Just One More Question

by Niall Tubridy

  At breakfast the next morning, they spoke about the night before. Conor seemed distracted, and she teased him for being hungover and mumbling. As he buttered the breakfast toast she saw his right hand shaking. She knew in her heart where this was going.

  For a while, she avoided Google for fear of what she might find, but after a few weeks she couldn’t hold back any longer. By the time she had finished searching, she was in tears. She had typed in ‘tremor’ and got Parkinson’s disease, as she had expected, but as she was led from one website to another she spiralled into a neurological nightmare of brain cancer and motor neuron disease.

  ‘Oh my God, Conor is going to die. Or worse, he’ll have to live as a vegetable,’ she thought. She rang their old friend who was also their GP and made an appointment for as soon as she could get one. She had not discussed this with Conor, who continued to work away. So often this happens: two people who have spent their adult lives together have the same fears at the same time but do not share them. It seems both protective and defensive – not wanting to face illness in yourself or your partner, hoping that nothing will change.

  When Conor got home to the news he was seeing the family doctor the following morning, they had the first of many arguments about it. He was outraged. He was embarrassed that what he thought was only apparent to himself was just as obvious to everyone else. Gráinne said she was worried about his shaking, and just wanted to make sure he was OK, but he was angry and felt betrayed. So they both spent a sleepless night in separate rooms.

  By morning, things had thawed, and they held hands as they waited to see the GP. Conor suddenly felt ancient, and later said he could only see ‘all the old people’ in the waiting room – and he felt like one of them.

  The doctor called them in, and I would guess had made the diagnosis by the time they had sat down. He had gone to rugby matches with Conor over the years, and many an International weekend was spent in Dublin or ‘on tour’ on the Continent, drinking a bit more than was good for them perhaps, but always having a great time. In front of him sat his good friend, who was only a few years younger than him, but who looked like he’d aged ten years since the last rugby season. He told them he couldn’t be absolutely sure, but suspected that Conor had Parkinson’s disease. Their world crumbled when they heard the actual words – even though they would later admit that this was exactly what they had suspected.

  A few weeks later, Conor and his wife came to see me. My secretary came into the office to tell me he was here and, sotto voce, said, ‘I think he has Parkinson’s.’ We had been working together for some time, and despite her young age she was well able to distinguish the worried well from the truly unwell.

  ‘Why do you think so?’ I asked.

  ‘He looks like one of those Hollywood stars who’s had too much Botox, and he walks like he’s seventy-five. His referral letter says he’s sixty-three.’

  I brought them in and as they sat down in the office I could feel the fear – theirs and, to a lesser degree, mine. Knowing that their GP had already brought up Parkinson’s, I asked whether they had been googling; he said no, but she quietly said, ‘Just a little bit.’ He scowled at her; it was plainly news to him.

  I describe Parkinson’s to patients as like a car running out of petrol, but the fuel in this case is dopamine, a neurotransmitter responsible for conveying information to specific nerve cells from the brain. We start out life with a full tank of dopamine, but for most of us, as we age, our reserves are running out. At some stage, people who develop symptoms of Parkinson’s have reached a critically low level of their natural dopamine.

  While Conor’s diagnosis was as immediately evident to me as it had been to their GP and our secretary, I had to be careful to make sure that I was not missing any other possibilities. Most people with typical symptoms of Parkinson’s disease – a hand tremor present when at rest that goes away temporarily when they move; bent posture; reduced swinging of one arm; an expressionless face – have what we call idiopathic or ‘regular’ Parkinson’s disease. This type usually responds well to therapy, at least in the early stages, and often for many years.

  There is a much smaller group of people who have what is called ‘Parkinson’s plus’ or ‘Parkinson’s “extra”’, who respond less well to treatment, and the outlook is much more gloomy, hence the ‘plus’. These conditions or syndromes look like Parkinson’s, bar a few subtle differences, found with a carefully elicited history and a detailed neurological examination. So some poor folk go to the neurologist fearing the worst – Parkinson’s – only to find out it’s not the worst at all.

  Conor and I walked around the room together. Doing this in an examination where Parkinson’s is likely allows us, and the patient’s watching relatives, to see any differences in posture, arm movements and the ability to turn quickly. Conor shuffled, but speedily, as if he were chasing something, or fearful he might topple over if he lost momentum, like a child learning to ride a bicycle. While most of us have a very fine tremor, if we look closely, when we hold our hands out straight in front of us, Conor had a very visible coarse tremor of his right hand at rest. He smiled briefly when his hand stopped shaking when he held it in a different position, but it soon started up again. When I lifted his right arm up and down it revealed the characteristic stiffness of Parkinson’s; it was like trying to bend a lead pipe.

  I asked Conor to tap his left foot on the ground as if he were listening to a familiar song. I turned away, the better to listen to the rhythm, and it sounded fine. When he tried the same trick with his right foot, however, it was as if his body had lost all sense of rhythm and the jerky tapping quickly dissipated into a barely audible slap of his foot on the ground. The difference was striking. This relative slowness of movement in one side of the body compared to the other is termed ‘bradykinesia’ and is a classic symptom, generally more or less diagnostic of Parkinson’s disease. Poor Conor could see and hear for himself the disease that he had been trying to cover up for so long.

  Conor’s eyes twinkled throughout our meeting. His face looked like he was trying to smile, but his expression was frozen. Gráinne sat by stoically. Now and again a tear would run down her face as she watched the whole process from across the room.

  Conor had all of the signs of ‘regular’ Parkinson’s disease and there was no evidence of anything ‘extra’. Some small solace for me, but not for him at that stage. He and Gráinne reacted calmly to the news that he had an early and, as yet, mild, form of Parkinson’s disease. In a sense there was some element of relief, especially for Gráinne, whose research online had uncovered far worse potential diagnoses.

  Conor decided there and then that he would retire, saying that he had better get busy living life to the full. I counselled caution; to think carefully about any big decisions over the coming months. I have many patients who have lived with the condition well into their eighties or nineties with relatively few problems; others deteriorate rapidly within a few years of diagnosis, and it is simply impossible to know which road any individual’s condition will follow.

  I said to Conor that he should take some time to digest the information, have a think about the treatment options, and engage with a physiotherapy class to which I’m accustomed to refer people in a similar situation. In addition, when someone is diagnosed with Parkinson’s, it can be incredibly helpful for them to talk to other patients. Being part of a class can lead to a friendly sense of competition, and I’ve seen patients come back and say, to their delight, that their classmates hardly believe they have the disease sometimes. (Conor took to this healthy sense of one-upmanship with great enthusiasm.) On the other hand, people who can see they are worse off than those around them can often become even more despondent, so we have to be very careful.

  When he returned a few weeks later to discuss potential therapies, Conor asked whether he could do without drugs for the moment – he felt great, and thought he didn’t need them just yet. I was gratified to see his optimism return, but having sugg
ested he reconsider retiring because the disease might not advance rapidly, I now had to remind him of the flip side: unfortunately, Parkinson’s is a progressive condition, so while a patient might feel fine at the moment, or even years from now, it will almost always disimprove.

  My petrol tank analogy – that Parkinson’s is caused when the tank runs low on fuel (the neurotransmitter, dopamine) – though easy to understand as a crude explanation for patients, is faulty. It’s not just that the tank is full and then runs out. In truth, a healthy brain commonly produces more dopamine than we need – just in case. And, because the human body is at its best a well-balanced system, it also produces enzymes whose job it is to break down the excess of dopamine the brain produces most of our lives. At first, when someone develops Parkinson’s symptoms, a neurologist may try to maintain what dopamine their patient still has by giving drugs that stop the enzymes from destroying the extra dopamine the patient now needs. Later, we might use a kind of ‘fake’ dopamine that stimulates the dopamine receptors – called dopamine agonists – where dopamine usually goes to prevent the disease. Later again, when a patient is struggling – say, to tie their shoelaces, do up buttons, shave in the morning – we will dispense dopamine itself in tablet form, to be taken throughout the day.

  While it’s true that there is no cure for Parkinson’s disease, the treatments can be very effective. It is the neurologist’s job to try and replenish the depleted dopamine with medication. Each drug regimen is specific to each individual, based on when the patient is at their best and their worst every day. This might mean when their tremor is most obvious or when they feel they are at their slowest in terms of their ability to move. We then tailor the amount and frequency of the medication to kick in at the low points (tremors, slowness) and fade away when they briefly feel more like themselves. It gives great hope to the patient and the doctor when they see their facial expressions return, their posture straighten and their tremor abate in the early stages of treatment. This has huge psychological benefit, which can be half the battle for most sufferers. With more optimism, people will willingly engage in physiotherapy. Learning some techniques to manage symptoms boosts the lowered self-esteem a diagnosis can bring. People will start to socialize again, meaning that the brain is stimulated and, crucially, distracted from spiralling gloomy thoughts.

  The cliché of the retired golfer has particular poignancy for people with Parkinson’s. Many of my patients have looked forward to endless days of trying to lower their handicap once they stop working, but it is on the golf course that they experience their initial symptoms. Difficulty pulling back the putter on the green seems to come first, as they have an issue initiating movements. Many then find it hard to chip the ball, hovering over it with their club, as though lost in thought, but really because they cannot get their brains to engage with their hands to get the ball airborne.

  Because golf is such a process-driven game, practised again and again with great attention to detail, it can be a particularly accurate lens through which to measure out a patient’s deterioration over time. Golfers cannot fail to face the truth of their decline when they go from playing eighteen holes a day to nine, to needing a golf buggy, to playing the six holes closest to the clubhouse. The perceived social humiliation is painful to hear about, and eventually many give up their hobby, and with it their social outlets, leading to further isolation and melancholy. A vicious cycle then ensues as the psychological fall exacerbates the physical torment.

  The longer a person with Parkinson’s is on dopamine, the more of it the brain will usually require. The more they need, the more likely it will lead to the development of dyskinesias, or unpredictable writhing movements. It looks like the person is practically dancing all the time whether sitting or standing. It can lead to disfiguring grimacing and, though people who are frozen to a standstill by the condition itself will still get some relief to be moving at all, they and their loved ones can become self-conscious. Simple things, like having a meal in a restaurant, take on enormous significance and I often wonder about the stress the person feels when such public events are arranged. Do they take an extra hit of dopamine before venturing out? If they do, are they worried this will cause their dyskinesia to worsen just as the soup arrives? Many families eventually give up trying to maintain a semblance of their pre-Parkinson’s lives, meaning that now it’s not only the patient who becomes isolated, but their immediate family as well.

  One of my mentors in London was an international expert and a pioneer in the field of Parkinson’s for many decades. His advice about the disease has always stayed with me.

  ‘Dr Tubridy,’ he exclaimed rather grandly, when I finished presenting the case of a man who had early Parkinson’s, ‘I have been studying Parkinson’s patients for over thirty years, but what dose of dopamine do you think we should give this man?’

  I mumbled a few suggestions. In those days, he did his clinics in front of an audience of about twenty visiting students from all over Europe, so it was fairly daunting for the junior doctors, not to mention the patients telling their stories. He smiled at my bumbling efforts.

  ‘Some good suggestions for the average person with Parkinson’s, but there is no such thing as “an average person”, let alone an average person with Parkinson’s disease. After much trial and error, over my many years treating many thousands of people with this condition, I have concluded that the only course of action for a neurologist in this situation is to provide the patient with a bucket of dopamine tablets and instruct them to take them as needed. After a few weeks they will reach an equilibrium, and that is when the neurologist who listens to his patients will know what dose that patient should have.’

  His advice to treat each patient as an individual in individual circumstances has stuck with me for over twenty years – though I try to be more parsimonious with the dopamine.

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  * * *

  TALES OF THE UNEXPECTED

  Anne Marie had swum competitively in her younger years and then coached children at her local swimming club, so she had had her fair share of shoulder injuries over the years. These had culminated in an increasing discomfort in her left shoulder when she tried to lift her arms above her head. This is commonly diagnosed as a ‘frozen shoulder’, and Anne Marie was referred to a physiotherapist. The stiffness eased a little, but after a few months she sought medical help again. An x-ray revealed degenerative changes in the shoulder joint – what medics euphemistically call ‘wear and tear’ – so she was sent to an orthopaedic surgeon. He injected the shoulder with some steroids, which did alleviate some of her pain, but she still couldn’t move her arm freely, particularly when she went for a long run in the morning. The surgeon, having run out of more conservative options, suggested that she might consider shoulder replacement surgery. As she pondered what she considered to be a rather drastic option, she mentioned that she was also now developing an intermittent shake in her left hand. The surgeon said he was not sure what was causing the tremor, and that he felt it was not linked to the shoulder problem. ‘I can fix the shoulder, but it probably won’t help the shake,’ he said.

  And that’s how Anne Marie ended up in my clinic. She was just fifty-one but had many of the early signs of Parkinson’s – she held her left arm stiffly by her side and had the tell-tale resting tremor. She spoke animatedly but in a hoarse whisper. She was married and had two children, but came to the clinic on her own. When I asked about her husband she went quiet. ‘Oh, he couldn’t cope with me complaining about my shoulder any more,’ she said, almost impassively. Did I detect some sadness in her voice?

  We spoke generally. Anne Marie just wanted to know how her years of toil in the swimming pool could have caused the shoulder problem, and how it in turn had caused the shake in her hand. She had not, she said, been on the internet, and this was clearly true, as she was not at all sure why she needed to see a neurologist.

  ‘Well, should I have the shoulder surgery?’ she asked.

  I pa
used. It is shocking for a patient who thinks they have one fairly straightforward problem to be told that they have an altogether more serious one that they will have to live with for the rest of their lives. To diagnose in the first consultation without further tests is risky, lest it be something else and you lose the already unsettled patient’s trust. But Anne Marie’s surgery was scheduled for two weeks hence, so I didn’t have the luxury of time to do tests or to introduce the concept of her condition gently. I was pretty sure, but not definite, that she had Parkinson’s and it looked like I was going to have to tell her without the tests to confirm it.

  In younger people like Anne Marie, we do blood tests and MRI scans and the more definitive dopamine transport (DAT) scan. This is a brain study in which the person is given iodine and a particular scan of their brain is performed. The area of the brain that would be affected by Parkinson’s lights up when things are normal, but appears less bright on the scan if Parkinson’s is present.

  I said I wanted to arrange for tests, and that maybe she should postpone the surgery until I could be more sure.

  ‘But why?’ she asked. ‘I’ve been in pain for ages and want to get on with my life. What difference will your tests make?’

  Perfectly reasonable questions, and I could not avoid telling her.

  ‘I think you may have an early form of Parkinson’s disease,’ I explained.

  She blanched.

  ‘That’s an old person’s disease. You must be wrong.’ She started to sob.

  I went through all the signs her body was displaying and did the foot-tapping trick. Her left leg was lacking the rhythm of the right, and when she saw it with her own eyes, she broke down. It was heart-breaking to see a relatively young woman observe her life fall apart.