Roanoke Times
Copyright (c) 1995, Landmark Communications, Inc.
DATE: TUESDAY, April 10, 1990 TAG: 9004100017
SECTION: EXTRA PAGE: E-1 EDITION: METRO
SOURCE: CHARLES HITE MEDICAL WRITER
DATELINE: LENGTH: Long
"Do you think the kids will stop making fun of me now that I can tell them what I have?" the 11-year-old asked.
For more than seven years, Scott had behaved in ways that made him an easy target for cruel jokes and taunts.
Just before his fourth birthday, he began an exaggerated blinking of his eyes. His parents took him to an eye doctor who pronounced Scott's vision fine.
"We thought it was just a nervous habit," says Patra Bedwell, Scott's mother.
In elementary school, other tics emerged.
Scott constantly sniffed and cleared his throat.
He jerked his head, mimicking the motion of someone trying to clear the hair hanging in front of his eyes.
He flipped his wrists with the motion of someone shaking down a thermometer.
On the soccer field, he stomped and kicked the ground as he waited for the ball. "He looked like a bull getting ready to charge," Patra Bedwell recalls.
He repeated commands. "Please close the door," Patra Bedwell would tell her son. "Close the door, close the door," Scott replied, apparently unaware of his behavior.
He had a compulsion to touch things. If he walked by the living room sofa, he needed to sweep his fingers lightly over the arm.
About this time, Patra Bedwell read an article in Parade magazine about a relatively unheard of disease. "As I read the article, I remember saying to myself, `This sounds a lot like Scott,' " she says.
A few months later, she watched an episode of the television show "Quincy" about the same disease. Again, she was reminded of Scott's behavior.
The article and television program prompted Bedwell and her husband, Ron, to take Scott to his pediatrician. The doctor thought Scott had a simple case of childhood tics that eventually would go away. He had never heard of Tourette syndrome, the disease described to him by Bedwell. But just to ease her mind, he referred Scott to Michael Sisk, a Roanoke neurologist.
In the past dozen years, Sisk has treated about 350 patients for Tourette syndrome. "If someone had told me that when I was in training, I would have laughed at them," Sisk says.
In the mid-1970s, when Sisk was finishing his residency in neurology, there was a lot of "fuzzy thinking" about Tourette, he recalls.
Until the late 1970s, Tourette was considered a psychiatric disorder. After all, the patients seemed crazy: They jerked, shrugged, barked, mimicked, squeaked and, sometimes, shouted obscenities.
The syndrome was named after the French physician Georges Gilles de la Tourette, who described the disease in detail in 1885. One of his first patients was a noblewoman who lived as a recluse and had symptoms throughout her life.
Tourette remained a rare diagnosis until the late 1960s and early 1970s, when it was discovered that the symptoms in many victims were greatly reduced by haloperidol, an antipsychotic medication. It was the first solid evidence that Tourette syndrome had a physical basis.
By 1980, Sisk says, Tourette was accepted as a neurological disorder with symptoms that often weren't as severe as those seen in the first documented cases. It was also discovered that symptoms changed in frequency and severity.
Estimates on the number of persons afflicted by the syndrome now range from 100,000 to 1 million in the United States alone.
The tics in Tourette typically begin in early childhood. The average age of the Tourette patient who comes to Sisk's office is 8.
The tics wax and wane over the years. About half of the victims can expect some improvement as they reach maturity. A sizable group can expect to continue to improve throughout their lives. Several medications that help control the symptoms are available.
Many victims are believed to go undiagnosed for years because Tourette is still unfamiliar to many physicians and teachers who confuse the disease with other disorders.
A majority of Tourette patients are self-diagnosed. Or, as in the case of Scott Bedwell, a family member may recognize the syndrome after reading an article or watching a TV show.
Unfortunately, says Sisk, the cases that get the media's attention are often the most dramatic and least typical. They leave the impression that Tourette victims always shout obscenities and have wild, violent motor tics, he says.
"The majority of these people can lead normal lives," Sisk says.
Fighting the intolerance
It was nearly a decade ago when Scott Bedwell walked out of Sisk's office with something that would change his life. He had a name for his disease. He had an explanation for his behavior. He had an answer for the kids who had been teasing him.
"A lot of kids at school made fun of me," Bedwell recalls. "Kids tend to rib kids a lot. If someone wanted to get to me, they would mimic my habits and movements."
If there's one thing she wants people to learn from Scott's disorder, Patra Bedwell says, it is to recognize the importance of tolerance and patience.
"The worst damage in Scott's case was his self-esteem," she says. "It was not the Tourette but how he was made to feel. He spent a lot of time alone."
Once the diagnosis was made, Scott's parents made sure his teachers were given a thorough explanation of his disease each year. That helped enormously.
Scott learned to meet the teasing head-on. He told his friends he had a neurological disease that sometimes caused him to do strange things. He told them it was something he couldn't control. If they wanted to make fun of him, so be it. Most kids understood.
Still, Scott had problems in school. He was well-behaved, but he had difficulty concentrating. The quiet environment of the classroom seemed to make his tics more difficult to control.
By the time he reached high school, Scott's uphill struggle with school work led his mother to have him evaluated by a psychologist. The psychologist concluded that Scott had a learning disability and also attention deficit disorder, a syndrome marked by lack of concentration and difficulty in listening and retaining information.
Attention deficit disorder has been linked to hyperactivity, a condition in which children are fidgety, disruptive, easily distracted, talkative and impulsive. It is now believed the two disorders can occur separately.
In recent years, hyperactivity and attention deficit disorder have been noted in children with Tourette. Some researchers estimate 50 percent of children with Tourette will show symptoms of hyperactivity/attention deficit disorder.
Whether children with Tourette should also be treated with drugs used in treating attention deficit disorder/hyperactivity has sparked a debate among researchers. There is some evidence that those drugs don't interfere with the medications that are used for controlling Tourette.
Scott Bedwell has used several different medications to control his Tourette. He started with haloperidol, the antipsychotic drug first found useful in treating the disease.
The drug made him tired, groggy and "out of it." Sisk switched him to clonidine, a medication first used to treat high blood pressure. For the past few years, Scott has been taking pimozide, a drug developed exclusively for controlling tics in Tourette.
Despite its frequent side-effects, haloperidol remains the drug used most often to treat Tourette. And it was haloperidol that first led researchers to look at the brain for the roots of the disorder.
A genetic link
Haloperidol is known to inhibit dopamine, a chemical that is critical in enabling the central nervous system to function properly. Dopamine is produced in an area of the brain known to be associated with several movement disorders, including Parkinson's disease, Huntington's disease and tardive dyskenesia.
Some doctors theorize that patients with Tourette are overly sensitive to dopamine. Researchers are focusing their attention on this area in their search for Tourette's cause.
Hereditary studies of families with Tourette have led scientists to conclude the syndrome is a genetic disorder. The disease is passed from one generation to another by a gene-carrying mother or father.
It can be passed to either sons or daughters, although male victims outnumber females more than 3 to 1. When one parent has Tourette or carries the gene for Tourette, there is a 50 percent chance each child the couple has may inherit the disease or become a carrier.
While researchers look for a way to identify and isolate the gene for Tourette, it has become clear from drug therapy that the syndrome is a complex disorder that apparently is closely tied to other behavioral problems.
Some Tourette patients simply don't respond to haloperidol or other dopamine blockers. Some do respond to drugs such as clonidine that have no affect on dopamine.
Besides attention deficit/hyperactivity symptoms, many Tourette patients also exhibit obsessive-compulsive behavior. They have recurrent or persistent thoughts and feel the need to perform certain tasks. Examples range from repeatedly checking to see if a door is locked or a stove is turned off to the need to touch an object with one hand after touching it with the other in order to even things up.
A song on the radio can become an obsession for Scott Bedwell. Often it is a tune he doesn't like. But for some reason, the melody haunts him. And the more he tries not to think about it, the more he can't get it out of his head.
Bedwell says a new medication - fluoxetine - seems to help lessen compulsive thoughts and behavior and allows him to concentrate more.
Many Tourette patients say their tics are like an itch that can't be scratched. But they've learned that if they really focus their attention, they can suppress the tics, at least for short periods of time. Their medication, they say, helps control the urge to scratch.
"My tics don't bother me at all," Bedwell says.
The most frustrating aspect of his disease now, Bedwell says, is his inability to do well in school work.
"I take tests very badly. I find my mind wandering a lot," says Bedwell, a sophomore at Roanoke College. "Sometimes I have to read a section of a book over and over before I can grasp what it's saying."
Still, Bedwell finds his disorder hasn't prevented him from leading an active life. He rides a mountain bike, is a rock-climber and an avid hiker, and he plays junior varsity lacrosse.
And Tourette hasn't deterred him from pursuing his dream of going to law school and becoming an environmental lawyer.
by CNB