Like myasthenia, Guillain-Barré is associated sometimes with a rogue antibody produced by our own defence mechanisms against infections, but where myasthenia attacks the junction between the nerves and the muscles, in Guillain-Barré syndrome the problem is a direct assault on the nerves themselves. So, to return to the postman analogy, whereas in myasthenia the rogue antibody blocks the delivery of a letter just as the postman is trying to put a message through a letter box, in Guillain-Barré syndrome the antibody wrecks the garden path and the postman hasn’t got a hope of getting near the letter box to make his delivery.
We performed a lumbar puncture straight away and found that Susan had excessive protein in the fluid surrounding her nerves. This is usually a sign of infection or inflammation in the fluid in which the nerves from the brain are bathed. A further scan with contrast (dye injected into a vein) confirmed the nerve roots were inflamed, causing her low-back discomfort.
We set up the drip for the immunoglobulin and started treatment right away. We also organized electrical tests, but this time for the nerves, not the muscles, and these confirmed our suspicions.
Within a few days Susan was on the mend. Against her better judgement, she had googled her condition, but stopped reading when she realized how much worse things could have been. Some people respond to treatment quickly, but others can spend months in hospital unable to walk. When I saw a young man with Guillain-Barré syndrome over twenty-five years ago he was staring at a fly that had landed on his left hand. The fly meandered up his forearm and then flitted on to his staring face. I swatted it away gently and he blinked rapidly at me, as if to say ‘thank you’. He was conscious; he could hear and see everything – and he could feel the fly on his body, but could do nothing about it. He had developed a rapidly ascending weakness of his legs, arms and facial muscles. He couldn’t breathe without the help of a ventilator. His frustration was palpable, and I could only imagine the helplessness he felt. The use of good antibodies for Guillain-Barré syndrome was then in its infancy, so many people who were diagnosed with the condition had to hope that their immune system would stop attacking their nerves, and that the nerves would recover slowly on their own. (That young man was in hospital for many months and eventually transferred to a rehabilitation facility. He survived but required a walking aid for many years and was never able to return to his job as a mechanic. I saw him for a few years for follow-ups, but then he stopped turning up for clinics.)
Susan responded rapidly to the ‘good antibodies’ and within a week she was walking with a Zimmer frame. ‘I thought I wouldn’t need one of these until I was eighty,’ she smiled ruefully. She recovered slowly over the following few weeks, and when I saw her in the clinic she walked in with a single crutch. Having seen many cases of the same condition with much worse outcomes – including death – I was delighted with her improvement. Her husband, having seen his beloved wife unable to move her arms or legs a few weeks before, was relieved she was alive.
Susan, however, was deeply traumatized by the whole episode. Her time in hospital had taken a serious physical and mental toll. She suddenly looked older than her years. Her recovery was slow but sure; nevertheless her transient dependence on others made her miserable. Her confidence in what she hitherto had taken for granted was shot. She said she felt diminished in front of her friends and family. She felt diminished in front of herself. She was fearful of going outside her own house, such was her loss of self-esteem and confidence. She would not go even as far as the local shops, although physically she appeared able to, as she could not overcome her fear of being seen in public feeling as vulnerable as she was.
It would take many months for Susan to return to some semblance of her former self, but even after a few years she was not the same person psychologically. Eventually I suggested she was recovered and no longer needed her annual check-up visits. I thought she would be cheerful – it is always a good thing, I tell patients, to say goodbye to your neurologist – but she became upset.
‘What if it comes back?’ she said.
‘That’s highly unlikely, but I’m here if it does.’
‘But would you not just see me, even briefly, once a year – for my peace of mind?’
Naturally, I agreed, and for many years after we met annually and discussed life and the universe – everything except her illness which gradually faded into the annals of her memory.
Often when I try to discharge people from outpatient clinics they will almost plead not to be set adrift. ‘Just in case’ is their mantra; ‘to be on the safe side.’ They ask to see me even though the only thing I’m doing is reminding them that they were once ill. They worry that the condition will come back, and need the metaphorical crutch that the doctors who helped them get well will be there if it does. It is sad to see such people – for they are no longer patients in my eyes, even if they remain so in their own – struggle to return to their previous selves.
I meet people many years after I have been involved in their care. Some come running over – always particularly welcome in the neurological world – to celebrate their good fortune with me. But, while many survive neurological illnesses like Guillain-Barré syndrome, some can be left with permanent damage to their arms or legs. I might see such a previous patient in the supermarket, limping away from my oncoming trolley, avoiding saying hello. It is disheartening, but understandable; their successes are my successes, but their sense of failure when their bodies don’t fully recover is, in a sense, my failure too.
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MORE THAN JUST A HICCUP
Something as mundane as a hiccup, which we all experience, can be a case of food going down the wrong way or it can herald a cataclysmic brain injury. So while most normal people will wait for an episode of hiccups to blow over, or hold their breath to stop them, a hiccupping neurologist will worry about his brainstem. No wonder we seem weird to many people.
A hiccup is an involuntary spasmodic contraction of the diaphragm followed by the rapid closure of the vocal cords. Hiccup, hiccough or singultus are interchangeable terms used to describe this phenomenon. What causes them and what function they serve in evolutionary terms is not clear. Generally people can get a brief bout of hiccups after consuming particularly spicy food, alcohol or even hot drinks, but they can arise as a result of any condition that irritates the nerves that control the diaphragm (the muscle that controls breathing).
As we all know, hiccups only usually last for a few minutes, or at worst a few hours. Most commonly, they stop suddenly for no obvious reason and some attacks can be stopped by blowing into a paper bag or drinking cold water. Hiccupping that goes on for more than two days is labelled ‘persistent’, and hiccupping that lasts more than a month is ‘chronic’.
The vast majority of cases are benign, but hiccupping can be associated with lung diseases like pneumonia, with kidney failure, and with disorders of the stomach and bowel. Hiccups can also be due to neurological damage in the lowest part of the brainstem, called the medulla. Such conditions include strokes, cancers and infections. So, for example, I have seen people present with hiccupping for days on end as the first sign that they’ve torn an artery in their neck, which has led to a small stroke.
I was called to Casualty to see Joseph, a 38-year-old Scot who was having difficulty swallowing. Earlier that day he had been doing the front crawl in the swimming pool and after getting out of the water he complained of some pain in his neck. He did not put too much store by it. He had been a rower and had had a fair few neck injuries in his time. But at home an hour or so later he started choking on his evening meal. He could not swallow the roast potatoes, however small he cut them. Then he started to hiccup, and did not stop.
Joseph’s hiccupping was the first sound I heard when I approached his trolley in a pretty chaotic Casualty. He and his wife both looked exhausted and troubled. I explained that I had been asked to have a look to see if there was a neurological cause for his swallowing problems and persistent
hiccupping.
His speech was very slurred and I found it hard to understand him, so I proceeded with the examination. The combination of the neck pain, the sudden onset of his symptoms and the slurred speech suggested the hiccups could be due to a vertebral artery dissection – that he had torn a small blood vessel in his neck. He was probably predisposed to a tear like that by his previous injuries and this vulnerability was possibly increased by his vigorous front crawl. As a result of this tear he had had a small stroke in the lower part of his brainstem controlling his swallow and some of his speech mechanisms. The hiccups were also due to this. These certainly seemed to be the most distressing symptom for him.
We arranged an urgent brain scan and identified the damaged blood vessel. The hiccups disappeared after two days and, with the aid of blood thinners to avoid further injury, Joseph was eating and talking properly again within a week.
A case like Joseph’s is straightforward, but sometimes hiccupping remains a complete mystery. When I was in Melbourne I met Ian, who had arrived to attend a routine appointment for which he had been waiting for some time. His wife accompanied him to the packed clinic on a sunny Thursday afternoon, and they were the picture of patience when I called them in.
When I asked Ian what the problem was, he said, ‘Can’t you hear it?’
‘Hear what?’
He had developed hiccups in his teenage years and still had them, aged forty-five.
‘So this has been going on for nearly thirty years?’ I asked incredulously.
He confirmed that it had. I didn’t have to take a history to work out that if he had been making this strange noise all of his adult life, then he must have married, held a job and conceived several children, hiccupping all the way.
‘Did you hiccup your way up the aisle on your wedding day?’ I asked, rather rudely. I was just bowled over by how unperturbed both Ian and his wife seemed to be.
‘You get used to it,’ his wife said in a matter-of-fact tone. ‘And, sure, is that not what love is? The good, the bad and the ugly.’
Ian could not remember the exact moment it started and said that he had woken up one day in his mid-teens and started hiccupping, and never stopped. He had seen many doctors over the years, and had had multiple tests of his lungs and stomach, but no cause was found. He was also seen by a number of psychiatrists in case there were some unresolved childhood anxiety disorder; nothing was found – he was a perfectly happy child with a loving family. Now, as a last resort, he had been sent to a neurologist. Of course, most cases of hiccups are not due to neurological disorders. Indeed, I had never seen such a case before, and have only seen a couple since.
An MRI scan revealed a small scar in the lower part of the stem of his brain. The scar was likely due to a small stroke as a teenager (most likely, but purely speculatively, caused by a tear or dissection of a blood vessel in his neck). As there was nothing that could be done to remove the scar without potentially causing untold nerve damage to the surrounding brain structures, we tried a number of medications to alleviate the symptom.
As is often the case, Ian felt the medication made him feel ‘stupid’ and ‘like a zombie’ and he decided to stop it, a reasonable thing to do in the circumstances. The hiccups returned.
There was nothing more I could do to help him. In my early days a case like this, which seemed to confirm the stereotype that neurologists ‘make a diagnosis and apologize’, would make me despondent. But getting an explanation for what had caused the hiccupping gave Ian and his wife peace of mind. They could finally stop worrying that something sinister, like a brain tumour, was growing in Ian’s head. He said he was so used to the symptom he could live with it now that he was sure it was not going to kill him. ‘So I am not mad, but I am unique,’ was his happy response. He was (almost) unique. I presume he is hiccupping to this day.
21
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BURNOUT
I never really considered, until I was much older, how my father must have felt driving home from his rounds on a weekend morning, puffing furiously on a Silk Cut purple. Ireland is a small place, and it is inconceivable that he would not have been socializing with some of his patients or their relatives. These were stories you would rarely hear told in any detail, particularly because of his work with people suffering from addictions. And there was never a shortage of patients for him in 1970s and 1980s Ireland. People came from all over the country with their own stories and those of their families being torn apart by alcoholism. So he talked in general terms – how marriages were destroyed by an alcoholic spouse resistant to his attempts at therapy; the abuse suffered by the children of an alcoholic parent who refused or was unable to stop drinking; or the havoc wreaked by a drug-addicted child on their parents and siblings.
Not everyone can be helped, and I recall in my late teenage years going for my first legal pints with my father in our local village. He would go in ahead and survey the room. ‘Not this one,’ he would whisper, shutting the door behind him as I waited in the street. We would try one or two more pubs before he would settle on one that he deemed safe. When I asked him about this strange habit, he explained that not all patients wanted to see the doctor who had ‘failed’ to cure them of their alcoholism, particularly while they were drinking in their local.
Some former patients could be abusive, and he did not want to expose me to an embarrassing scene. How sad, I thought. He was doing his best to help people, but those whose treatment was not successful were actively unpleasant to him. I would come to understand in time that this was part of the downside of being a doctor, particularly if you are a local doctor, although he denied that it upset him when I asked him many years later.
I also remember sitting with him over a few pints one night when the door of the pub was thrown open by a well-known figure in Dublin at the time. He gave my father a barely perceptible nod, and walked briskly around the pub, checking every nook and cranny. He left as quickly as he had arrived, alone. ‘What’s he doing?’ I asked.
‘The poor man is probably looking for his wife,’ my dad said grimly as he took a sip of his pint. ‘He will probably repeat the same routine in the next four or five pubs until he finds her.’ He paused briefly. ‘And then he will do the same tomorrow night, and the night after that.’ I knew better than to ask more.
As he got older my father seemed to withdraw socially, and preferred instead the company of a small group of people in the comfort of his own home. Obviously, this isn’t unique – our circle of friends tends to diminish as we age and the vagaries of life, children, jobs take priority – but I think my father’s world outside work began to shrink over time. He lived and worked in the same small area of Dublin all of his professional life and, in seeing thousands of patients from the area each year, there must have been few people left who had not either attended him as a patient themselves or been affected by someone who had. I think he just wanted to avoid any awkwardness for them or himself and so he went out less and less as time passed.
And in the end, I think, Dad fell out of love with the job of being a doctor. He was frustrated by the changes that were coming into his daily working life. The increasing need for paperwork did not appeal to him (does it appeal to anyone?), and the influence of hospital managers on doctors of his vintage was a particular bugbear. Now and again he would lament how his influence was being undermined by people many years his junior and with no medical experience at all. He kept these frustrations private but it was clear he was losing his enthusiasm for the job.
I think he also wearied of the unrelenting human suffering he had to deal with every day. He worked in the same post for thirty-odd years, and every hour of every working day he would listen to the tragic stories of those afflicted by alcoholism, by drugs, by depression and other diseases of the mind and body. Such consistent exposure to the frailties of your fellow man must be a heavy personal burden to bear, I now think. The nature of the doctor–patient relationship means that it is a burden you bear alone.<
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Of course, he still enjoyed some parts of his working life. I remember the occasional party when his registrars would come back to his house, singing and laughing late into the night. He liked being around the younger doctors, much like I do now. Their sense of energy and idealism is a reminder of your younger self, and the wide-eyed look of a young doctor on first witnessing a condition that you have seen many times before jolts you back to the enthusiasm of your early days. And, however he felt about his own career in his latter working years, he was proud of any achievements I might have had, especially becoming a consultant and living just up the road from the house where I had spent my medical school years under his tutelage and protection.
When it came time to retire, he did not want a retirement party or an event to mark the occasion – he was too self-conscious for the attention it would bring. I think his colleagues tried to organize some sort of celebration, but only a few days before he finally left the hospital building for the last time he had another heart attack. I was then living in Melbourne but luckily had travelled to London for a meeting so I got home quickly. It was so sad to see him in the coronary care unit, though he was upbeat as always. Because I had been qualified for many years by that stage, I felt more comfortable talking to the cardiologists about my father’s prognosis than I had been that time in Galway when I had just finished pre-med.
Just One More Question Page 13
