"Rewriting the Myths, Redefining the Realities"
by Lorra Tamplia
“I believe that attitude is the real disability! I have Tourette Syndrome. But ‘attitudes’ affect profoundly: society’s attitude toward people with Tourette’s, how I allow someone else’s attitude about TS to affect me and, perhaps most importantly, how I maintain a positive attitude about myself and my ability not only to survive with Tourette’s, but to THRIVE with Tourette’s,” says Joanne Cohen, 38, of Massachusetts.
Tourette Syndrome (TS), sometimes called “Tourette’s,” was first documented in 1885 by the French neurologist, Georges Gilles de la Tourette. According to the Tourette Syndrome Association (TSA), about 100,000 Americans have TS, which favors no race or geographic region. Other research groups name the incidence as one in 200. This includes people who have very mild cases and/or never get diagnosed.
Symptoms of TS reside on a continuum of frequency, intensity and type: the person with TS experiences involuntary, rapid and sudden movements and vocalizations - all known as tics.
Tics may occur continuously on a daily basis, or be absent for weeks or months at a time, and everything in between. Tics may also shift in variety and intensity: an individual may have one set of tics for several months, and then they’ll disappear, only to be replaced by a whole new set of symptoms.
Movement (motor) tics may be “simple,” such as facial twitches and eye blinking, or “complex,” such as jerking back the head or shoulders, hand clapping, finger snapping, tapping objects, or flopping or waving the arms.
Tics can occur out of the blue during any activity, but stress, excitement and fatigue may increase their frequency.
Some tics belong to specific categories:
Echolalia - repeating the words, phrases or sounds of others. Example: “John, please pass the biscuits.” John responds, “The biscuits, the biscuits.”
Palilalia - repeating one’s own speech or sounds. Example: “Hi Mom, I’ve missed you so much…much…much.” (This is not stuttering.)
Coprolalia - utterance of foul language, racial slurs or other socially inappropriate language. (This occurs in only 5 to 10 percent of cases.)
Echopraxia - imitating movements or gestures of other people.
Copropraxia - making obscene gestures.
None of these behaviors are performed to get attention or out of anger (though stress can increase frequency).
Whether physical or vocal, symptoms are not 100 percent involuntary. The
person with TS becomes swamped with an abundantly overwhelming urge to
express the movements or vocalizations. Eventually, the individual must give
in to this overpowering urge. It’s extremely exhausting to simply ignore
these urges, even while at a formal banquet or PTA meeting.
Cohen is a Licensed Certified Social Worker (LCSW) who works extensively with TS families, gives training seminars in Massachusetts and New England school systems, and is past president of the Massachusetts Chapter of the TSA. She exhibits many motor tics involving the head, neck, shoulder and arms, plus coprolalia.
Some people in the general population apply “will power” to quit giving in to a variety of urges or addictions, such as nail-biting, knuckle-cracking, junk food bingeing and cigarette smoking.
But “will power” isn’t enough to keep TS tics restrained permanently. This is because holding back the tics causes the person physical discomfort, and this feeling escalates until the tics must be “released.” The discomfort, during the restraint, never subsides.
Cohen explains, “The sensation many people feel just before a tic, is called the pre-monitory urge. I can usually feel a tic just before it happens. It’s a split-second feeling, like the feeling you get right before you sneeze, except much more subtle. If I try to suppress a tic, I’ll feel a pressure or tension building, either in my stomach, or maybe my throat, or even my shoulder muscles. The sensation becomes so uncomfortable that eventually the tics have to come out.”
Imagine having the hiccups and being told not to let them out. Even if you were able to subdue the hiccups, you’d feel ready to explode. Or imagine trying to hold in a sneeze. “There is a brief sense of relief after the tic is expressed, but this is temporary and soon builds up again,” Cohen says. “Some people say they can tell when I’m about to have a loud vocal tic, because I take a deep breath and there’s a short pause. Even one of my dogs seems to recognize this, and he ‘prepares’ himself for what’s coming!”
Cohen adds that the pre-monitory urge is experienced in many different ways, and some people with TS don’t feel it.
In no way are any of TS’s motor or vocal tics indicative of a person’s moral values or lack of self-discipline. Coprolalia in kids and teenagers should not be seen as an indication of poor parenting.
Cohen says, “Many people can control the words or find ways to camouflage them, such as saying them under their breath softly or mentally to themselves,” or altering the inappropriate word at the last second.
Even some children can restrain their tics temporarily. Wesley Powell is only 8 but can hold in his tics (mostly head-jerking) at will, says his mother, Barbara, of Overland Park, KS. Every hour or so Wesley asks his teacher to be excused to get a drink or use the restroom. “He does this to release his tics privately,” says Powell. The teacher knows this, and Wesley returns within five minutes.
Wesley’s tics also include eye-rolling, which once made a teacher think he was being disrespectful. His tics may occur two to seven or more times in a row, and increase with stress or excitement. “Last Christmas morning,” says Powell, “he jerked his head up to eight times every time he opened a gift.”
Onset of symptoms usually appears around age six or seven. Only last
fall was Wesley diagnosed, along with his father. “Wesley was greatly
relieved,” says his mother. “Wesley said, ‘I’m glad to know it’s
called something.’”
Even some modern day doctors continue to overlook TS as a possible diagnosis in their patients. Tic conditions that don’t qualify as TS are fairly common in the general population, so the tendency is to suggest that the symptoms will subside with age.
Tics are sometimes suppressed in a doctor’s office, and if physicians believe that TS is rare, it will less likely occur to them as a diagnosis. Lastly, sometimes the tic behavior is attributed to “nervousness.”
Powell explains, “When we first sought a diagnosis for Wes, we met with a child psychiatrist. He easily recognized Wes’ Attention Deficit Disorder symptoms, but when I asked about his tics and night-time fears, the doctor blamed them on the fact that Wes’ dad and I are divorced. Even though we have a positive relationship and co-parent extremely well, the doctor’s ‘divorce diagnosis’ was very painful.”
Proper diagnosis requires that the tics be present periodically for over one year, and both motor and verbal tics must be present. “Verbal tics often take the form of sniffing or clearing the throat,” says Jack Southwick, LCSW for the Joshua Center in Kansas City, MO., a nonprofit organization that services people with TS. He has worked with TS patients for over 15 years, and adds, “Frequently these tics are initially misdiagnosed as an allergic reaction or a frequent cold.”
Cohen’s symptoms began at age 9 “I was misdiagnosed for four years, while my parents took me to one doctor after another. They all told my parents, ‘It’ll go away.’ One of my more bizarre complex tics was on a skating rink, where I simultaneously skated across the ice, bent down, and touched my chin to the ice, with my arms out to the sides. You don’t see THAT in the Olympics!”
Cohen frequently sees children whose pediatricians continue to insist that the child, who clearly meets the diagnostic criteria for Tourette’s, is being misdiagnosed with a milder tic disorder - Transient Tic of Childhood. Over 30 percent of children get this, and it disappears within a year or less.
At the left side of the spectrum is Transient Tic of Childhood (duration of tics less than one year), and at the far right is TS. In between are other tic disorders, such as Single Vocal Tic, Single Motor Tic, Multiple Motor Tic, and Multiple Vocal Tic.
Cohen says, “Simple vocal tics involve
sounds such as humming, squeaking, chirping, clicking tongue, grunting, making
small noises, etc. More complex vocal tics might be, ‘Uh oh!’, ‘Oh boy,’
‘Watch out,’ ‘Help!’ and ‘Wow!’” These can occur during silence as
well as conversation.
Wesley is being tested for his school’s gifted program, and gets
mostly A’s. He has played soccer and basketball, and now prefers tae
kwon do, a martial art. “He is quite agile,” says Powell. “The
only problem I’ve seen is that his tics would slow his response in a
game. I have felt great anxiety watching him run down a basketball court
with his head involuntarily jerking up and down, for fear he’d run
into something. He has never had an accident.”
Brian, a teenager from New Jersey, is quite an athlete; he earned a karate black belt during grade school, and currently snowboards, skis and wrestles. His mother first began noticing frequent eye blinking in her son when he was 5. Over time, new tics appeared such as throat clearing, facial twitches and mouth opening.
“He tries to hold in the tics at school, which can be exhausting, and then release them at home,” says his mother. “There is a physical tug when you have to tic, and a release when you finally do.”
Brian masks the open-mouth tic by making it look like a yawn. “Brian’s tics are worse when he’s excited or stressed, so we weren’t surprised that on the day of his Bar Mitzvah he was opening his mouth real wide throughout the service and the four-hour party, all of which was videotaped.” During his Bar Mitzvah speech Brian announced that a portion of his gifts would go to the TSA.
“The element of unpredictability, both in the path the tics will take and in not knowing what tic your child will wake up doing on any particular day, make this an especially unusual disorder,” says his mother.
About 60 to 70 percent of people with TS have varying degrees of Attention Deficit (Hyperactivity) Disorder -
AD(H)D. They are also more likely than the general population to have Obsessive Compulsive Disorder
(OCD), Dyslexia and other learning disabilities, plus sleep disorders and temper control problems (anger outbursts, defiance behavior). People with TS almost never physically lash out at others.
“It’s often some combination of these other disorders in addition to TS that causes the most difficulty for the individual,” says Southwick. Wesley has ADD and OCD. Powell says, “He is more challenged by the ADD and OCD symptoms, which are largely misunderstood by casual observers.”
Brian has been somewhat tic free lately, but his mother has concern with his TS related “rages in the face of frustration,” such as not being able to attend a school dance. The “rages” began when Brian was 13, involved screaming only, and lasted about 30 minutes. TS related rages do not reflect inadequate parenting methods.
The prevalence of mental
conditions - Schizophrenia, Paranoia, Manic Depression and Mental Retardation -
in people with TS is no greater than in the general population. A person in
public who is releasing prominent tics may very well be a well respected
businessman, attorney, plumber, college student or high school cheerleading
captain. People with TS hold all kinds of jobs. “Extreme concentration can
cause the tics to subside,” says Southwick. “I have known surgeons, figurine
painters and sports figures who get into a zone, and do not tic for these
periods of time. They also report that the tics come out later.”
The genetic components that cause TS are being hunted down. Researchers believe that, in addition to a genetic component, an environmental factor such as infection during a critical neurological development stage of a young child may be at play.
TS is linked to a flaw in the regulation of the brain neurotransmitters dopamine and serotonin, and three-quarters of people with TS are male.
The greatest hardship for people with TS may be the way people - particularly those who make no effort to educate themselves about the variety of conditions that can affect human beings - view them.
Cohen says she uses humor to
diffuse a tense public situation. Recently she was in a chain discount store
waiting in the checkout line when all the cash registers broke down. “I don’t
handle waiting in line very well and my tics began to get louder, and people
started turning to look at me. I looked at the ceiling and said, ‘I was FINE
when I came in here. There must be something in the vent system. I think I’m
getting Tourette Syndrome!’ Imagine being able to make 50 people stuck
at
the registers burst out laughing!”
Cohen has other strategies that she uses to delay or minimize tics, such as chewing gum to occupy her mouth, sipping water during a lecture, and doing deep breathing or relaxation exercises.
Various cognitive behavioral methods exist that many people with TS use to help manage the condition. “These techniques are used quite often with great success,” she says. These strategies “often help a person temporarily reduce, modify, or camouflage tics for short to moderate periods of time.”
Cohen explains, “Libraries, quiet movie theatres, even funerals present a great challenge for me.” She employs creative solutions, such as using the drive-through teller at a bank, calling a library ahead of time so staff can have material waiting for her, and attending drive-in movies.
TS isn’t a disability in the strictest sense of the word. Physical mobility, internal health, life span, immunity to disease, and cognitive skills are not at all affected.
If people would accept TS as an uncontrollable tic disorder, just as they accept that a paraplegic cannot walk up a flight of stairs, a person with TS wouldn’t have to manage, restrain or use coping mechanisms to hold back anything, asserts Cohen. “TS is a disorder only in the sense that society often cannot completely deal with it,” she says. With more understanding from society, TS can one day be nothing more than an inconvenience.
“Living successfully with TS
requires that the person focus on his or her strengths and passions,”
continues Cohen, who sells her captivating photos of outdoor scenery. “Since
the Tourettic mind and body are almost constantly in motion, I often find that
in order to slow down, I need to get back to nature.”
“Stress, excitement, fatigue and sensory stimulation make the tics worse,”
says Southwick. “If the child and parents come to realize this, they may
be able to do some things which manage the situations in which these occur,
better. Tics make a person look different. The person may even be teased by
others. Therefore, part of the counseling work is to help build self-esteem.”
With healthy self-esteem, a child or teenager with TS might actually enjoy the attention he gets. Brian likes when people ask him about his tics. “Brian tells people he has a defect in his brain that causes these behaviors and they’re not under his control,” says his mother. “He suggests it’s similar to other peoples’ habits, such as biting nails.”
Cohen educates everyone in the system about TS, including siblings, school bus drivers, teachers and doctors, as well as the child and parents. “I give training in schools and people are amazed at how much they thought they knew about TS, but didn’t!”
Powell does whatever it takes to get Wesley past his rages. She bypasses the options to reason with or discipline him with consequences. Instead, she waits for the episode to pass, then talks gently to him about the experience. “I also tell him I know the rage was painful.”
Southwick says, “People do not outgrow TS. It’s life-long. Most
people develop an acceptance of self, which reduces personal stress and
therefore a lessening of tics.”
Many medications are available, but some have side effects, and not all provide relief. No medication can completely rid a person of tics. No known surgery is recognized as a treatment for TS.
Currently under investigation and not yet approved by the FDA for TS
treatment is the tic-reducing effect of nicotine patches. This research
is headed by the University of South Florida’s Doug Shytle, Ph.D.,
Assistant Professor of Neurosurgery; Paul Sanberg, Ph.D., D.Sc.,
Professor of Neurosurgery; and Archie Silver, M.D., Professor of Child
and Adolescent Psychiatry.
Typical nicotine patches can produce side effects, so the researchers are turning to using “nicotinic receptor blockers,” which mimic nicotine’s effect on brain neuron receptor sites. “In a recent retrospective case review of 25 TS patients, most of which were also receiving medication that was not fully controlling their symptoms, we found that mecamylamine (a nicotinic receptor blocker that the three researchers have a patent on) is therapeutic in TS, particularly for those with behavioral problems, such as rage outbursts,” says Dr. Silver. “We emphasize these findings need to be confirmed with additional studies.”
People with TS must seek advice and consultation with their doctors before using any kind of nicotine patch or nicotinic receptor blocker, says Dr. Shytle.
Humor is often the best way to diffuse unplanned situations and enhance acceptance of TS. Cohen says:
"In Switzerland I was on top of Mt. Zermatt, and I had a tic so loud my echo came back to me about one minute later from across the mountain range. For a moment, I thought there was another woman on the other side with Tourette’s!”
“A single friend once asked if I felt my biological clock ticking. I told her that my biological clock was the only part of me that WASN’T ticcing!”
“Sometimes, when I speak a little too fast, I say something punny or make up a word. When people look at me, I say that I can do that because I have Tourettic License!”
And Wesley has actually named many of his tics. The tic that involves tugging up on his top right lip is named “Elvis.” The shoulder shrug tic is called “I Don’t Know.” Eye-rolling is named “Bored.” And head-jerking is the “Yes” tic. Powell says, “Humor puts it all in perspective.”
Retired NBA player Mahmoud Abdoul-Rauf
(formerly Chris Jackson)
Retired Major League baseball player Jim
Eisenreich
Mort Duran, M.D., a leading general
thoracic surgeon in Canada.
Michael Wolf, former band leader for
Arsenio Hall.
Shane Fistel, a Canadian sculptor.
The premier resource for TS is the Tourette Syndrome Association, which maintains a crisis hotline, physician referral listings, up-to-date information on TS, consumer services, a quarterly newsletter, additional literature, and many other avenues of assistance.
This nonprofit voluntary health organization has 54 chapters in the U.S. and over 40 contacts in other countries, and was established in 1972. TSA’s address is:
TSA, Inc.
42-40 Bell Blvd., Suite 205
Bayside, NY 11361-2820
Phone: 718-224-2999 Fax: 718-718-9596 Email: tourette@ix.netcom.com.
Web site: http://tsa.mgh.harvard.edu
For information about presentations or seminars on Tourette’s, please contact Joanne Cohen at:
jocohen@mediaone.net
Copyright 2002 A&H Publishing Corporation