Ticked

by James A. Fussell

  “Well, Jeff,” his dad said. “After this operation you might not be able to share so many of your funny stories with us anymore.”

  “Speaking of those stories,” Jeff said. “Why don’t we tell a few now?”

  People with Tourette’s always had stories. And while waiting for surgery, Jeff delivered his like a stand-up comic. He told about the time at the dentist when he ticked so hard the curved, plastic suction device flew out of his mouth and began sucking on his neck. He told about the time when a pharmacist thought he was flirting with her when he tried to hand her a twenty-dollar bill only to have an arm tic repeatedly pull it back before she could take it.

  “And one day I was walking in the parking lot at Target—” Jeff said.

  “Oh, you guys are gonna love this one!” Steve said, a smile of recognition crossing his face.

  “And I see my friend Pete from college,” Jeff said. “He’s walking with his parents. I see them approaching, and I’m like, ‘Oh no.’ At least Pete knew about my tics. So, they come up and Pete introduces me, and his father extends his hand, you know, like, ‘Nice to meet you, Jeff.’ It went fine at first. I put out my hand and shook his. But then—”

  “Uh-oh,” someone said, sensing the story was about to take a weird turn.

  “My hand contracted really hard, and I put a death grip on him, squeezing him so tightly he couldn’t break free.”

  “Oh no!” Aunt Suzie gasped.

  “Oh yeah!” Jeff said. “And then I yanked my arm—and his—back really hard,” he said, clenching his right fist and pumping his arm violently back toward his body two times in a row. “He couldn’t understand what I was doing. He just screamed. You know, ‘Aaaah-hhh! How dare you, you freak? What are you trying to do to me?’ And then his wife yelled too. ‘What are you doing to my husband? Help! Helllllp!’”

  His family broke out laughing. “Oh, God,” Jeff said. “I told him I was so sorry. But he wasn’t in any mood to listen. When he finally managed pull his hand out of my sweaty grasp, he started quickly back for the car holding his hand as if he had just been mauled by a grizzly bear. His wife ran after him. ‘How’s your hand? How’s your hand?’” Jeff said, affecting an elderly woman’s voice.

  “Pete just looked at me. He was like, ‘Don’t worry about it. I’ll explain it to them on the ride home.’”

  The stories just kept coming. He told of the time he felt a massive tic coming on while riding alone in a department store elevator. When he finally expressed it—bending at the knees and clenching his fists as he screamed, red-faced, with all his strength—the doors had opened to reveal a little old woman with eyes the size of silver dollars. She dropped her purse, started screaming, threw her hands up in the air, and ran the other way.

  His family couldn’t catch their breath. After several more stories, they were all laughing so loudly a nurse had to tell them to pipe down because they were disturbing the other patients.

  Jeff was just thankful for the laughter. He didn’t want the last memory his family had of him to be that of someone who was worried or scared.

  AND THEN IT was time.

  As Maciunas washed up, two doctors in powder-blue scrubs with white masks hanging loosely around their chins—one young, the other middle-aged—walked in the room.

  “Ready to get this show on the road?” they asked.

  “Today’s the beginning of a new life for me,” Jeff said. “Let’s do this.”

  With Jeff sitting at a forty-five-degree angle, the doctors put the side rails of the gurney up and got on either side.

  “Do you want your family to walk with you?” one asked.

  “They’re coming with me because they’re going to live my new life with me,” Jeff said.

  His family walked beside him as the doctors rolled him slowly toward the operating room. With each squeak of the gurney’s wheels, it became increasingly more real. This was it. This was happening. Tears began to fall. He looked into the faces of the people he loved the most in the world. They were more emotional than he had ever seen them.

  They didn’t know what to think. Were they wishing him good luck on a new life? Or saying good-bye to him for the last time? Jeff maintained his composure. He didn’t cry. He even managed a smile and an “I love you” as he gave each one a kiss.

  “I’ll see you on the flip side,” he said. “And you can quote me on that.”

  He turned and flashed one last smile as the doors to the operating room closed and locked with a loud click. For Jeff it was a powerful moment. The doors symbolized the end of one chapter of his life and the beginning of another. This was the new frontier. This was hope.

  Jeff looked back through the small windows in the doors and whispered to himself.

  “My past is out there,” he said. “My future’s in here.”

  AS A SMALL group of doctors and nurses swirled around him, Jeff couldn’t help but wonder if all was all going as planned. He wanted to ask but didn’t dare interrupt them, especially during their most delicate work.

  He needn’t have worried. The operation, which lasted roughly eight hours and ended at 4:00 PM, went as well as his doctors could have hoped. Working slowly and carefully, they followed a meticulous plan and detailed computer models to successfully implant the tiny electrodes, called stimulators, deep in his brain.

  There was no infection. No excessive bleeding. And while they still couldn’t be sure it ultimately would work, all indications were that they had hit their target. It wouldn’t be long before they would know for sure.

  Eleven days later, Jeff would undergo a second surgery to have two rectangular battery packs implanted in his pectoral muscles. The plan was to use those battery packs, connected by fine wires to the stimulators, to send electrical pulses into Jeff’s brain in an attempt to interrupt and correct the misfiring signals thought to be causing his tics.

  After the operation, nurses removed the halo around Jeff’s head and wheeled him into a recovery room in the intensive care unit. As he struggled to open his eyes in the dimly lit room he saw members of his family coming in. Drugs kept him relaxed, while the swelling in his brain worked to reduce his tics.

  He looked at Debra with searching eyes and spoke in a groggy voice.

  “Did it go OK?” he said.

  With tears welling up, Debra looked at her husband and bent down to kiss him on his cheek. She took his hand.

  “Yes, honey,” she said. “The doctors said it went well.”

  Later Jeff asked his doctors the same question and got the same answer.

  Still, he kept asking. “You’re sure it went OK?” he asked Debra again.

  “Yes, dear,” she said. Debra wanted to project a confident and strong front. But inside she was scared. Sure, this operation had worked. But what about the next one? And then what? Would they work like they all hoped? Would Jeff be able to be the husband and father he longed to be? Or would he go back to being bedridden again? She swallowed hard. There were no guarantees.

  But as concerned as Debra was, it was Jeff’s father who felt the worst after the operation. “Did it work?” he asked the doctors following the operation. “Is … is he better?”

  “We won’t know that until after the next operation and we can see if everything works the way we hope it will,” Dr. Maciunas said.

  Jim Matovic’s head dropped. Next surgery? Jeff had not told his father much about deep brain stimulation. He had not told him it wouldn’t work right away. He had not told him it had two parts. And he certainly hadn’t told him that it had never before worked on someone with Tourette Syndrome. After the disappointing news, Jeff’s father had to walk off by himself to be alone with his emotions.

  Jeff spent thirty-six hours in the ICU before being transferred to a regular room. As time crawled, his mind raced. Monitors hummed as he stared at the ceiling and thought about his future. He could hardly believe it. This was really happening—and he had already made it past the hardest part!

  He thought abou
t his next surgery and the exciting times ahead.

  “Just a matter of time now, he thought. “Just a matter of time.”

  42

  Turn Me On, Tina

  TWO WEEKS AFTER doctors implanted battery packs in his chest walls, Jeff was ready for the biggest test of all.

  March 4, 2004. For weeks he had stared at the date on his calendar. It was either going to be the best day of his life or the worst. That was the day he would find out if the operation worked, the day he would find out if he had a future, the day they finally would switch on his stimulators.

  That morning Jeff pulled on gray shorts and a white T-shirt, and his father drove him to the hospital. They parked on the fourth floor of the parking garage and then took the elevator down. As they walked up the steps to University Hospitals, Jeff’s body went into a spasm, moving him up and down, up and down. Jim Matovic reached out to help his son, to no avail. It was like holding hands with a yo-yo. Jeff grunted, forcefully expelled air from his mouth, then unleashed violent clenched-fist punches into the air that could have could have put a heavyweight boxer on his butt. Jeff walked like a drunken sailor with exaggerated, pounding steps. Sometimes they looked purposeful and angry, as if he were on a mission to do harm. Other times it looked more like he was trying to stamp out a cigarette butt. As he walked, his eyes twitched, his head rolled, and his neck snapped.

  Once inside, he and his father were quickly ushered into a small room in the neurology department on the hospital’s sixth floor. They were joined by neurologist Brian Maddux, Jim Adler of Medtronic, and clinical nurse specialist Tina Whitney.

  Jeff sat in a chair as best he could. A small crowd gathered around—Maddux to his right, Whitney and Adler to his left, and his father directly in front of him.

  “Are we ready to begin?” asked Whitney, a small, blonde-haired specialist who took the lead role in adjusting Jeff’s stimulators.

  Jeff nodded yes, and everyone seemed to lean in just a little closer.

  Armed with a stylus and a black device slightly larger than a smartphone, Whitney activated the stimulators and began the slow and artful process of tuning him in like a radio. She used her handheld computer to communicate, one by one, with two pulse generators implanted just under Jeff’s collarbone. The computer communicated by telemetry through Jeff’s clothes and his skin. Each electrode in Jeff’s brain had four leads. Tuning the orchestra in his head, as his doctors often put it, would be neither easy nor quick.

  Maddux didn’t know what to expect. He didn’t know if the stimulation would have an immediate effect, a delayed effect, or no effect. Whitney adjusted the amplitude (the strength) and the rate (the frequency) of the pulses-per-second stimulation, which could go as high as 185 pulses per second. She also adjusted the length of each pulse, measured in microseconds. Over several hours she patiently tried countless combinations. Able to work on one side of his body at a time, she tried low frequencies and high frequencies, narrow pulse widths and wide pulse widths. One adjustment made Jeff’s vision blurry. Another made him dizzy. After a third, he couldn’t swallow correctly. But after almost an hour of adjustments, his tics hadn’t improved.

  Although Jeff had been told the process could take some time, his head dropped. He couldn’t help but worry that the operation had failed. Oh well, he thought. At least I tried. And that was worth something.

  But just as he convinced himself nothing was going to happen, it did. The feeling was subtle at first, but it was something. He had never felt anything like this before. It was indescribable—a calmness, a relaxation of tension, a modicum of control. Was he imagining it? No! Whitney’s adjustments had made a difference. Suddenly Jeff’s tics began to lessen. Whitney noticed. So did Jeff.

  “Is that better?” she said.

  “Absolutely!” a stunned Jeff said.

  And then, with a few more adjustments, Whitney hit frequency-modulation gold! Within thirty seconds the four leads on one of the electrodes in Jeff’s brain began producing a series of pulses that caused his tics to virtually disappear on the left side of his body. He stopped twitching. He stopped punching. He stopped jerking and kicking and clearing his throat. More important, the urges that drove those movements disappeared as well.

  Jeff said nothing, almost unable to process what was happening in his body. As wonderful as it was to have the tics gone on his left side, he had now become a strange creature, with powerful movements still ruling his right side.

  His father shook his head as tears rolled down his cheek. He couldn’t believe his eyes. Half of his son was calm, relaxed, silent. “It’s like you glued two different bodies together, but kept the clothing the same,” he said.

  Just then Whitney spoke. “Sorry, but I have to try another combo,” she said.

  A singular thought ran through Jim Matovic’s mind: Are you crazy? Leave it here! It works!

  At the same time Jeff had a fearful thought. What if Whitney couldn’t repeat the success on the right side? But she wasn’t about to let that happen.

  Two hours passed.

  Nothing. Jeff kept ticking and kicking … until …

  Whitney noticed a change in Jeff’s motions.

  “Did you feel something there?” she said.

  “I think so,” Jeff said. “Yeah. I think so.”

  After a few more adjustments, the tics on Jeff’s right side noticeably decreased. Then she added the settings for the left. In thirty seconds the last of his tics—on both sides—simply and quietly melted away. His arms and legs fell quiet. No one talked. In the silence of the room Jeff’s body became perfectly still. Mouths hung open. Against all odds it had worked. For the first time in history, deep brain stimulation had completely worked on someone with Tourette Syndrome!

  Jeff looked stunned as his new, calm body actually responded to his control. He breathed as if taking a breath for the first time. His muscles relaxed and his eyes fluttered as if he were waking up in a whole new world. Maddux looked at Whitney in shock. There were no words. Out of nowhere, Geppetto’s puppet had come to life!

  Jeff’s father bent in front of his son like a catcher in a crouch, mouth open, eyes wide with wonder. His son was back! After thirty years it was more than he could take. Weeping openly, he couldn’t have imagined a greater miracle.

  Neither could Jeff. “I … I’ve never experienced this before,” he said.

  Jeff tried to talk further but stumbled over his words. His eyes darted left, then right, looking at nothing. With all his attention focused inward, he kept waiting for a tic, a twitch, a jerk, a spasm. He kept waiting for his tics to come back, for his speech to be interrupted.

  It felt wonderful but foreign. All the sensations he had grown used to disappeared in an instant. It was strange looking at his father. Rather, it was strange looking at him straight on, without having to correct for blinking eyes or a violently jerking head.

  He was not used to this. This was not him. But it felt unbelievable!

  Jeff hugged his father, then began to cry. An odd sense of peace washed over every muscle in his body. And he could talk—fluidly, without interruption. It was impossible, like some sort of dream, but it was true. He was normal again.

  A shocked Brian Maddux called his partner, neurosurgeon Robert Maciunas. “You’ve got to come and see this!” he said.

  After a few more moments, it was time to take Jeff 2.0 for a test drive. Sure, Jeff felt better while he was seated. But how would he do when he was really moving? He walked several steps around the office. His muscles felt smooth and under control. Whitney shone a laser pointer on the wall.

  “See the red dot?” she said. “Point at it.”

  Boom! No hesitation. Jeff put his finger dead on it.

  “Jeff, now I’d like you to walk down the hallway,” Maddux said. And just like that, Jeff did. He walked, calmly, leisurely, down the hallway. He smiled as he stopped to look at and read everything on the wall. No punches. No kicks.

  Jim Matovic just shook his head, and look
ed at Dr. Maddux as if to ask, Do you see what I see? And do you believe what you’re seeing?

  Thank you, God! Jim Matovic thought.

  Jeff couldn’t believe his sudden stillness either. He picked up a magazine.

  “Dad, I can read this!” he said.

  “I know! I know!” his crying father said.

  “Look! I can hold a cup!”

  For several minutes he kept stating random things he could do to anyone who would listen. Back in the hall he made eye contact with a nurse who was passing by. “Hey!” he said in an uncomfortably loud and cheerful voice. “How are you today?”

  “Umm, OK.” the nurse said. “How are you?”

  “FANTASTIC!” Jeff said.

  He continued walking around the hallway, interacting with anybody or anything, doing things he never thought he’d ever be able to do again. An ocean of stillness took over his body.

  Suddenly everything was amazing. He stood up. Standing was amazing! He sat back down. Sitting was even better! After a few more minutes, Maciunas walked in the room and looked at the man he had healed.

  There’d be time for thank-yous later. Right then, Jeff was too busy pointing out things he could do. “Dad, Dad,” he said, putting his finger on a hospital evacuation plan on the wall. “If there’s a fire, this is where we need to go! And this is where we are!”

  “That’s great, Jeff!” his father said.

  “Want me to turn the lights on?” he said. “Off? On? ’Cause I can do it!”

  He stood on his right leg and held out his arms while lifting his left leg in front of him at a forty-five-degree angle. “Dad, check this out,” he said.