Imagine that after decades of relatively healthy life, and perhaps before an age where health scares are on your horizon of possibilities, you are told suddenly that you have an aneurysm – an expanded blood vessel – that could tear, with fatal consequence, at any moment. Imagine, further, that you are told it is likely that you have a condition that can explain this aneurysm, that you have had this condition your whole life without knowing, and that this condition explains the little bodily abnormalities that you always thought were just slightly unique parts of your anatomy. That is, roughly, the situation I found myself in seven months ago (a situation I wrote a little about here). I was in New York City. The aneurysm was in my aorta, the body’s largest blood vessel. And the condition was something called Loeys-Dietz Syndrome. *

After being joined in New York by mother and girlfriend, and going back to work for a week to complete an internship, I was told I could fly back safely to where I was living in Oxford – but that I should plan for surgery in the near future.

I travelled alone, and I thought the experience would be frightening. As the plane took off the tarmac at JFK, I realised that if anything went wrong mid-flight, there would be no guarantee that anyone could help me during the flight. I’d be twenty, thirty, almost forty thousand feet above the ground, away from a hospital. I swallowed some pills that were meant to lower my blood pressure, reducing the risk of a fatal dissection, and hoped for the best.

The ten or so hours that followed were not nearly as bad as I’d expected. On the Amsterdam to London flight, a little girl – maybe 4 or 5 years old – started quizzing me. Her older brother on the other side of her looked embarrassed, and stared out the window; the girl’s mother in the seat behind told her I’d probably want to get some sleep. But I was happy to talk. “Do you always wear brown jackets?” the girl asked, as if familiar with the entire contents of my luggage, and pointing to the brick-coloured anorak I was wearing on the plane. “Let’s do some colouring-in,” she demanded, giving me a pad and colouring-in pencils. I immersed myself in colouring-in for the first time in fifteen years, remembering how to shade colours in, and taking instructions from this authoritative girl. Something about the experience of rediscovering my inner child took my mind off the deep anxiety of the day before.*

Back in Oxford, though, the fears rushed back. On top of the concerns about a fatal dissection, I began to worry about my body. At a special clinic for people with connective tissue disorders, a diligent cardiologist (who would prove to be a dependable medical companion for me over the coming months) confirmed after a physical check-up that it was highly suspicious that I had Loeys-Dietz Syndrome (pronounced ‘Low-eez Deets’), but that I’d have to wait for the results of genetic testing, which I’d had done in the States.

They also looked at my flat chest, my spidery fingers, and flat feet. I couldn’t help but feel like a bit of a freak, a specimen on show.

I remember that word he used, “suspicious”: a curious term, I thought, which put doctors in the position of detectives trying to settle on a medical culprit after lining up a few alternative candidates. And the physical check-up left me questioning my own anatomy, though this was not the intention of the cardiologist or anyone else in the room. “Oh yes,” the cardiologist said, after spotting my bifid uvula – a split in the reddish bean that dangles at the back of most people’s throats. “Do you mind if I show the medical students here this?” he asked politely. “Of course,” I said. The two medical students peered into the back of my throat as I opened my mouth wide, and then looked slightly taken aback when they spotted it. They also looked at my flat chest, my spidery fingers, and flat feet. I couldn’t help but feel like a bit of a freak, a specimen on show.

And when I went home, I found myself taking a second glance at my lanky frame in the mirror. I’d never been blessed with the best looks in the world (though I’d like to think I took something from my Mum’s grace and my Dad’s astute posture). Moles, shortsightedness, a biggish nose – I have all of these imperfections. But being told I had Loeys-Dietz Syndrome made me look again at all my features in a new light. Those long fingers, those skinny legs; that particular facial structure; the shape of my chest – did all my features represent not just irregularities but differences that put me in a separate category to other people? After all, I was told I had a condition. How different was I? The way we define attractiveness as a society is, of course, warped. And I knew, in my more rational moments, that Loeys-Dietz Syndrome was just a collection of symptoms that put people at heightened risk of aneurysms. But I still felt a sinking loss of confidence that came with my appearance being certified as different. My abnormalities were no longer points of difference (“those lovely piano fingers of yours!”) but features with medical significance.

A few weeks later, the results of my genetic testing arrived – not, as they might have once done, in an envelope in the mail, but in the modern way: as an attachment to an email I read on my cellphone. I stood outside a second-hand bookstore with a friend, and let him go inside as the email loaded up on my phone. The subject line read simply ‘MH’, and the results, when I read them through, were initially confusing. I did have a genetic mutation – “TGFBR1 (Ala202Val, c.605C>T)” – but it was a mutation that the laboratory had not seen before. One prior patient with this mutation had had aortic complications, so it was still recommended that I go ahead with surgery, but nothing was said about whether or not this meant I had Loeys-Dietz Syndrome. As I spoke to more medical professionals, however, the position became a little clearer. One told me that I was in the “dreaded grey area of genetic testing”, but most agreed that it was likely that I had the syndrome.

I paused. “Mum … Are you crying?” A long silence followed. “I’m sorry,” she said, clearly upset. I shook my head, and heard my voice shaking, too.

Sharing this news was complicated and, at times, emotional. I remember phoning my mum, who was back in New Zealannd. This is good news, she suggested; this genetic mutation has never been seen before. She sniffed in between sentences, seemingly holding back a cold. Maybe you have something else, she went on, and this aneurysm is just a freak accident. She sniffed again. My Mum is a nurse, now working in a senior healthcare management role, and I knew her view deserved weight. But something felt not quite right about this – it sounded like she was resisting what I read the genetic test results to be saying: that I had Loeys-Dietz Syndrome, or something very much like it. The syndrome was, after all, I said, just a collection of symptoms that predispose people to sudden heart events (and other complications). In some ways the question of whether I “had” the syndrome was academic. I had the collection of symptoms – flat feet, flat chest, long arms and legs, bifid uvula, now a dilated aorta – that put me at increased risk. My Mum seemed to accept that this was the case, and we moved on to talk about next steps. But I paused. “Mum … Are you crying?” A long silence followed. “I’m sorry,” she said, clearly upset. I shook my head, and heard my voice shaking, too.*

Before long, after we settled on a surgeon in Oxford (who told me that “we’d go into the surgery together” and “give it our best shot”), a surgery date was set in early December. In the weeks prior, my mind had been hyper-sensitive to pains in my body – it was as if I had decided that the key challenge was responding alertly to a dissection if it happened before surgery, and getting quickly to the hospital. But with the date for surgery approaching, I realised that it was far better use of my mind’s energy to drum up courage for the operation itself: a complex open heart procedure, with a 2-3% mortality rate, which involved replacing the expanded part of the aorta with a dachron tube.

My girlfriend Julia helped me through the terrifying weeks before the surgery. I read books to take my mind off the operation. And when my thoughts drifted back to the thought of sudden death through dissection, or that 2-3% mortality rate that burned in my brain, she reassured me. “You’re not going to have a dissection,” she insisted, one night as I trembled before sleep on the top floor of a wooden villa where we were staying. Going to sleep was one of the hardest parts of the day. I had read of people dying of connective tissue disorders in their sleep, and I had been reminded by my dad of what my grandfather once said: that every time we go to sleep, a small part of ourselves dies; we give ourselves to the gods or to other people. Those words lingered with me as my thoughts blurred into darkness every night.

Around this time, I went to a Quaker meeting with my cousin. Six or seven young people sat in a small circle in silence for about an hour, as is customary. We all dipped into books that were lying around on a table in the middle of our circle. I picked up the Quaker Advices & Queries booklet, and my eyes fastened onto the following line: “Accepting the fact of death, we are freed to live more fully.” I thought that might be my challenge in the weeks ahead before surgery: to accept the fact of death. This was, surely, something everyone would have to come to terms with at some point in life: couldn’t I steal a march on everybody else by coming to terms with death earlier?

I had been reminded by my dad of what my grandfather once said: that every time we go to sleep, a small part of ourselves dies; we give ourselves to the gods or to other people. Those words lingered with me as my thoughts blurred into darkness every night.

Unfortunately, reckoning with death was not so easy for me in the lead-up to surgery. What I did know was that I wanted to live – through the next few weeks, and through the surgery. A few friends had asked me whether I felt that what had happened to me in the preceding months was unfair. I’d always rejected this idea: we’re all given strengths and limitations in life, and the health challenges I was facing were simply some of ‘the bad’ that you take with ‘the good’. What I did think was unfair was that there was a chance that I might not make it through to surgery, or through surgery itself, when I wanted to live so much. I felt like there should be some kind of natural symmetry between the strength of our desire to live and our actual chances of survival. But, of course, that’s not true of any of us.

Max before taking the All Souls exam.

I got the opportunity to think more on mortality in late September, when I decided to sit the All Souls Examination Fellowship exams. These were exams I’d heard about since I’d started studying at Oxford – exams that involved broad, challenging questions and encouraged imaginative answers. I signed up for them, with no hope that I’d get through – Julia and I planned to return to New Zealand, and the exams just seemed a fun way to cap off my time at Oxford. I sat near the door, prepared for something to go wrong during my writing. And I remember worrying that no invigilator stayed in the room over the course of the four three-hour exams: the supervisors stayed for the first fifteen minutes, and then returned just before the end of time. Images flooded through my mind of me having a sudden heart episode, and ruining the exam for others – or creating significant awkwardness as they decided whether to put their pens down or continue writing. Thankfully, I made it through the exams. And luckily for me, there was a question about death – a subject I had been thinking about in the previous weeks. “Death is nothing to us, since so long as we exist, death is not with us; but when death comes, then we do not exist” (Epicurus). Discuss. I recounted some of my experiences in hospital in New York and afterwards, and tried to argue that this cold, rational view of death doesn’t capture the fear we can all feel about the possibly permanent closure of our lives.

I knew that any moment I would lose consciousness. I tried to absorb everything around me, and I remember thinking: ‘this could be it’.

I could have spent the days leading up to my operation drowning in anxiety. But my family’s arrival in the UK meant I ended up spending the time before surgery laughing, as my brother and sister mocked me for my characteristic clumsiness and general ineptitude. My mother and father offered supportive words. I still remember my father telling the surgeon, “You know, our son Max means very much to us” – a line that was slightly too close to a threat for my liking, but nonetheless evinced a deep love for me.

The operation was scheduled for the early morning of November 12 at John Radcliffe Hospital. I had slept soundly the night before in my hospital gown. My family arrived before I was taken downstairs. This is all a slight haze in my memory – it was early in the morning, and I don’t remember everyone from around the time of the surgery – but I know I said ‘goodbye for now’ to my family, before being wheeled downstairs to the anaesthetist’s room. The gentle small-talk started there: “so, what programme exactly are you studying … ?” And I knew that any moment I would lose consciousness. I tried to absorb everything around me, and I remember thinking: ‘this could be it’.*

To my elation, I woke up some six or eight hours later. I regain consciousness with a tube in my mouth that prevented me from speaking, and I am told that I scrambled around for paper and pen, and offered prayer-like gestures of gratitude to the nurses around me. I still have the notes I made from these moments. “You are all champs,” I scribbled in very mangled writing. And I wrote: “I am so happy to be alive.”*

The seven days that followed in hospital were at times a reminder of the value of the NHS and the importance of free public services, and at other times a very confusing experience. I had a scare in my first night after surgery, when apparently my heart rate was consistent with activity during a heart attack – while the rest of my body appeared fine. I was somewhat bewildered by this, and was told by my nurse that all was relatively normal, though I found out later that this might have been a charitable interpretation of the truth.

On the night of the third day, I woke up convinced that I was in a luxurious room, associated with royalty.

The operation also affected my mind. I had been told that having to go on a heart-lung machine could have unusual effects on people post-surgery. For the most part, it didn’t impact me. I was able to function relatively normally, reading books and maintaining conversation. But there were oddities. On the day after my surgery, I was unsure about whether the surgery was going to take place the next day. I had spent so long trying to build up mental strength for the surgery that I couldn’t quite believe the operation was over. On my second day recovering, with my brother holding my hand by my bed, I saw a man striding through the room who looked just like an old friend from New Zealand, Will Fotherby. I thought I heard him say, “Max Harris”? – only to realise after some time that this probably wasn’t Will. On the night of the third day, I woke up convinced that I was in a luxurious room, associated with royalty. I even looked on Google Maps to check whether I was in London. When it told me I was in Oxford, I thought I might be in a special room in my surgeon’s house. It was only as day broke, and sunlight streamed into the room, that I slowly accepted I was still in hospital.*

I regained my strength in the weeks that followed. It helped that I quickly recognised that the hospital-supplied recovery booklet (written by elderly patients) would not prove to be much use, since the activities listed were things like “use of the watering-can in the garden”, “putting”, and “breast-stroke”. Instead I found my own benchmarks for recovery. I wrote a book review eight days after the surgery, and a blog about a biography I’d read in hospital.

Of course, the worries about my body and general anxiety about my health didn’t entirely disappear. I now had a long thin red scar down my chest. And when I volunteered to be a patient at an examination session for trainee doctors, I was reminded that my body has its quirks. The first doctor who reviewed me looked at my slightly skinny arms, and examined my chest, and then turned to the examining doctor and said: “First thing I notice is some muscular under-development in the arms, possibility of wastage …” Muscular under-development?! And “wastage”! Those were perhaps features for which I could not blame Loeys-Dietz.

Even now, I worry occasionally about the implications of Loeys-Dietz for my life ahead. The syndrome is so new, and cases are so varied and on such a spectrum, that it is difficult to predict how my future will unfold. My heart episode could well prove to be the most serious symptom I face for at least decades, and I could well live a very long and healthy life. This could be it. Or the years ahead could see new challenges emerging for me. What I know is that I am safer for knowing more about the syndrome I have, and that as time goes on medicine addressing both the syndrome and its symptoms is likely to improve. What I also know is that while what I have been through has caused great stress for my family and others around me, it has also been an enriching, challenging, strengthening journey for me. It is, perhaps perversely, a journey for which I am extraordinarily grateful.

Max Harris is researching law and political theory, and working on a book on the future of New Zealand politics, as an Examination Fellow at All Souls College, Oxford.