PSP and Personal Responsibility

I have always believed in personal responsibility. If you make a mistake, own it. If you hurt somebody, apologise. If you are wrong, say so. If your behaviour needs to change, change it. I have tried to live that way. I have also tried to instil it in my children and in the many employees I’ve trained.

None of that has changed. But I am starting to.

I am angrier than I used to be. My fuse is shorter. I become frustrated more quickly. There are moments when I hear myself speaking and do not entirely recognise the person speaking.

That frightens me.

PSP is not simply attacking my balance, movement, eyes, speech and swallowing. It is a neurological disease. It damages the brain. The same brain that moves my legs also regulates motivation, restraint, emotion, judgement and behaviour.

The science is clear that behavioural change can be part of PSP.

For example, a study of 154 people with PSP found behavioural abnormalities to be common, with more than half experiencing symptoms including apathy, irritability and disinhibition. An earlier Neurology study found apathy in 91 per cent of patients and disinhibition in 36 per cent. More recent research has linked irritability and agitation or aggression in PSP with changes in specific areas of the brain involved in emotional regulation.

Another study of apathy and impulsivity found that the two can coexist in PSP. That sounds contradictory, but it is not. A person can struggle to initiate one action while finding it harder to inhibit another.

This is not simply a patient becoming awkward, lazy, rude or difficult. In so many cases, it is damaged brain circuitry changing how that person responds to the world.

More importantly, I have read countless accounts from husbands, wives and children of people with PSP. They say that the person they love has changed. Some describe anger, apathy, impulsivity or behaviour that would once have been unthinkable. Occasionally, they describe aggression or violence.

That does not mean every person with PSP becomes aggressive. Many do not. It certainly does not make harmful behaviour acceptable.

But it does make the question unavoidable.

If I become angry with my loved ones, is that PSP, or is it me?

Am I responsible?

I think the honest answer is deeply uncomfortable. It may be both. PSP is already part of the mix and will probably become a larger part of it.

PSP may explain why my fuse is shorter. It may weaken the brakes that once controlled my reactions. Pain, exhaustion, fear, loss of independence and the sheer daily frustration of living inside this body may add still more pressure.

But an explanation is not automatically an absolution.

If my words hurt somebody, they still hurt. My family should not have to pretend otherwise because I am ill. They are allowed to be upset. They are allowed to tell me that my behaviour was unacceptable. They are allowed to protect themselves.

To my current self, PSP should not be a get-out-of-jail-free card. At the same time, it would be equally wrong to judge neurological behaviour as though the disease played no part in it. We recognise that someone with Alzheimer’s, another disease involving abnormal tau protein, may no longer be functioning with their previous levels of memory, judgement or understanding. We should also consider what damaged frontal brain networks in PSP may do to judgement, motivation and self-control.

Responsibility, therefore, may no longer be all or nothing.

Perhaps my responsibility now is to do everything I still can while I still can.

To recognise the change. To listen when my family tells me that I have crossed a line. To apologise without immediately defending myself. To identify the triggers. To reduce unnecessary stress.

To ask doctors whether medication, pain, fatigue or progression may be contributing. To put safeguards in place before I need them. To give the people around me permission to step away when my behaviour becomes too much.

Most importantly, to keep fighting for the person I want to remain.

I also know that, at least for now, I am fortunate to be able to recognise the threat and fight it.

There are many people living with PSP who no longer have that ability. They may have lost part of their character to the beast without even knowing what has been taken. If they still possessed the necessary insight, they might desperately want to exercise personal responsibility, resist the change and reclaim the person they once were.

But the disease may already have taken away their ability to see it.

That is not a moral failure.

It is part of the cruelty of neurological disease.

I know that there may come a point when my own insight deteriorates. I may lose the ability to recognise what is happening or to explain it. Others may then say, “That was the PSP.”

Part of me will be grateful that they understand.

Another part will be wounded by it.

I do not want my family to face two equally cruel choices: to blame me entirely for behaviour shaped by disease, or to excuse everything by erasing me entirely.

The truth may live somewhere in between.

I am still Ben. PSP is also becoming part of how Ben reacts, thinks and behaves. The border between us is not always clear, and it may become less clear with time.

That is one of the most frightening things about this disease. It is not only taking over my body. It sometimes feels as though it is trying to negotiate ownership of me.

I do not know whether I can win that battle.

I do know that I am still fighting it.

Perhaps the struggle itself matters. The fact that I worry about my responsibility means that my values remain alive. The fact that I feel remorse means that I still recognise the man I want to be.

I cannot promise that PSP will never change me.

It already has.

I can promise that, for as long as I retain the awareness and ability to do so, I will not casually surrender my responsibility to it.

PSP may explain some of my behaviour.

It must never become an excuse I am eager to use.