A six-pack of courage: one man's surrender to a terminal disease

I still remember the first day I realised something was wrong. The day when my life's path would be irreversibly altered and I would leave my role as caregiver and doctor and begin my gradual surrender into the role of patient.

There was nothing special about that day, just an average workday within a busy hospital haematology ward, managing patients diagnosed with blood cancers.

I was leading my usual eight o'clock ward round, examining my patients and investigating any issues that had developed during the night. Seeking and managing symptoms or side effects from the chemotherapy I had prescribed.

Little did I know that my attention to detail would set off a chain of events leading to my diagnosis, and ultimately to where I am now.

That day one of my patients was complaining about a "funny feeling" in his left foot, a dysaesthesia we call it. The night-time resident doctor had seen him and had dismissed this new symptom as a somatic manifestation of the patient's extreme anxiety.

It turned out this patient had every right to be scared.

In the morning, I made my own assessment. I worked my way through a neurological examination, testing his peripheral and central nervous systems, looking for signs of pathology. I got him out of bed and asked him to stand on his toes and then on his heels to demonstrate the strength within his lower leg muscles. He was unable to stand on his heels. He had a "foot drop", weakness in the muscles that lift the toes and foot off the ground.

He was horrified, I'm told. The chemotherapy had indeed damaged his nerves and it was obvious for all of us to see. But I missed his reaction. I was transfixed by my own legs. He couldn't stand on his heels and neither could I.

That night my wife Mel and I walked our two dogs, and during the walk I could hear the faint sound of my left foot slapping the ground with each step. It was still there, it wasn't just a pinched nerve, I hadn't just slept funny. We got home and I lay on the bed and I asked my then fiancee, who is also a doctor, to examine my legs. I was 33 and it would turn out that I have motor-neuron disease (MND).

The road to a diagnosis for most MND patients is long and arduous, sometimes taking years. I was fortunate. I had a shortcut. I quietly approached one of my colleagues for help and within two days I had undergone nerve-conduction studies, a muscle and nerve biopsy, a lumbar puncture, a battery of blood tests, and a whole-spine MRI. All motor-neuron patients ultimately understand that each element of this is a grasp at hope, a grasp for an alternative, a grasp for something else – please, anything else.

MND is a diagnosis of exclusion; there is no one test, no one defining feature. Often sufferers are left for months or years with a title of "possible" or "likely" MND, with time ultimately being the final arbiter. Thanks to my training, I had inside knowledge about this disease and no matter how much I longed to see the distorted anatomy or bright intensity signals on my MRI that would point to a simpler, more treatable cause, I knew deep down they would not be there. I remembered that first night after our walk. Mel had examined me, I saw the signs. She had demonstrated them to me. I was good at neurology, this was MND.

That night I answered her question honestly when, with tears in her eyes, she asked me, "Could this be anything else?" My training, knowledge and gut feeling said no.

By the time we saw the neurologists I was certain. The preceding hours and days were spent going through alternatives in my head and I undertook an extensive library and internet search. Nothing else had come up.

Hearing the first and then the second neurologist actually mouth the words in some ways was a relief, legitimising my fears, but at the same time, as he said those words, my life, my future, collapsed. All was replaced by unfamiliar emotions: dread, fear and, on the drive home, panic. I knew about this disease. I knew what lay ahead. I was, and still am, terrified.

Motor-neuron disease was originally described by the French neurologist, Jean-Martin Charcot, in 1869, and is also known as amyotrophic lateral sclerosis (ALS) or Lou Gehrig's disease. It is one of modern medicine's failures. That's a personal and obviously biased opinion, but I think most in the medical community would agree. It's been 71 years since Lou Gehrig, the famous New York Yankees baseball player and self proclaimed "luckiest man on earth", died from this disease. He died on my birthday, June 2, in 1941, and as a doctor I am ashamed to say that from a practical point of view for patients, little more is known about the cause, and certainly no truly effective therapy is on the horizon.

MND is a progressive neurodegenerative affliction, a gradual imprisonment, where an accumulation of disability leads to a gradual but inevitable reduction in independence. Patients live on average two to three years after being diagnosed.

In the simplest terms, the disease attacks and kills the motor-nerve cells within the brain and spinal cord, destroying the means by which messages are sent to the body's voluntary muscles. When they are gone, the muscles atrophy and die, taking with them our ability to move. Forever. My mind and intellect will not be affected. I will bear witness to it all. There are about 1400 to 1700 people living with the disease at any time in Australia, but we don't live long enough for the prevalence to rise.

Statistics have shown the number of deaths from MND is rising – there were 450 in Australia in 2000 compared with 596 in 2009 – but the cause of this increase is unknown.

Sure, it's a rare disease, but in real terms, every day at least one person will be diagnosed, and another will die as a consequence of this disease. It strikes at anyone, often with no warning. Ten per cent of sufferers have an inherited form of the disease. The remainder, like me, grapple every day with the often and inevitable question, "why me?". The disease is now mainly in my legs. I have a brace and use a walking stick when my leg becomes tired. I know it's spreading. My muscles constantly twitch. Intermittent cramps in seemingly unaffected parts of my body signal that the disease is spreading. I feel these butterflies under my skin, in my arms, back, and left side of my face. I just have to wait for the weakness to begin.

The only treatment available is largely ineffectual. Borderline efficacy data has allowed the drug to reach the market, but for many people the cost and side effects are prohibitive. The medication prolongs the average life expectancy or time to needing a ventilator by about three months. I take it. I'm not sure I really want to prolong the back half of this disease, though, and I take it in the hope of slowing progression. I don't know if it is working. The disease marches on regardless. Despite MND being granted "orphan disease status" by the US Food and Drug Administration – which is meant to encourage research – there has been little advance. Developers of so-called "orphan drugs" aimed at treating rare diseases get more leeway with statistical analysis, potential tax incentive and enhanced patent protection and marketing rights. But drug developers have still been slow to get on board. Patients are diagnosed with MND at nearly the same rate as many other neurodegenerative diseases, but the fact stands: we don't live long enough to create a burden on society, so there is no incentive for governments or pharmaceutical companies to pursue new treatments. There isn't enough profit to be made. Cynical I know, but nevertheless true.

To live with this disease, I have come to the realisation that I need to achieve two goals: complete acceptance and complete surrender.

Firstly acceptance. This is a constant process, one in which I am continuing to reframe my perspectives on this disease and how I let it affect my life. The progressive disability needs to be incorporated into a constantly evolving acceptance. I think I have the internal strength to do this. I hope I do. However, one of the hardest things has been recognising that others will need to accept the disease as well, and in turn I need to accept and acknowledge their response, in whatever form it comes. In a recent discussion with a psychologist, the issue of secrecy came up. I have kept my diagnosis secret for almost a year, with only the closest of friends and family aware. Surely telling others was one of the first steps to true acceptance, so why then have I kept it secret? I told myself that eventually everyone would know, the disease would see to that. My body would betray my secret, but until then I didn't want people to treat me differently.

This element, not wanting to be seen as a sick version of my former self, now interests me, and it turns out I'm not alone. The psychologist referred me to a 1988 book, Stories of Sickness by Howard Brody. In the chapter about the relationships of patients with those around them, Brody refers to the "Gregor effect", in reference to German author Franz Kafka's 1915 short story The Metamorphosis. In the story, the protagonist Gregor Samsa turns into an insect. His family and friends, although at times empathetic and still loving, find ways to avoid Gregor, to distance themselves from his "illness". Psychologists ascribe this "Gregor effect" to how some view the terminally ill. We make you nervous, uncomfortable and scared, and at times make you challenge your own mortality. I know this firsthand.

My work in haematology means I deal with patients suffering from terminal diseases. I would comfort them, advise them, treat them, and prognosticate for them with clinical efficiency at work. I was never cold, I was professional, I could empathise, but I always kept them distant. If I saw my patients outside of office hours, I would often turn around rather than interact with them and face how they made me feel.

On a social level, I feared the awkwardness of the situation. I worry about this now happening to me. Will people fear interacting with me, and treat me differently? I fear walking into a room. You can probably see a recurring theme here – I need to accept fear.

Second is surrender. This one seems easy. I'm not talking about surrendering to the disease and surrendering all hope. That will not happen, not any more. Rather I mean surrender to inevitability and accept my future disability. Trust that others will look after me. Easier said than done.

More recently I find myself looking for new inspiration. I must admit I am much more easily inspired these days. I search for renewed passion, for something I can try to excel at, something where I can aim to leave my mark on this world when I can no longer work as a doctor.

Again, it turns out I'm not alone. This phenomenon is known well to geriatricians, and they call it "generativity". It is common among the elderly at the end of their productive lives and, as I am finding, common among the terminally ill. It describes the emotions and regrets a person has when lost opportunities are mourned and individuals find themselves longing for more time, endurance and chances. They wish to have produced something meaningful, something to have made a difference. Anything to make others proud, to make them remember. For some it is through professional endeavours. I will very likely not finish my training and will not know what this feels like.

For others, it is through starting a family and creating a legacy. Again, with much heartache I know this will not be possible. So I need to focus on what I can do here and now, and enable others to help the cause when I'm gone.

Inspiration, hope and purpose can come from strange places and for me it was tasting an artisan ale. I guess you could call it my epiphany ale. I had been searching for something, anything I may be able to do – something that did not require my legs. Something that would need my brain, my passion, my drive. I turned to my medical school hobby of home-brewing beer. I will not go into detail, but the brewers among you know the satisfaction I'm talking about.

On my birthday this year, a mixed six-pack of craft beer from my sister-in-law showed me the way.

The very first ale I tried was perfect. Why not make a beer for MND? Why not use beer to raise awareness of the disease across Australia and to raise funds for research and equipment?

I had to make this happen. So I found my epiphany ale's website, emailed the brewers, and to my delight the guys from Cavalier microbrewery responded in less than 24 hours. They would help, and most importantly they were excited by the idea. From then on Steve, Heath and Andrew have been amazing, and our plans for launching the beer this year are well under way. The name we have decided on is the "Cavalier Courage", with the concept and design of the beer based on the myth of Sisyphus. Albert Camus's essay on the ill-fated king resonates with me and probably all people with MND. Condemned to the underworld, Sisyphus's punishment was to push a boulder up a steep hill, only to have it roll back down to the bottom each time he reached the top. For me, this represents what MND patients go through.

This disease takes something away from us. We adapt, but when we think things are stable it takes something else. The rock rolls back down. I now use a cane intermittently and a knee brace and I am back at the top of the hill. But I know my rock is delicately balanced.

Despite thinking that Sisyphus should be the most miserable man ever, Camus proposed that we should think him as "happy", in that the process of pushing the rock, no matter how draining and depressing it seems, can be enough, if we accept it, to fill us with purpose. He wrote: "The struggle itself towards the heights is enough to fill a man's heart."

This beer will pay tribute to all MND patients and hopefully give incentive for all of us to start pushing again. I am not alone in this battle. I have gained much wisdom, passion and encouragement from other sufferers. One person who touched me early after my diagnosis, and during one of my darker days, was Scott Sullivan. He is an MND sufferer, a Queenslander, a father, and an absolute hero. He founded the MND and Me Association and raises funds for research and awareness. The other is the Zo-ee association in Melbourne, named after the Greek word meaning life, formed by Karen Mustica and Gina Gonis after Karen's mother was diagnosed with MND in 2005. The profits from Cavalier Courage Ale sales will go to these two great charitable associations. Where I will be in one week, one month, or even one year is anyone's guess.

It is however, a surety that I will get weaker. I will experience good days and darker times, but I can now see some sort of light, and it's the ratio of the two I'm working on. I continue to gain strength with the help of family, friends and, more recently, strangers.

I often think of a quote by the Jewish-Austrian neurologist, Victor Frankl, from his book, Man's Search for Meaning, written after he was released from a concentration camp in 1945. Mel and I first became aware of Frankl's work while visiting the concentration camp museum at Dachau in Germany. Even in this place, the first of Nazi Germany's death camps, the human spirit could endure.

The quote is: "Everything can be taken from man but one thing: the last of the human freedoms – to choose one's attitude in any given set of circumstances, to choose one's own way."

This disease is going to destroy me physically. It will try to take everything from me. To a large extent it will succeed. My independence, my dignity, my career and my plans of a family and growing old with Mel will be taken. This quote makes me realise, however, that some things are off limits. I've never been a religious man, but I do believe in an individual's spirit and the disease can't have mine. I may not yet see myself as "the luckiest man alive", but I'll keep working on it, Lou. Until then, with the help of my family and friends, I'll continue to live in the present tense . . . It just makes much more sense.

cavalierbeer.com.au; mndandme.com.au; zo-ee.com

The story A six-pack of courage: one man's surrender to a terminal disease first appeared on The Sydney Morning Herald.

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