7
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LOCATING THE DAMAGE
I love the flow of the neurology exam. Listening to the heart is in and of itself beautiful but limited. Listening to and examining the respiratory system or laying your hands on an abdomen are also interesting but nothing, in my view, compares to the long-playing record that is the well-performed neurology exam. From my student days I found the theatre of it instantly appealing. But quite apart from the flowing aesthetic, the realization of how we move, talk and even think is thought-provoking on an existential level.
A careful observation of each patient’s gait is still my favourite part of the process – though it may not be the patient’s favourite, particularly if they’re walking in front of student doctors. When I was training we would stand like a small army and watch the patient walk the length of the ward corridor and back as if they were on a catwalk.
There then follows an intricate examination of twelve cranial nerves on either side of our head that orchestrate how we see, speak, smell, smile, blink, nod and hear, among other things. This part of the exam also looks at how we eat, how we swallow and even how we move our tongues. It is always mind-boggling to see the intricate ways that these things we take for granted when well can go wrong with the smallest of brain insults. A broken wire here can lead to a dropped face, or an infection there can cause the whole system to collapse.
The examination then moves on to the limbs, examining the motor system connecting our brains to our hands and feet, and then the sensory system running in the opposite direction. I always see it as a railway line that runs from the right brain to the left foot and from the left brain to the right foot. From head to toe the examination looks at the fidelity of the train track. Is it intact and, if it is not, whereabouts has the train been derailed? Was it on the outbound trip or the return journey?
Motor testing assesses the power and tone of each of the muscle groups. It is designed to establish the integrity, or otherwise, of the wiring or neural tracts that run from the brain to our hands and feet. Testing involves a set of simple dance-like moves that, when a patient is showing symptoms of neurological disease, can look a little like a wrestling match. I grasp the patient’s arms and legs gently at various points and move the limbs in different directions to assess muscle tone. I then wield a tendon hammer – a long stick with a round rubber head – to elicit the reflexes. You could visualize the various reflex points as being in a Christmas tree formation from the top of the body down. The reflexes mark various branches along the nerves’ journey to the arms and legs – and, if absent or exaggerated, highlight areas where the fault may be localized.
Patients often gasp at their knees flying up in the air when tapped with a tendon hammer (‘at least that is working’ is the universal comment), like they did as children when they tapped their knee. I remind young students not to express similar delight when they ‘get’ a reflex for the first time – very uncool in front of patients and hardly reassuring if they appear bemused by their own competence.
At the end of the motor examination, I run a key or orange stick firmly along the soles of the feet. You may recall that in chapter 1 Jenny’s big toes springing upwards told me that she had suffered serious nerve damage. This is a reflex called the Babinski (or plantar) response and testing it is an integral part of the neurology exam. It’s a peculiar-seeming thing to have done to you and I can only wonder what patients think when they come to a doctor complaining of visual problems or headaches and he or she asks them to take off their shoes and socks and proceeds to scrape their feet firmly. (I have learned to explain what I am doing in detail after a young woman asked many years ago whether I had a foot fetish.) When we are born our big toes go up when the sole of the foot is stroked. After a year or so, stroking the sole causes the big toe to bend downwards in keeping with the development of our nervous system. If the nervous system is damaged in certain areas later in life, as can be the case in MS and many other neurological conditions, the toe assumes the ‘baby state’ and goes up again when the sole is stroked. It is one of the most important signs in clinical neurology, in my view, and toes curling up are never a good thing for the patient.
Having completed the motor testing I then do the sensory exam to assess the wires running in the opposite direction – from the hands and feet to the brain. Two of the main tools used for this are a tuning fork and a tooth pick, asking patients whether they can feel the vibration of the tuning fork or the sting of the tooth pick as I work up from their feet to their head. There are two main sensory lines. These determine how we respond to the world around us and explain how we react to pain, to hot plates and even how we feel our feet touch the ground. Again the goal of the sensory examination is to ascertain whether the return journey is interrupted and, if so, to work out where the ‘train’ has been derailed.
Once the location of the problem has been established, we try to determine what caused it. To do this we perform scans of the location, like mechanics taking photographs when they survey the site of a train crash. Only when we have been able to diagnose the site of the damage can we begin to try to fix it.
Precisely what to tell patients, and when, about what their condition might be is a delicate process. Naturally enough, they are extremely eager to have an immediate and full picture of what’s going on. Sometimes I’ll explain how I might suspect a certain diagnosis, because I’ve seen it many times before. Aware that I could be wrong, I won’t go into the intricate details while we’re waiting for tests to be performed. This inevitably leaves the patient with the impression that I am hiding something from them. And in a sense I am – but not with malice. I’m trying to protect them from unnecessary anxiety, in case my initial suspected diagnosis is wrong. If I were to list all of the possibilities – everything that might be wrong, called the differential diagnosis – I could scare them witless. Worried patients tend to have a heightened awareness of a doctor’s mood and tone, and any apparent furtiveness leaves them feeling vulnerable. Patients’ fear that they are losing control is an important part of the interaction too, especially for men it seems to me, where a sense of emasculation can be at play. No one likes the idea that their fate is in another person’s hands.
Apart from judging how much information to give patients, choosing the right words can also be a challenge. On the one hand, your patients need to understand that you know what you’re doing. On the other, you must be careful not to overwhelm them with jargon. There is a line between trying to engender confidence in your abilities and sounding arrogant. Like most doctors, I have inadvertently crossed that line, of course. When you are trying to explain very complex things that you have been studying for many years, it can be hard to give people a clear picture of their diagnosis and your understanding of it in a single consultation.
So, in delivering news, I try to get the balance right with every patient. And I fail with regularity. For instance, I can explain how we are going to do tests to rule out serious illnesses, only to hear them outside on their mobile saying, ‘He thinks I have cancer.’ And if I remain vague, either because I need more information from test results or I am suspicious that something serious is going on and I want to be absolutely sure before breaking bad news, I will hear them telling friends, ‘He hasn’t a clue.’
James’s father and grandfather had Huntington’s disease, as had two of his three brothers. Huntington’s is a hereditary brain disease that leads to abnormal writhing movements, called chorea, and then dementia. One of his brothers had taken his own life at forty-three years of age when his brain began to disintegrate and he could no longer dress himself. James was forty-six and had been referred because he had begun to get ‘the shakes’ when presenting at business meetings in recent months.
We shook hands and he sat down in front of me. He had nicked himself shaving that morning, and the third button on his shirt was undone. I asked him to describe his symptoms, and he did so in a detached way, seemingly only mildly perturbed by h
aving to sit on his right hand during meetings. At times he felt he was unable to control it. I asked about stress; he held a fairly high-powered post that meant long hours and sleepless nights worrying about ‘the bottom line’. He had three children, the eldest of whom was heading to college in the autumn. James had worked hard to provide for himself and his family and happily admitted to a fairly comfortable lifestyle.
I asked about his family history and he went through it dispassionately; obviously he had always been keenly aware of Huntington’s and its symptoms. Throughout the conversation he would intermittently rotate an arm in a very subtle but definite writhing movement (as we all do when scratching an itch and don’t want to draw attention to the fact), or his leg would jerk forward very slightly from the chair he was sitting in.
I asked how long he had had these shakes.
‘About a year or so, but they haven’t bothered me that much till recently.’
‘And has your wife noticed anything?’
‘I’m not sure if she has or not. I don’t think so. She hasn’t said anything.’
I was fairly certain that James’s wife had noticed, but exploring the extent of a patient’s denial is worthwhile; I need to know how best to deliver bad news.
‘Well, what do you think it could be?’ I asked.
‘You’re the doctor,’ he said, smiling. ‘Just as long as it’s not what my dad and brothers had, I’ll be able to deal with it.’
Huntington’s can come on at a variety of ages, and at forty-six, James thought the family disease had passed him by. It is a particularly cruel condition because it is inherited, but the chances of the children of a parent with the disease inheriting the genes is 50 per cent. A very accurate test can confirm whether you have the relevant genes; if you do, you will get the disease, but with what symptoms, or at what age, we can’t be certain. James’s brothers had developed symptoms in their early twenties, so James had reasoned that he was now too old to have inherited ‘the family curse’, as he called it. If he did, his three children would have a 50 per cent chance of getting it themselves. This can be the hardest part of a diagnosis for parents; the guilt they carry with them can be as hard to bear as the physical suffering of Huntington’s and other genetic disorders. Symptoms typically start with unusual shaking and evolve into squirming movements. Eventually, it leads to dementia.
After talking to James for a few minutes, I had no doubt that he had the condition, but his denial was so deeply ingrained, I knew that the diagnosis would be particularly traumatic for him. You try to be straight with people without being too harsh, but there are occasions when dancing around what is self-evident can be unnecessarily cruel. I told James that I thought he might have the disease, but that other conditions could mimic Huntington’s, so I would look for those too.
I arranged a variety of blood tests and a brain scan in the hope we could uncover an alternative diagnosis with a less grim prognosis, but I knew it wasn’t to be. Doctors get scared of a terminal diagnosis too and I needed some time to come to terms with having to condemn him to the disease he’d always dreaded, and having to inflict panic on his wife and children. The family history was telling us what was staring him in the face. He signed the consent form for the genetic tests after I had explained the implications of a positive result.
‘I think you are wrong on this one,’ he said. He was still smiling, but less convincingly now. I hoped he was right.
A few weeks later he returned and a different personality stood before me. Gone was the jocular, confident businessman of a few weeks previously. He looked pale and drawn. He had discussed it with his wife and they had hardly slept with the worry waiting for the test result. They came together for this appointment and looked at me with desperation. They both knew there was to be no reprieve.
They cried as they held each other, and I promised meekly to see them again soon to go through things further when they had had time to gather their thoughts. At times like that there is nothing you can do other than to say you are sorry to be giving the news. I sat there, shell-shocked myself, as they left the room. After a few minutes the clinic nurse peered around the corner of the open door. ‘The patients outside are getting restless,’ she said. I scanned the next chart, stood up and went to the waiting room.
8
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SLIDING DOORS
I was on call one evening as a senior house officer and met a lovely elderly lady who told me she was from Galway. When I told her my name, her face lit up and she proceeded to tell me a story about my grandfather, Seán Tubridy, who hailed from Oranmore. He had qualified as a young doctor in Galway, and was training to be a GP. He wanted to make some extra money, and successfully applied for a post in the remote coastal area of Connemara called Beal an Daingeain, where the senior GP was about to go on his annual holidays and needed cover.
Seán arrived the night before he was due to start and was going through the handover of his duties with the GP when a call came looking for an dochtuir from one of the Irish-speaking islands nearby. I can only imagine his excitement – he’d have had little chance of ‘home visits’ by boat until now – but the local doctor said he’d take the call himself to ‘clear the slate’ for Seán. The senior GP set out with some local boatmen in a currach, a small rickety boat made in the traditional way, with animal skins stretched over a wooden frame. It was a rough night at sea and none of them made it back. Their bodies subsequently washed up on the mainland.
This is our genealogical ‘sliding doors’ moment. If my grandfather had been on that boat, my father would never have existed and I and my siblings would never have existed. I had never heard this story before. We’ve since verified it and it has stayed with our family ever since. The element of chance in all of our existences is both freakish and extraordinary. Seán went on to be a well-known GP in the Galway area and eventually was elected to the Dáil to represent the people of Galway.
Years later, another elderly patient mentioned the ‘Tubridy stones’ in her area of Connemara.
‘What are they?’ I asked innocently.
‘Oh, don’t you know?’ she asked conspiratorially. ‘Your grandfather would go on his house visits to very remote areas of the countryside. Most of our families had little or no money, so he would be paid with a meal and a drink or two.’ She smiled. ‘There was no such thing as electricity or street lighting at that time. There might have been the odd occasion when he was given’ – so generous were her words – ‘a bit too much to drink, so we would paint the stones in the road white to help guide him on his way!’
‘Are you saying he was a drunk, then?’ I asked.
‘Not at all,’ she said. ‘That was the way of things back then.’
My grandfather died aged forty-two, apparently from a heart attack. Since hearing about the events of his first locum I have sometimes wondered if the trauma of his near miss affected him psychologically and whether he felt guilty about surviving when others perished. Maybe his premature death was hastened by the stress of surviving and too many drinks.
My father practised psychiatry for many years in Dublin. His speciality was helping people with alcoholism. I always wondered whether he chose, subliminally or otherwise, to treat alcoholics because of the stories about his father, who died when my father was three years old.
When we were younger, my siblings and I would go along with my father and our excitable Irish red setter on his weekend hospital visits where he did his rounds. It gave my mother a precious break for a few hours from the havoc created by five young children. He would park in the hospital car park and we would run wild in the grounds. We would gather pine cones in St Gabriel’s or St John of God’s for burning in our fire in the living room that evening, and the dog would chase whatever unfortunate birds were resting in the otherwise tranquil surrounds. Not infrequently, a janitor, nun or priest would come out to investigate the commotion being caused by these feral children and their mad dog. It is funny now to think of my poor dad goi
ng into the austere surrounds of the psychiatric wards, switching from parent to doctor mode, while we ran riot outdoors.
Occasionally, it wasn’t so austere on the ward, however. Much later, I once witnessed mayhem on the locked unit of one of the hospitals in which he worked, and I thought how jarring it must have been, having to switch from the domesticity of children and a dog on a Saturday morning outing to the seriousness and desperation of the patients he was visiting. He would return to the small car exhausted and light up the first of many cigarettes, the five children and the red setter be damned, like a scene out of Mad Men.
In the days before mobile phones and the internet we had a single house phone and it is funny to recall the excitement the loud ringing in the hall would generate. We had to go ex-directory after a series of late-night calls from a few of his patients who had fallen off the wagon and were angry – at their own failure, at the doctor who had failed them – and indulging in the ultimate ‘drunk dial’. The phone ringing was such a big deal that we would all wake to the racket. I knew how deeply it upset him and he would just be a bit quieter the next morning but life would go on as usual for us.
In the 1970s and 1980s psychiatry underwent some seismic changes and our father was sometimes described as ‘old school’ by the trainees I’d later meet – a back-handed compliment, perhaps, but his philosophy was a simple and straightforward one: listen, listen and listen some more. The film One Flew Over the Cuckoo’s Nest became part of the social vernacular when it came out in the mid-1970s. Electroconvulsive therapy, popularly referred to as electric shock therapy, for people with depression so severe that anti-depressant drugs could not help them, was in relatively common use in psychiatric hospitals throughout the world, and Ireland was no exception. The changes wrought upon the Jack Nicholson character, ‘Randle McMurphy’, in the film sent a shiver down the spine of most audiences, and scared the world at large as to what could happen in ‘the loony bin’. And any nurse who was anything less than sweetness and light every hour of every shift was cruelly dubbed ‘Nurse Ratched’ after the heartless nurse in the film.
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