Progressive Means Sinister: It Creeps Up on You

One of the things I find most unsettling about Progressive Supranuclear Palsy is that the word progressive sounds almost harmless. Clinical. Predictable. As though symptoms advance in a neat, orderly fashion that you can observe and prepare for.

The reality feels very different and this is a large factor in the Anxiety I feel.

Progressive means things can change at such a slow pace that you don’t notice them changing at all. Your brain quietly adapts to each small loss until one day you suddenly realise something has been taken from you and you have no idea exactly when it happened.

It creeps up on you.

What is strange is that this happens within a disease that is moving relatively quickly (understatement). It feels as though PSP delivers a thousand tiny cuts to the body and mind, each one small enough to adapt to, until eventually you step back and see the cumulative damage.

Over the past few years, PSP has already changed my relationship with my eyes/vision in several different ways. There is the photophobia that can make bright light uncomfortable. There is the involuntary blinking and eye closure that has become one of my more frustrating symptoms and which, thankfully, now seems to be responding to Botox treatment. Then there are the eye movement problems, particularly my inability to look up and down without moving my head, which has been documented in my medical notes for years.

Those problems are familiar now. I don’t like them, but I understand them.

What I hadn’t realised was that, alongside all of those changes, something more sinister had been quietly emerging.

Over the past few months, the world around me seems to have become narrower.

I first noticed it after my birthday. My daughter had two photographs of us, at her wedding, framed and we placed them beside my chair on my right-hand side so I could see them easily. They are right next to me, yet I often don’t notice them at all. I can’t see them unless I make a deliberate effort to turn towards them.

Because the muscles in my neck and shoulders are becoming increasingly rigid, even that simple act is not always as straightforward as it once was.

At first, I thought I just thought about it in the context of the photos. Then I started noticing the same thing with people. There are so many things happening that is goes to the background.

The moment that finally made me pay attention happened this morning during my neurology appointment. I was waiting for my third round of Botox injections. My wife was sitting immediately beside me on my right-hand side, yet I couldn’t really see her.

A nurse entered the room on my left.

I couldn’t see her either.

Obviously, I knew they were there. If I consciously turned my head, I could find them immediately.

In some ways, though, that’s what made the experience so striking.

People and objects that once existed naturally within my visual world increasingly seem to disappear unless I actively go looking for them.

I originally described this as a loss of peripheral vision, although I’m now a little more cautious about that label. PSP is well known for affecting eye movements and, as those movements become slower or more restricted, it can create the sensation that the edges of your world are shrinking.

I don’t know exactly what mechanism is responsible for what I’m experiencing.

For comparison, with normal vision, one eye can typically detect something roughly 100 degrees out to the side. With both eyes together, many people have a horizontal visual field of around 180 to 200 degrees.** **I don’t know my precise amount but it is so much less.

That doesn’t mean you see everything sharply at the edges. You don’t. But you would usually still be aware that a person, movement or large object was there.

My usable visual world now feels dramatically smaller than that.

I haven’t measured it formally, and I don’t yet know whether this is true loss of peripheral visual field, restricted eye movements, reduced scanning, or some combination of them.

But functionally, the difference is enormous.

Someone can now sit immediately beside me and effectively disappear unless I turn to find them.

I don’t know the medical terminology, but I do know what it feels like.

It feels like the world is slowly closing in.

I have come to realise that this is a major driver of the anxiety.

It isn’t one dramatic event. It is the steady accumulation of change across so many fronts, physical and mental, often happening before I fully recognise it.

To me, that is the truly sinister nature of PSP.

I discussed it with my neurologist and there is very little that can be done if this is part of the progression of the eye movement problems caused by PSP. There isn’t another pill. Botox doesn’t help with this.

The answer seems to be adaptation rather than treatment: finding ways to compensate for what my eyes no longer do automatically.

Hearing that was difficult.

I think I already knew it intellectually, but there is something different about suspecting that PSP is quietly taking something else from you and then having a neurologist confirm that there may be very little you can do to reverse it.

Ironically, that concern almost overshadowed the good news from today’s appointment.

Because the Botox appears to be working.

A few months ago, I would probably have been the last person in my family anyone would have predicted would be having Botox.

Without question. Today was round three. Not for wrinkles.Not for vanity.

It is injected around my eyelids to help with the involuntary blinking and eye closure that PSP has kindly added to its growing collection of attractions. No – 10 injections in th eye lids is not fun but it is not too bad.

And, at least in that particular battle, things seem to be getting better.

My eyes feel more open. The blinking is less intrusive. The involuntary closing seems reduced. The neurologist also noticed improvement in my eyelid control, which has been a real problem in the past.

There is obviously no control-group Ben sitting next to me with identical PSP who hasn’t had the injections.

So I can’t prove exactly how much difference the Botox has made.  But I can compare myself with myself. And I think it is helping.

The FL-41 glasses are helping too. They haven’t changed the disease, but they have made the photophobia easier to manage.

So today’s appointment delivered two very different messages. Some eye problems are responding to treatment. Others appear to be progressing.

And perhaps that is the reality of living with PSP.

Not that everything gets worse all at once, but that change is constantly unfolding in the background, often unnoticed until it has already happened.

You celebrate the victories while understanding their limits.You treat what can be treated. You adapt to what can’t. And occasionally you discover that something has changed before you even realised it was changing.

That is why progressive feels so sinister.

It creeps up on you or at least it does to me.