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CHARGE syndrome: an educators' primer.

Abstract

This paper introduces educators to CHARGE syndrome (CS), a multiple anomaly developmental syndrome that is usually accompanied by some degree of hearing and visual impairment. We describe the defining medical characteristics of the syndrome, and following this, outline the behavioral features commonly seen in individuals with CS. Throughout, we highlight the implications for the inclusive education of children and youth wth CHARGE syndrome. Recommendations focus on Positive Behavioral Support strategies, and we provide an illustrative school-based case study.

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The prevailing philosophy of inclusion for children with special needs within educational settings (Slee, 2001) means that educators must be prepared to address the needs of children with developmental conditions that are both complex and unfamiliar. CHARGE syndrome is one relatively newly described complex developmental syndrome. Children with CHARGE syndrome face many challenges (e.g., medical, behavioral, and learning) that can interfere with their educational success. There is limited literature related to educational best practices for children with this condition, but a recent surge of behavioral research (e.g., Bernstein & Denno, 2005; Hartshorne, Hefner, & Davenport, 2005; Smith, Nichols, Issekutz, & Blake, 2005) has yielded information that may enable educators to anticipate better the types of programs that could benefit students with CHARGE syndrome.

For the present purpose, an extensive, systematic literature search of electronic databases related to the fields of education (Educational Resources Information center [ERIC]) and psychology (PsycINFO) was conducted for articles related to CHARGE syndrome. This paper integrates this current psycho-educational literature with medical information about CHARGE syndrome, providing an overview of the syndrome and its behavioral manifestations, with a specific focus on educational implications. Of note, this paper is designed for educators, and is intended to provide information on CHARGE syndrome that will allow them to better understand, and to provide educational programming for a student with this syndrome. Specific information on medical and behavioral characteristics is included, as well as positive support strategies and suggestions for the classroom.

Introduction

CS was first identified only in the late 1970's (Hall, 1979). The acronym CHARGE was coined (Pagon, 1981) to highlight the association of a group of symptoms existing from birth:

* Coloboma of the eye is a cleft or failure of closure during development of the eyeball. It can affect the lens, choroids, optic disc, retina and/or iris, and may cause blindness, restricted vision, acuity, and many other related problems of a child's visual field.

* Heart Defects are common and vary in type and severity. Treatment includes surgery and ongoing medical management.

* Choanal Atresia is a blockage or narrowing of the nasal passages by bone or cartilage. A complete blockage of both passages is a neonatal emergency requiring urgent surgery and periodic follow-up. This problem may result in difficulty with breathing and eating.

* Retarded Growth and Development occurs in most children, although the extent of either type of delay varies.

* Genital Hypoplasia is typically observed in males (e.g., small penis, undescended testes). It is common for both males and females to receive hormone therapy to help them achieve puberty.

* Ear Anomalies/Deafness are frequent in CHARGE syndrome. Children often have both unusually shaped external ears and some degree of hearing loss (due to abnormalities of internal structures).

The acronym represents the most common features of CHARGE syndrome as it was initially described. Previous diagnostic criteria required four of the six features to be present. However, over the years, other features commonly associated with those first identified in "CHARGE" have been reported, notably cleft/lip palate and cranial nerve dysfunction. Revised diagnostic criteria have been suggested, and include both major and minor categories of features (Blake et al., 1998; Blake & Prasad, 2006). Distinctive facial features (e.g., broad forehead, square asymmetric face, high nasal bridge and full nasal tip, small mouth, arched eyebrows, facial palsy, and malformed ears) are also common to individuals with CHARGE syndrome (Blake et al., 1998; Blake & Prasad, 2006).

CHARGE syndrome is a heterogeneous condition; that is, children may exhibit various combinations of features, differing in number and severity. CHARGE syndrome is often estimated to affect 1 in 15-20,000 babies born, although Canadian data suggest a much higher rate, up to 1 in 8,500 live births (Issekutz, Graham, Prasad, Smith & Blake, 2005). CHARGE syndrome is associated with a genetic abnormality in a substantial proportion of cases (Sanlaville & Verloes, 2007; Vissers et al., 2004). Ongoing genetic research may lead to a better understanding of the origins of CHARGE syndrome.

Presentation of CHARGE syndrome

As indicated above, it is common for children with CHARGE syndrome to have chronic physical challenges that interrupt many aspects of development and overall functioning. In the years following the first descriptions of CHARGE syndrome, many children who were affected by the more severe medical manifestations died. Now, with better understanding, earlier identification, and improved medical care, the survival rate has improved greatly. Surgical intervention early in life can stabilize and/or correct many of the potentially fatal anomalies. In a series of 42 cases of children with CHARGE syndrome over the age of three years (mean age 8.6 years, range 3-23 years), 27 (64%) had undergone between one and six operations, and 15 (36%) had experienced more than seven surgical interventions (Blake, Morris, & Smith, 2006). In addition, Blake & Brown (1993) reported that children with CHARGE syndrome had an average of 8 out-patient medical appointments each year. It is likely that interruptions of family routines and typical early childhood experiences, including socialization and educational opportunities, are common for these children due to their extensive medical involvement.

Early CHARGE syndrome research focused exclusively on diagnostic and medical issues. In both the short and long term management of CHARGE syndrome, Blake and colleagues (1990) reported that a multidisciplinary approach helps to ensure that these children reach their full potential. However, organizing a multidisciplinary approach is often challenging. This situation is further complicated by the sheer number of specialists and professionals who are typically involved with a child with CHARGE syndrome. For example, it is not uncommon for a team to involve eye, ear-nose-throat, and heart specialists, paediatricians, speech-language pathologists, occupational therapists, physiotherapists, and special educators. In Blake and Brown's (1993) study, many families experienced fragmented services and were often left in the difficult role of coordinating their child's services themselves. Despite all the stress that families experienced, only some families reported receiving support from community-based services (Blake & Brown, 1993). As identified in other populations of children with special needs, helping families to find appropriate formal or informal support services or groups may be beneficial (Acorn, 1993; King, Cathers, King, & Rosenbaum, 2001). It is generally accepted that children with CHARGE syndrome will experience many challenges throughout their lifetime. Many children have multiple sensory impairment (hearing and vision impairments, often with associated vestibular-balance problems) that may range from mild to profound (Brown, 2005). These difficulties can lead to further complications across various developmental domains, including motor development, language and communication, and cognition or learning. Initially, intellectual disabilities were expected in all children with CHARGE syndrome (e.g., Pagon et al., 1981). Now that understanding of the disorder has evolved, a wide range of both cognitive and language functioning is evident for these children.

Our own data show that more than 90% of children with CHARGE syndrome are affected with hearing impairment, and more than 80% experience some degree of visual impairment (Blake et al., 1998; Issekutz et al., 2005). When only one sense is affected, a child may be able to compensate more adequately. However, when there is dual or multi-sensory impairment, compensatory alternatives are greatly reduced, a situation that is further complicated when the child may also have motor and/or cognitive deficits (Sobsey & Wolf-Schein, 1996). Understanding the functional implications of hearing and/or vision and vestibular/balance problems is critical when working with children with CHARGE syndrome. Depending on the extent of impairment of multiple senses, specific consideration for educational programming should be taken.

The degree of visual impairment an individual experiences is measured by both acuity (that is, the ability to perceive detail) and visual fields. Visual acuity is generally measured with an eye chart, and is typically represented as a fraction, although can be represented other ways (Sobsey & Wolf-Schein, 1996). The numerator indicates the distance the individual is from the chart, while the denominator indicates the distance at which an individual with normal sight can read the chart. For example, if an individual has 20/40 (6/12) vision, he or she can read letters at 20 feet that an individual with normal sight can read from 40 feet. That is, the more impaired an individual's vision, the larger the denominator (Sobsey & Wolf-Schein, 1996; see Table 1 for visual impairment definitions). As indicated above, children with CHARGE syndrome often experience visual impairment due to colobomas of the iris and/or retina, which have been considered cardinal features of the disorder. The implications for functional vision depend on the location of the coloboma. For example, a coloboma of the retina may cause deficits in vision. Losses commonly occur in the upper visual field, which can cause children to rely on very limited peripheral vision (Brown, 2005). Such restrictions may have a significant impact on learning in the classroom, and could affect how well information is received from different positions (e.g., writing on the blackboard, signing in front of a bright or distracting background, standing vs. sitting). Interestingly, visual acuity and visual fields are often not affected by colobomas of the iris. Rather, a coloboma of the iris can cause light sensitivity (photophobia), making it difficult for the eye to adjust to bright light. For children with photophobia, sunglasses, visors, or peaked hats are helpful when outside or in a bright room. In addition, environmental factors such as room lighting or the visual contrast in presentation of certain materials may need to be considered (Lewis & Lowther, 2001; McMain et al., 2008). A variety of other visual abnormalities (such as strabismus and reduced depth perception) have also been reported in individuals with CHARGE syndrome (McMain et al., 2008), rendering a comprehensive visual examination a crucial part of the assessment of any child with CHARGE syndrome. Educators will require interpretation of the functional implications of the findings for any individual child.

Table 1

Visual Impairment Definitions, Based on Visual Acuity.

Mild Vision Loss (visual acuity (VA) 6/6-6/18)

Severe or profound problem in one eye only, but other eye is normal.

Moderate Vision Loss (VA; 6/24-6/36)

Able to read print with simple aids and/or education assistance. Usually a defect of at least half visual field (hemianopia). VA may be normal.

Severe Vision Loss (VA; 6/60-3/60)

Unable to read large print without intensive educational assistance or the use of sophisticated aids. Severe visual field defect with impaired VA

Profound Vision Loss (VA; > 3/60)

Very little useful vision. May be able to count fingers, and/or see light perception and hand movements.

Note. Adapted from Blake et al. (2005); Sobsey and Wolf-Schein (1996)

The degree of hearing loss that an individual experiences is characterized in terms of both sound frequency (hertz, Hz) and intensity levels (decibels, dB; see Table 2 for hearing impairment definitions). It is important to note that both environmental factors and the functional use of other senses may influence an individual's hearing (Sobsey & Wolf-Schein, 1996). Two children with CHARGE syndrome whose hearing losses are identical may understand speech differently, depending on factors such as their useful vision and cognitive ability. For example, speech perception is facilitated when one is able to watch the communicative partner's oral and facial movements (MacLeod & Summerfield, 1987; Munhall, Jones, Callan, Kuratate, & Vatikiotis-Bateson, 2004).

Table 2

Hearing Impairment Definitions

Mild Hearing Loss (20dB-40dB)

Able to hear most speech and other environmental sounds, but generally benefit from classroom modifications (e.g., amplification).

Moderate Hearing Loss (41dB-70dB)

Generally require speech, hearing, and language support. Amplification is generally useful.

Severe Hearing Loss (71dB-95dB)

Generally require speech, hearing, and language support. Amplification is only sometimes useful. Often language alternatives are helpful.

Profound Hearing Loss (95 dB or greater)

Typically require oral language alternatives, and intensive language and educational support

Note. Adapted from Blake et al. (2005); Sobsey and Wolf-Schein (1996)

Furthermore, due to anatomical anomalies and physiological dysfunction, children with CHARGE syndrome often have difficulty swallowing, chewing, and making facial movements. These difficulties may impede the development of both speech and nonverbal communication through facial expressions. It is also common for these children to have gastrostomy feeding tubes to help ensure that they receive appropriate nutrition (Blake et al., 1998).

Most children with CHARGE syndrome experience delays in motor development and disturbances in vestibular function due to abnormal development of the inner ear. Many children with CHARGE syndrome do not walk independently until three or four years of age, and may continue to have difficulty maintaining their balance. These motor and sensory difficulties may further restrict the child's ability to explore the environment and thus impose additional constraints on their opportunities for learning (e.g., social engagement with peers, movement around the classroom).

Research has indicated that many school-aged children with CHARGE syndrome demonstrate significant delays in multiple aspects of adaptive behavior (that is, in communication, self-care, socialization and motor skills), and their academic functioning is also often compromised (Smith et al., 2005). Impairment in gross-motor skills (e.g., walking), as well as the presence of sensory deficits, are strong predictors of delayed adaptive functioning in CHARGE syndrome (Salem-Hartshorne & Jacob, 2005).

Challenging Behavior and CHARGE syndrome

As noted above, much of the early research on CHARGE syndrome focused on the children's medical difficulties. However, with improved medical outcomes, families, educators, and health care providers have expressed concerns regarding the challenging behaviors often exhibited by these children. Parents of children with CHARGE syndrome have reported that once their child's medical conditions were stable, psychological and behavioral challenges interfered most with development (Lauger, Cornelius, & Keedy, 2005). For example, concerns have been expressed regarding disruption of such adaptive abilities as formation of reciprocal relationships, complex communication, and self-care. More globally, challenging behaviors compromise the children's experience of educational success.

The relationship between medical factors and behavioral challenges in CHARGE syndrome is unclear (Brown, 2005); findings in this emerging area require cautious interpretation (cf. Vervloed, Hoevenaars-van den Boom, Knoors, van Ravenswaaij, & Admiraal, 2006). Additional research is needed to examine associations between patterns of CHARGE syndrome features and behavioral symptoms. For example, it is unknown to what extent these behavioral characteristics may reflect the impact of neurological differences that also result in deaf-blindness or in CHARGE syndrome. Conversely, it is possible that these behavior patterns may result from children's histories of atypical sensory experiences, or as a consequence of medical interventions and hospitalizations (cf. Lewis & Lowther, 2001). Little evidence is available with which to evaluate these alternatives, and indeed the effects may be additive or interactive. Bernstein and Denno (2005) identified four categories of behavior frequently seen in children with CHARGE syndrome: self-stimulatory behaviors, maladaptive routines/behaviors, tics, and obsessive-compulsive symptoms. Patterns of behavioral symptoms associated with obsessive-compulsive disorder (OCD), autistic spectrum disorders (ASD), attention deficit hyperactivity disorder (ADHD), and Tourette syndrome have all been observed in children with CHARGE syndrome (Bernstein & Denno, 2005; Fernell, Olsson, Karlgren-Leitner, Hagberg & Gillberg, 1999; Hartshorne & Cypher, 2004; Hartshorne, Grialou, & Parker, 2005; Hartshorne, Hefner, & Davenport, 2005; Johansson et al., 2006; Smith et al., 2005). In particular, ASD symptoms may be related to specific neural pathology (involving the brainstem) common to CHARGE syndrome and Moebius syndrome (Johansson et al., 2006). Finally, Hartshorne, Nicholas, Grialou, & Russ (2007) identify executive function deficits (e.g., difficulty with transitioning, using flexible problem solving, monitoring their work, and acting on impulse) in children with CHARGE syndrome, as in many other developmental disorders including ASD. Educators should consider the presence of these behavioral characteristics when developing and implementing educational programs for children with CHARGE syndrome.

Educational Implications

The Individuals with Disabilities Education Act (IDEA) of 1990 and its 1997 and 2004 amendments placed emphasis on education in inclusive environments (Downing & Eichinger, 2008). A great deal of evidence supports the goals of this legislation, indicating that students with multiple disabilities are most successful when included in general education classrooms (e.g., Downing & Demchak, 2008). Although some students with multiple disabilities may appear to have basic needs related to their learning (e.g., simple communication skills, sitting and sustaining focus, manipulation of objects) they can benefit from the learning opportunities that typically occur in general education classrooms. For example, rather then teaching skills in isolation, the classroom provides an age-appropriate context in which to learn (Eichinger, Downing, & Hicks, 2008).

Following an inclusive education model is ideal in theory, although can be demanding to implement; the frequency of such varies from country to country. For example, in the United States, the 2004 Annual Report to Congress reported that only 51.9% of students with disabilities ages 6-21 spent at least 80% of their time in the general classroom. Conversely, in some Canadian provinces almost all students with disabilities spend their time in general education classrooms (Ferguson, 2008). When considering the inclusion of children with CHARGE syndrome, patterns of both physical and behavioral challenges have a number of educational implications. As indicated previously, intellectual disabilities were once expected in all children with CHARGE syndrome (e.g., Pagon et al., 1981). However, it is now apparent that cognitive and language functioning varies considerably in these children and requires careful assessment. It is important to note two key factors with respect to the cognitive abilities of children with CHARGE syndrome. First, they may experience developmental delays secondary to other factors (e.g., repeated illnesses, hospitalizations and surgery, sensory impairment, and feeding difficulties), rather than as a fundamental feature of the condition (Blake & Brown, 1993). Second, the extent of early developmental delay is not a reliable predictor of later intellectual ability, as some children with CHARGE syndrome make large gains once they develop mobility and an effective communication system (Blake et al., 1998).

Thus, although delayed skills are common in early childhood, comprehensive early intervention tailored to the child's specific needs can alter the picture dramatically. Valid conclusions about cognitive functioning are not only best reserved in the early years, but also depend on the use of assessment tools and processes that accommodate the child's sensory and behavioral differences.

Education of children with CS

There is little published literature regarding school-based strategies for children with CHARGE syndrome. This is problematic for parents and educators, both of whom need support in facilitating the learning and development of these children. The existing literature (Griffen, Davis, & Williams, 2004; Jones & Dunne, 1988; Lewis & Lowther, 2001) suggests educational modifications in areas such as visual impairment, communication, social development, and motor development. For example, Lewis and Lowther (2001) and Jones and Dunne (1988) recommended consultation with a teacher who has experience with visually impaired students, modification of the classroom layout, and use of a variety of different teaching materials (e.g., large-print books, textured materials), and specialized equipment (e.g., CCTV equipment), if available.

Lewis and Lowther (2001) identified sensory impairment as the main barrier to communication, based on the impact on the child's expressive and receptive abilities. Their suggested interventions included providing the child with extra time to process information and to attempt communication. Furthermore, they recommended alternative modes of communication (e.g., picture symbols, photographs) augmenting sign and spoken language as appropriate. Griffen et al. (2004) recommended the use of a "communication shelf" for children with CHARGE syndrome who have language impairments, presumably for children who are the most impaired. The shelf is a concrete analogue of a picture schedule, and can be used as a framework for organization of the child's schedule. The technique involves providing, on a shelf, objects that are representative of events or activities in the child's day. Also, for children with limited communication skills, observing the child in order to identify subtle ways in which he or she may be communicating (such as with body language or gestures) may be beneficial (Lewis & Lowther, 2001). These observations may also prove valuable in gathering relevant diagnostic information. For example, for a child with CHARGE syndrome who does not use gesture despite adequate motor and cognitive skills, and who has rigid and repetitive routines, one might consider whether a co-existing autistic spectrum disorder is affecting the child's development. That said, differential diagnosis of specific developmental disorders in children with sensory impairments is complex and difficult (Smith et al., 2005; Wing, 2005).

Lewis and Lowther (2001) further suggested that classroom social skills interventions should consist of group activities aimed at gaining a better understanding of self and others and developing positive social behavior (e.g., learning to initiate interactions appropriately, play skills). To increase motor development and vestibular functioning, strategies focussed on environmental exploration have been recommended, as have sensory integration activities (Griffen et al., 2004).

Literature related to the education of students with CHARGE syndrome is limited in a number of ways. First, opinion rather than evidence is provided as the basis for most suggestions. In particular, no support is provided for the view that the social differences observed in CHARGE syndrome result from deprivation of social experience, although this may indeed be a factor. In light of observed associations between CHARGE syndrome and autism (e.g., Fernell et al., 1999; Hartshorne & Cypher, 2004; Johansson et al., 2005; Smith et al., 2005), at a minimum other explanations need to be considered. Second, suggestions tend to be brief, general, and not to address the underlying principles of intervention. Practitioners working with these children need to understand that recommended strategies are not to be used in a prescribed manner, but rather need to be tailored to the individual child, with consideration of his or her strengths and needs. For example, an individualized assessment of a child will indicate whether his or her functional vision is conducive to the use of pictures or objects in a visual schedule, as well as the optimal presentation conditions. Third, interventions are most often recommended for visual impairments, without consideration of hearing impairment and the different implications of these (Freeman & Groenveld, 2000; Orelove & Sobsey, 1996; Orelove, Sobsey, & Silberman, 2004). In fact, the evidence indicates that hearing impairment, or combined hearing and visual impairment, is most prevalent and has the greatest impact on children with CHARGE syndrome; visual impairment alone is uncommon. Finally, as was the case in medical care (Blake & Brown, 1993), the critical role of collaborative multidisciplinary efforts in the development and implementation of effective educational programming for children with CHARGE syndrome and other complex conditions must be acknowledged.

Given the dearth of literature related to the education of individuals with CHARGE syndrome, it is important to draw on what is known regarding the education of children with multiple disabilities in general.

Education of Students with Multiple Disabilities

There is a large literature base concerning the education of children with multiple disabilities, including deaf-blindness. Drawing on the body of evidence related to the education of these children is valuable, given the high percentage of children with CHARGE syndrome who do have dual or multi-sensory impairments (i.e., hearing, vision, vestibular).

Inclusion of children with multiple disabilities in general education classrooms poses a specific set of challenges, and requires substantial support and collaboration from a variety of sources, including parents, health and education professionals, and peers (Downing, 2008). Parent-professional teams are essential to the child's education and will help to ensure that the child's specific needs are consistently met. Collaborative teaming for children with sensory impairments is most successful when two levels of participants are involved. The first level involves parents, teachers, administrators and specialty school staff (e.g., psychologist, speech-language pathologist). The second level involves specialists and experts outside of the general education setting (e.g., health care providers, sensory or deaf-blind educational consultants) who are available, when needed, for consultation and program planning. The specific composition of a team depends on the individual needs of the child, as well as on the service model that is currently in place within the child's school system. Factors that encourage successful collaboration include establishing parity among team members, conducting in-services specific to the student's condition and needs, maintaining open communication and keeping valued outcomes (e.g., the best interests of the student) at the forefront (Minke & Anderson, 2005).

Children with multiple disabilities in general, and specifically those with deaf-blindness, have unique educational and support needs in the areas of communication, mobility, social skills and hard-to-manage behavior (Downing, 2008; Haring & Romer, 1995). The children may find it difficult to remain seated and focussed if the relevance of the material being taught is not obvious. They tend to learn best when they are actively involved in the learning process and are given many opportunities to perform meaningful tasks throughout their day (Downing & Eichinger, 2008). Identifying ways to teach specific skills in naturally occurring routines (e.g., teaching appropriate social and communication skills during recess or lunch) is one example of how to do this (Eichinger, Downing, & Hicks, 2008). The curriculum for students with multiple disabilities should be based on a developmental model with a large focus on the acquisition of functional skills (Eichinger et al., 2008). Teaching methodology should be evidenced-based, and in the United States, for example, IDEA requires that schools use research-based intervention strategies. These may include such methods as direct and systematic instruction as well as the use of positive behavioral support techniques. Evidence-based teaching strategies such as prompts, corrective feedback, and the use of reinforcement are also recommended (Downing & Demchak, 2008).

In addition to a variety of teaching strategies, adaptations and accommodations are critical in helping students acquire valuable skills. Activities as well as materials can be adapted to enable participation based on students' strengths and needs, including levels of functional hearing and vision (Downing & Demchak, 2008; Udvari-Solner, Causton-Theoharis, York-Barr, 2004). For example, in the area of curriculum, adaptations may include reducing the amount of work the student must complete, or increasing motivational variables (e.g., providing incentives) to help the student better attend. Environmental adaptations are also common and may include adapting the student's seat or proximity to teacher.

Finally, given the broad range of functioning of these children, it is necessary that their education programs are individualized, that is, tailored to both their current abilities and needs. Information from the child's entire support team regarding strengths and vulnerabilities will be essential (Downing & Demchak, 2008). Undoubtedly, some children with CHARGE syndrome require minimal educational supports, and will be included relatively easily, whereas others may require more substantial supports to function successfully within a classroom setting.

The use of functional assessment and positive behavioral support strategies continues to increase as a result of legislative mandates (e.g., IDEA, 2004) and extensive school-wide initiatives (Alter, Conroy, Mancil, & Haydon, 2008). These strategies provide a useful framework for teams collaborating to develop individualized behavior programs for children who have multiple disabilities such as CHARGE syndrome.

Functional Assessment and Positive Behavioral Support

Functional assessment and positive behavioral support strategies are the methods of choice when responding to the challenging behavior exhibited by individuals with developmental disabilities or emotional/behavioral disorders (Alter et al., 2008; Billingsley, Huven, & Romer, 1995; Umbreit, 1997). Research has supported the use of these methods for developing classroom-based interventions because of the explicit link between assessment and intervention. Determining the functional relationship between a problem behavior and its antecedent and consequent events improves the likelihood of selecting an appropriate intervention, and thus having a successful outcome for the child (Gresham et al., 2004; Matson & Minshawi, 2006; Watson, Ray, Turner, & Logan, 1999). Due to the complexity of the needs of children with CHARGE syndrome (e.g., dual or multi-sensory impairments, physical, cognitive and/or communication limitations), it is essential to examine the functions served by specific behaviors in order to implement interventions that produce long-lasting meaningful change (van Dijk & de Kort, 2005). For example, examining the function of a child's disruptive behavior is critical. Often, disruptive behavior serves a communicative function and the child can be a taught a more appropriate replacement behavior (e.g., signing or exchanging a picture to indicate "all done" or "help") that is more effective and efficient than the problem behavior. For further information on how to conduct a functional assessment, see O'Neil et al. (1997). For more specific reading related to functional assessments within classroom settings, see Gresham et al. (2004) and Watson et al. (1999).

Positive behavioral support (PBS) refers to a comprehensive, research-based approach that uses behavioral principles to help promote adaptive behavior for students with challenging behaviors (Albin, Lucyshyn, Horner, & Flannery, 1996; Gresham et al., 2004). PBS plans are often developed based on information gained during a functional assessment and focus on changing setting events, antecedents, consequences and teaching new skills. A key component to successful implementation and follow-through with a PBS plan is to ensure it has good contextual fit. That is, the PBS support plan works well (i.e., is a good fit) for the individuals and environments where it will be implemented (Albin et al., 1996). Consideration of the following three factors help to ensure good contextual fit: 1) characteristics of the individual for whom the plan is designed, 2) variables related to the people who will implement the plan, and, 3) features of the environments and systems within which the plan will be implemented.

Educators often require PBS support plans to integrate children with challenging behaviors successfully in their classrooms. Given the learning and behavioral challenges that some children with CHARGE syndrome face, the implementation of PBS plans to support new skill development may be beneficial. Ruef (1998) suggests a few PBS strategies that are proactive and teacher-recommended. The following six strategies are adapted from Ruef (1998), with examples of applications for a child with CHARGE syndrome.

1. Alter the classroom environment.

Educators can modify their classroom at an individual level (e.g., place desk in proximity to teacher, door, etc.) or at a classroom-wide level (e.g., classroom arrangement) to best suit their students' needs. For example, it may be necessary to alter the classroom arrangement to accommodate a child with CHARGE syndrome who has delayed motor development/planning or a restricted visual field. Additional environmental alterations may need to be considered for a child with CHARGE syndrome. For example, the classroom lighting may need to be adjusted for a child who has light sensitivity, and materials may need to be arranged to be more accessible (Downing & Demchak, 2008). Similarly, technology (e.g., CCTV) may be necessary to enhance written text for a visually impaired child with CHARGE syndrome (Lewis & Lowther, 2001). For a child with hearing impairment, instructional strategies may need to be modified (e.g., increase the use of visual supports) as well as specific classroom arrangements (e.g., position of child, carpets to minimize external noise; Downing & Demchak, 2008), and other technology (e.g., sound amplification systems) may be required.

2. Increase Predictability and Scheduling

Educators can implement strategies to increase predictability and scheduling within their classroom at an individual level (e.g., an object or picture schedule illustrating a student's daily routine) and at a classroom-wide level (e.g., a class schedule). These tools help reduce anxiety and promote better learning for students who experience difficulty with change or transitions. They may be particularly helpful for those individuals with CHARGE syndrome who have limited communication and cognitive abilities. For example, a child with CHARGE syndrome who has limited verbal language, is deaf, but has some useful vision, could follow a visual schedule. Likewise, a deaf-blind student with CHARGE syndrome may benefit from a communication shelf (Griffin et al., 2004). These suggestions can help with prediction of daily routines and with transitioning between activities.

3. Increase Choice-Making

Individuals with special needs, particularly those with limited motor or verbal skills, are often not provided with choices; rather, they are told what to do. This can increase the likelihood of challenging behavior. Educators should examine the daily schedule and provide their students with opportunities to make choices throughout the day within their classroom routines. For example, a child with CHARGE syndrome can choose which activity she or he wants to complete first, what incentive he or she is working toward, and whether his or her break should be five or six minutes.

4. Make Curricular Adaptations

It is important for educators to make curricular adaptations for their students with special needs to prevent and reduce frustration. For example, a teacher of a student with CHARGE syndrome would modify programming given his or her cognitive abilities and strengths and needs. As well, for a student with CHARGE syndrome who has vision and hearing limitations, it is important to provide time to solidify and process what is often fragmented information. As a final example, for a student with CHARGE syndrome, consideration of his or her adaptive behavior and functional skill set will be important, and goals related to these may need to be incorporated into the curriculum (e.g., independent dressing, eating, toileting).

5. Teach Replacement Skills

Many students with special needs exhibit challenging behaviors because they do not have a more appropriate means to achieve an outcome. For example, a student with CHARGE syndrome may engage in disruptive behaviors to escape or avoid the completion of a task. He or she may be taught to give a 'break' card to the teacher, which is a more appropriate, alternative behavior that serves the same function. Teaching appropriate replacement skills (determined through functional assessment) will likely reduce the incidence of challenging behavior, and increase the student's skill set.

6. Appreciate Positive Behaviors

Many students with special needs have limited skills (e.g., social, behavioral) that facilitate success in the classroom. Thus, it is important for educators to notice and reward students for their positive behaviors, helping them to learn the appropriate way to behave. For example, a teacher of a student with CHARGE syndrome could praise a child for communicating with a visual picture that he or she needs a break, rather than reacting to his or her disruptive attempts to escape the task. Similarly, acknowledgement or praise for on-task behavior can be provided for a student with CHARGE syndrome who has difficulty sustaining attention and focus.

We illustrate these principles with a brief description of the results of a school-based behavioral consultation for a youth with CHARGE syndrome.

Case Illustration

David (a pseudonym) was an older adolescent who exhibited many major features of CHARGE syndrome including coloboma of the eye, heart defect, choanal atresia, ear anomalies, and small stature, as well as a cleft palate and facial paralysis. Among children and youth with CHARGE syndrome, David was quite severely affected. He had profound sensory-neural hearing loss (but often refused to wear his hearing aids) and in addition, moderate-to-severe visual acuity deficits, as well as restricted visual fields bilaterally. These sensory impairments had a serious impact on David's ability to gain information from his environment. However, when single letters or shapes were presented within his intact field with good contrast and no visual clutter, David was able to see 12-point print. He was able to walk independently and to navigate obvious barriers (e.g., furniture). Therefore, it was apparent that David, like many students with CHARGE syndrome, required many supports and accommodations to help him appropriately function and learn within a classroom setting.

David attended a regional high school as one of four students in an adapted classroom with facilities for functional skill development (e.g., kitchen, laundry). The other students were non-communicative and without independent mobility. Similar to many other individuals with CHARGE syndrome, David had a history of challenging behaviors (e.g., kicking, biting, scratching) which affected his participation at school. David spent a lot of time in the classroom completing table-top activities such as puzzles, sorting objects, and tracing; programming that used principles of direct and systematic instruction. Going for walks within the school, sitting in a comfortable chair, and folding laundry were other more common activities for David. Thus although his classroom, school, and the surrounding community provided opportunities for functional skill development, it was evident that not enough time was spent teaching meaningful, functional skills (e.g., Downing & Eichinger, 2008).

David's school-based team included his classroom teacher and educational assistant, and school administrators, as well as consultants from a regional educational agency serving students with sensory impairments. These consultants visited frequently and provided an abundance of materials and resources for David and the team. Despite support and collaboration from these resources, school personnel had often attempted to address David's problem behaviors using consequences that were intended to be negative procedures (time-out or send home), but perhaps were not to him.

The school-based behavioral consultant became involved to help David's team conduct a functional assessment to better understand the purpose of his disruptive behaviors and to help them to plan and implement more appropriate interventions based on PBS strategies. Disruptive behaviors impede learning and compromise a student's experience of educational success. As indicated above, research strongly suggests the use of functional assessment and PBS strategies when responding to the challenging behavior of individuals with multiple disabilities (e.g., Alter et al., 2008). A formal functional assessment identified several setting events including such factors as David's sleep patterns and various health concerns, and two summary statements were developed:

1. When David does not comprehend task demands, or when structure is insufficient, he will exhibit problematic behaviors. Following this, David is generally re-directed or given a negative consequence procedure (e.g., time-out, sent home).

2. When David has difficulty with a task or activity, he will exhibit problematic behaviors to escape/avoid them. Following this, David is generally re-directed or given an intended negative consequence procedure (e.g., time-out, sent home).

Target behaviors were identified based on the Functional Assessment Interview. The objectives were to decrease the specified challenging behaviors (biting, kicking, scratching), increase positive functional behaviors (communication), and to monitor several behaviors (jumping, teeth grinding, visual self-stimulatory behavior) the functions of which were unclear.

School personnel were given both general and specific information about PBS strategies for David. One suggestion included introducing more structure and routine to David's daily activities through the use of visual supports throughout the classroom environment and embedded within his learning. Literature on the education of children with multiple disabilities (e.g., Downing & Demchak, 2008) indicates that visual supports enhance receptive understanding and therefore better allow students to anticipate events and facilitate their learning. Their use is often associated with a reduction in disruptive behavior.

Additionally, it was suggested to provide David with a greater variety of functional and interesting activities and to ensure that his educational program was comprehensive (especially incorporating more daily living skills). As indicated previously, highly relevant material and frequent opportunities to perform meaningful tasks, as well as inclusive community-based activities, benefit children with multiple disabilities. David's communicative and cognitive limitations suggested that these functional skills, rather than academics, should have been the focus of his education program.

A final recommendation, based on the outcome of the functional assessment, included teaching key communicative behaviors such as requesting 'wait', and 'help' through the use of visual supports, while avoiding the use of procedures that may have inadvertently reinforced David's inappropriate behavior (e.g., being sent home or given a time-out). The hypothesis was that once David learned new communicative skills his disruptive behavior would diminish. All of these topics were known to have been discussed previously with the school personnel by the external consultants, and as indicated in the literature cited above should be considered when programming for individuals with multiple disabilities.

Following the implementation of positive behavioral support strategies with coaching from the school-based behavioral consultant, the school team observed positive changes in David's behavior. In post-intervention interviews, they reported that examining David's environment had been useful, enabling them to prepare an environment around his individual needs. They expressed that engaging David in more functional activities was better for him because it gave him a sense of purpose. Often if David started the day with a functional activity, he would be more settled to do routine table work later on. Consistency was another component that school personnel found useful. They reported that David required routine and boundaries, and without consistency, his behavior was more challenging. David's independence had increased; they noted that he could now walk independently throughout the halls in the school. Additionally, they indicated that he independently initiated the sign 'yes' (previously used only after modelling), as well as spontaneously requesting 'bathroom,' 'walk,' and 'break' through the use of visual symbols. School staff reported that David appeared more motivated and excited because his activities were more interesting, and that he was correspondingly easier to calm down if he became agitated. David responded well to the use of visual supports throughout his environment, and on a "good" day he would use these independently. Challenging behavior decreased in frequency, although staff expressed the view that difficult behavior would always be present to some degree. In particular, mealtimes (i.e., snack and lunch) were still problematic, as David continued to exhibit challenging behavior such as throwing food and refusing to eat. In contradiction to the plan emerging from the consultation, the school continued to give "time outs" and to send David home following disruptive behaviors. Continued use of such consequences may have maintained some of David's problem behaviors. Ongoing education and support of school teams may be necessary to maintain the consistent use of positive behavioral support strategies.

Conclusion

This paper has presented an overview of basic information about the development and behavior of children with CHARGE syndrome. CHARGE syndrome is a complex congenital disorder typically involving multiple sensory systems, as well as other developmental differences. A Positive Behavior Support framework for inclusive educational practices was provided, with particular reference to educational needs and the potential for challenging behavior. In addition, readers were directed to a variety of written and web-based resources relevant to the education of children with CHARGE syndrome. Armed with these fundamentals, educators are encouraged to devise strategies that merge an understanding of the specific strengths and challenges of individual children with CHARGE syndrome with general knowledge of best practices in inclusion. A case illustration exemplified this process. Such a solution-focussed approach promises to enhance the experiences of both students with CHARGE syndrome and educators.

Resources Box 1

CHARGE Syndrome

* The CHARGE Syndrome Foundation http://www.chargesyndrome.org

* CHARGE Syndrome Canada http://www.chargesyndrome.ca

Functional Assessment and Positive Behavior Support

* Association for Positive Behavioral Support: http://www.apbs.org/main.htm

* Special Connections (see Teacher Tools): http://www.specialconnections.ku.edu

* http://www.kent.ac.uk/tizard/subscribernet/positivebehavioralsupport.doc

* http://www.centerforautism.com/Resources/ChallengingBehaviorMayl6_07Rochester.pdf

* Book: Functional Assessment and Program Development for Problem Behavior: A practical handbook. Edited by R. E. O'Neil, R. H. Horner, R. W. Albin, J. Sprague, K. Storey, J. S. Newton--2nd ed.

* Book: Parenting with Positive Behavior Support: A Practical Guide for Resolving your Child's Difficult Behavior. By M. Hieneman, K. Childs, J. Sergay.

* Book: Positive Behavioral Support: Including People with Difficult Behavior in the Community. By L. K. Koegel, R. L. Koegel, G. Dunlap.

Education of Children with Multiple Disabilities

* Book: Educating Children with Multiple Disabilities: A Collaborative Approach. Edited by Fred P. Orelove, Dick Sobsey, Rosanne K. Silberman--4th ed.

* Book: Including Students with Severe and Multiple Disabilties in Typical Classrooms: Practical Strategies for Teachers. Edited by June E. Downing--3rd ed.

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Katherine G. Smith

IWK Health Centre

Isabel M. Smith & Kim Blake

IWK Health Centre

Dalhousie University

Correspondence to Dr. Isabel Smith, Dept. of Pediatrics, IWK Health Centre, 5850 University Ave., PO Box 9700, Halifax, NS, Canada B3K 6R8; e-mail: Isabel.Smith@iwk.nshealth.ca.
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