Medical Syndromes in Special Education Populations Research Paper

Introduction

Many special education students are found to suffer from a host of medical syndromes, most of which are genetic disorders that are diagnosed by “the specific constellation of physical anomalies apparent to the trained eye” (Reynolds et al, 1987, 1565). Special educators are most directly concerned with those physical anomalies that can limit an individual ability to have a normal social and family life. These physical anomalies can happen in the following categories: visual, hearing and physical handicaps, health problems and mental retardation. Hence medical syndromes that cause such physical anomalies are especially relevant to the field of education. Some such syndromes include Down syndrome, Prader-Willi syndrome, William’s syndrome, Asperger’s syndrome and Tourette’s Syndrome. According to the Individuals with Disabilities Education Act (IDEA) 2004, the education environment provided to a student with a medical syndrome must be based on the educational needs of a child not a specific diagnosis (Fetsko, 2008). Due to recent advances in the fields of genetics and child psychiatry, etiology-based interventions are becoming increasingly possible for children with different genetic mental retardation conditions especially in the field of special education. Hodapp and Fidler (1999) say that though such interventions are still in the primitive state in special education work, there is much promise of these interventions becoming more and more developed in the years to come. This paper examines the etiology, symptoms, learning/educational implications, and the implications for educational intervention for various medical syndromes in the special education population.

Down’s syndrome

Of all medical syndromes, the Down syndrome is the most common genetic (chromosomal) cause of mental retardation with a prevalence of 1 in 700 to 1,000 (Rozien, 1997). All cases of Down syndrome result from partial or complete duplication of chromosome 21 in the genome. About 95% of the cases arise due to standard trisomy, involving duplication of chromosome 21. Over 90% of these cases are genetic in nature, and the extra chromosome is of maternal origin due to non-disjunction during meiosis. However there may be other rare causes such as the translocation of chromosome 21 material to another chromosome (usually 13 or 18) and mosaicism (transmission of a cryptic trisomy 21 cell line from an unaffected parent (Wallace et al, 2007). One of the most striking epidemiologic characteristics of Down syndrome is the marked increase in risk with increasing maternal age – from approximately 1 per 1400 live births at ages 20-24 to 1 per 600-700 live births at ages 41-45. Increased availability of prenatal diagnosis followed by therapeutic abortion have reduced the number of infants born with Down syndrome. But the increased longevity of persons with Down syndrome has lead to a rise in the prevalence of Down syndrome in adolescents and adults (Wallace et al, 2007).

Most persons with Down syndrome have a number of physical and behavioral symptoms including a characteristic facial appearance and frequent heart and respiratory problems (Rice and Warren, 2004). Most individuals with Down syndrome function in the mild to moderate range of mental retardation, displaying IQs between approximately 35 and 70 (Chapman & Hesketh, 2000). Their cognitive profile shows a weakness in auditory short-term memory relative to visual short-term memory and other aspects of cognition (Chapman, 2006). Special education students with Down’s syndrome show difficulty in acquiring the forms and contents of language and the extent of the difficulty depends on the domain of language (e.g., vocabulary or syntax) and modality (i.e., receptive or expressive) (Rice and Warren, 2004). Fidler et al (2002) point to the fact that children with Down syndrome display specific problems in linguistic grammar, expressive language, and articulation and find it difficult to progress grammatically beyond the 3-year level. Similarly, they also have delayed skills in expressive language, as opposed to those in receptive language (Fidler et al, 2002). Conversely, they have high levels of visuospatial short-term memory that are one or more years advanced over identical auditory or verbal short-term memory tasks (Hodapp, Evans, & Gray, 1999). A special education teacher must provide a positive environment for the student afflicted with Down’s syndrome and must have honest two-way communication with parents and family of the student. The focus on the teaching intervention must be on reading as it helps the child to visualize language and overcome difficulties associated with listening. As the child has more time to process the text while reading, it is easier for it to read than to understand what is said. Speech processing is a short-term stimulus which can be lost if the child’s auditory short-term memory is not very effective. Reading also helps the child to understand syntactical rules, word morphology and grammar and it has a profound effect on the child’s self esteem, independence and quality of life. Children with Down syndrome have difficulty writing or talking because of difficulties in organizing thoughts and transferring it on paper or through speech and difficulty in sequencing words to form a sentence. Their have good social interactive skills but poor speech sound production. Moreover, language learning is hindered by the fact that children with Down’s syndrome often have hearing difficulties. The slow rate of language learning affects progress in all aspects of cognitive development – particularly learning, remembering, thinking and reasoning. The best learning intervention for them would be one that would encourage them to be social and communicate with others through words, signs and gestures. Buckley, Bird, and Byrne (1996), understanding the strength of these children in visual perception have suggested that reading, as much as hearing, is a crucial device for learning new words in children with this syndrome. They suggest that educators might capitalize on these children’s “visual reading” and sight vocabulary (the ability to recognize certain whole words without sounding them out) (Hodapp and Fidler, 1999). It is important they are not grouped together and isolated because that would retard their learning process. Vocabulary can be built through games and specific activities. Articulation and phonology should be targets for intervention from the first year of life right through to adult life. Grammar will be acquired with increase in vocabulary. The evidence from available research does suggest that improving the quality, quantity and sensitivity of the talk to children with Down syndrome will help them with language learning especially if it is accompanied by teaching specific vocabulary and sentences. Dodd and colleagues in their study have found that using targeted words and using groups for intervention work with parents and children are beneficial. In their study they targeted whole words and then sentences, worked with 10 functionally important words chosen in discussion with parents and noticed a dramatic improvement in the children’s phonology after just 12 weeks. Groups provide a mutually supportive atmosphere in which parents are more responsive, and more likely to change attitudes and learn new procedures.

Prader-Willi Syndrome

First described in 1956 by Swiss physicians A. Prader, A. Labhart and H. Willi, Prader-Willi syndrome (PWS) are a complex disorder and a rare birth defect. Roughly 60% to 70% of people with PWS have a partial deletion of a section of the long arm of chromosome 15. PWS affects about one in every 10,000 to 20,000 individuals (Butler, 1990). Prader-Willi syndrome is caused by missing genetic material from the Chromosome 15 derived from the father (either a deletion on the paternally derived 15 or two Chromosome 15s from the mother). The main clinical features included poor muscle tone during infancy with improvement by 9 months of age and obesity with onset between 6 months and 6 years of age, with an average age of onset by 2 years of age (Thompson et al., 1996). The most outstanding characteristic of PWS is the individual’s constant preoccupation with food and the obsessive desire to be eating all the time (hyperphagia). They may even eat spoiled meat, rotten vegetables or cat food and indulge in behaviors such as foraging, stealing or gorging food (Reynolds et al, 1987). Though people with PWS have a developmental disability, they do not necessarily have mental retardation (Thompson et al, 1996). Many children with Prader-Willi syndrome have been found to be weak in tasks involving sequential processing, but strong on tasks that involve simultaneous processing. Dykens, Leckman, and Cassidy (1996) in their study involving 100 individuals with Prader-Willi syndrome, found that 94% showed obsessions (thoughts) and 71% showed compulsions (behaviors). Combined with overeating (98%) and temper tantrums (88%), these children showed extremely high levels of maladaptive behavior (Dykens and Kasari, 1997).

Educational intervention for individuals with PWS should being in early childhood with a program that supports parents and children in managing eating behaviors. The classroom must be rid of all food including pet food and using alternate reward and reinforcement systems other than food reinforcers must be instituted (Reynolds et al, 1987). Physical activity should be encouraged and academic weaknesses should be addressed as well. Particular attention should be paid to eliminate or modify temper tantrums or extreme stubbornness using a behavior-modification approach (Cassidy, 1984). Academically, children with PWS have better reading abilities than arithmetic abilities. A subsequent analysis of 232 people with PWS conducted by Greenswag (1987) found that 75% of the participants had received special education services. These persons typically performed at the sixth grade level or lower in reading and the third grade level or lower in mathematics indicating clearly that they acquired reading skills easier than numerical skills.

Williams Syndrome

Williams Syndrome (WS) is a rare genetic condition, first identified in 1961 by Williams and his colleagues (Williams, Barratt-Boyes, & Lowe) and sometimes known in Europe also as Beuren’s Syndrome. Children with Williams Syndrome exhibit several medical anomalies: mental retardation, facial dysmorphology (characteristic elfin like face), abnormalities of the cardiovascular system, as well as of the renal, musculoskeletal, endocrine, and other organ systems. Generally they are born underweight and have frequent vomiting during the first few months. WS children also suffer from an unusual sensitivity to certain environmental sounds (hyperacusis), and they are extremely social, showing marked friendliness to adults including strangers. Genetically, Williams syndrome is “a sporadic, autosomal dominant disorder with submicroscopic deletion on the long arm of chromosome 7 affecting about 20 genes” (Boller et al, 2000, 131). Due to variations in the deletion pattern, the phenotypic outcome varies. The deletion revolves around the gene for elastin and this abnormality is responsible for the classic cardiovascular anomalies typical of the disorder. Children affected with Williams syndrome also have a distinctive pattern of brain anomalies: they have good language /verbal and face recognition skills but a diminished IQ and severe deficits in visuo-spatial processing. Older children are strong in expressive language skills especially those involved with the use of grammar and morphosyntactic rules. They face difficulty in the lexical and semantic features of speech. Additional characteristics of Williams’s syndrome include over friendliness, affective speech patterns and symptoms of ADHD (Boller et al, 2000). The incidence of this syndrome is very low, approximately 1 in 20,000 to 1 in 50,000 live births (Greenberg, 1990), and until very recently it often went undiagnosed until adulthood. As WS is characterized by language delay and a number of other language problems, speech language therapy (SLT) is recommended for virtually all children with WS (Semel and Rosner, 2003). Besides trying to rectify language problems and develop language skills, speech language therapy can also help the students perform better in other areas such as word finding, relational terms, and figurative speech, and with syntactic problems of personal pronouns and complex sentence forms. Students with WS have a wide range of language abilities, deficits, problems, and difficulties. Hence it is best to have customized interventions based on their psycho-educational profiles. Many of the children with WS tend to be responsive to verbal mediational techniques, especially dramatization, storytelling, and self-instruction. These children are very cooperative and amenable to work and definite signs of progress can usually be observed by SLTs, parents, teachers, and others depending on the severity of the problem, use of apt techniques and experienced providers (Semel and Rosner, 2003). Keeping in mind the short attention span of individuals with Williams’ syndrome, Reynolds et al (1987) make the following suggestions: flexibility in study timings; frequent breaks in work time; a high motivation curriculum, minimal distractions, rewards for attending behaviors, providing some autonomy in activities and encouraging group activities.

Asperger’s Syndrome

Asperger’s syndrome is basically understood as autism with normal intelligence. It was first recognized by Hans Asperger of Austria in 1944 but only in 1994, it has been accepted as a specific developmental disorder by the American Psychiatric Association (APA) only in 1994. Individuals with AS lack appropriate social skills, have a limited ability to take part in reciprocal communication, and do not seem to understand the unwritten rules of communication and conduct. They “perceive the world differently from everyone else” (Attwood, 1998, 9). The essential features of AS include qualitative impairment in social relationships, impairment in verbal and nonverbal communication, and a restricted range of interests or lack of flexibility in thought. In addition they may exhibit: insistence on sameness, poor concentration, poor motor coordination, academic difficulties, and emotional vulnerability. They may also lack common sense and be unable to focus on the relevant stimuli. Children with Asperger’s syndrome face academic difficulties because they lack higher level thinking and comprehension skills and tend to be very literal (Williams, 1995). Comprehension is frequently at the factual level. They also lack problem solving skills and face difficulty in generalizing knowledge and skills (Barnhill, 2001). Students with Asperger’s syndrome are likely to go unnoticed because of their pedantic style, advanced vocabulary, and parrot-like responses (Myles & Simpson, 1998). However they need to be taught in a way that is compatible with the way they learn and process information. Students cannot handle oral presentations well as they have problems with auditory input. Moreover, they cannot process visual and auditory information simultaneously. They may require additional processing time in order to understand words used in the context of sentences and paragraphs (Myles & Simpson, 1998). It is suggested that the educational intervention should be based on social skills training, social stories, and structured teaching. One published report of a social skills program for individuals with AS (Marriage, Gordon, & Brand, 1995) in New Zealand suggests the use of role playing of common social interactions (greeting, conversational skills) using videotape for feedback, “show and tell” of special interest areas, and cutting out pictures to help identify emotions in cooperative groups. Parents viewed the program positively and the authors felt that the “show and tell” activity as most effective. Social stories are a strategy found to be useful in the case of younger children with AS and those with severe language delays. Here, illustrations paired with simple sentences are designed to facilitate understanding of situational social cues. Swaggart and Gagnon (1995) found that when social stories were coupled with social skills training, rates of aggression and ignoring were reduced; sharing and greeting behavior were increased. Structured teaching involves using a set of routines, schedules, physical organization of material, visual boundaries defining space, etc to facilitate learning. There are two critical actions that should be initiated by practitioners. Stephen S. Safran (2001) suggests that all students at risk of AS must be screened using either the Autism Spectrum Disorders Screening Questionnaire (Ehlers et al., 1999) and those identified of suffering from AS must be referred to a mental health professional as early as possible. Educators must inform families with care and sensitivity.

Tourette’s Syndrome

According to the Diagnostic and Statistical Manual of Mental Disorders (APA, 2000), Tourette’s syndrome is a neurobiological disorder that is characterized by involuntary motor and vocal movements and sounds, called tics (Prestia, 2003). Tics can be significant academic, social-emotional, and physical challenges and may lead to social isolation. Approximately 1 in every 2,500 individuals have TS, with boys diagnosed more frequently than girls (LinguiSystems, 1999). Generally ticks are first noticed in early elementary-age children (Bronheim, 1991) and the most common tics are eye blinking, head or arm jerking, throat clearing, or sniffing (Prestia, 2003). As the child develops and matures, tics may become more complex and may appear in facial gestures or movements that imitate others (LinguiSystems, 1999). Tourette’s syndrome is a lifelong disorder with an unpredictable course (APA, 2000) and many people suffering from TS are found to have learning disabilities, obsessive-compulsive disorder, attention-deficit/hyperactive disorder, or Asperger syndrome (Ottinger, 2003). Studies show that many students with TS have average or above average IQs. Still, approximately 40% of individuals with TS are reported to have learning problems (LinguiSystems, 1999). They may show incomplete work, poor quality of written work, be inattentive and disorganized in class or have difficult understanding verbal instructions. An Individualized Education Program (IEP) developed by a multidisciplinary team is the best intervention for a child with Tourette’s syndrome. Prestia (2003) suggests that the assignments should be broken down into smaller sections and if the student has problems in writing, the questions should be framed in multiple choice format or the student may be allowed to use a computer. Tourette children may need a private study area, extra time to complete assignments, privately held exams, or oral exams if the child has difficulty writing (Zamula, 1988). Reading disorders are six to seven times more common in TS children than in the general population, so timed tests often throw them into a panic (Zamula, 1988). Hence care should be taken to eliminate distractions, timed tests, and to make lessons more visual and give limited homework depending on the student’s rate of task completion. Students suffering from TS will have problems interacting with and being accepted by peers. To create a cooperative environment it is important to inform peers and faculty about TS and help the student make social bonds with peers. (Prestia, 2003) Direct teaching of basic social skills must be incorporated into the weekly curriculum plan. Moreover, the problem of tics has to be handled. Students may be given physical assistance in tasks such as toileting, eating lunch, or putting on a jacket when they face problems in executing them due to the interference of tics. The academic and social success of students with neurological disorders is interdependent and it is important that the social emotional needs of a student are met before his academic needs (Prestia, 2003).

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