One of the many challenges of 2020 has been coming to terms with a loss of control. The future has always been uncertain, it’s true—but a global pandemic has a particular way of bringing that unpredictability to the forefront.
However, for people living with Tourette’s Syndrome (TS), a neurological disorder characterized by involuntary motor and vocal tics, unpredictability is the status quo: living with forces outside of their control is a daily, bodily reality. For them, this year has been even more difficult.
“Stress and Tourette’s are not a good mix,” says Andrew Joannou, a 52-year-old man with TS speaking to me by phone in October from his New Jersey home. “I speak for my fellow sufferers when I say the pandemic has caused a lot of stress, which has caused our symptoms to flare up.” Andrew’s verbal tics are still under control with the help of his medication, but his physical tics—the involuntary twist of his neck, the flinging of an arm—have gotten worse.
When I met Andrew and his wife Amy in 2017, Andrew’s TS symptoms were vastly improved from where they had been just five years before. Andrew and I were introduced through the Longitudinal Experience to Appreciate the Patient’s Perspective (LEAPP) program at the Perelman School of Medicine, which pairs a patient with chronic disease (Andrew) with a medical student eager to learn about chronic disease (me). My first conversation with him was notable more for his humor than his tics, which at that time were limited to jerking movements of his head and occasional spitting. He told me to watch the 2007 documentary Tourette’s on the Job to get a sense of how far his symptoms had come. Like a dutiful med student, I watched it that evening.
The documentary chronicles Andrew’s journey to find steady employment, from horticulture jobs, which he loved “because plants can’t discriminate,” to “dirty jobs that no one else wanted to do,” to his current position as a mail sorter with the US postal service, where he still works today. Out of the four professionals interviewed in the documentary, Andrew’s symptoms are the most severe; we learn that previous neurologists have dubbed his condition “Super Tourette’s.” Throughout the program, Andrew is seen hitting himself on the head with a phone (“I need a rubber phone,” he says), slamming his arm into tables, and buckling his knees as he walks down the hall. “I have pretty much everything,” Andrew says in one scene, and he goes on to describe his arm and leg jerks, his echolalia (repetition of phrases), copraxia (obscene gestures), and coprolalia (obscene language). Though coprolalia is typically the most salient feature of Tourette’s in pop culture, it is actually rare, present in only about 10-15% of people with TS. In a scene showing Andrew speaking on the phone, he pulls out what sounds like a stock phrase: “I have Tourette’s Syndrome so I may curse and shout at you; please don’t be alarmed, okay?”
Andrew was born and raised in London and shortly after birth exhibited what his doctors called “fits” or “seizures.” As he got older his symptoms waned, but then returned with a vengeance at age six or seven when his tics became louder and more physical. He was sent to boarding school at age eleven and was finally diagnosed at age thirteen. “That’s common with Tourette’s—the late diagnosis,” he says. At the time, there was only one medication to treat TS: haloperidol, an antipsychotic drug developed in the 1950s. “Back in the 1980s, when I got diagnosed, they put you on very high doses of haloperidol, basically to zombify you,” he says. “They didn’t know what to do with Tourette’s or how to treat it.” He spent his adolescence in a medicated stupor, failing many of his classes.
Andrew tried other medications in his 20s and 30s, with dangerous side effects: “All the medications made me suicidal. My neurologist told me the depression was part of Tourette’s. They didn’t understand back then what the deal was. I decided to stop all the medications cold turkey because I knew there was something more to the depression than just Tourette’s—something told me there was more to it. So I stopped them all, and within a week the depression totally left. And then I went back to my neurologist, and apart from having my head chewed off for doing such a dangerous thing, he understood.”
But staying unmedicated had its dangers, too. By the time Andrew quit medication altogether, he was living alone in England, and his tics soon became unmanageable. “If I was chopping food, I’d cut myself. If I was cooking a meal, I’d throw the saucepan of food I was preparing at the wall.” He was attacked on public transportation and bullied at work. He found solace at church and found relief in swimming, cycling, and martial arts, cycling up to 100 miles a day. When he was active, his tics lessened. But he couldn’t stay on his bicycle forever.
One of Andrew’s doctors suggested a lobotomy, an experimental surgery at the time, and he almost went through with it. “When I was on the list for this so-called surgery, I made an expensive phone call from the UK to the Tourette’s Association of America to get more information. They sent me the information, and it was basically a study report on 30-something cases, and all the outcomes varied from not working at all to a couple of people being brain damaged. I said, ‘I can’t do this.’” He ended up trying Risperdal, an atypical antipsychotic. “It took away my personality. I was existing, not living. I was like this for six or seven years before I finally went back to my doctor and said, ‘This ain’t working.’” He adjusted the dosage, eventually reaching an unsatisfying middle ground: “I was still having tics, but I wasn’t so dopey that I couldn’t function. But I wasn’t fully alert, either.”
It was during this time that Andrew met Amy. He came to the US for a Tourette’s Conference in 1991 and stayed in New Jersey with a friend, whom he accompanied to work one day. There he met Amy’s mom, who asked him why he wasn’t dating anyone. “I told her that I had never met anyone who could see past the Tourette’s,” Andrew says. “And she said, ‘Well, you haven’t met my daughter yet.’” When Andrew visited the US again in 2000, he and Amy had an opportunity to meet. After a successful blind date, he returned to England, where he emailed Amy for months before returning to the US to stay with her and her family. The rest, as they say, is history; their twenty-year wedding anniversary will be in January 2021.
Of course, romance hasn’t been easy. Once, when they were still dating, Andrew bent down to kiss Amy on the bridge of the nose and had one of his biting tics, leading him to chomp down and break her nose. That same evening, he flung a cup of hot tea at her.
“That was my test,” he says with a grin, “to make sure Amy was being genuine, that she was right for me. She passed the test.”
Once settled in New Jersey with Amy, Andrew continued to search for the right neurologist. “They either weren’t very kind or said that I was too of a severe case for them to deal with,” he says. His tics were so severe at that point that even sitting next to Amy was a risk—his arms would frequently fling out and hit her. “It didn’t bother Amy, but it bothered me.” The chronic pain from his physical tics, due to whiplash in his neck and in his knees, was debilitating.
Desperate for a better solution than medication alone, Andrew wrote to a trusted friend, the late neurologist and writer Dr. Oliver Sacks. Throughout his career, Dr. Sacks wrote frequently about TS and spoke about his kinship with those with TS, calling himself an “honorary Touretter.” Andrew had connected with him through the Tourette’s Association of America, and they met in Dr. Sacks’ New York office in 2001. “At the end of the visit,” Andrew says, “he said he didn’t want to be my doctor—he wanted to be my friend. So we exchanged email addresses.” It was a unique correspondence: Andrew sent Dr. Sacks emails, and Dr. Sacks wrote letters in return. “His handwriting was atrocious,” Andrew once confessed with a laugh.
Andrew had written to Dr. Sacks years before, asking what he thought about Deep Brain Stimulation (DBS), a procedure in which a pair of electrodes are surgically implanted in the brain and attached to a device that emits electrical impulses—a “pacemaker for the brain,” as it’s sometimes called. DBS had been FDA-approved for Parkinson’s Disease, but was, and still is, experimental for severe Tourette’s, though Andrew knew of someone with TS who had undergone the surgery successfully. At the time, Dr. Sacks told him to wait; more research was needed. But years had passed, and this time, Dr. Sacks put him in touch with a neurosurgeon at Mt. Sinai, Dr. Brian Kopell.
When Andrew met with Dr. Kopell, he learned that DBS would help with the physical symptoms but wasn’t likely to improve his verbal tics. Andrew wanted to pursue it anyway, and he started the process to get approval from his insurance company, which was far from a guarantee. In Andrew’s case, approval was swift: “I went to a church prayer meeting and prayed it would happen. I spoke to the medical director and my neurosurgeon on Monday morning. When I returned to work on Wednesday morning, I got a call from Mt. Sinai, telling me my insurance company approved the surgery. If you know insurance companies, they don’t approve things in three days.” To Andrew, it all seemed heaven-sent. Speaking of Dr. Sacks, Andrew says, “He was my angel. They don’t come like him.”
“Leading up to the surgery, I wasn’t scared or fearful—nothing. I was at total peace about it all. There was a chance I could die, but I felt like if I died, it wasn’t my problem,” he says. Dr. Kopell and his team performed the series of surgeries in the summer of 2013: two brain surgeries, a month apart—one for each hemisphere—and then another surgery weeks later to place the battery-operated pulse generators in the chest. The surgery was an overwhelming success. Surprisingly, the first symptoms to disappear were the verbal ones: no more coprolalia. His physical symptoms improved, too, so much so that he was finally able to walk hand-in-hand with Amy without fear of hurting either of them. Since the surgery, his tics have decreased by about 80-90%.
Andrew knows that his challenges are far from over—adjusting his medication regimen and DBS settings, with the help of his neurologists Dr. Andres Deik and Dr. Daniel Kremens, continues to be an ongoing journey. Several years ago, when Andrew’s mother died, the settings on his DBS device had to be adjusted temporarily to account for the stress-related increase in his tics. A disruption in his meticulous sleep schedule can wreak havoc, and his sleep apnea, a common comorbidity of TS, can make quality sleep difficult to achieve. Since the onset of the pandemic, he has had to switch from an antipsychotic that he has used for fifteen years, Geodon, to a similar medication, Zyprexa, to keep his tics under control. He still rides his bike, but lately his neck and knee pain have been interfering with his exercise regimen. When he can’t exercise, he reads his Bible or listens to music. “Music calms the savage beast,” he says, “and it definitely calms me.” British ‘80s bands are his favorite: Tears for Fears, WHAM, Ultravox, Thompson Twins. “Do you even know these bands?” he asks, laughing. (Honestly? No. But still the dutiful med student, I looked them up.)
Though research is growing by the day, there isn’t a wealth of data on long-term outcomes for TS patients who have undergone the DBS procedure that proved so transformative for Andrew. He is in uncharted territory—which, in many ways, is where he’s always been. He believes his life’s mission is to share his story with the medical community, particularly with trainees. In addition to participating in the LEAPP program at Perelman, he gives lectures to medical students throughout Philadelphia and New Jersey. He told me once about a lecture in which he turned off his DBS device for fifteen minutes to show the students how severe his tics are without it. When I asked him if that was a scary thing to do, he shrugged it off.
“I’ve met doctors who should never have been doctors,” Andrew says. “They should have been a butcher, because they treated me like a lump of meat. I have the right to say ‘I’m never seeing a doctor again,’ but instead, I choose to turn the whole situation around and use the bad experiences I had to educate the new doctors-to-be, to make them better doctors than what I experienced in the past. That’s why I do this. That’s my goal.”
In a year riddled with uncertainty, I can say for certain that getting to know Andrew has made me a better doctor-to-be. Over the past three and a half years, Andrew has given me a first-class education in how to approach a life marked by unpredictability: with resilience, generosity, and an unwavering sense of humor.
Special thanks to Andrew’s neurologist Dr. Andres Deik, who pointed me to the University of Florida’s database of Tourette’s patients who have undergone DBS, which will be used to analyze long-term outcomes and reduce some of the uncertainty for people like Andrew moving forward. And to Andrew, for dealing with my unbridled Oliver Sacks fandom.
Jennifer Wineke is a MD/MBE student at the Perelman School of Medicine. Jennifer can be reached by email at [email protected].
