Tourette's Syndrome, a benign tic disorder once thought to be rare, afflicts as many as one in one thousand people according to new research (Suchowersky). Usually diagnosed at about age six (Baron-Cohen et al.), its episodes seem to escalate and peak during the teenage years. Most victims (60%), however, are free of symptoms, except for a few episodes, during their adult years (Park et al. 1712). Tourette's is described in terms of its obvious manifestations but, from a medical perspective, it is described in terms of what it is not, unlike most other diseases. Sadly, it often goes misdiagnosed (Kurlan et al. 699) or undiagnosed altogether.
Tourette's Syndrome includes multiple migrating tics, including a vocal tic, which have persisted for over one year (Park et al. 1712). Medically, all blood work of the subject is negative, CT Scans and EEGs are non-symptomatic and no evidence exists of other abnormalities. In other words, if the tics are manifest and there is nothing wrong with the patient, a diagnosis of Tourette's is probable. Because it is neither life-threatening nor contagious, little time or money is expended on research for its causes or cures. Current consensus suggests that it is probably organic in nature (Brookshire et al.), meaning there is an existing mal-function at the cellular level. The subject was probabaly born with the abnormality which may be heriditary.
For those afflicted with Tourette's, it is anything but benign. It is socially stigmatizing (Lishman 581). It undermines self esteem and confidence in personal sanity and can present almost unsurmountable obstacles for educating a healthy, intelligent mind (Kurlan et al. 699). Illness, stress, fear and other strong emotions can trigger episodes of Tourette's manifestations that prevent one from functioning and interacting in any kind of "normal" way. Thus, once a diagnosis is confirmed, it becomes a challenge to the family to help the child gain and maintain a moderate emotional state. It must be recognized that as a victim tries to force such a state it proves to be counterproductive to the outcome.
When our daughter Elizabeth was five, she displayed typical tic abnormalities that were not too remarkable. If she became excited, she would throw her hands, fingers outstretched, up and to the side at about shoulder height and "shake" them. The affectation was quaint at first but over time began to take place at seemingly inappropriate moments. Although the hand movement looked like excitement, it also presented itself when she should have been very sad, such as when her kitten died. We did not realize, at the time, that she was not throwing her hands but that they were moving involuntarily to her will. We began telling her, "Don't do that," but the more pressure we put on her to stop her inappropriate behavior, the worse it got.
Then the vocal tic began. A "vocal" tic is anything that causes air to pass over the vocal cords (Kurlan et al. 700). It is not necessarily, as it is usually portrayed in the media, words, either socially unacceptable or otherwise. For Elizabeth, it was clearing her throat. Since she was still a preschooler and spent most of her time playing, it was an easy symptom to ignore. Easy, that is, for all but her teenage sister who shared a room with her. "Mom," Karen would complain, "she's driving me crazy! Can't you do something?" I talked with Elizabeth to find out why she felt it necessary to clear her throat every ten to twenty seconds. She gave somewhat plausible, if not exactly confident, explanations: there was "spit" in the back of her throat and she needed to clear it; she felt kind of like she was gagging. I talked with her, explained how irritating it was to Karen, and asked her to try very hard not to do it. She agreed but again, the harder she tried, the worse it got. Although she seemed healthy, there was definitely something wrong and I made an appointment with our family doctor. He checked her over and gave her a clean bill of health but suggested that the problem might be caused by "post nasal drip." He prescribed a mild, over-the-counter decongestant with instructions to give it to her only at night so that she would not be drowsy. Because they were so unremarkable, I did not think to mention the hand movements that had begun months before. We tried the decongestant but it seemed to make no difference. After four weeks we quite using it because I do not believe in medicating children unnecessarily.
Elizabeth began throwing her head back and to the right. It happened so frequently that I asked her about it. "I don't know," she answered. After that initial confrontation, each time she did it, she would raise her right hand immediately in a gesture as if to brush her hair back. Again I asked her about it. "My bangs are getting in my eyes," she responded. "Well," I told her, "if that's the case, lets cut your bangs so they will quit getting in your eyes." I cut her bangs very short, but it did not reduce either the incidences or Elizabeth's explanation of them. Although she did not understand and could not explain what was happening, she felt a need to make her uncontrollable physical actions appear less "odd" and therefore more socially acceptable. This is very typical of all people who suffer from Tourette's: a tic will be followed by a voluntary movement to minimize the socially stigmatizing effect it creates.
At this point in the progression of the tics, Elizabeth herself had perceived that something was wrong with her but she lacked the experience and vocabulary to explain it. She felt like she was going crazy although she was too young to express that emotion. The consequences were severe. Unable to ask for help, she became terrified of what might be wrong. An avalanche of tics followed. Her vocal tic became audible coughs and sniffs. In addition to her hand and head movements, her eyes began rolling back in her head without any loss of consciousness. Friends began asking their mothers, "What's wrong with Elizabeth?" Mothers and relatives began asking me, "What's wrong with Elizabeth?" I had no answer. Elizabeth, sensing the fear and distancing of her friends, began to control, with visible effort, the rolling of her eyes.
Many people with Tourette's Syndrome can control episodes for specific, short periods or for specific occasions with nonspecific time frames (Lishman 581). There is, however, a "pay-back." If episodes would naturally have occurred during those controlled times, they will manifest themselves when the control is relinquished. This was clearly evident for Elizabeth. She took ballet and ice skating lessons. While performing either on stage or on the ice, she did not miss a step. When her performance was done, however, she could be rendered nonfunctional as her head and hands and eyes all jerked and shook of their own will.
Our next trip to her doctor resulted in a far different diagnosis. We talked about the first evidence of the hand movements and subsequent problems. He referred Elizabeth to a neurologist who scheduled a multitude of tests. All blood work was negative. Her EEG and CT scan were as perfect as textbook study models of the normal brain and normal brain waves. The diagnosis: Tourette's Syndrome. Options included medication to induce a more relaxed state, simply coping as a family and helping Elizabeth to accept and understand her affliction, or medications that are given as a group of three, all personality altering, and each to counteract the negative consequences of the others. We chose to accept Elizabeth as she was, beautiful, healthy and intelligent, and to work at helping her to adapt to the worse aspects of the manifestations.
A positive diagnosis was the most important step in Elizabeth's treatment and adjustment. Just knowing that there was nothing intrinsically "wrong" with her helped to assuage the doubt and fear. She regained much emotional equilibrium; the episodes abated although they did not disappear. We talked with her openly about the condition and encouraged her to use the skills she already possessed to control the manifestations if it was necessary. Control came not from concentration on the control itself, but in complete and total concentration on something else. Most people, and children are no exception, function at optimal capacity under a certain amount of stress. For Elizabeth and others with Tourette's, any degree of stress can have a negative influence on a favorable outcome. It is imperative that they learn not to place unreasonable expectations on themselves yet many suffer symptoms of obsessive-compulsive behavior and the "just right" phenomena of perceptions commonly associated with such behavior (Leckman et al. 675).
Educational challenges are of particular concern. Within one school district, 27% of all children in Special Education classrooms were diagnosed with Tourette's (Kurlan et al. 701). Many of these children also suffered from attention-deficit hyperactivity disorder, arithmetic disabilities, disruptive behavior and school problems in addition to obsessive-compulsive behavior (Park et al. 1713). Tourette's is typically diagnosed in the first grade for very good reasons. As in Elizabeth's case, the episodes did not interfere notably with our family life and, if they had not escalated, could have gone unremarked. In a classroom setting, however, those same episodes can be particularly disruptive. Since they become socially stigmatizing, a child is placed under additional stress and they are magnified. Learning, in the classroom setting, is new and challenging; challenges cause stress; stress increases head and eye movement making reading and/or following instructions difficult to impossible; failure at assigned tasks creates additional stress. The problem becomes a giant spiral, trapping the child in a cycle of failure. Taking away the stress completely requires that the child do nothing. No learning is expected and therefore there are no failures in performance. We watched this pattern taking place in Elizabeth's life. Our healthy, intelligent child was not able to learn in the traditional classroom and proved disruptive to the learning of others as well. We live in a small, economically depressed city in rural Oregon. Our school lacks the resources for special tutors or trained educational assistants. Many schools, particularly those in larger districts, recognize the needs of Tourette's children and are prepared to allocate their resources for specialists and tutors who can support and encourage children to reach their true potential. Unfortunately, our school's answer was to place Elizabeth in a remedial class that put no pressure on her in order to minimize both the episodes and their disruptive quality. It also absolved the teacher of the responsibility of teaching. It guaranteed that Elizabeth would not fail, but neither would she learn. We chose an alternative not available to all parents: home schooling. I am not a teacher by training or education, but I am able to spend a concerted amount of time with Elizabeth. It strains all of my creative talents to come up with ways of walking the tightrope between successfully challenging her to learn and creating stresses that prevent her from learning. One anomaly is that unlike most children, success does not encourage her to believe that she will experience success at the next level. Each day is a new challenge and therefore a new stressor. Reading a page of her book one day does not guarantee her that she will be able to read it the next day and the same page can present itself as a brand new challenge. Although she has a wonderful memory, when it comes to schoolwork, her brain refuses to let her build on past learning with any consistency or confidence. To a great extent, our efforts have been successful, although we are forced to confront the same challenges with each day and with each task. Elizabeth must first, every time, overcome the fear of performance failure . . . expectations she places on herself.
At the outset of the diagnosis, Elizabeth acknowledged that she already controlled the eye-rolling although it was hard and physically tiring (the "payback"). She had seen the reaction of those around her who went "ballistic" when her eyes rolled, and sensed that it was a social necessity. As a family, it became imperative that we reinforce her personal concepts of self-worth. She needed to know then, at an early age, that her value was not determined by a peer group but by personal integrity and adherence to principles and morals. At the same time, she needed to learn to be open with friends and acquaintances who were curious or concerned. Although she functions well in small, intimate groups, Elizabeth does not do well in large groups. She is comfortable giving demonstrations of cooking, for instance, and speaking one-on-one with a judge, but is unable to speak before a group to explain what she is doing. The stress at that level of performance makes her incapable of successfully accomplishing the task.
For Elizabeth and, I suspect, for others with Tourette's an additional challenge not often discussed is the problem of medical care. Most children are somewhat fearful when visiting with a doctor and Elizabeth is no exception. She does very well when shots and routine treatments are needed but other procedures, which she does not understand and has no previous experience with, can present extreme obstacles. Having her teeth cleaned at the dentists office was not a problem. When she went for routine treatment, however, it was a complete failure. Her dentist was able to give her a shot to numb her mouth, but then she could no longer control the fear. She continually cleared her throat, swallowed, sniffed and coughed. Her hands went uncontrollably to her face. Her dentist gave up. To have baby teeth pulled, it required a general anesthetic. We are uncertain, at this time, what will be required for two small fillings.
Vocal tics often turn to words in the teenage years and those words may or may not be socially acceptable (Lishman 581). We have tried to keep unacceptable words out of her life believing that if she didn't hear them, she could not possibly vocalize them. In today's society, it is impossible to so completely control a child's life. She hears "bad" words at the store, in the post office, on the television, in the waiting rooms of doctor's and dentist's offices and from neighborhood children. Words I have always found unacceptable are constant parts of our life although not by choice. How difficult it will be for her if, as a teenager, she suddenly begins saying words she would never choose to use. We are known by what comes out of our mouths. It remains to be seen what is in store for Elizabeth but we are hoping "bad" words will have such minor significance in her life that they will not be the ones which take the form of a tic.
She has gained praise from her dancing teacher for her diligence. She is a perfectionist when it comes to her dancing (part of the "just right" perception perhaps), and is willing to accept the "payback." Her friends, too, are now familiar with the manifestations of Tourette's episodes and have quit worrying about her. Elizabeth's social circle is small and intimate. She has included people she trusts. She also has some friends who are cruel about what she "does." Elizabeth, at nine, considers that they simply don't know any better but quietly avoids them when she can.
Elizabeth has adjusted very well. She has accepted her affliction as one accepts that some people have red hair and some are blonds. She has successfully redefined what "normal" is for her and acknowledges when her limits of coping are exceeded. In other words, certain episodes are expected but when there are more than that number or kind, she reevaluates and makes necessary adjustments. Sometimes she can make changes independently; sometimes the adjustments require discussing the problem with her father or me or her older sisters to regain balance. The wisdom she has gained is in knowing the difference and seeking help when she needs it. She does not value herself on the basis of what other children say or do. She has learned that good apples and bad apples all exist and each needs to be evaluated on its own merits.
.For Elizabeth and others with Tourette's Syndrome, the disease is not benign. Without adequate emotional and educational support, it can prove to be devastating. It alters life and one's perception of it. It changes how its victims relate to themselves, to other people, to learning experiences and to society. They must redefine their own goals and expectations based not on accepted norms, but on their own capabilities. They must alter their own environment in order to grow and mature healthy and well-adjusted. Elizabeth is learning and doing, gaining self-confidence and self-esteem. For her, the future is bright, as it should be for all children.
