Diary: 1st May 2018 – What is PCA?!

What a week. This is not the update I wanted to write. I wish I’d written one on Sunday or yesterday before I got home. I had started to really process everything and felt that I had finally reached some sort of positive acceptance. It took a few days and a day off work to get there but I was actually “ok”.

Unfortunately, we’ve been unlucky and another curve ball came in out of nowhere, by way of the official doctors letter when I got home from work last night. The official diagnosis, sat there staring at me;

Dementia of Alzheimer’s Type, early onset (Likely Posterior Cortical Atrophy Variant) 

Reading that was bewildering; I’d never heard of Posterior Cortical Atrophy. The doctor didn’t mention anything about it in the meeting?! Google can be a horrible thing in these situations, but quickly answered my question, “What is Posterior Cortical Atrophy?”:

“Posterior cortical atrophy (PCA) is a rare form of Dementia that usually begins by affecting a person’s vision…”Many people diagnosed with PCA will often be registered as blind during later stages of the disease.”

What?!? Blind?!?

I cried for the best part of an hour.

Atypical Alzheimers is what we were told. And yes, it’s factually accurate but the candid details of what this meant in real life terms had never been disclosed. I was furious. One of the key aspects of PCA, I discovered (thanks, Google), is its brutal deterioration of vision and spacial awareness (an area that had in fact been highlighted as problematic for Dad). This is not caused by the eyes themselves but in fact how messages are relayed by the brain.

For me, it was like a light bulb going off; the issues with the iPad, the falls… it’s not his glasses that are the problem – something I’ve been consistently having a go about, for months on end – it’s this f-king disease causing him further pain and anguish.

My father is too intelligent for his own good sometimes. He’s found ways to cover up his difficulties as far as is possible, just short of lying about them. He’s a proud man and not one to want to cause any unnecessary commotion. But the words “I can’t see what you’re talking about” from a few weeks before, hauntingly rang through my ears in that moment. 

I’m grateful to a very dear friend who let me cry and shout down the phone, the majority of which were incoherent rants. Honest and true friendship right there. I was so angry. So heartbroken. So unprepared for another swipe at his dignity.

It was too late in the evening to speak to the doctor but I called first thing this morning; I couldn’t wait. I needed answers, now. She profusely apologised for not going into specific detail regarding the specific etiology / cause of his “atypical Alzheimers” but that she had focused our time on treatment options (however frugal they might be…) and believed vision didn’t appear to be such a key problematic area for him.

After I explained how much I had chastised him regarding the need to go to opticians and a number of specific incidents, we both realised how much had gone a miss throughout the testing phase (even despite the lengthily review process).

We agreed, he’s far too smart, my Dad. He still manages to out-smart us all. His cunning and impressive tactics and have covered up just how far the disease has progressed and sadly, may even mean the stage at which he’s been diagnosed at is inaccurate. 

Following our lengthy discussion, the Doctor has now pointed me in the direction of a PCA support group and I will be looking to discover as much as I can about this little known Dementia and how we can fight it together for as long as is possible. 

Note: For more information on PCA, other Rare Dementias and support available, please reach out to RDS here: https://www.raredementiasupport.org/

Diary: 25th April 2018

It took until this morning whilst brushing my teeth for those tears to arrive – I mean what the hell?! Brushing my teeth!? 

I have no idea why or what possible trigger set that off. It seemed to come from nowhere and continue on and off over the next few hours including at work in front of co-workers… I feel stupid. What has changed?! I knew this was coming in one form or another; it’s not shocking or unexpected.

A few people knew the appointment was yesterday and have kindly checked in to see how it went; saying he has “Young Onset Atypical Alzheimer’s” brings an interesting variety of responses but with one common theme; no one really knows what to say. What do you say to that? I’m not even sure what my response would be if someone said that to me now. “I know what you’re going through” would seem feasible and also predominantly untrue – what I’m learning is that everyone’s relationship to, experience, symptoms and understanding of Dementia is different. 

No one believed me when I said something was wrong.

It’s probably just stress” 

Maybe he just has a vitamin deficiency

When I mentioned Dementia was being explored, I even had:  “I very much doubt it’s anything like that, he’s too young”.  Words I will never forget. 

From family to Dad’s own GP, their lack of understanding delayed my fathers treatment. This perhaps is the most upsetting part of this story; had knowledge on the subject been more freely understood, we could have possibly delayed the crushing reality that (at the age of 61) my fathers ability to follow or contribute to conversations has already reduced predominately to pre-rehearsed platitudes. 
It’s taken 9 months to get here. 9 months from the first GP appointment.

Since then we’ve had:

  • Vitamin and blood tests (September)
  • Return GP appointment to confirm “everything’s fine” (October) 
  • First appointment with the Memory Clinic including an hour and half of initial testing  (December) 
  • Two follow ups with Memory clinic for 2.5 hours of neuropsychometric testing (January)
  • MRI (January) 
  • Initial diagnosis meet at Memory clinic (March), now referred to PET Scan
  • PET Scan (March)
  • Final diagnosis appointment April 24th
  • Treatment due to start May 1st

This is just too slow for a disease that progresses as the rate it does and only when the resulting diagnosis has been delivered, can potential treatment options even begin. 

Diary: 24th April 2018

D day, except the D here stands for the one that I hate. 

Finally, almost 9 months since the first GP appointment we have a diagnosis;

Atypical Young Onset Alzheimer’s disease.

A string of words with devastating consequences. 

The “atypical” aspect wasn’t something I had thought of or had fully considered. This means the development of the disease doesn’t begin the way the majority do, with the “reduction” of the hippocampus (the part that stores memories), it can start elsewhere. Note: Biology was never my forté, so it still feels weird knowing the terminology.

We’re told that although only 5% of over 65’s diagnosed with Dementia present in this way, in Young Onset Dementia (those under 65) it is more than two thirds (!) which I wasn’t previously aware of (learn something new every day and all that) but no wonder it’s so bloody impossible to diagnose. 

I haven’t cried today. I’m not even sure why. I feel the need to cry but there’s nothing.
I guess there’s a strange sense of relief. Of the two possible expected outcomes, we’re told this was the better one. There are possible treat options for Alzheimer’s, with the goal to potentially slow it down. Nothings for certain – apart from it’s a terminal progressive disease, that’s the fact. We just need to take it step by step now and enjoy what we have while we have it. 

I did surprise Dad with an iPad today. He’s never had one before and it gave me so much joy. Despite concerns over his ability to engage with new technology, his disconnection from the world could not continue. I had to try. I set him up with a brand new Apple ID and showed him how with two clicks my (pre saved) contact card would allow him to video call me and show me what’s around him too..
Communication is becoming more and more difficult and hopefully this can provide an opportunity to show things vs putting himself under further stress and inevitably exasperating the issue.

As he grasps to hold onto his independence for as long as possible (stubborn as ever, like his daughter!), I’m going to damn well do everything I can to help him achieve that.

Diary: 23rd April 2018

The appointments been moved up; it’s tomorrow and I’m dreading it in some ways but in others, we just need to know.

They’ve said they *think* it’s FTD or Alzheimer’s due to his young age and primary issues with communication. Each (and all forms of Dementia) come with their own painful paths but our doctor even said “we hope [its Alzheimer’s]” as FTD has no treatment options and would progress much faster.. I guess we shall see what diagnosis we walk out with.

But I spoke to my Dad today and I can hear (and feel) how quickly his condition is progressing. Repeating questions, no knowledge of previous conversation, regular incorrect use of words and phrases and then the announcement; he’s just purchased himself a new alarm clock, that he has spent so much time trying to set up, to no avail. I can tell he’s upset, something so simple and that you can’t even blame on the complications of modern technology. He says the “paperwork is too small for him to read”. I don’t understand why there’s paperwork… I take a second and realise what he means to say is that the buttons, to adjust the time etc, are too small;

Let me walk you through this:

  • Paperwork = provides instructions and directions on how to set up things
  • Buttons (in this context) = provide a way to adjust settings and set up said alarm clock 

This kind of decoding of conversation is becoming more common than not. 
My very smart father, knows what he means – he’s not any less intelligent – he just doesn’t necessarily have all the tools to explain it in his previously impressive vocabulary.

This is a very sad but unfortunately – I’m coming to learn – a common fact of life for those with Dementia and one that I am slowly adjusting to. 

I just try not to forget that he’s still the same – he’s still in there – something I try to remind him every day. I will remind him tomorrow, no matter what. 

Diary: 10th April 2018

At long last, Dad’s “final” diagnosis appointment is confirmed for May 4th. At which point, we will hopefully understand more about the specifics of his condition and what that means for the future.

This will be almost 8 months since his first doctors appointment and close to 2.5 years that I’ve been saying and screaming that somethings wrong so.. let’s see what cards we are dealt with and we’ll have to just go from there.

Diary: 9th April 2018

They say the grieving process – and yes you are grieving! – includes a number of stages;

Acceptance, this is where I ‘think’ I’m at now… Either that or just flat out denial.

It’s been 6 weeks since that formal diagnosis of the D word. Dementia, a subject at this point I feel like I already know far too much about, has turned our world upside down. 

Since then, Dad’s had another hospital appointment. This time a PET scan where radioactive components are injected into the arm to see how the brain digests them and allows you see which areas of the brain arent working how they should be. He’d already had an MRI but it was inconclusive.

One of what feels like an endless list of slaps in the face when it comes to Young Onset Dementia, is the rigorous testing needed to confirm the underlying disease. We know it’s Dementia, but what type? I didn’t know there were at least seven (!), each with their own unique and evil trajectory. All with the same inevitable, heartbreaking outcome. Honestly, one I wish not to consider at this point but unfortunately that’s not an option; Ive been told there are legal, safety and financial aspects that we have to start dealing with now, before he no longer has the ability to make such decisions..I was given a folder (“a handbook”) on all the things that need to be sorted. Not in the least bit overwhelming, sure… 

But then, as we’ve already found, there are smaller issues that present themselves along the way, that you don’t even think of.

Modern technology, for example. As much as it brings so much joy and opportunity, for someone with Dementia it is an on-going obstacle course of confusion and unnecessary anguish. Something as simple as a mobile phone has become of topic of distress. The short term memory deficiency makes it almost impossible to remember new words and names, let alone learn new technology. As each mobile phone comes with its new applications and “toys”, it becomes increasingly difficult for Dad to understand. As luck would have it, backed by consumer nostalgia, Nokia have recently released some of their original phone lines (3310s with buttons and all!). Here’s hoping this could be a serendipitous opportunity to re-stimulate old memories and increase and extend his independence in the months and (I hope) years to come. 

Diary: 16th March 2018

That word. Dementia. I didn’t think it was possible to hate a word.

I hate what it is, what it does; the chaos, the pain, the fear, the confusion.

Most of all I hate what it’s taken and continues to steal from us every single day. Memories of the past and time from the future. And that’s just my selfish point of view. What it’s taken and continues to take from Dad every single day is inhumane. 

I honestly don’t know a man more proud or with more intelligence than my father. An encyclopaedic knowledge of history and the world around us, during his life he went from the Air Force to CEO of a UK Trade Association and predicted so much about how things would change around us; I still remember him talking to me about something called “Bluetooth technology” c2001 and how one day we’d be able to use it and the internet to program home devices such as our microwave, heating, or lights remotely… We recently just bought light bulbs for our flat that are turned on and off via an app or through Alexa – I had to smile that day. 

What I feel is worth mentioning is Dad is currently 61 years of age. Not even legal retirement age. And it’s taken us the best part of two years to hear that my worst fear had been realised; he has Young Onset Dementia. The Doctor’s best guess is he’s had this for 3 years now; slowly and quietly doing catastrophic and irreparable damage until no one could deny the problem any longer. 

My dad still has difficulty explaining the key moments when he knew something was wrong but mine started at least two years ago:

In that first year, there wasn’t one specific thing that stood out, it was lots of little things: slower responses, confusing things, struggling to recall a specific memory –  I remember having a conversation with someone and saying “something’s just not right”. Based on my explanation, you might think nothing of it (a lot of people did) but it was just the start. 

Then in October 2016, I took Dad for a holiday to Rome. It was his 60th birthday and we had never really had a holiday just the two of us, so it was my treat. During that week, the realisation of how regular those “little things” really were, was quickly apparent. I had already subconsciously started adopting my behaviour like I was the parent and needed to look after him. He also began to talk about family memories a lot more (the reason for which became clear later on…) and death. So much about his own morality. 

By early 2017, I was seeing signs that I look back on now as the most obvious. Communication issues (mixing up words) and repeated conversations for example; sometimes a few days apart, sometimes within 15 minutes. I was really concerned but no one else seemed to be picking up on what I was experiencing and at times, I even felt like perhaps I was being dramatic. But then in September 2017, a series of events in a single afternoon meant that what was happening, could no longer be ignored. It ended in tears and Dad locking himself in the bathroom crying.

The GP was called the next day.

More than 6 months later, here we are. He’s been given a formal diagnosis of Young Onset Dementia, although yet still waiting to hear exactly what type. Today we were on the phone and it happened again… My career was something we talked about at length. It was so important to him that I did well. We were talking about (more) hospital appointments and he said, “I know there was something else we talked about yesterday that I was supposed to ask you about.” It was an interview that I had coming up tomorrow. A small thing to others, but to me crushing. 

I recently built up the courage to watch the movie “Still Alice”. It’s hard to watch, I wont lie, but the story was obviously very close to home. Of the (many) quotes that resonated with me was when she tells her husband, “I wish I had cancer”. 

Please, don’t take this the wrong way. My family has had to fight the horrible C more than once in the last few years and we have generational history of cancer in our family, so I would never diminish the pain or fight that comes with this. 

But the education and understanding of Dementia (both within the general and scientific community) is not where it needs to be. Latest numbers reveal 800,000 people (!!) in the UK alone fighting this disease, sadly non of whom will recover. It’s a progressive disease with no cure…Gradually robbing the individual of everything they once knew about themselves and the world around them. How can this be?

My anger here is palpable.