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A progressive disease moves in a steady line until, every so often, it does not.
For two evenings in a row, my brain appears to have put on something I can only describe as a freak sideshow.
But that is not the main event.
The main event is far more predictable, and far more ruthless. Day by day, in a steady, almost disciplined progression, this disease is taking things away. My eyes are worsening, my stiffness increasing, my movement and balance deteriorating. The need for a carer, the withdrawal from noise and crowded places, the risk of falling and choking and a number of indignities I had not wished for.
That is the real story of this condition. Not chaos, but consistency. Not spikes, but slope.
And yet, alongside that slow, relentless decline, there are these moments.
Moments that feel completely disconnected from the underlying pattern. Moments so out of place that, if they were happening in isolation, I would genuinely question whether I was imagining them.
But I am not, nor are the people with me.
On Friday night, as my carer helped me stand to transfer to my wheelchair for a bathroom trip, I froze. Completely. I was pulled back into my chair, unable to move. For the next seventy to seventy‑five minutes, my eyelids kept my vision from working. Added to that, my head rocked back and forth at a steady and sometimes increasing pace. My teeth chattered intermittently. My foot tremored. My arm tremored.
I was fully conscious throughout, trapped inside it. It was horrible to experience, and it must have been horrible to watch.
My mother said it looked like a seizure. My neurologist, with whom I had an email discussion after Shabbat, said it did not look like a typical PSP reaction and that we should keep an eye out for seizures. The head bobbing has continued in a much more measured way and looks to me like a form of dystonia (involuntary movements) regularly experienced by PSP patients, as has the eye closure, as has the occasional powering down – all again are typical symptoms.
In the early hours of Sunday morning, something different appeared, but no less out of place. My left hand swelled significantly, accompanied by pins and needles. It was not a bite. I was discussing it on a live WhatsApp chat with a doctor friend in another time zone I thought would be awake, and who was, and she advised me in real time to raise it. It eased. Then, just as that subsided, my left foot began to swell. I raised that too, and gradually it settled. I am very grateful to her.
As I write this, it seems to have faded.
These episodes sit alongside recent hospital visits for Bell’s Palsy and a suspected TIA (mini‑stroke). I am discussing all of this with my neurologist, and I have an MRI scheduled for next week.
But clarity is in short supply.
This is the contradiction I am living with. On one hand, a disease that progresses in a steady, almost predictable line, earning every letter of the word “progressive” in Progressive Supranuclear Palsy. On the other, these episodes, exaggerated, irregular, entirely out of keeping with that line. Not the core description found in any textbook, and yet not entirely unheard of either.
A few years ago, and I would recommend this as a default for most patients if there is any serious doubt or danger, any one of these episodes would have sent me straight to the emergency room or appropriate medical attention. Now, it no longer feels like something that will be resolved in a regular hospital setting late at night. A swollen hand and foot, a frozen body for over an hour, alarming, but not actionable.
That, too, seems part of the progression.
One further thing, I think of which may or may not be shown to have to do with the atypical nature of some of these freak show events is the fact that this all started in atypical fashion. This all began six weeks after COVID (I was an early patient – no 305 in Israel long before vaccines and was treated for it in hospital). The onset of what is now PSP was rapid. And from the beginning, despite presenting with classic PSP features, vertical eye palsy, backward balance, choking, my case has consistently been described as atypical. This is a Pandora’s box I have no intention of opening. I draw no conclusions. But it remains part of the story.
It is well documented that each PSP patient seems to have a slightly different nature and ordering of experience. There are many subtypes, and that seems to me to be the point. If the progression is the main event, steady, inevitable, uncompromising, then the sideshows are still real. Still experienced. Still worth documenting.
Over the weekend we discussed the two trips I made last May, to Japan with my son and to London for a funeral, both with a walker, but independently. I cannot believe the change in a year. Nor can I say I am particularly looking forward to what is coming, given the speed of that change.
I continue to write this as my way of trying to make sense of it, and to also show the patient experience. I would be interested in hearing if others have had anything similar.
This may feel like a freak sideshow at times.
But it is not fiction.
The main event continues.
And the progression, quietly and steadily, goes on.
