Tag Archives: PSP

of dancing, rare disease, and a boy scout

I’m not so good at the ME ‘pacing’ thing. This will come as no surprise to the regular reader. I do all the things I’m not supposed to. I’m usually flat out busy or flat out recovering in bed. I want to do all the things, usually at once. I don’t recommend it, but it seems to be my way.

That means that my brain make up over the last few months has probably been:

35% rare disease events, training, support group, survey, what else can I do, of course I’ll do that

5% wee blind doggy literally bouncing off walls

5% Irish dancing things dress alterations, new dress, competitions, summer trip to France, fundraising, make the sandwiches

50% I can’t remember anything, what did I say I would do, wake me tomorrow, who, me?

5% the rest of life.

(Bloggy buddies, you fit in the final 5%. Not only I have I not been writing here (or anywhere) but I haven’t been able to keep up to date with y’all. I may have read your posts, but not commented. I may not have read anything at all.)

It’s true, for my own sanity I need to address the balance of my mind and my energy. I will, I will, I will.

In other news, Ditzy and I were out last night at a rare disease symposium at one of the universities. We did mingling and chatting and listening to talk about genetics. We may not have understood everything, but such is life. I was asked a question by a young woman who responded to my answer with ‘You don’t really know, do you?’ Harsh, but accurate. She was a biomedical scientist and my grade C in O’level biology from 1981 was no match. I need a crash course in the science bit.

bob chaos

Somehow, together, Ditzy and I attract oddness. Individually we’re sensible people. She’s a retired accountant, a proper grown up, and I’m vague, anxious and a bit mouthy, but together we operate in (create?) a world of chaos. We tried to get into the car park, but the barrier remained closed. Buttons were pressed. Nothing. We wondered if the event had started. More buttons. Still nothing. Ditzy put on her little old lady face and approached the car whose exit we were blocking. She wore the man down until he came to rescue us by pressing a different combination of buttons. Ditzy smiled and simpered and fluttered about, thanking him for rescuing two old ladies (She meant me! We spend so much time together she really thinks I’m her age!)

But what else could the man do- he was an actual scout, in uniform. Toggle and all. He couldn’t ignore distressed white haired women, no matter what age, or the whirlwind of mayhem surrounding them.



driving miss ditzy and mr david

You’d be hard pressed to squeeze more driving related crises into a morning. Anxious, giddy drivers, transporting a relative stranger around the region in the rain.

It started early. Miss Ditzy, a retired genteel sort, is not used to morning traffic. The 15 minute journey to my house took 50 minutes. By the time we gathered up Mr David at 9am, she’d been on the road for an hour and a half, and was past herself. While I was distracted looking up a picture of an MP with a fetching tie, we took a wrong turn or two and headed up the busiest road in town. In the wrong direction. A slip road, another road, a roundabout, more lights, the other slip road and eventually the right way. By 9.15 we all needed a stiff drink. It had already been a long day.

We’d spent the previous day with Mr David also. We’d met a neurologist, and held a huge support group meeting. 25 people in a room designed for 12. Borrowed chairs and big boxes of Swedish biscuits. A walking stick with added laser; sadly not a light sabre. There was only a minor parking incident on that day.

Miss Ditzy and I carry on like family- we giggle, squabble and wind each other up. I felt for Mr David, dropped into the middle of a strange place, with two hyper volunteers he can’t get rid of. Maybe he can commission research, by a mental health professional, on Life After PSP?

found at: http://www.pinterest.com/pin/212865519860265448/
found at: http://www.pinterest.com/pin/212865519860265448/

The second support group meeting of the week had smaller numbers, powerful stories, and a baby. A cuddly baby who was content enough on my lap to let the group do its thing. Eventually, I gave the baby back, we gathered the light sabre and the biscuits, and hit the road. This time, I was driving.

I have driven Miss Ditzy’s car before. Once I remember about the handbrake, all is well. We knew roughly where we were going. We had plenty of time. What could possibly go wrong?

The sky was low and dark. The rain kept coming. Heavy, wet missle.  Grey, splashy roads.

I realised I’d never needed the lights on Miss Ditzy’s car. Where was the switch?

-That’s the wipers.

-No, not there, that’s the indicators.

-To your right!

-This wee twiddly thing?

-No! Turn the thing!

I did. I turned the thing on the right.

Unfortunately, it was the ignition key. In three lanes of traffic, approaching a roundabout (with no lights on), I turned off the engine.

Fearing imminent crash, chaos and injury, Miss Ditzy and Mr David had conniptions.

I turned the not light back the right way, and proceeded round the roundabout while my travelling companions quelled their heart rates and struggled to restore their breathing. When Miss Ditzy could speak again, I pulled over and she pointed me in the direction of the huge big turny knob thing on the dashboard, nowhere near the steering wheel. There was light.

There was only one stall, at a ‘tricky’ roundabout, between us and the afternoon meeting.

There is a moment in every support group meeting when I am reminded why I do this. The hassle and the tiredness fades into the background, and I know that the effort is more than worthwhile. What we do makes a difference. People connect, get and give support, and realise that they are not alone. It is an privilege to be in the room when that happens.

The long journey home was without incident. Mr David avoided further panic by closing his eyes and praying/ meditating sleeping. Miss Ditzy and I blathered on, as we do.

Next time, we’re taking the bus.


A possible title for this post was ‘Thelma and Louise’, but that may cast Mr David in the Brad Pitt role. Only a young Brad Pitt can do that.

snakes, slowly

Progressive Supranuclear Palsy is a terminal, degenerative, brain condition. There is no treatment and no cure.

My mother received this diagnosis in 2007. Surprisingly, it wasn’t a hard to hear diagnosis- we didn’t understand. We didn’t know enough to be freaked by “Progressive”. We’d never encountered neurological illness, so we still expected that there’d be something to be done. That delusion wasn’t helped by the fact that the consultant gave us the name and no other information. No fact sheet, no phone number, no website, no specialist nurse. We went home, happy to have a name. Eventually, with the help of Dr Search Engine, reality began to dawn. We found limited information and support. Before long, we learned that we were the experts- how could that be possible?


Medusa, Carravagio

PSP is like a series of snakes, active in the brain. Sleeked. Slowly, deliberately, without compassion, squeezing themselves round proteins and neurotransmitters, tangling the communication systems. Squashing the the ability of the brain to tell the body what to do. A slow onset paralysis.

Medusa, a beautiful woman transformed into a mask. Powerful and raging, affecting anyone who looked at her.

The eagle eyed reader may have noticed that my input to the Health Activist Writer’s Month Challenge has been, at best, sporadic. It seems I’m not yet beyond setting unrealistic targets. This post was in response to #HAWMC prompt 8 “If the health condition of a loved one was an animal, what would it be?”


#HAWMC day2

Introduce your condition(s) to other Health Activists. What are 5 things you want them to know about your condition/your activism?

  • My mother got me interested in rare disease. Not on purpose. She had other things she wanted to be doing, but instead we all learned something about Progressive Supranuclear Palsy. The word ‘progressive’ gives a clue as to how much fun that was. I first wrote about herself in April 2011. She died 3 months later.
  • PSP affects mulitple body systems and can be hard to describe without the listener drifting off (eyes, movement, speech, swallow, blah, blah, snore…) This video gives some idea of its impact.


  • There are over 6,000 rare diseases, affecting 1 in 17 of the population. In Northern Ireland, that approximates to 100,000 people- roughly the size of a city such as Derry/ Londonderry.Collectively, rare diseases are common. Unfortunately, that’s not how it feels when a loved one is mysteriously ill and no one seems to know what it is; or when they are eventually diagnosed with a disease that nobody has ever heard of.  As Eileen, who is living with a rare disease, says: “When newly diagnosed the impact on the person/ family is like a bomb going off- everyone is stunned, the air is cloudy and you can’t see the way forward. You feel stuck in time, stranded, isolated, victimised, life as you know it will never be the same. The rest of the world carries on around you, and you have to find a new way to slot back into it. This is made even more difficult because nobody explains to you how to do it, when to do it, what to do if you need help, where to go for help, how to get there, who to ask when you get there.This is even more of a problem when the condition is rare, because nobody knows about it. It doesn’t fall under any umbrella so there is no system, set of guidelines, leaflet, specialist in place that can guide you.It is a very lonely, bleak, soul destroying place to be.” 
  • Although collectively rare diseases are not rare, people living with rare disease are particularly disadvantaged within the health and social care systems because of the lack of information, training or joined up services. Children born with some genetic conditions may not live to see their first birthday, others struggle to gain a name (and correct treatment) for their condition and those that are, happily, living longer than in previous years do not have access appropriate adult services. In Northern Ireland, both children and adults are living with rare disease. Many of those people do not know anyone else with the condition; nor do their neighbours, their GPs, nor their range of Allied Health Professionals. Those people are isolated and anxious, they may be receiving inappropriate treatment, and they have very limited support.  More than ¼ of individuals wait between 1- 5 years for a diagnosis, with many people receiving multiple incorrect diagnoses, and some patients waiting more than 10 years for an accurate diagnosis. The lack of awareness of rare diseases leads to some individuals receiving inappropriate medical treatment and poor social support; this may severely impact people’s quality and length of life.
  • I can campaign on rare disease issues because I had to give up work due to my own chronic ill health. I have issues with fatigue, cognitive disfunction and pain. I’m unreliable, unpredictable and prone to mood swings. I volunteer my time and energy to raising awareness of rare disease issues because I can do a fair bit of that from home, in my pyjamas. Nobody need know that I am the great unwashed, in a scruffy hoody and slipper socks.
  • Major life changes, loss of health (and job, plans for the future, confidence, salary etc), bereavement; it’s hardly surprising my mental health has been affected. Marian’s writing on mental health and cake is wonderful. I find there’s no rhyme nor reason to depression- there’s no easy algorithm or magic wand. I resisted medication for years and have now embraced it with great enthusiasm; it makes an incredible difference to my world and enables me to function. I muddle through.

stephen fry


I’m sure you were so enthralled by the writing you didn’t notice that was 6 points…. great points, all