You can only imagine where patients’ thoughts go at a time like this. Unprepared patients can go into a trance-like state. Asking whether they have any questions is basically useless – most just want to run away from you to try and process what you have just told them. I will then get an email a day or two later, asking an array of questions. Typically it explains how they were too shocked at the time to take in anything I said. By this point they have been trawling the internet, further traumatizing themselves. The time of day the email is written is also telling, as are the typos – they are not thinking straight, and writing in the small hours of the morning when sleep is an impossibility.
Anne Marie, however, regained her composure quickly, which in itself was unusual. She dried her eyes, and said, ‘OK, what’s next?’ I was taken aback at her apparent stoicism, but went through the tests I planned to arrange. I told her she should postpone the shoulder surgery. I promised to see her in a few weeks’ time to clarify where we were once we had the results. She appeared to be listening, but I couldn’t help but notice her mind seemed to have wandered.
I suggested that next time Anne Marie might bring her husband as support, and to help take in the information, but when she returned a few weeks later she was alone once again. The tests confirmed the diagnosis, and ruled out alternative possibilities. She definitely had Parkinson’s.
It was discombobulating to see a completely different person in front of me when I went out to the waiting room to call Anne Marie into the office. She jumped up, smiled warmly and all but hugged me. I had spent a few sleepless hours the previous night myself worrying about how best to break the news. I had assumed there would be tears and perhaps some anger, but I got the opposite. She sat down and I said I wouldn’t drag things out, and confirmed that the tests had shown that she did indeed have Parkinson’s. We would have to start with medication and physiotherapy for her left arm and leg.
She smiled. ‘Fair enough,’ she said nonchalantly. ‘Should we start dopamine now?’
‘You seem to be taking this all very well,’ I said. I asked, had something happened since I last saw her? ‘I’m always keen to learn how someone turns bad news into good in their own head, both for myself and for patients in the future.’
‘Well,’ she said, still smiling, ‘it’s a funny thing, but this Parkinson’s thing has been a bit of a blessing.’
Now that was a new one.
‘I was devastated, initially, after our last meeting. But since then I’ve come to terms with it. I have been unhappy with my life for the last few years, and for the last six months I’ve been in contact with an old flame on Facebook. Myself and my husband had grown apart and I was constantly wondering if this was it. Everything felt stale. So, when the ex-boyfriend got in touch, it was exciting. There was a frisson. I won’t lie: we had planned to meet up after the shoulder surgery. I was all set on having an affair with him.’
I was not following at all.
‘So, how does all this make you seem almost … happy that you have Parkinson’s?’
‘I was planning on potentially wrecking my life … and my husband’s … and my children’s. I was going to risk everything for a stupid fling. I know that now. I looked around for the first time in years, and it was like I finally had my eyes opened. I began to realize how lucky I am. When I thought about all that I had to lose, I cancelled the rendezvous with my ex. I said nothing about the diagnosis. It doesn’t matter to him.
‘That’s why I didn’t bring my husband with me; I wanted to come to terms with the diagnosis on my own, to think about what life would be like with Parkinson’s. I know he’ll be there for me and I will bring him to the next appointment. So, basically, Parkinson’s has kind of saved my life.’
It was one of the most uplifting and thought-provoking encounters I’ve ever had.
‘Gunslinger’s gait’ was the term used by a group of researchers a few years ago to describe how ex-KGB men walked, with one arm swinging and one arm straight down by their side. Rumours on the internet suggested that President Vladimir Putin might be in the early stages of Parkinson’s because, like Conor, the Russian president did not swing his right arm when he walked. Ultimately, the research team unearthed a KGB training manual that taught the agents to keep their right hand close to their chest while they were walking, the faster to draw their gun if threatened. When they looked back over the news footage for other possible signs of the condition, they soon debunked the myth that President Putin had Parkinson’s.
The lesson was clear; regardless of the patient, it’s an occupational hazard of neurologists that our training to hunt for clues can lead us to read too much into behaviours; without taking an adequate clinical history and, crucially, putting behaviour and history in context, a strong diagnosis isn’t possible. I have diagnosed Parkinson’s in someone sitting opposite me on a bus or a train, only to realize when they get up and walk off briskly that it’s a Monday morning and their glum, expressionless face is merely showing how they feel about the week ahead.
Having said that, sometimes the signs of Parkinson’s can be extraordinarily slight. I once met an engineer who, on retirement, was given a very fancy hi-tech watch. He wore it with pride and loved telling people how it was motion-sensitive; he joked that he knew he’d be dead if it ever stopped. Within a year of retirement, the watch stopped, but Mike was not dead. He brought it to be fixed, but they could find nothing wrong. It seemed to work well again for a while but then stopped again. Frustrated, he gave it to his son, thinking it might work better for someone else. His son wore the watch for two weeks, and sure enough, had no problems. He felt a twinge of conscience wearing his father’s expensive gift, and returned it to his dad, telling him that he was imagining the faults. Mike wore it for a few more days, but once again it stopped.
Mike is the first and only person I have come across so far whose watch told me practically the exact time he had developed Parkinson’s. The lack of movement in his left arm was indistinct but, like Conor, he was not moving the arm naturally when he walked, so his bradykinesia was such that the watch could not detect any motion. The onset of Parkinson’s can be that subtle.
Of course, not every tremor turns out to be Parkinson’s. Jason was eighteen when his mother, Yvonne, noticed that he had a problem. In fact, she heard it before she saw it. Jason was being the dutiful son, she thought wryly, bringing her a cup of tea while she watched Downton Abbey one Sunday evening. She was as specific as that because she was watching the screen rather than Jason and got distracted by the rattling of the cup on the saucer in his hand.
‘What’s wrong with you?’ she said irritably. She assumed he was still recovering from his Saturday night excesses, having arrived home from ‘gallivanting’ at three in the morning. Jason assumed the same.
‘Oh, stop giving me a hard time,’ he said, and it blew over quickly.
Over the next few months, though, Jason began to notice that when he had indeed been out gallivanting, his hands were shaking a lot the next day. He googled ‘shakes and hangover’ and discovered the phrase delirium tremens (DTs).
‘Oh God,’ he thought, ‘I’m an alcoholic.’ He vowed to cut back on the booze, and he did.
The shaking improved for a while, but soon he realized that he’d shake under any pressure, particularly in front of the class at school. As time went on, the shaking was happening more and more, and now almost regardless of the social setting. It would go away when he was at rest, such as when he was watching television, but as soon as he tried to do anything that required a modicum of precision, like texting or writing, it would start again.
His second round of googling convinced him that he had Parkinson’s, and he watched footage of Michael J. Fox on YouTube for a glimpse of what he thought would be his future self. He decided that he would die young, and that he might as well enjoy himself. So he went back to his late nights and boozy weekend binges. In fact, he quickly discovered that once he’d had three pints, his shaking hands were ro
ck-steady once again – but the next morning the shakes were twice as bad as before.
It was at least six months before he told Yvonne. She looked at his outstretched hands trembling and recalled the cup and saucer rattling so loudly that night she was watching Downton. She recalled how her own mother had a shake in her hands most of her life, but it only caused her difficulties when she was in her seventies. She remembered being embarrassed when they were out and her mum would spill food down her front as she tried to bring a fork to her lips. Her mother had died ten years before from cancer, but she had wondered occasionally whether she had had undiagnosed Parkinson’s.
Yvonne arranged to get Jason an appointment to see me. As it did not appear urgent from the GP’s referral letter, the appointment was scheduled for some months hence. In the meantime, Jason’s tremor was worsening. Yvonne was worried sick and shared her suspicions about their mother’s shakes with her siblings.
‘Oh, that’s the family tremor,’ her elder brother, Brian, told her. ‘I have it, John has it, but Mary doesn’t.’ Relieved but confused, Yvonne wondered why no one had mentioned this ‘family tremor’ before, and how come she had never even noticed it in her brothers over all these years.
‘Mine only started a few years ago, and only affects me from time to time so I just get on with it,’ Brian said.
As a result of her detective work, Yvonne was calmed by the time she brought Jason in to see me. And it was indeed the case that he did not have Parkinson’s. Instead, Jason had a type of shaking called a benign essential tremor. The benign bit and the tremor bit are self-explanatory, but the ‘essential’ part means, well, essentially nothing. We don’t know what causes it exactly. (It is typical of us medics to come up with complicated explanations for questions we can’t answer. We use ‘idiopathic’ and ‘cryptogenic’ a lot too, and again these terms mean, in essence, that we don’t know what causes a condition or symptom.)
Such ‘essential’ tremors tend to run in families and are genetically referred to as autosomal-dominant, meaning that they are transmitted via our genes, so half of the patient’s children might expect to be similarly affected. That said, essential tremors also have what is called variable penetrance, so can affect only one or two of a sufferer’s children or may even skip a whole generation altogether.
The tremor is not, as in Parkinson’s disease, present when the hands are at rest. It comes out when someone is performing a delicate task, like trying not to spill the tea; the cup rattling on the saucer can be a sign. It is made worse by stress, caffeine and, as with Jason, withdrawal from alcohol. ‘So you’re telling him he should give up booze altogether?’ Yvonne asked, hopefully.
I explained that alcohol sometimes actually helps the shaking.
‘So you’re telling me I should drink more?’ Jason jumped in.
It was funny to watch the mother–son interaction as they shot playful digs at each other through me. It was a relief to us all to share a laugh over such a relatively positive diagnosis.
Yet there was no cure for his shaking and the first thing we advise is to cut back on caffeine and alcohol – Yvonne clapped her hands in victory – ‘particularly as many people in the past have become progressively more dependent on alcohol to overcome their shaking’, I said. Jason looked miserable, but cheered up when I reminded him that he did not have the Parkinson’s he was worried about.
There are some drugs that we can use for this type of tremor. Often we use beta blockers, which work mainly by improving the body’s receptors for rushes of adrenalin, the hormone that reacts to stimulating or anxious environments by making the heart go faster and putting you on edge. Beta blockers are familiar from their one-time prevalence in sport for helping with performance anxiety. Until they were largely prohibited, some sportspeople used these drugs in an attempt to control their shaking at critical moments; for instance, when they feared being unable to complete a shot in snooker, make a putt in golf or release a dart.
Generally, once someone is reassured that their shaking isn’t part of the serious or fatal illness they feared, their anxiety subsides, and the tremor improves. And if they then avoid other provoking factors, things can improve even further. I tend to advise medication to help control a tremor only if it’s substantially affecting their quality of life; the tablets I prescribe only mask the symptoms. Most of the people I have seen take the medication for a while, and then gradually wean themselves off it or take it only when there’s cause for anxiety on the horizon – a father-of-the-bride speech is a common reason.
Martin’s shaking came on when he was standing at a pedestrian crossing waiting for the green man. As the cars pulled up and Martin set off to cross the road towards his office he lost control of his body. He later described it as being like a bolt of lightning going through his body and, although it didn’t hurt and he was fully conscious, he became frozen with the shock. Confused, he was roused from his very brief dreamlike state by the car horns of the angry morning drivers. He realized he was standing in the middle of the road and the green man had turned red again.
‘I must have blacked out,’ he thought, but he was still standing and reasoned he would have fallen over if he had truly lost consciousness. He waved apologetically to the angry drivers and headed on into work.
He tried in vain to come up with anything he had done out of the ordinary, but despite his youth – he was just nineteen – Martin was a creature of habit and that weekend he hadn’t ventured far from his usual set of bars and clubs. And though he had taken the occasional ecstasy tablet in the past, he hadn’t taken anything that weekend.
He had a cup of coffee at work and looked at his colleagues in the hope that they wouldn’t notice anything different about him. It seemed that they didn’t, so Martin just went about his day. Still, he was deeply disturbed by this ‘bolt of lightning’ and for the next few days worried he was coming down with something. It was with some trepidation that he came up to the same traffic lights on the mornings that followed. When nothing further happened, he decided he must have imagined the whole thing.
Weeks later he set out on his evening run. Martin was a fit young man who had already finished three Dublin marathons and planned to do Boston the following spring. He did a few stretches and closed the door of his flat behind him. Just a few steps from the door he lost control of his body again. He appeared to be having, he thought, some sort of fit. He stood stock-still, and the twitching movements subsided, but when he took some tentative steps, it happened again. He was sure he faced an early death.
He was grateful that no one had seen this fit, which was as brief as the first. He went back into his apartment, took off his runners and fired up his computer. He googled ‘fits’ and ‘epilepsy’ and decided this was the problem. He recalled a distant cousin who had epilepsy, but his cousin’s seizures were like the ones he saw on television medical dramas, and Martin’s weren’t like that.
His head was full of questions: ‘What will I tell my folks?’, ‘Will I lose my job?’, ‘Will I be able to drive?’ – the websites all pointed out that epilepsy could severely restrict your chances of getting a driving licence – ‘Am I going insane?’
He ran through the gamut of possible neurological diseases as he scrolled. ‘Oh God, I have Parkinson’s. Or multiple sclerosis.’ Finally, ‘I have motor neuron disease’ – the searches nearly always end with motor neuron disease. But Martin kept going until he came across a blog about Huntington’s disease. The blogger’s description of his involuntary movements seemed to match his own experience, though Martin’s twitches were brief and passing.
He closed his laptop and started to cry. His life was over. He would never run the Boston marathon. Suddenly his hopes and expectations of life narrowed down to crossing his fingers and praying to God that he wouldn’t end up in a wheelchair.
When Martin came in to see me the dark circles around his eyes testified to his sleepless nights. He came to see me alone and, perhaps having watched too many fast-talk
ing TV medical shows, he explained his problem at speed. After a few short minutes, he caught his breath, looked at me grimly and said, ‘Give it to me straight, Doc. Don’t sugar-coat it. I can handle it.’
I felt so sorry for him but I struggled to stifle a smile at his Americanisms, gleaned from a combination of Grey’s Anatomy and House, and his attempt to simulate what so-called ‘brave patients’ are supposed to sound like. There was no family history of Huntington’s, and the only person with a neurological problem he knew of was his second cousin with epilepsy. He didn’t smoke, and was very healthy apart from his weekend binge-drinking and the occasional ecstasy tablet. I examined him and could find nothing untoward, so reassured him that I did not think he had MS, Parkinson’s or MND.
After a heartfelt sigh of relief and shedding a brief tear, he said, ‘So, am I going mad?’
It had crossed my mind, and I do see a lot of deeply anxious young people, but I kept that to myself. I explained that I would request some tests and I asked him to keep a record of further attacks while we looked into it.
‘But what if it happens again? Is there no medication you can give me?’
As I didn’t know what I was treating, I couldn’t yet offer a solution. Crestfallen, despite having been reassured that his worst fears were unfounded, he left the clinic room angry and deeply unhappy. He was sure I didn’t believe him, and I didn’t know how to convince him that I did.
I expedited all his tests, and within a few weeks he was back in my office.
‘It’s happened at least ten times since I saw you last. I kept the diary you asked me to, and the only time it definitely doesn’t happen is when I’m watching TV or am in bed.’
He showed me the diary and his extensive notes. ‘Monday afternoon, bolt of lightning when out walking to lunch with colleagues … Thursday morning, halfway across the road on way to work … Saturday morning, two minutes into my run …’ And on it went. ‘It seems when I’m still, I’m fine, but when I move, my body can sometimes go into a spasm like I’m possessed, but not every time.’
Just One More Question Page 21