Paul Trotman
How are you?
Are you OK?
How are you feeling?
When I was in my late teens I developed a real dislike for these phrases and I’ve had a love hate relationship with them ever since. I was quite a sickly teenager. On the first day of my last year of high school I was dragged screaming and kicking to the GP after a couple of mysterious vomiting episodes – the last straw being when I threw up at the dinner table. No warning, no nausea just bleeeuch: succulent flakes of baked fish all over the place.
The GP took one look and said ‘Aha, kidney failure’. Apparently, I was a classic case. I wondered how malfunctioning kidneys could make me throw up, as my basic knowledge of biology told me the kidneys and the gut weren’t connected. He explained that if the kidneys aren’t working, toxins build up in the body, which makes you vomit. I was sent for urgent blood tests. In the lift to the blood lab my dad told me the initials on the form – ESRF – stood for End Stage Renal Failure. Uh-oh.
It turned out that I had been sick my whole life. I had been diagnosed with kidney reflux when I was four. But my parents were told reflux was ‘pretty benign’ and just to ‘keep an eye’ on me. By the time I was 17, more was known about reflux. There are two kinds, a gentler version that usually presents with urinary tract infections but nothing else, and a second version – the one I had – where the kidneys are scarred and there is protein in the urine (proteinuria). This second type often progresses to full on kidney failure.
After that GP visit, my lifetime of treatment began. First I was prescribed daily medications (I’ve taken pills every day of my life since). Then I had surgery. A few months later, I went on dialysis. I vividly remember my first dialysis session. The specialist struggled to get the lines into my femoral veins. An hour into the treatment I got a headache. I spent the next two hours vomiting and feeling like I was going to die. I tried counting to keep my brain ticking over and lost count around 600. I did manage to doze a bit after that.
Despite the diagnosis and the demands of treatment, I was determined to be seen as ‘normal’ and I was pedalling like mad behind the scenes to maintain that appearance. ‘Aggressive Normality’ I came to call it. ‘Obnoxious Independence’ would have been more accurate. Being asked ‘how are you?’ all the time isn’t welcome if you are working so hard to be normal, and I quickly came to loathe the question. A typical inward-looking teen, I had no idea how difficult it must have been for my parents to balance my need for independence with the need to look after my health. Eventually I told them to stop asking how I was, and that they should assume I was fine unless I said otherwise. Ouch!
With the help of my Aggressive Normality, and a lot of support from my parents (who were taught to assist me with dialysis), I managed to finish high school. But what next? By then I’d had a lot to do with the healthcare system, and I thought becoming a doctor would be a good idea. I told my plan to a class of medical students who were reviewing my case as part of their education. They laughed politely. The medical school wasn’t so sure either. Dialysis is hard, even with family around to help. After a couple of hours of treatment it becomes hard to focus, and I needed to spend five hours, three times a week, hooked up to ‘The Machine’. Basically, I would have very little available time to study. Nevertheless, with the support of some kindly professors I was able to give it a go, and there were only a few bumps along the way. At the end of my fourth year of study, I received a kidney transplant from a 29-year-old man who had died in Napier Hospital’s intensive care unit. At last I was truly ‘normal’ – apart from the handful of pills I took twice a day, of course. The transplant enabled me to finish medical school, pursue my medical career and have a family.
When my second child developed reflux, proteinuria and scarred kidneys, I spoke to a geneticist who told me that kidney reflux is a multi-genic problem with ‘variable penetrance’. In other words, some people with the genes will get it and others won’t. It can even skip generations. All eyes swivelled to my grandmother who came from a large family with many dying before they made it out of their teens. Even at the time my daughter was diagnosed there wasn’t much that could be done to head off the inevitable. However, taking ACE inhibitor blood pressure tablets did seem to offer some protection, delaying serious damage to the kidneys. This treatment wasn’t yet mainstream, but I argued long and hard for an ACE inhibitor to be given to my daughter. Finally the paediatrician indulged me – and in doing so bought her an extra seven years of healthy life.
My daughter entered the final stages of kidney failure last year. As part of her work up, the renal team did some high-end genetic analysis. They found a single DNA code letter misspelling in the PAX2 gene. This gene is involved in the way the ureter divides and connects with the kidney. In one of my daughter’s PAX genes, Guanine is replaced by Adenine at position 74 – a single letter error which leads to a single amino acid change in the resulting protein, making Glycine replace Glutamic Acid. One tiny spelling mistake is the cause of both our problems.
As my daughter starts on dialysis and waits for a transplant, everything has come full circle. She is finishing her Master’s degree and is going to do dialysis in her student flat, on her own, with no assistant. You have no idea how scary that sounds to a dialysis kid from the 1970s and ‘80s. There was no way anybody could do that in my day. So now, finally, I understand the complex balancing act my parents faced. I find myself constantly wanting to ask her, ‘How are you feeling?’ and ‘Are you OK?’. I know it’s annoying, and it takes all my energy, love and admiration for her and what she has achieved to leave her alone to pedal like crazy to maintain her own ‘normal’ life.
But still, sometimes I still can’t stop myself.
How are you?
Paul Trotman is a doctor, writer, film maker and lifelong patient, although not necessarily in that order. He has spent his entire medical career working in rural hospitals. He has developed, written, produced or directed over 40 hours of commercial television, numerous short films and two feature documentaries – Donated to Science about human dissection and Behind the Mask about frontline workers during the Covid-19 pandemic.
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