Needs of a Child With Tourettes Syndrome - Research Proposal Example

Tourette syndrome Introduction Tourettes syndrome is described in much of the literature as an inherited tic disorder. (Merck, 2001). However, other recent literature indicates that only 50 to 70% of the diagnosed cases are hereditary (Meyers, 1998). Research suggests the remainder of the cases, acquired Tourettes syndrome (or Tourettism), might be related to several things including environmental, infectious, and psychosocial factors. Stell, Thickbroom, and Mastaglia (1995, p.729) relate Tourettism to "inflammatory, toxic, metabolic, and structural lesions of the central gray matter of the brainstem." Meyers (1998) reports "a growing number of children have been identified with stimulant induced TS. That is, stimulant medications commonly prescribed to hyperactive children (e.g., Ritalin, Cylert, Dexedrine, etc.) can sometimes precipitate TS in predisposed children, according to the Food and Drug Administration."(p.4) This condition is neither degenerative nor terminal (Meyers, 1998). Diagnosis of TS To be diagnosed with TS, a person must present with both motor tics and vocal (phonic) tics. The motor and vocal tics do not necessarily occur concurrently. (Chouinard & Ford, 2000; Marneros, 1983). Tourettes syndrome symptoms fall along a continuum from barely noticeable to blatantly noticeable and debilitating. Frequency of tics may range from those who tic only when they are anxious or fatigued to those who might tic as much as 30 to 100 times per minute (Murray, 1997). For many years clinical interventions and research focused almost exclusively on people with the most severe cases of TS. This focus may have created a distorted perception of the TS population, i.e., TS was thought to be a very rare condition that a practicing physician might see only once throughout his or her practice of medicine (Zinner, 2000). However, It is now recognized that most cases of TS are in the mild to moderate category. Many people affected by TS may never seek medical intervention (Hendren, 2002). Tourettes Syndrome There is a child in your class who is very perplexing. She is bright, friendly, anxious to please, generally well-behaved and polite. However, for no apparent reason, she disrupts the class with snorting noises. She also blinks her eyes constantly, even though the eye doctor says she doesnt need glasses. She also persists in jumping around in her seat. You have spoken to her and her parents about her behavior, but she has persisted. You wonder: Is she looking for attention because her parents have recently separated? Is she unusually anxious about something? Does she have some emotional problem that is not obvious? Finally, someone suggests to you that the child may have Tourette Syndrome. The symptoms of Tourettes syndrome (TS) have been reported since antiquity. The syndrome was first documented in the 1400s; however, the diagnosis at that time was possession by the devil. One of the earliest accounts, written by two Dominican monks in 1489, concerned a priest who suffered from multiple complex motor and vocal tics (Lohr and Wisniewski 1987, p. 175-179). In all his behavior he remained a sober priest without eccentricity, except during the process of any exorcisms; and Tourettes Syndrome when these were finished, and the stole was taken from his neck, he showed no sign of madness or any immoderate action. But when he passed any church, and genuflected in honor of the Glorious Virgin, the devil made him thrust his tongue far out of his mouth; and when he was asked whether he could not restrain himself from doing this, he answered: "I cannot help myself at all, for so he uses all my limbs and organs, my neck, my tongue, and my lungs, whenever he pleases, causing me to speak or to cry out; and I hear the words as if they were spoken by myself, but I am altogether unable to restrain them; and when I try to engage in prayer he attacks me more violently, thrusting out my tongue." (Lohr and Wisniewski 1987, p. 191) Many theories of causation were proposed between 1886 and the 1950s. Some pointed to psychological dysfunction or inflammatory disease (Lohr and Wisniewski 1987). Currently, theories and treatments tend to fall into the psychiatric, neuropsychological, biochemical and educational realms, far removed from religious theories and treatments. Since 1885, when Gilles de la Tourette described this disorder, made up of multiple tics, phonic and verbal productions, and behavioral changes, there has been a cascade of research studies. The result has been a clearer appreciation of the phenomenology, natural history, etiology, physiology, and treatment of TS. "Tic," the central word in TS, derives from the word ticque, which originally was used in veterinary medicine to describe the movements made by horses when restrained. The term did not enter the medical literature until the middle of the nineteenth century. By the start of the twentieth century, the term was used to refer to convulsive, inopportune, and excessive movements (Mikkelson, Detlor, & Cohen, 1981). Currently, the word "tic" is used to describe a variety of involuntary movements extending from simple muscle contractions to complex spoken phrases. The major features distinguishing tics from other conditions are the rapid and transient nature of the movements, the lack of evidence of underlying neurological disorder, the disappearance of tics during sleep, and the ease with which they may be voluntarily reproduced or suppressed. Their rapidity and lack of rhythmicity differentiates tics from the stereotyped or manneristic behavior seen in some autistic and mentally retarded children (Corbett and Turpin 1985) The tics of TS seem to increase as a result of tension or stress and decrease with relaxation or concentration on an absorbing task. Some Facts Prevalence While the incidence of TS is low (estimates range from 0.0003 to 0.5 percent among schoolchildren), transient tic behaviors are common among children, especially between the ages of 7 and 11 years. An estimated 25 percent of children between 6 and 12 exhibit transient tics, and 5 percent have been estimated for tic occurrence between the ages of 7 and 11. Mild tics occur in about 10 percent of children at some stage of development (Corbett and Turpin, 1985). Thus, while tic behaviors are fairly common among children, tics that meet the defining criteria for TS are not. Gender Incidence is greater in boys than girls. There appears to be a male to female ratio of about two to one. Onset If the disorder is going to appear, it almost always does so before age 21, and sometimes as early as two years of age. The norm is from six to seven years of age. The most common first symptom is a facial tic, such as rapidly blinking eyes or mouth twitches. For some individuals, the disorder begins abruptly with multiple symptoms of movements and sounds; throat clearing and sniffing or tics of the limbs may be the initial signs (Hendren, 2002). The factors that influence the continuity of tic disorders from childhood to adolescence to adulthood are not well understood. There is probably an interaction between maturation of the central nervous system and emotional distress along the maturational course. Prognosis When TS was first identified, the prognosis was thought to be poor, and the majority of cases were assigned to long-term hospitalization. Today, the outlook for TS generally is considered to be good; most individuals experience their worst tic symptoms between nine and 15 years of age. More recent studies suggest a more favorable outcome. Improvement appears to be related to age of onset and initial severity. From the perception that TS was a rare eccentricity of nature at the turn of the century, we have moved to appreciate a now common, frequently mild disorder compatible with a full, rewarding life (Glen Hendren, 2002). In summary, TS is understood generically to be a complex tic disorder with a lifelong course. A triad of components is necessary to make the diagnosis; the presence of generalized tics and involuntary utterances that may be obscene or suggestive, onset in childhood and a course that involves a fluctuation of signs throughout the life span but typically is not severely disabling. TIC DISORDER Diagnosis Despite understanding that TS is, essentially, a neurological condition, diagnosis is made by observing symptoms and evaluating the history of their onset. No blood analysis or neurological testing exists to diagnose this disorder. Some physicians order an EEG, MRI, CAT scan, or certain blood tests to rule out other ailments that might be confused with TS. Rating scales are available to assess tic severity (Tourette Syndrome Association 1994). Definition. TS are a disorder characterized by tics, which are involuntary, rapid, sudden movements or vocalizations that occur repeatedly in the same way. The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a span of more than one year. First, both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently (Mikkelson, Detlor, & Cohen, 1981). MULTIPLE MOTOR TICS Second, the tics occur many times each day (usually in bouts) nearly every day or intermittently throughout a period of more than one year, and during this period there has not been a tic-free period of more than three consecutive months. Tics can occur at various times. The frequency ranges from a few tics per week to over 90 tics per minute (Lohr and Wisniewski 1987; Leckman et al. 1994). The forcefulness of motor tics and the volume of phonic tics vary from behaviors that are not noticeable (a slight shrug or a hushed guttural noise) to strenuous displays (arm thrusts or loud barking) and exhausting (Leckman et al. 1994). Genesis of TS Numerous factors have been considered in the genesis of TS. Eight of these are described here. First, temperamental factors have been mentioned together with a history of related behavioral symptoms, such as hyperactivity, impulsiveness, and increased muscle tension and reactivity. These symptoms may indicate an underlying temperamental vulnerability to TS. Second, precipitating factors also have been noted. Varied stressors, such as seeing a pet dog run over, sexual assault, serious home conflicts, serious frights, and acute physical conditions, have been mentioned. In most cases, no acute factors have been evident, but chronic stress is seen in a large proportion of cases. Acute factors seem more justified in cases of simple tics (Peterson, 1996). Third, familial and genetic factors have been cited. Studies suggest a dominant gene; a high proportion of first-degree male relatives show chronic tics. Cases of TS have been found in mothers and sons, uncles and nephews, identical twins, and among sisters. About 30 percent of persons with TS have a family history of the disorder (Lohr and Wisniewski 1987). One study (1987) found (through longitudinal study) increased rates of tic disorders among children having first-degree relatives with TS. Fourth, neurological factors have been implicated. About half of TS patients showed soft neurological signs, and eight out of 15 children with TS showed soft neurological signs. TS are a neurological disorder, specifically a defect in the basal ganglia. Fifth, brain chemistry has been implicated. Current research presents considerable evidence that TS stems from the abnormal metabolism of at least one brain chemical, the neurotransmitter dopamine. Other neurotransmitters, such as serotonin, may be involved ( Tourette Syndrome Association 1994). Physical structures in the brain may change during behaviors associated with TS. Sixth, speech and language difficulties have also been implicated. Recent research has focused on linguistic aspects of vocal tics, suggesting that such tics do not occur randomly but are located according to the clausal boundaries or at points of low information within. Other research indicates word finding and speech volume regulation difficulties. Seventh, psychopathology, or emotional disturbance, has been associated with TS. Two specific psychological disorders associated with TS are obsessionality and sleep disorder. Eighth, the relationship between epilepsy and TS has been examined by several authors. Some believe that epileptic seizures or other epileptic phenomena do not occur in this condition (Peterson, 1996). Interventions Currently, there are broad-spectrum interventions for TS encompassing biochemical, psychological, psychiatric, and educational types. The majority of people with TS do not require medication; however, there are medications available to help control the symptoms when they interfere with functioning. Some effective drugs are haloperidol (Haldol), clonidine (Catapres), pimozide (Orap), fluphenazine (Prolixin, Permitil), and clonazepam (Klonopin). For obsessive-compulsive symptoms that interfere with daily functioning fluoxetine (Prozac), clomipramine (Anafranil), sertraline (Zoloft), and paroxetine (Paxil) have been effective. Stimulants such as Ritalin, Cylert, and Dexedrine are used to help with attentional difficulty, but these drugs are controversial because they may increase tics (Glen Hendren, 2002). Summary: Developmental Challenges Socially, the student with TS frequently experiences rejection. Odd and sometimes aggressive behaviors do not help in making friends or getting along with teachers. A verbally hostile student, particularly one whose outbursts are unpredictable, is not readily accepted by peers or adults. Several authors have noted general difficulties in social adaptation at school and the problem of maintaining age-appropriate social skills has been recognized among adolescents with TS (Leckman et al. 1994). Making Friends Psychologically, some of the behaviors shown by a student with TS may be alternative expressions of an obsessive-compulsive disorder or of affective and anxiety syndromes. TS have been associated with panic attacks, phobias, stuttering, and rapid talking. Defining the limits of tic disorders in regard to other forms of psychopathology remains one of the most controversial and difficult areas (Leckman et al. 1994). The psychological difficulties of students with TS add to specific developmental challenges related to identity, independence, and self-esteem. Verbal outbursts and motor tics may give the student a negative reputation and be incorporated into identity. Students with TS have difficulty becoming independent if they become anxious in new situations. Phobias place limitations on ones ability to function autonomously. Self-esteem is likely to be poor as much of ones self-evaluation depends upon feedback from others; when others express repugnance at compulsive swearing, snorting sounds, or other odd behaviors, the TS student incorporates these data into his self-assessment, with resultant low self-esteem. Students with TS typically have cognitive difficulties, notably cognitive inflexibility. While the general mental ability of most students with TS can be expected to be in the normal range, there is evidence that these students can "get stuck" cognitively. For example, students with TS may be unable to complete work because they reread or rewrite one word over and over. The analogy is made with a computer program loop, something that cycles back on itself repeatedly. For the student with TS, this "looping" may have special meaning. Cognitive sticking may mean an inability to switch smoothly from one activity to another, to relinquish a negative thought, or to recognize persons or events if out of context (Stell, Thickbroom, & Mastaglia, 1995, p 234-247). Cognitive Inflexibility Cognitive inflexibility can adversely impact independence. A student who becomes disoriented in new places is not a good candidate, say, for a drivers license, and would be expected to have difficulty even using public transportation. There may be significant educational/occupational impairment. For example, many students with TS have learning disabilities. A disproportionately high number of youngsters treated for TS have some form of learning disability, particularly a visual-motor integration deficit, which makes completion of work and productivity difficult. Severe reading problems, especially poor retention of what was read, were extremely common among TS patients. Also, obsessional thoughts can impede the educational or occupational progress of an individual with TS as a result of his or her inability to focus on tasks. Learning Disability Students with TS also find it difficult to cope with demands to target an occupation. These students have unrealistic ideas, or do not process feedback from teachers and guidance counselors. It is difficult for a student with TS to select appropriate courses. For example, a developmental challenge for adolescents is self-advocacy. These youngsters must not only come to grips with their identity, but they must assert themselves to get what they want. For a TS student, who may have poor self-evaluation ability and unrealistic goals, the progression from school to work is uneven, perhaps even arrested. The student with TS faces special challenges in working toward gainful employment (Davidovicz, 1994, p 345-348). Future Outlook The student with TS also has special challenges in regard to dating and romance. Implicit are the social problems, as articulated above, that often result in isolation. While the individual with TS may experience an abatement of tics during orgasm, this is a relatively brief part of an intimate relationship. If these youngsters cannot get into relationships, they will remain sexually inactive. TS and the Federal Definition of Ed According to the guidelines of the Individuals with Disabilities Education Act (IDEA) TS is not a category of special education. However, this does not mean that a student may not be included within its parameters. There are, in fact, numerous characteristics of TS that meet the federal ED criteria. One criterion, an inability to learn that cannot be explained by intellectual, sensory, or health factors, relates to TS in various ways. Their inability to learn is not a result of intellectual, sensory, or health limitations (Davidovicz, 1994, p 324-329). Rather, learning is impeded by a collection of factors that relate to their inability to build or maintain satisfactory interpersonal relationships with peers and teachers and inappropriate types of behavior or feelings under normal circumstances. Students with TS have difficulty building or maintaining satisfactory interpersonal relationships because of their odd behaviors. Peers do not want to interact with a student who snorts, grunts, barks, or uses profanity, particularly when profanity is used out of context. Youngsters tend to be frightened by unpredictable behavior. A student whose voice rises and falls for no apparent reason, who isolates himself, or who jerks his head, grimaces, and touches another inappropriately does not endear himself to peers. Furthermore, self-destructive, aggressive, antisocial, and oppositional behaviors make relationship building difficult. Even if students with TS can find compatriots with similar behavior problems, bonding with groups will be difficult because their behavior is unpredictable and sometimes repugnant (Murray, 1997, p 567-573). Teachers, too, find themselves put off by these students. A student who tells them they will "go to hell" for caring about others does not gain interpersonal ground. A student who has attention and impulse control problems, or low frustration tolerance, will be difficult for the teacher to manage and, consequently, will tax the teachers patience. Certainly, the TS penchant for perfectionism will not charm the teacher who tries to move a classful of students forward Bernstein, 1997, p 178-183). The TS student who shows inappropriate types of behavior or feelings under normal circumstances also meets a federal criterion for ED. Within the normal routine of the classroom, a student who suddenly jerks his body or shouts out an obscenity shows inappropriate behavior. A short temper and a tendency to be confrontational are also abnormal behaviors; they indicate a lower than normal emotional threshold. Also, a student who is phobic about being in groups exemplifies inappropriate feelings under normal circumstances. Finally, students with TS may be found eligible for special education services under IDEAs multiple disabilities category: a combination of conditions causes such severe educational problems that these students may be eligible for special education even though they cannot be accommodated in special education programs for just one of the impairments. Jed, for example, was determined to be eligible for special services as a student with multihandicaps, and he was placed through the individualized education plan (IEP) process in ED, learning disabilities, and speech-language programs. According to the Tourette Association of America (1994), identification as other health impaired (OFH) may occur under federal law. Such identification entitles the student with TS to services that address specific educational problems in school (Cohen, 1998, 234-246). Suggested Educational Strategies A student with TS requires a multifaceted teaching approach that addresses needs for acceptance/approval, external control, internal control, appropriate engagement, consistency/routine, and encouragement. Stress and tension should be prevented as much as possible. As there are differences of opinion about whether or not a tic is a simple learned response that is self-perpetuating or an outward expression of inner conflict, both processes must be addressed because there probably is a causal interaction. Some suggested educational strategies are the following. 1. Teach the student with TS to be self-aware regarding mental and physical signal symptoms, for example, a violent image prior to a tic. Heightened self-awareness can promote development of coping strategies, say the use of socially acceptable movements to replace unacceptable ones. 2. As this student typically feels anxious regarding self-control, do not put him or her in anxiety-provoking situations. For example, the stress of public speaking may evoke tics. Allow the student to present information in an alternative format. Flexibly schedule oral presentations when the tics are less severe or waning. 3. Strategies that have been effective with stutterers may apply. For example, the gestalt approach is recommended. This approach views stuttering not as an isolated symptom but as an organized self-system arising within the whole person. Like tics, stuttering may be maintained by the individuals experience as an inadequate, deviant stutterer (or ticquer) and by his or her inability to contact those aspects of the self that feel competent, speak (or behave) fluently, and interact in comfort. Consequently, the self system is locked in by the individuals negative experiences. In gestalt therapy, the individual is encouraged to become aware of the here-and-now experiences that maintain stuttering/tics. Tension, breathing, eye contact, and all other moment-to-moment behavior is focused upon so that those parts of the self that maintain the stuttering/tic system may be explored. For example, the student with TS may discover that his or her tics relate to fear of criticism from a particular teacher. Self-awareness promotes understanding of the individuals role in creating his or her own fear and, thus, tics (Mikkelson, Detlor, & Cohen, 1981, p 250-262). 4. Promote the teacher-student relationship. Anxiety and tics can be reduced through the relationship. Specifically, feelings should be labeled verbally; the adult should be accepting and nonpunitive; and specific suggestions should be made for better alternative behavior. Students will respond positively when they are able to relate to a caring person. 5. Reinforce the student for communicating in an appropriate manner based on the length of time he or she can be successful. Gradually increase the length of time required for reinforcement as the student shows success (Mikkelson, Detlor, & Cohen, 1981, p 250-262). Conclusion "Tourette syndrome symbolizes one of the fastest increasing diagnoses in North America" (Kushner, 1999, p.4). Increased understanding of TS and the more regular diagnosis of TS augment the possibility that treatment personnel will be asked to offer services to people who have TS. Tourette syndrome is a very multifaceted condition. It frequently engages comorbid conditions. There is no recognized cure for TS at this instance. Nonetheless, continuing research is offering a wealth of new information concerning this condition. It is significant for rehabilitation personnel to stay side by side of the research results so that they can better comprehend TS and, therefore, provide a high quality of service for those people with TS who might seek their help. References Bernstein, J. (1997). Coping with TS in early adulthood. Tourette Syndrome Association, Inc. Cohen, J. (1998). Disability etiquette: Tips on interacting with people with disabilities. Eastern Paralyzed Veterans Association. NY. Davidovicz, H., (1994). Learning problems and the TS child. Tourette Syndrome Association, Bayside, NY. Merck Manual Home Edition (2001) Retrieved March 3, 2001 from the World Wide Web: HTTP:HMERCKHOME EDITION.COM/INTERACTIVE/D.../0606705.HT Meyers, A. (1998). Serving clients with Tourette syndrome: A manual for service providers. Tourette Syndrome Association, Inc. Bayside, NY. Stell, R., Thickbroom, G. W., & Mastaglia, F. L., (1995). The audiogenic startle response in Tourettes syndrome. Movement Disorders, 10. American Psychiatric Association. (1994). Diagnostic and statistical manual of mental disorders, 4th edition. Washington, D.C., American Psychiatric Association. Chouinard, S. & Ford, B. (2000). Adult onset of tic disorders. Journal of Neurology, Neurosurgery, and Psychiatry, 68. Peterson, B. S. (1996). Considerations of natural history and pathophysiology in the psychopharmacology of Tourettes syndrome. Journal of Clinical Psychiatry, 57, suppl. 9. Zinner, S. H. (2000). Tourette Disorder. Pediatrics in Review, 21 (11). Glen Hendren, 2002. Tourette Syndrome: A New Look at an Old Condition Journal article; The Journal of Rehabilitation, Vol. 68, 2002 Leckman J. F., and Cohen D. J. 1994. Tic disorders. In M. Rutter, E. Taylor, and L. Hersov (Eds.), Child and adolescent psychiatry ( 3d ed.). London: Blackwell Scientific. Leckman J. F., Walker D. E., Goodman W. K., and Pauls D. L. 1994. "Just right" perceptions associated with compulsive behavior in Tourettes syndrome. American Journal of Psychiatry 151 (5). Lohr J. B., and Wisniewski A. A. 1987. Movement disorders. New York: Guilford. Tourette Syndrome Association. 1994. Questions and answers about Tourette Syndrome. Bayside, NY: TSA. Kushner, H. I. (1999). A cursing brain? The histories of Tourette syndrome. Harvard University Press, Cambridge, MA. Mikkelson, E., Detlor, J., & Cohen, D. (1981). School avoidance and social phobia triggered by Haloperidol in patients with Tourettes disorder. American Journal of Psychiatry, 138 (12). Murray, J. B. (1997). Psycho-physiological aspects of Tourettes syndrome. The Journal of Psychology, 131 (6). Read More