Just One More Question

by Niall Tubridy

  From the rest of the neurology examination we found a right-sided palsy (or paralysis) of the tenth, eleventh and twelfth cranial nerves – those that enable us to speak and to swallow food and water. Closer examination revealed a small rubbery lump in the right side of his neck. Jack probably had some form of cancer, but how was it connected to the neurological presentation and personality change? If the cancer had spread directly to his brain surely it would have shown itself months ago?

  A lumbar puncture revealed some abnormal white cells and an excess of protein that, though non-specific, indicated that whatever was causing the lump in his neck was now attacking his brain and the nerves coming from it. A scan of his upper body showed abnormal lymph nodes or glands throughout his chest and in his armpits. A brain scan showed more inflammation there. Finally, a biopsy of the rubbery lump identified that Jack had lymphoma (Hodgkin’s type).

  With chemotherapy Jack recovered within months but could not remember any part of his initial hospitalization. By the time he was ready for home, his cognitive score had risen to twenty-seven and his brain scan was greatly improved.

  I saw him in the clinic a few months later. He came alone. Although he was, once again, living independently and working full-time, his wife had had enough and had left him and he was barred from seeing his children. Although physically he had survived cancer he was a broken man psychologically and socially.

  We never found any cancer cells in his brain and we assumed that the cancer had caused his immune system to produce rogue antibodies, similar to Tom’s case, but all the ones we knew of and had tested for were absent in Jack’s blood and spinal fluid. The diagnosis of limbic encephalitis was similar to Tom’s but we had not found the antibody to confirm it definitively in Jack’s case.

  One of my colleagues had been deeply involved in the case and it is to her credit that she continued to show interest many months after Jack had been discharged from hospital. She read an article in a neurology journal about Ophelia syndrome, which I had never heard of to that point. It described a new antibody that attacks the brain and can appear, very rarely, in people with Hodgkin’s lymphoma. We tested some of Jack’s spinal fluid from the time of his original presentation in Casualty and to our astonishment it proved to be positive for these antibodies.

  Jack’s cancer had been insidiously over-stimulating his own immune system, causing personality and memory changes many months before the cancer itself became apparent. He had descended, temporarily, into a Shakespearean form of madness.

  24

  * * *

  ANTHONY’S STORY

  Anthony stepped out of his car, caught his foot in the car door frame and crashed on to the wet pavement outside his home. He cut his forehead badly on the concrete and was bleeding profusely from his left hand when he came into the kitchen.

  His wife gasped. ‘Were you in a fight?’

  ‘I just slipped getting out of the car,’ he said, embarrassed, his pride more injured than anything else.

  Looking back later, Anthony knew this was the moment when he had become sick. Over the following weeks, it became evident to him that his left hand was slow to heal. His grip on the steering wheel was off – perhaps he had done more damage in the fall than he had thought – but he ignored it. He assumed he had sprained his wrist and things would settle.

  After a month, his hand was cramping when he lay in bed at night, and he found it a handicap at his work as a carpenter. A left-hander, he couldn’t seem to use his drill as adroitly as he did before and began to worry he may have damaged the nerves in his hand. He decided to see his doctor soon, but kept putting it off.

  ‘Too busy,’ he explained to me later. ‘Work is insane at the moment and, as I am self-employed, I cannot afford to turn down any jobs. I didn’t have the time to arrange an appointment.’

  He had never been one for doctors anyway, and didn’t want to waste his time or money seeing a doctor who would only tell him what he knew already: that he had sprained his hand and it just needed some anti-inflammatories and rest (and the latter wasn’t going to happen). He carried on as usual and bought some strapping in Boots to form a makeshift splint for his sprained wrist and hand. He started applying some gels to ease the cramps and took Nurofen at night to ease the pain.

  He nearly seriously injured his apprentice some weeks later when he dropped the drill on the poor guy’s foot. That did it. As soon as he finished that job, he booked an appointment with his GP. His arm had been ‘complaining to him’, he said. What he meant was not that it was bothering him, but that when he took off his splint, he could see the muscles in his forearm rolling around spontaneously ‘like they were waving at me, trying to get my attention’.

  His GP arranged x-rays and an appointment with an orthopaedic surgeon. ‘Probably just a sprain,’ the surgeon agreed, and the x-rays showed nothing had been broken. He thought it likely the weakness of grip was due to pain, so, as Anthony had predicted, he was told to take anti-inflammatories and rest the arm as much as he could. He was also scheduled for a physiotherapy session: ‘fecking useless’ was his judgement, and after three sessions he abandoned the exercises.

  As his hand got weaker, Anthony realized that something was seriously wrong. He didn’t tell his wife. Put off by his first experience with the doctor, he started googling ‘arthritis’ and ‘nerve damage’. He convinced himself he had a variety of medical conditions – carpal tunnel syndrome; repetitive strain injury; and even early rheumatoid arthritis. Meanwhile, his hand was undeniably getting weaker, and he had been forced to adapt his drilling technique at work. He started driving his car almost entirely using his right hand. Three months after he had fallen from his car he tripped again. He had been focused on his useless left hand, but suddenly realized that his left leg was weak too. He panicked – ‘I must be having a stroke’ – and drove himself to Casualty.

  Anthony sat in a room on his own, listening to the comings and goings of Casualty outside. He hadn’t told anyone else he was coming. The very act of coming to the hospital had nearly broken him. All of his attempts at denying that something serious might be wrong had folded as soon as the nurse saw him. She knew instantly that his symptoms were ominous, and fast-tracked him on to a trolley. He had then seen a young doctor, who noted the ‘waving muscles’ in his arms and also knew this was not going to end well. The young doctor explained that Anthony’s symptoms did not appear to be related to his joints but were due to a nerve problem. Then I was called in.

  I knocked on the door. ‘Come in,’ he whispered. Tears streamed down his face as he sat scrolling through his phone. Most people do not know the difference between a neurologist and a neurosurgeon, so my patients are often frightened to see me, thinking that they’re about to be wheeled off to brain surgery. When I explain that neurologists listen to patients’ stories, examine them and order tests, as relieved as they might be, they probably think that sounds pretty useless by comparison to surgery.

  He composed himself, and I listened to his story. He had admitted to himself that the fall from his car was a symptom, not the cause of his weak left hand. He had begun to notice his left leg following suit. His voice was shaking and, at times, I could sense the excess of saliva at the back of his throat. He saw me looking, and jumped in with, ‘– and I can’t seem to stop all this spit building up! It’s so embarrassing. I carry boxes of tissues with me to avoid looking like a dribbling mess.’

  As we talked, it came out that he had four young children, and he showed me a photograph of them all together at the foot of the Sugarloaf mountain, where they went for family picnics in the summer months. I knew the area well, and recalled many years of my own family doing the same thing. He stood up so that I could examine his gait, and we could both hear the slap of his left foot on the floor. The cap of his left work boot was scuffed and worn down. This had clearly been going on for months, and he pointed out that he would catch the foot on cracked pavements and was tripping on the stairs all the time unless he con
centrated. ‘I never thought I would have to think about climbing the stairs,’ he told me. He was only forty-six years old.

  He took off his T-shirt and we both looked at the undulating muscles in his arms and all over his chest. This is what he meant when he described his muscles waving at him. These ‘fasciculations’ are invariably a bad omen. The nerves supplying the ‘electricity’ to the muscles were failing throughout his body, and I could see the rippling tissue in his legs, his arms and even on his tongue. This burly man now had visibly atrophied muscles in his hands, which looked like they belonged to a much older and frailer man. He could not even grip the piece of paper I proffered to test his dexterity.

  This was the beginning of the end for poor Anthony, I thought to myself. He could recognize it in my eyes, seeing through my attempts to hide my concern for him. ‘How bad, Doc?’ he asked. ‘I really need to know. I have to look after my wife, my kids, the business, the mortgage … everything.’ My heart broke for him. I knew it was doubtful that Anthony would be alive a year from now.

  One might think that all the worries of life would dissolve into insignificance at moments like this, but the practical nature of people astounds me. He had hardly talked about himself and his own fears at all; he just wanted to make sure that his family was going to be OK. We went on with the exam. His reflexes were brisk despite his failing nerves. ‘At least that’s a good sign, right?’ he asked hopefully. I scratched the soles of his feet and both of his big toes sprung upwards. None of these were good signs at all.

  Motor neuron disease had been much in the news as the bogey-man of all neurological conditions and it is the worst nightmare of all neurologists. As a diagnosis, it is a death sentence, but the condition can vary in terms of how aggressive it is. Typically, patients have a year of symptoms before a diagnosis is made, and then it is a year or two until death, but I have seen people live for years with this cruel condition and battle grimly in the face of the loss of control of their arms, their legs and eventually their speech and ability to swallow.

  When and where do you tell someone they are going to die? Is it in a side room of Casualty, when they’re alone? Should you make it more obvious by asking them to call their loved ones in, and explain it to them when they are all together? Do you gently hint at a diagnosis over a few days? Or end the misery of the patient’s not knowing and risk seeming cruel by getting straight to the point?

  Of course, there is no right or wrong answer, no manual. You just have to try to judge each case on its own merits and hope you get it right – or at least not horrendously wrong. Anthony was smart and, though very upset, he wasn’t angry. What he wanted were answers, immediately. There are some conditions that mimic motor neuron disease, and that are treatable. As a result, I would never say straight out that a patient had motor neuron disease until I had exhausted all the other possibilities. This involves getting blood tests, electrical tests of the muscles and MRI scans. The tests serve to make sure I am not fooled by an unusual presentation of some other rare condition.

  I told Anthony that I was worried about a number of conditions – sometimes a white lie is kinder than the blunt truth when you have not yet confirmed a diagnosis. It may seem unfair but it would have been worse, I think, if you told someone they had an incurable disease before investigating thoroughly.

  This time, though, the tests would only confirm what I already knew. I described how the nerves or wires to his muscles were failing.

  ‘Like a dodgy light switch?’ he asked. ‘Well, who is the electrician and how can we get him to fix the switch?’

  I outlined the tests we would do and that he should plan to stay in the hospital to speed up a diagnosis. ‘I haven’t time for that,’ he replied. ‘I have a communion and a confirmation this weekend; I have two big jobs on at the moment. I don’t have the time to be sitting around hospitals. Will it be quicker if I pay for the tests?’ he asked.

  ‘No. It’s not a question of money, but one of time. We need you to stay in hospital and get everything done smartly so we can plan ahead if there are any treatments for you.’

  And there it was. If.

  ‘What do you mean, “if”? What is this “if”? Are you saying there might be a chance this is not treatable? Do you think I have cancer?’

  His voice had risen an octave. Before my eyes I watched him grapple with his own mortality, all in an instant. He was both heartbroken and terrified.

  ‘I am worried,’ I said reluctantly, ‘that you might have motor neuron disease.’ I paused.

  The tears rolled down his cheeks and he said nothing.

  I went on. ‘There are other conditions that look a lot like motor neuron disease and are treatable, so I am asking you to stay in the hospital so that we can look for all treatable alternatives.’

  ‘But what about the communion? The confirmation? The kids will be in bits if I’m not there,’ he said. He’d returned to worrying about everyone else again – it’s a lot easier to do, in a way.

  ‘You can still go out to both,’ I told him, ‘but you will just have to come back to the hospital afterwards while we work this all out.’

  ‘Jesus,’ he exclaimed as the gravity of what was confronting him began to sink in. ‘Motor neuron is that one with the ice buckets, isn’t it? No one survives that, do they?’

  While Anthony was being admitted, I went on to see the other patients who had come in through Casualty that day: a young girl with migraine; an old woman who had suffered a mini-stroke; a worried mother with a shaking hand; and a 32-year-old man with dizzy spells. Then I dropped back to see Anthony. He wasn’t on the neurology ward. The nurse told me he had gone out for a cigarette. ‘He has been on the phone constantly since you left,’ she said.

  I went out to the hospital entrance, where he was talking animatedly into his phone. ‘It’s going to be fine,’ he was saying. ‘They just want to do some tests. I will be at the communion on Saturday, so don’t worry. But better bring me in some pyjamas and slippers if I have to stay in this kip.’ He caught my eye as he said this and tried to hide his cigarette by cupping it in his hand. Smoking is the least of your worries right now, I thought, as I waved him over.

  ‘How are you feeling?’ I asked, rather uselessly.

  ‘Well, Doc, it is what it is,’ he smiled grimly. ‘Let’s just get on with the tests and see how it goes. I’ve been sorting out the jobs at work so they’re covered for this week. The wife will be in later after the school runs. Life goes on, doesn’t it?’

  It was just hours since I had suggested the diagnosis, and he was almost buoyant.

  ‘We are going to fight this thing, aren’t we?’ he said.

  As doctors we can fool ourselves into thinking that someone is coming to terms with their illness. It certainly makes it easier to deal with for the doctors and nurses looking after people if they seem to have somehow made peace with where they find themselves. That’s because, with terminal diagnoses, the futility of much of our work is never far from our thoughts. We do an awful lot of good and cure the majority of those we see, but there are times when modern medicine is still no match for an illness. And motor neuron disease is one of those illnesses.

  I smiled and agreed that, of course, we would fight it all the way. What else could I say?

  Over the following week I saw Anthony at least twice a day. He was the life and soul of the ward, where he organized an evening card game. I would catch him off guard sometimes wandering back to the ward on his own from his daily smokes – ‘I promise, I am cutting down – I just need a few each day to help the stress of all this,’ he’d explain bashfully.

  The hospital must feel like a prison at times. Your day is ordered for you. You are woken for breakfast and pills. You are called for tests and therapies. You watch the doctors and nurses arrive in an entourage each day and discuss your progress. You observe the medical students unable to hide their emotions at your diagnosis. You are away from all the comforts of home and your loved ones. When they do visit,
they come in with heads tilted and awkward conversation ensues. Their daily news of who did what at school or who called around to the house must seem extraordinarily mundane, compared with what you’re facing, so visits can be trying for everyone, though patients are lost without them. You make friends (and indeed enemies) among your fellow patients – the camaraderie that develops can be wonderful – and then watch as some head home having been given the all-clear. When will it be my turn to go home? When does my sentence end? For some, it doesn’t.

  It is beyond sobering for longer-term patients when one of their circle dies on the other side of the curtain opposite them, surrounded by an army of doctors trying and failing to revive them. The next day someone new will have moved into the recently vacated bed. No one talks about the events of the night before, as it’s all too close for comfort. A silent breakfast follows. The daily routine resumes and by nightfall there is someone new at the card table who never asks how they gained their place there.

  A week or so on, and Anthony had had his scans, electrical tests and therapy. ‘She may be a sergeant major, but I am definitely feeling the strength back in my hands,’ Anthony told me of the physio, delighted with the success of his therapy sessions. I walked with him to the side room with his wife, whom I had asked to come in. They already knew the result in their hearts. From this moment, they couldn’t deny it any longer.

  ‘The tests confirm what we were most worried about,’ I explained. ‘You have motor neuron disease.’ During his week in hospital, Anthony had convinced himself that things would be OK.

  His wife burst into tears and started shouting, ‘No, no. You must be wrong!’ Anthony took her hand. ‘I don’t think he is, love,’ he said. ‘Can I go home now, Doc?’ he asked. ‘Is there anything else we can do?’