Eyes and Ears and Mouth and Nose

For some strange reason, I found myself thinking about a nursery rhyme just now.

Those who knew me before PSP arrived will know this sort of mental detour is not entirely new territory. My brain has always worked in peculiar ways.

It goes, well the British version anyway: Head, shoulders, knees and toes, and eyes and ears and mouth and nose.

To parents who have spent years listening to this rhyme on endless repeat, I can only apologise for now lodging it back into your head.

Anyway, I was thinking about the verse probably because I have Botox treatment in hospital tomorrow to address the involuntary closing of my eyelids. I stopped because it struck me as quietly profound.

That simple children’s rhyme, which starts with head, shoulders, knees and toes, is in fact an almost perfect map of what PSP does to a human body. Systematically. Comprehensively. With considerable lack of apology.

It is also convenient for a Brit, because it leaves out uncomfortable parts of the body about which no British person would dare write. I once mentioned a non-British word, constipation, and it caused more feedback and comment from my British readership than recent posts about far more logically scary stuff. I appear, somewhere along the way, to have swallowed the British pill whole.

A lot of attention, understandably, is focused on the headline symptoms of PSP, often both the ones that resemble Parkinson’s and the ones that distinguish it from Parkinson’s. The balance, the falls and the cognitive changes feature highly. But there are other symptoms. Strange ones. Ones that don’t make it into the brochure. I thought this was a useful way of describing them.

Eyes

I have written about eyes at some length, and I will not repeat everything here. But it is worth noting that the name Progressive Supranuclear Palsy contains three medical concepts, two of which are derived directly from what the disease does to the eyes.

Supranuclear refers to the location of the neurological damage above the nerve nuclei that control eye movement, and palsy refers to a paralysis that results, usually used in the context of eyes and especially vertical gaze palsy. The eyes are not an incidental casualty of PSP. They are written into its very definition.

The damage is broad. Vertical gaze palsy means I cannot move my eyes on the vertical axis, only horizontal, so looking up or down requires moving my entire head. My eyelids close involuntarily due to neurological misfiring, which is what brings me to the Botox appointment tomorrow. I experience episodes of rapid, uncontrollable blinking. My eyes tear unpredictably. They crust. They dry. They do, in short, a considerable number of things that eyes are not supposed to do, and decline to do several things that they are.

It is a limited list only in the sense that I have chosen not to write the longer one.

One other recent example springs to mind. I had an MRI for the suspected TIA two days ago, which thankfully looks clear of any lasting damage from a stroke. However, they played a presentation on screen during the scan and a single correction box on the screen slowly became, over the course of twenty-five minutes, two distinct and equally clear boxes. Double vision, from one.

Ears

In the early days, when we still thought this was Parkinson’s, my late neurologist identified what he called a “startle response”, something documented in both PSP and Parkinson’s for certain proportions of patients.

I am acutely noise-sensitive. Loud sounds at certain moments cause genuine pain. I often wear earplugs or noise-cancelling headphones because, without them, a freeze can be induced. So can bright light, which I probably should have mentioned above.

But the startle response is something else entirely.

My favourite illustration involves my son surprising me by opening the door when I was on a client Zoom call many years ago, at the start of this journey. I screamed, dropped the computer, and was neither on mute nor off video.

But it is perhaps better illustrated by something that just happened yesterday. I was being wheeled by my wife and carer to my physiotherapy session. It was bright outside and I was wearing sunglasses. We moved suddenly into a patch of shade beneath some trees. No car passed. No sound occurred. Simply light became dark. And I screamed at the top of my lungs.

There were also one or two specific occasions last week when I was just falling asleep and my wife came into the room. I screamed so loudly that it was genuinely frightening. I also hallucinate, but I will leave that for another time.

This is, for some PSP patients, relatively normal. It does not happen to everyone. It happens to some of us. I am, apparently, one of the some.

Mouth

I will not dwell on my issues with choking, not because the fear isn’t real but because I have addressed it many times. My throat muscles are weakened. The neurologist suggested a feeding tube. I resisted. My diet, adapted and carefully managed, has to date kept me away from serious choking. That sentence contains more anxiety than it appears to.

What I will mention, because it came up in a PSP support group just today and made me smile with recognition, is something rather different.

One member talked about her husband growling, moaning and groaning. I do that from time to time, and I also hum, often for hours on end, to the considerable annoyance of my family, especially at the meal table. I cannot begin to explain it.

Nose

I have no sense of smell. Scientifically, perhaps a fraction of a percent remains.

Once I did a sniff test for a research project and was given twelve smells and four options. I could only genuinely smell one. It wasn’t whisky, ethanol, mint or anything obvious. It was pineapple.

Since then, pineapple has gone too. But for a moment, I smelt it fully. I cannot explain that either.

The practical consequences include the following. When a toaster fire broke out behind me at the kitchen table, I noticed nothing whatsoever until my children came in and pointed out, with some urgency, that there appeared to be flames emerging from relatively close to me. They simply couldn’t understand how I could not be aware of it.

On the plus side, before confinement to a wheelchair, I was given the role of clearing up unpleasant substances left by child or dog for a long time. Every condition has its compensations. Or did, until the wheelchair removed that particular advantage.

Head, Shoulders, Knees and Toes

I won’t dwell on the head. The brain is the core of this disease and the driver of everything above.

The shoulders represent the axial rigidity I have written about recently. My neck, shoulders and trunk are as stiff as anything a number of therapists have encountered.

And right now, the sharpest daily pain lives in my legs: hips, knees and feet. A combination of muscle atrophy and the neurological impact PSP has on the limbs themselves. It aches. Persistently. It is real pain.

And being the Brit I am, I have left out all the stuff in between, which carries many issues.

So. Botox tomorrow.

I am almost certainly the last person who ever expected to be the first in his family to have it.

Eyes and ears and mouth and nose. And tomorrow, someone is going to inject poison into the lids of mine in the hope that they start behaving.

The nursery rhyme never mentioned that part.

I will report back.

And no, the wrinkles and lips are going to be left well alone.