receptive language in young children with fragile X has many implications for educational and treatment strategies… to furnish rich and meaningful opportunities to learn and practice expressive activities” (p.215). One example strategy is the use of visual representation systems as discussed by Susan Stokes (n.d.) in her article “Assistive Technology for Children with Autism.” Such visual representation systems include objects, photographs, realistic drawings, line drawings, written words and other graphic symbols to help nonverbal children communicate. For many of these children their receptive skills are quite strong, resulting in the ability to remember many different symbols and pictures of everyday objects and thus being able to communicate by choosing which picture represents their specific need at any given time.
To create an even stronger connection between these children and the instructional technology, in this example printed pictures, the different factors associated with the wide variety of pictures available must be examined. These factors include: color verses black and white, line drawing or symbols verses realistic photographs and other visual features such as shape, texture and size. In the 2009 article “Recognition and Use of Line Drawings by Children with Severe Intellectual Disabilities: The Effects of Color and Outline Shape, author Jennifer Stephenson cites Dixon’s (1981) findings that children with intellectual disabilities ability to match objects to photos was improved when the photo was cut into the actual shape of the object resulting in an even greater connection to the real object. This finding is a specific example of the many different variables that are associated with successful connection of children with FXS to the assistive instructional technology that best matches their abilities.
The types of assistive instructional technologies that may be utilized to help children with FXS can be divided into three categories: “low” technology, “mid” technology and “high” technology. Stokes (n.d.) describes “low” technologies as non electronic or battery powered device-typically low cost and easy to use; “mid” technologies as battery operated devices-typically easy to use and “high” technologies as more complex and high cost. Examples of “low” technologies include: white boards/dry erase boards, clipboards, 3-ring binders, folders, envelopes, photo albums, laminated photographs, magnets, highlighters, picture blocks and more. Examples of “mid” technologies are: tape recorders, headphones, CD and DVD’s, overhead projectors, view masters, timers, calculators and more. Finally, examples of “high” technologies include: video cameras, computers, software, hardware, voice output systems, video tablets and more.
Facilitation of Verbal and Nonverbal Communication
Some specific types of assistive instructional technologies for the facilitation of verbal and nonverbal communication are: the Picture Exchange Communication System (PECS), hardware such as Assistive Technology (AT) speech generating devices or Voice Output Communication Aids (VOCAS) like DynaVox Maestro, Go Talk Pocket, digital picture boards such as MyVoice and software programs such as Picture This and Boardmaker by Mayer-Johnson. These examples represent a range of low to high technology that may be used to help facilitate verbal and nonverbal communication for children with FXS, Autism, Attention Deficit Disorders and other development and spectrum disorders.
For children who have some form of verbal communication, helping them to better articulate their wants and needs is very important. According to Guess, Benson, & Siegel-Causey (1985), “Without an effective means of communication, individuals with moderate and severe intellectual disabilities can experience the phenomenon of learned helplessness” (as cited in Pinto, Simpson and Bakken, 2009). This learned helplessness can have an effect on all areas of their lives. Karen Banks (2010) author of the article “Autism Augmentative Communication & Assistive Technologies” writes that communication lays an important foundation for individuals with autism [FXS and other spectrum disorders] to succeed in their lives. In addition, it plays a vital role in social and personal development. Matching an individual with an assistive instructional technology is an important foundational stone toward building quality of life and realization of potential. Along with learned helplessness, children can also experience frustration if their attempts at connecting with others are consistently ineffective. Simply because these children may not have full capacity at expressing themselves does not diminish their need to be heard.
For children with no form of verbal communication, it is also important to provide them the best tools that can be offered; so they may be successful in communicating their wants and needs. The use of assistive instructional technologies can open up ways of communication for these children who have no voice. “This inability to express themselves as others does not indicate that these individuals have nothing to say, nor does it diminish their need and right to communicate…” (Pinto et al., 2009, p.99). It should be universally understood that just because they cannot speak does not mean they have nothing to say, nor does it mean that they cannot understand. Kelli Ngariki (2010), mother of two boys with FXS and author of the article “To Understand, To Be Understood: The Power of Augmentative Communication” discusses that she has used a combination of communication aids such as sign language, the Picture Exchange Communication System (PECS), picture schedules, interactive computer programs and MyTalk on iPod to help her sons establish better communication. She goes onto state, “Most parents and speech language pathologists who have used augmentative/alternative communication (AAC) with children see it as a bridge to verbal communication” (p.26). The Picture Exchange Communication System (PECS) is just one example of a widely used low tech instructional technology that is proven to help build important bridges for nonverbal children to achieve verbal communication.
The Picture Exchange Communication System (PECS) was developed in 1985 by Andrew S. Bondy, Ph.D. & Lori Frost, M.S., CCC/SLP. It is an alternative form of communication typically used with children who are nonverbal. “The PECS teaching protocol is based on B.F. Skinner’s book, Verbal Behavior, such that functional verbal operants are systematically taught using prompting and reinforcement strategies that will lead to independent communication” (What is Pecs?, n.d.). The Delaware Autistic Program was the first to implement PECS and since then it has received worldwide recognition for its unique foundational aspect of focusing on the initiation component of communication. It was created with everyday families, educators, care givers and therapists in mind (What is Pecs?, n.d.). PECS is simple to use, inexpensive and readily adaptable to many settings and circumstances.
By design PECS is the “exchange of the icon as a form of communication, not the picture itself” (Autism-Help, 2008, n.p.). PECS uses picture images, usually graphics or line drawings but realistic picture may also be used, and typically starts with desired images such as food or toys. PECS then moves through six stages: Phase 1”How to Communicate” teaches the student to spontaneously request an item by exchanging a picture with a “communicative partner”, Phase 2 “Distance and Persistence” teaches the child to generalize the new understanding to apply to other objects and activities, Phase 3 “Picture Discrimination” teaches the student to select two or more pictures from a binder or Velcro strip to intentionally build to more complex sentences, Phase 4 “Sentence Structure” encourages the construction and use of sentences using the “I want” picture and pairing it with the picture of the want or need, Phase 5 “Answering Questions” teaches the student to use PECS to answer the question “What do you want?”and finally Phase 6 “Commenting” encourages the student to comment when asked questions such as “what do you see?” or “what do you hear?”(Autism-Help, 2008; What is Pecs?, n.d.). PECS is not an immediate process but rather can take months to implement. However, it is a proven effective and successful means of communication. In a study done by Schwartz, Garfinkle and Bauer (1998) results found that all 31 children in the study learned to use the system in an average of 14 months. In addition, 44% of the children showed an increase in spoken language (as cited in Pinto et al., 2009, p.100). PECS is just one example of an easily accessible and applicable instructional technology that can help children with FXS communicate.
Hardware such as Speech Generating Devices (SGD) or Voice Output Communication Aids (VOCAS) have proved to be successful in helping generate communication. These options vary from the lower tech systems such as PECS and sign language because they provide the child their “missing” auditory voice. They are also more universally understood, since they verbalize the child’s want or need to the parent, family member or teacher making it unnecessary for them to have to learn special skills associated with other options in order to interpret the child’s needs (Missouri Assistive Technology Advisory Council, n.d.). In addition, experts who have implemented such devices, have found other positives such as, the devices only requiring simple motor movements to operate and these devices can help preempt difficult behavior and other communication frustrations due to them being quick and consistent means of expression (Missouri Assistive Technology Advisory Council, n.d.). Some specific types of SGD’s or VOCAS are DynaVox Maestro, Go Talk Pocket and thedigital picture board MyVoice.
The DynaVox Maestro gives a variety of image pages designed in a familiar computer keyboard layout. When the user pushes the picture symbol that reflects what they want or need it is translated through the device and into spoken words. The Maestro also contains a camera for individuals to take their own images, providing an even more personal connection to their communication. Go Talk Pocket is shaped similar to a smart phone. “It is lightweight, rugged and conveniently pocket sized…it has six message keys with five levels that give the user a variety of communication options” (Autism Speaks, 2005-2011). While some of these devices can be quite expensive, most are affordable, convenient and easy to use.
MyVoice was designed by parents with an autistic daughter. In their About Us statement titled Our Story they state:
We experimented with many different methods of communication, from sign language to icon boards, and none were satisfactory… they all suffered from the same flaw: they required an abstract connection between a thing and a concept that our daughter wasn’t getting. A picture board solved this to some degree but was neither portable nor easily modifiable. Enter MyVoice. It allowed us to create a “digital” picture board as well as let her hear the phrase spoken every time she requested something (MyVoice, 2011, n.p.).
This personal example not only highlights the importance of matching each child to the specific technology that best works for them, but also shows that the child’s range of cognition must be taken into account. Not all children can make the abstract connection between something and its matching concept. MyVoice was specifically designed to help these non-verbal, lower cognitive children communicate their wants and needs. “It is the digital big brother of a “picture board” a communication method that has been shown to work extremely well with many non-verbal children” (MyVoice, 2011). It is also readily available to use on multiple devices such as Galaxy Tab, Android, iPhone, iPad and iPod.
Picture software programs such as Boardmaker and Picture This by Mayer-Johnson, have also proven very successful in helping aid communication. This software is currently utilized in schools and by teachers, parents and other professionals. Boardmaker software offers a 3,000 Picture Communication Symbols (PCS) library in either black and white or color (Stokes, n.d.). These pictures can be accompanied by written words or messages and can be resized to fit the user’s preference. Boardmaker helps produce and design printed materials such as communication boards and schedules. The user can choose the topics, symbols, labels, colors, borders, languages and more to aid in the progress of successful communication (Boardmaker Product Overview, 2011). In contrast to Boardmaker, Picture This software allows for the use of real photos. Picture This works with Boardmaker as an additional or accompaniment tool. This software provides thousands of realistic, clear and simple-to-use images to help individuals communicate for those who prefer realistic representations.
Many assistive instructional technology options are available to help facilitate communication for children with Fragile X. The Picture Exchange Communication System (PECS), Speech Generating Devices (SGD) or Voice Output Communication Aids (VOCAS) like DynaVox Maestro, Go Talk Pocket, digital picture boards such as MyVoice and software programs such as Picture This and Boardmaker by Mayer-Johnson are just a few of them. It is important to connect the child to the right assistive instructional technology that best meets their functioning level and personal needs. Once this match is made, children who previously had no way of communicating or had continued frustrations at their level of communication being continuously misunderstood will be able to establish new levels of self confidence, independence and self expression.
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As an instructional designer my focus is the design and development of effective online instruction, interactive learning objects, instructional alignment and the visual aspects of instructional materials. Specifically, how the inclusion of visuals within instruction can help facilitate learning.