Out of control

Watching Robbie suffer was like having one of those nightmares where you know what’s going to happen but there’s nothing you can do about it. After years of being merely debilitating, his symptoms had finally become unmanageable. I knew he couldn’t continue living this way, nor could we, his parents. Having already tried everything else and failed we were desperate. Our saga began when Robbie was three years old and started to experience small verbal tics—involuntary sounds like grunting, barking, coughing, hissing, sniffing, snorting or clearing his throat—that worsened as he grew older. At the age of seven he was officially diagnosed with Tourette syndrome, but no one could have predicted just how severe his case would turn out to be. Tourette’s, as we learned, is a rare neurological condition that affects up to 1 in 100 people, and is most frequent among people of Mediterranean descent and Ashkenazi Jews. Characterized as a movement disorder, its sufferers lack muscle coordination that leads to tics and other involuntary movements that are often selfinjurious.

Usually diagnosed between the ages of seven and ten, boys are affected three times more commonly than girls. Some of the symptoms can be quite bizarre: frequent and involuntary swearing; obscene and inappropriate gestures and drawings; and repeating the things that other people say. Even stranger, these symptoms are sometimes only exhibited in the presence of others.As the doctors explained it, Tourette syndrome is caused by abnormal circuitry in the brain. Neurons, or nerve cells, fire and misfire out of whack, sending the wrong messages and wreaking havoc. While it not entirely known what causes Tourette’s, both environmental and genetic factors are known to play a role. The resulting tics can be mild or extreme, and sometimes disappear on their own after a few years. Unfortunately, that was not the case for our son. Robbie’s Tourette’s was so severe that it was almost unbearable; his behaviors were just so extreme.

By the time he was eight, the tics were taking over his life. He was pulled out of school, no longer able to function in a public setting. Robbie not only had severe and pronounced tics, but every seven days like clockwork he would inflict serious harm on himself. During these episodes he would repeatedly punch his body, gouge at his eyes and spasmodically contort himself into horribly uncomfortable positions, all the while shouting at the top of his lungs. These major fits would last for two to three hours, and often resulted in broken bones and ruptured discs.  All we could do was try to protect Robert from himself, laying him down on the floor and surrounding him with pillows. We would stay in the house and wait for it to be over. By the time Robbie was a teenager he couldn’t be left alone. My poor son had been robbed of everything by Tourette’s. Tormented by his peers, he had no friends. Shunned by people on the street who were shocked by his behavior, the condition deprived him of a normal existence.

For all intents and purposes he was banished from society. “It’s not something I can control,” he once told me, trying to describe what it felt like to be in the grip of an irrepressible urge. “I’m conscious and totally aware of everything that’s going on, but I have absolutely no control over my body. It feels like someone else has taken it over by remote control.” The doctors told us that while there is no cure for Tourette’s, medications are often successful in controlling the symptoms. Over the years we had tried everything: Botox injections to paralyze muscles and nerves as well as over 40 different medications, including anti-depressants and anti-hyperactivity drugs. Some of these drugs had severe side effects, causing Robbie to hallucinate, experience depression and gain enormous amounts of weight. In fact, the side effects were so extreme that he attempted suicide three times while taking them.  But it was all for nothing, as none of the medications helped him at all. We were at our wits’ end. In 2011, when Robbie was 16 years old, we went to see Dr. Alon Mogilner, a neurosurgeon at the Movement Disorder Center at North Shore University Hospital in Manhasset, on Long Island. (Dr. Mogilner has since moved to the NYU Medical Center in New York, where he is director of its Center for Neuromodulation.) As a last ditch effort he recommended a procedure called Deep Brain Stimulation (DBS), a radical surgical treatment.

While something similar was already being done to control epilepsy, depression, Parkinson’s disease, dystonia and other movement disorders, the procedure had yet to be approved by the FDA for Tourette’s and was still considered experimental. We were faced with an agonizing decision—subject Robbie to the risks of surgery or watch him miss out on a normal life.As the procedure seemed to be Robbie’s last chance at getting his life back, we ultimately decided to go ahead with it. The operation wasn’t covered by our insurance so we held fundraisers. Dr. Mogilner described Robbie’s case of Tourette’s as “the worst he had ever seen.” Accordingly, he would have to be completely sedated, unlike many other candidates for the surgery. The doctor would implant two brain pacemakers in Robbie’s abdomen. Two wires would be run through his body, up into the neck and deep into the area of the brain identified as the source of the problem, where the electrodes would be implanted. In theory, the tiny electrical jolts would stop the overactive nerve cells from misfiring, similar to the way a defibrillator acts on the heart. The continuous stimulation would provide Robbie with “white noise” for the brain, thereby altering the abnormal activity and stopping the tics with the flip of a switch. If the deep brain stimulation were turned off, the symptoms would resurface. The only caveat was that it didn’t work for everyone.

After the operation, we were beyond ecstatic to find that Robbie had been practically cured. The results were even better than anyone could have predicted—and the doctors believe that the effects are permanent. Today, Robbie still occasionally experiences a few small tics, but he manages them with a small external remote control device. Whenever he feels a tic coming on he can turn on the current and stop it in its tracks. “I always dreamed of a time when I wouldn’t have to live with the tics, but I never thought it would come true. I feel like a new person,” Robbie says. Shortly after the surgery our son reached an unbelievable milestone: At the age of 16, Robbie was able to stay home alone for the first time in his life. The operation was truly a medical miracle and turned Robbie’s life around. “It’s like night and day!” he says. “I can now do things that other people take for granted. I can walk down the street without having people stare at me like I have two heads and without having to hear their hurtful comments. I can start living the life of a normal person, making friends and not having to explain to them before we go out what is inevitably going to happen. I got my driver’s license. I’ll be able to get a job. I would actually like to become a doctor so I can help others like me. It’s incredible!” As an added bonus, Robbie lost 120 pounds after stopping his medications. There is happiness again in our home. Robbie is regaining his confidence and enjoying life to the fullest. He’s back at school— and driving there himself. You have no idea how overjoyed we are to watch him grow and chase his dreams.

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