ROCKLAND, Maine — Ben Schesser cannot remember a time when his body didn’t twitch and jerk uncontrollably. The 24-year-old Rockland man was just a toddler when he began exhibiting signs of Tourette syndrome, a neurological disorder that causes repetitive involuntary spasms and vocal outbursts known as tics.
Two years ago, Ben’s tics began to worsen. His medications stopped working. His body twitched so relentlessly that he could no longer complete simple tasks such as sweeping the floor or stacking wood. His condition forced him to quit his job at a local deli.
Ben’s sister and two brothers watched as their once fun-loving sibling became sullen and withdrawn. They understood his struggle all too well. All three of Ben’s siblings — Matthew, 26; Caleb, 17; and Caitlin, 20 — also have Tourette’s, though their symptoms are less debilitating.
The Schesser children live at home with their mother, Mary Wiebe, who cares for them full time.
She, too, watched as Ben’s symptoms intensified. He tumbled down stairs, fell off of chairs, and suffered tics in his sleep that at times kept him awake for three straight days at a time.
“I saw this young man who was so vivacious and happy and full of life and outgoing turn into a withdrawn, irritable person who was staying in his room all the time and couldn’t function at all,” Wiebe said. “I knew something had to be done that was going to be drastic for him.”
Ben’s older brother Matthew, distressed by Ben’s deteriorating condition, researched treatment options. He discovered an experimental surgery that had transformed the life of another young man with Tourette’s who had appeared on ABC’s “Good Morning America,” along with his doctor.
Wiebe watched the episode, looked up the Manhattan doctor’s name, and called his office. A week later, Ben had an appointment.
In December, Ben plans to return to New York City for the potentially life-changing surgery that involves implanting small electrodes in his brain to interrupt the signals thought to be triggering his tics. Called deep brain stimulation, the procedure has been used to manage Parkinson’s disease, but has not been approved by the U.S. Food and Drug Administration for treatment of Tourette’s.
The surgery is experimental and carries risks. But for Ben, it also offers the promise of the simple joy of finally sitting still.
“I’m a little bit scared; it is brain surgery,” Ben said, his voice halting from his tics. “But I am hopeful that it will help me get this under control a little.”
Deep brain stimulation
Sitting recently with his family at the kitchen table in his Rockland home, Ben described how he hopes the surgery will change his life. He struggled to speak as his head pitched forward repeatedly and his jaw twitched.
Most of Ben’s days are spent at home.
“Basically I just sit around trying not to hurt myself or thinking of ways of what I can do to help and reading a lot of books,” he said.
Ben, as well as his siblings, also suffers from disorders than can occur in conjunction with Tourette’s. All four Schesser children have been diagnosed with obsessive-compulsive disorder, attention deficit hyperactivity disorder, anxiety, sleep deprivation and impulsivity.
“We’ve been each others’ support group for a long time,” Ben said of his brothers and sister.
A Civil War buff, Ben envisions attending college to become a high school history teacher. He hopes the procedure will improve his life enough so that he can do that.
He has reason for optimism. Some patients who have undergone the surgery have seen dramatic improvement, according to Dr. Michael Pourfar, Ben’s neurologist at New York University’s Langone Medical Center.
Pourfar’s colleague at Langone, Dr. Alon Mogilner, will perform Ben’s surgery. Ben will be the team’s fifth patient to undergo the procedure for management of Tourette’s.
“Our outcomes have been remarkably encouraging,” Pourfar said. “Everyone has done much better. We’ll cross our fingers of course that that’s the case for Ben.”
Of three patients who formerly had severe tics, Pourfar said, “If you saw them on the street now, you would never notice.” It’s too soon to assess the fourth patient’s results, he said.
The procedure, reserved for patients with a severe form of Tourette’s, will target Ben’s thalamus, a relay station for nerve impulses that serves as “the Grand Central Station for the brain,” Pourfar explained. After the electrodes are implanted, they’ll be attached through wires that will run along the back of Ben’s head to a pacemaker-like battery placed under the skin of his upper chest.
Electrical impulses transmitted through the electrodes should help to control the misfirings in Ben’s brain believed to cause his tics.
“By stimulating that part of the brain, in ways that are not entirely clear, we seem to be able to not completely normalize but improve the brain circuitry and reduce the impulse for these unwanted movements,” Pourfar said.
Other Tourette’s patients who have had the procedure, however, have seen no improvements at all, he said.
“It’s not a surefire thing,” Pourfar said. “There are still questions about who’s the best candidate and where’s the best part of the brain to stimulate.”
None of his patients has suffered adverse effects from the surgery, he said. Deep brain stimulation, as a wider treatment for depression and other conditions, carries about a 1 percent risk of neurological complications, such as stroke, he said.
Ben will have a good sense of his long-term results in about six months, after his doctors determine the best settings for his electrodes. The equipment will be implanted in his body for life, and he’ll need periodic minor surgery to replace the batteries.
Living with Tourette’s
Ben’s surgery will cost about $200,000. He has disability benefits under Medicare, but because the procedure is considered experimental, Medicare may not pick up the tab.
Regardless, her son is getting the surgery, Wiebe said.
“I can’t not do it,” she said. “I don’t care if I have to pay it until I die.”
Wiebe has witnessed the burden of Tourette’s on her children’s lives. The three boys were all under age 5 when they first developed symptoms, but their mother didn’t know what was wrong. She was pregnant with Caitlin when Matthew, her oldest, was finally diagnosed.
Then, at age 7, Caitlin began having tics — against the odds since Tourette’s is far more predominant in males than females.
Tourette syndrome has a genetic component, and the condition’s severity and how it manifests can vary widely among different people. The outbursts of swearing and obscene gestures often associated with the syndrome are a known but fairly uncommon form of Tourette’s tics.
“A lot of people think that Tourette’s is swearing and being loud and abusive and obnoxious, but that’s not it,” Wiebe said. “There’s so much more to it than that. It affects every area of their life.”
The family has relied on prayer, the support of a church, and each other through their trials with Tourette’s, Wiebe said. They all sing together, often contemporary Christian or gospel songs.
“They have beautiful voices; they can do incredible harmonies,” she said of her children. “It’s a blessing.”
While they’re anxious about the upcoming surgery, Ben’s family hopes that in the new year, they’ll have the old Ben back.
The youngest of the four, Caleb, with a round face and hair dyed bright blue, looks forward to going out to a movie or mowing the lawn with Ben, like they once did.
“I miss doing that stuff with him,” he said.
Anyone wishing to donate money to the family may do so through an account at The First bank on Park Street in Rockland designated as “Ben’s Cry for a Cure.”