Day Nineteen

Being in a household with a Prader-Willi person means you do learn to filter stuff out.

I can wilfully, skilfully ignore a level of background blatherings that would cause a random visitor (remember them?) to remark: “How can you hear yourself think?

When my husband and I do our unspoken tag team thing - otherwise known as when I sidle out of the room just after my daughter corners him in a particularly repetitive conversation cycle - I still vaguely register the conversation, but unconsciously filter it out to a background hum.

This happened yesterday, but my filter gradually failed as my daughter’s voice grew more panicky, and my husband’s started to sound as if his teeth were being well and truly gritted. I flipped my ear switch from ‘ignore’ to ‘earwig’, as I detected potential entertainment.

Her dad seemed to be explaining to her what ‘pixellating’ meant. He sounded like he was coming to the limit of the number of times he was prepared to answer the same question. I surmised this was what the last 15 minutes of ‘background hum’ had been about, but I couldn’t work out the context.

“I’m not going to tell you again, because I’ve already explained it, A LOT,” he said firmly, his exasperation clearly audible. “If you want me to watch ‘The A Word’ with you, you need to stop asking me that and PLAY the programme!”

Cue another anxious barrage of questions from her about ‘pixellating’. I started heading towards the door, motivated by a combination of nosiness, support, and the desire to take the piss. And just as I got there, the mystery of where the question had come from was solved.

“But it says in the listings that Joe is pixellated on his teacher!” said Josie, the confusion clear in her voice, possibly because she was trying to understand why Joe’s face would need to be disguised.

“All this time you didn’t think to tell me it’s in the listings?” came my husband’s accusatory voice, followed first by a pause as he located the remote and checked the programme synopsis, and then by a very large sigh.  “No,” he said, in the voice of a soldier, tired of war, “it doesn’t say that. It says Joe is fixated on his teacher. Fixated!”

"Oh!" came her unabashed reply. "Well, I know what that is."

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Song is Céu - Amor Pixelado - if you watch the video, you'll see she starts off well with the social distancing, but lets herself down by the end. Where's your 'common sense', woman?

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. I know I keep banging on about it. You could say I'm pixellated on it.

Day Eighteen

The amount of forward planning that goes into these blogs ranges from negligible to non-existent.

But I did actually think about today’s subject in advance, noting that it would be particularly appropriate for International Nurses Day. Yep, the International Nurses Day that was yesterday.

I’ll blunder ahead anyway.

It’s a simple story, about one encounter with one nurse. It’s not about the palliative nurse who took such care to explain everything when my mum was dying. It’s not about the nurse that helped distract my daughter as she had to fast for a morning when doctors tested her growth hormone levels. It’s not about the nurse that put her arm around me as my girl went in for her spinal operation and I almost crumpled. It was a definite crump, but not a full crumple. 

No, it’s this one.

Seven years ago, I spent five hours in a waiting room on a busy Saturday morning, hoping  a consultant paediatrician was covering what seemed like half the wards in the hospital could hold good on his promise to squeeze my daughter into his list. It was at a time when my girl had started going through bouts of sleepless nights, culminating in hallucinations, outbursts of laughter, anger, and even swearing. For f***’s sake, no-wonder I was worried - she hates swearing. Yes I do know it’s hard to believe with her being the daughter of Potty Mouth McGrew here. The sudden, terrifying, and totally uncharacteristic behaviours had me desperately worried that she might have a brain tumour. Spoiler alert: she didn’t. It turned out to be a mood disorder, which has since been managed fantastically well with medication.

Soon after we arrived, exhausted from four nights without any real sleep, feeling pretty hallucinatory myself, I asked to speak to a nurse in a side room, away from my daughter, so she couldn’t overhear. I told her what had been happening, and everything hit me and I broke. I absolutely broke. I’m not certain if I actually fell, but if I did the nurse caught me, because I ended up being held up and hugged tight. I couldn’t speak, she didn’t offer any platitudes, she just held me and waited. It took a while.

That’s it. There’s no spectacular ending for you. She didn’t perform a life-saving operation. She didn’t discover a hitherto unknown medical disorder. She just did exactly what was needed. Then got me a cuppa.

And that’s why nurses are f***ing awesome.

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is Etta James - Something's Got A Hold On Me

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. The PWSA UK works with medical professionals, you know, to help them know exactly what do when it's a little more specific to Prader-Willi. 

Day Seventeen

I don’t know about you, but I could really do with a pint. 

My phone served up a random memory from my photo feed today - the lovely sight of my girl manning the pumps behind the Rovers Return. Although I would have preferred it if it had actually served up a pint, instead of a pic. When is technology going to match my expectations? Yes, we’ve got the internet, but why can’t I materialise a Mojito in my hand? Is it too much to ask? And while I’m at it, where the hell are our jetpacks? I blame Elon Musk. I don’t know why; maybe it’s because he looks like a waxwork dummy of Rick Astley that’s been left next to a hot radiator since 1987, but I blame Elon Musk.

My hostelry-yearning has been sparked by lockdown life and my daughter plaguing me about going to the pub. 

“When will the pubs  open Mum? When can we go?”

If social services overheard the number of times she’s asked this in the past few days, I’d be put on both a reckless mother list and a 12 Step programme. 

Perseveration, it’s called. People with Prader-Willi Syndrome often perseverate. It’s when an idea, a word, a phrase, or a question gets stuck in their mind, and recurs, even when the stimulus is no longer there. My girl just can’t shift gears sometimes, and repeats and repeats and repeats herself. Answering the question doesn’t help, even if you’ve got a nice black or white response. She’ll still repeat her query, over and over - and when the answer is ‘I don’t know’, then we’re in for Perseveration, The Director’s Cut.

“So when will the pubs open, Mum? When can we go?”

I probably should explain: her desire is actually for a specific family tradition - various members heading to the pub on a Saturday morning to have a coffee with Grandad. My uncle and aunt are regulars. Different combinations of nieces and nephews of mine intermittently attend. And my daughter is a stalwart of the pub troop. The coffee is often followed by a Guinness for Grandad and half a cider for my girl, possibly not unconnected with the fact that the Saturday Club has also been witness to some extremely ‘interesting’ pronouncements, declared by my daughter at high volume in no filter mode (telling her cousin she needed to visit a sperm bank, being one of the most memorable).

“So when will the pubs open, Mum? When can we go?”

I don’t know, sweetheart. 

The pub club is on hold, like so many things are at the moment. thanks to the insidious Covidious coronavirus. 

Like Covid-19 her perseveration is catching. She keeps reminding me of what I’m really missing: the warmth, the company, the hugs, the laughs, and the simple loving heart of these visits.

Plus the beer.

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Related blog posts: Corrie, Baster

Song is Amos Milburn - One Scotch, One Bourbon, One Beer

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. And while you're at it, write to Elon Musk, would you, and get him to knock the space travel on the head and invent a beer materialiser, please. 

Day Sixteen

I’ve been thinking about adjectives today. 

Not all day, that would be weird. 

If you want to know, my other thoughts have included: ‘The modern day definition of a Sisyphean task is trying to get a refund out of yer Ryanair bollix Michael O’Leary’; ‘Although I have finally got hold the Holy Grail of self-raising flour, I’ve forgotten how to bake a cake’; and ‘I must, at some point, clear up the dog sick in the garden’.

Right, so, back to the aforementioned adjectives - this morning I had to help my daughter identify them in some sentences as part of the English work set by her college. She also had to think of some adjectives of her own - one beginning with every letter of the alphabet. A dictionary was permitted, which was a godsend when it came to X and Z. (Xenophobic and Zesty, if you’re interested).

She was hesitant at first, and needed a few prompts and prods from me to really think about which words were the ones describing something. She worked her way through the exercise, gaining in confidence. You might say she applied herself to the task determinedly, stubbornly, resolutely, unshakably, and bigly (no, damn you Donald Trump,  you tangerine succubus, bigly is not a word).

I realised something. In doing these daily blogs for the 2.6 Challenge, and trying to raise awareness about Prader-Willi Syndrome, it’s so easy to fall into the trap of using negative adjectives. The condition comes with a mass of challenges, and sugar-coating them doesn’t help anyone. (With PWS sugar-free not sugar-coated is definitely the way to go).

But sometimes you have to take a step back and celebrate the positives. Because there’s a plethora of positive adjectives that can apply to my daughter, despite of, because of, in addition to, and in conjunction with her syndrome.

Joyful, bright, happy, endearing, loving, funny, quirky, distinctive, delightful, engaging, beautiful, courageous, cheeky, loyal, astonishing.

Magnificent.

Yeah, that’s her adjective, right there.

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is The White Stripes - Sugar Never Tasted So Good

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. They are more than a little responible for producing some of the positive adjectives mentioned above. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. Oh, and Hippopotomonstrosesquippedaliophobia, that word I told you about in yesterday's blog? It's the name for the fear of long words. 

Day Fifteen

I’ve not caught the clap.

There you go, I’ve admitted it. During the coronavirus pandemic, people across the UK have been standing on their doorsteps every Thursday night, clapping to recognise the efforts of NHS workers and carers.

I, however, haven’t, because I hate nurses.

Not really. Jesus, learn to take a joke, would you?

Look, it's not a competition. Just because you clap doesn't mean you love the NHS more than me. Just because I have a child with Prader-Willi Syndrome doesn't necessarily mean I love the NHS more than you. (But I probably do. Deal with it). I expressed my love for the NHS as best as I could in a blog post marking its 70th anniversary: There. Give There it a quick read if you think I’m being an old cynic here.

But there’s just something about the whole clapping - a nice idea for a one-off show of appreciation - that now feels like a empty gesture. Does an exhausted consultant, coming off a 12 hour shift, really feel better because Bob and Brenda from No. 22 banged a saucepan on Thursday night? I’m not sure that they do. Particularly if Bob and Brenda then invited Trevor and Sue and the kids round for a VE Day barbie because if there’s one thing that renders a ribonucleic acid-based virus non-contagious, it’s a bank holiday.

I love the NHS and I know and love people who work in the NHS. They don’t want to be thought of as angels or heroes - they just want the resources to do their job, save lives, and care for people safely. 

So I just can’t bring myself to clap along with Boris, our glorious ‘led by the science’ leader who seems to have achieved hero status for contracting and surviving the virus, despite boasting at a press conference about shaking hands with everyone, including hospital patients, but who still insists that government messaging throughout this crisis been clear, consistent and responsible. 

If clapping brings you comfort and hope, and you think it helps  - even if it’s only good for your own mental health, or if the kids find it fun - please carry on. Maybe I'm a miserable old cow, out of step with the rest of you. Maybe I deserve your opprobrium* (*for my opinions, not for my vocabulary - I'm smashing my 'learn a new word a day during lockdown' challenge! Tommorow, I'll tell you what Hippopotomonstrosesquippedaliophobia means).

For now though, and for the foreseeable future, eight o’clock on a Thursday - when others are applauding and pan-bashing - will just happen to be the time I choose to chuck my empty booze bottles into the recycling bin. 

Because if no-one can hear the chink of empties then it means I’m not an alcoholic, right?

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is The Meters - Handclapping Song

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. And if you've already given, but are annoyed by what I've written today, I'm sorry, no refunds. Do come back, though. Remember: Hippopotomonstrosesquippedaliophobia, you know you want to know.

Day Fourteen

Today I want to talk about incomplete sexual development. No it’s not the answer to ‘Why do men buy sports cars?’ or ‘What could he possibly be over-compensating for by holding a large snake in his Tinder profile?’

No, I want to talk about it being one of the characteristics of Prader-Willi Syndrome. Sexual development in PWS is affected by the diminished or disrupted production of sex hormones. It’s called hypogonadism (the failure of the gonads to function properly - specifically the testes in men and ovaries in women). 

Firstly, I challenge you to say gonads without sniggering. Secondly, if you’re female - did you have ANY idea you have gonads? You live and learn, don’t you, Gonad Girl! *sniggers*.

So what does it effectively mean for someone with PWS? 

Boys typically need an op for undescended testes, and both boys and girls usually have delayed and/or incomplete puberty. Confusingly, they often go through premature pubarche (development of pubic hair) earlier than the average, caused by something called adrenarche (these words, man, so many words), which isn’t actual puberty (just the stuff like hairy bits, oily skin etc. Hairy bits isn’t one of those official medical terms, in case you were wondering). So that’s helpful, when you’ve tried to explain carefully to your child that they might not go through all of the same changes as their peers so, apart from that one that they already have gone through but isn’t actually what it looks like. Clear? Good, I’m glad someone is.

Testosterone treatment for boys and oestrogen therapy for girls can be used to bring on puberty (either to induce it, or to kickstart and maintain it when it stalls). 

For my daughter it involves taking an oestrogen pill, which finally brought on her periods at the grand age of 20. This is been an...interesting...development to deal with, not least because of the fact that her periods, although quite regular don’t start EXACTLY 28 days apart. And she does like to know EXACTLY when things will happen.

One thing that is extremely unlikely to happen, however, is related to fertility. My girl will be unable to have children. I could explain more, but I’d have to use more of those medical words, and to be honest, I peaked with gonads. 

There have only been a handful of women with PWS in the entire world who have had a baby. It’s something we’ve been honest about with her since she was old enough to understand. She’s pretty matter-of-fact about it. 

“I can’t have babies, can I, Mum?”

“No, sweetheart.” 

“But I am coping very well with my periods, really, very well.”

“You definitely are, yes."

So, many years from now, it’ll be down to her brother to ensure our family line lives on, God help us. Is it wrong to make a mental note of suggesting Gonad as a name for our first grandchild? It is, isn’t it?

https://www.justgiving.com/fundraising/carolyn-s-2-6-challenge1972

Song is Arrested Development - People Everyday

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. And keep coming back and reading. I mean, where else are you going to find out you've got gonads you never knew you had?

Day Thirteen

I’ve seen lots of jokes and memes on social media during the coronavirus lockdown about how people have no idea what day it is. I disagree. If you’re attempting to home-school, you know how far from the weekend you are, exactly.

So today is definitely a Friday, but not just any old Friday. It’s Bank Holiday Friday switcherooed for Bank Holiday Monday due to the 75th anniversary of VE Day, and I am mainly celebrating it for being a ‘no school’ day in a time of no school. No school squared, if you like.

I attempted a bit of mild parental responsibility this morning by chatting to my kids about what VE day meant. We cut out some paper to make bunting and got covered in oil pastels. I made a tiny bit of a ‘jingoistic isolationism leads to very bad things’ point by making sure we included not only Union Jacks but also flags from some of the other nations who had just a little bit to do with defeating the Nazis - you know, the ALLIES.

But my daughter wasn't entirely focused. She binned five pennants after colouring white bits blue and blue bits red, all because she has been concentrating on one thing, which was most assuredly not celebrating the anniversary of the end of the deadliest conflict in human history. No, it was watching a TV programme.

She has waited two years and four months for the third series of BBC drama The A Word to be made and aired, checking the TV guide every week to make sure she knew when it finally dropped. “When will Series 3 of The A Word be on?” is a question that might haunt me on my deathbed, alongside: “Will sleeping on my back make me shorter?”. On Tuesday it hit the screen, and she watched it on catch-up the next day (as it aired past her bedtime). 

Today, she’s been plaguing me and her dad to watch it with her. 

“Yes, we will watch it with you,” I explained. “But I’ve got to get Grandad’s medicine/cook the barbecue/write my blog/have a poo* first...” (*these activities were carried out consecutively, not concurrently, you’ll be pleased to know). 

So I’m off now, to curl up on the sofa and watch a drama about the family of a little boy with autism. “Like me, Mum, I’ve got a diagnosis of autism too, haven’t I, and people with Prader-Willi have a bit of autism don’t they?” my girl will tell me, numerous times throughout the show. Trust me, she won't be able to help herself. I might put the subtitles on so I have a chance of getting some of the dialogue.

Once she’s in bed, it’s my boy’s turn to choose the viewing, and tonight will be the sixth movie in his grand rewatch of the Marvel Cinematic Universe in chronological order of the narrative. So it’s Thor. Which just makes me look forward to that time in the future when cinemas will open their doors again and I can march up to the desk and request: “Four for Thor 4”. What a time to be alive!

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Song is Julian Cope - Trampoline (from the soundtrack of The A Word).

As part of the 2.6 Challenge (which is asking people to fundraise and donate towards small charities that are threatened with closure because of the effects of the Covid-19 crisis) I'm currently writing 26 blogs in 26 days.The PWSA UK is a charity which is absolutely vital for people with PWS, their families, carers and professionals who work with them. Without urgent help, PWSA UK will fold. This charity saves lives and for some people makes lives worth living. If you can, please go to my Just Giving page and donate anything you can spare - a few pence or a few pounds, it all counts. Think of all that money you're currently saving not going to the cinema. You'll still have enough left for four for Thor Four, honest.