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This week left me with more questions than answers.
Not just about Progressive Supranuclear Palsy, but about the decisions we make when living with a progressive neurological disease that moves at the pace PSP moves.
How do you know when a symptom is “just your condition” and when it is something entirely different? At what point do you call an ambulance? And if one arrives, should you always go to hospital?
It used to be easy – ACL. Dislocated Shoulder etc – the decision was clear.
Now, I don’t know. That is the honest starting point, and it may also be the ending one.
The episode
Over the past year I have developed a symptom that has now been witnessed by my neurologist, an emergency department neurologist and my physiotherapist. Nobody has yet offered me an explanation, and my specialist has been honest in saying it doesn’t fit the standard profile.
It happened again this week, after an intense emotional moment. We had been saying goodbye to my in-laws (whom I love deeply and enjoyed their visit) at the end of what had been a wonderful visit.
I was sitting in my chair. Not walking, not standing, not moving. I suddenly became unable to move at all. I couldn’t communicate. I couldn’t open my eyes. I was, to all intents and purposes, frozen.
Yet throughout, I remained fully conscious and aware of everything happening around me.
The nearest word I can find is akinesia. It doesn’t quite fit. Akinesia, in the textbooks, is difficulty initiating movement, usually when standing or walking. Feet stuck to the floor. Freezing in a doorway. My episodes happen while I am sitting still and last several minutes.
My PSP has never quite read the textbook.
I have the classic features. Vertical gaze palsy, poor balance, broken sleep, sensitivity to light and the rest of the catalogue. Alongside them I have had symptoms that make my specialists pause. Earlier this year I temporarily lost the use of my right arm and right leg, an episode serious enough to send me to hospital. MRI, CT, EEG, ECG, consultation after consultation followed. Nobody could say with confidence what had happened. Whether that episode and these frozen minutes are the same thing wearing different clothes, I still don’t know.
PSP doesn’t typically manifest itself down one side, yet the chances of having a multiple neurloglcal conditions is simply an even lower probability of having PSP.
This week introduced something new.
My tongue rolled out towards the left-hand side of my mouth. I remember it rolling and my wife very much saw it.
Two versions of the same event
To my wife, that meant one thing. A possible stroke. She called an ambulance. Had our positions been reversed, I would have done exactly the same.
What she saw and what I experienced were not the same event.
Outwardly I couldn’t move, speak or open my eyes. Inwardly I was calm. My mind was clear. I heard every conversation in the room and understood all of it.
That is not to say I wasn’t thinking.
For a few moments it crossed my mind that this might not be temporary. That this was the moment my ability to communicate had finally gone. That it might be the beginning of something worse. Quietly, in my own mind, I recited prayers no one wants to say.
Oddly, I wasn’t frightened. I was calm and at peace with whatever came next, although I tried to get out of it. Perhaps living with PSP changes your relationship with mortality. When you have spent years knowing that your world is gradually becoming smaller, the possibility that it might suddenly change direction is strangely less shocking than it once would have been.
The paramedics arrived while I was still unable to respond. They tried repeatedly to rouse me, vigorously. Just as they were preparing to lift me onto the stretcher, the fog began to lift.
Within moments I could communicate again, and I was making what would prove to be the biggest decision of the day.
The decision
I refused to go to hospital. I signed the waiver.
It wasn’t bravado and it wasn’t denial. I was exhausted by the thought of another emergency department, another battery of tests, and quite possibly another conclusion of, “We’re not sure.”
My reasoning seemed sound at the time. I had been here before. Previous investigations had found no stroke, no seizure, no obvious explanation. Another long night would probably end the same way. More tests. More uncertainty. No different treatment. Perhaps this was simply another manifestation of my PSP, unusual though it might be.
By the time I spoke to the paramedics I was fully lucid. I could tell them my normal blood pressure reading and answer everything else they asked. I recovered completely. Part of me felt vindicated.
Another part of me isn’t so sure.
The tongue movement was new. New neurological symptoms should never be dismissed lightly, least of all by someone who already has a neurological disease and has grown comfortable explaining things away. Perhaps that one additional feature changed the calculation entirely.
Perhaps I should have gone.
What the research does and doesn’t say
I have spent some time since looking for anything that describes what happens to me.
The literature on freezing in PSP is almost entirely about walking. It doesn’t describe a man sitting perfectly still in a chair, locked in, listening to everyone around him.
There are, however, a small number of published reports describing seizure-type events in people with PSP. Estimates of how often this happens are inconsistent and scarce. That isn’t a diagnosis. It is simply another sensible question to ask my neurologist.
Reading the literature has reminded me of something else. Neurology contains several conditions that can produce episodes of apparent unresponsiveness while consciousness is preserved. Whether any of them applies to me is entirely a matter for my neurologist. But it reinforces that there may be explanations beyond simply saying, “It’s my PSP.”
What I actually learned
Living with PSP changes your relationship with risk.
Every new symptom brings two competing thoughts. One says, “It’s probably just the disease again.” The other whispers, “What if this time it isn’t?” Both can be reasonable. In the moment, you have to choose.
One of the unexpected challenges of a rare disease is that you become an expert in your own body. You recognise patterns nobody else has seen. That expertise is genuinely valuable. It is also dangerous. Familiarity breeds confidence exactly where caution is needed. I have thirty years of professional experience telling me I am good at reading situations quickly. That experience is worth very little at four in the afternoon with my eyes shut and a paramedic shaking my shoulder.
What I need is not a better instinct.
It is a plan.
I need another conversation with my neurologist. Not only about what happened, but about what my family should do when it happens again. Should we always call an ambulance? Should we always go to hospital? Which signs tip the balance, and which don’t?
These are no longer theoretical questions. They are decisions that may need to be made in minutes, by people who love me, while I lie there hearing every word and unable to contribute a single one.
Perhaps the hardest part of PSP isn’t losing certainty in your body.
It’s losing certainty in your decisions.
This week I chose to stay at home.
I still don’t know whether that was the right decision.
My hope is that before there is a next time, my family and I will have a plan agreed with my neurologist, so we won’t have to navigate those minutes alone.
IMPORTANT NOTE – This is not medical advice. A tongue pulling to one side is part of the standard stroke assessment and should always be treated seriously. If you see it in someone you love, call an ambulance and follow the advice of the paramedics and medical team. I refused hospital. I am still not sure I was right.*
