Day Nine

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Day Eight

My hour-long daily dog walk gives me time for some deep contemplation about life and the beauty of nature. Although this morning I had to pick up a labrador turd from next to a dead pigeon, so, there’s that.

It was a fragrant start to what, by my calculations, is the 42nd day of UK lockdown measures, and Day Eight of my charidee blogathon. Only Day Eight and I’m writing about dog poo. God help us all.

Yesterday I spoke about food, and the insatiable appetite that affects people with Prader-Willi Syndrome. 

Today, it’s another biggie - emotional immaturity.

I’m not talking about simply having a short fuse, like my brother did when we were kids and I used to be able to make him lose his shit instantly, with one laser-targeted insult. It was as easy as snapping my fingers, which was ironic, as he did, on one occasion, snap my finger. And burst a blood vessel in my eye when his wild attempt to give me a dead arm missed and connected with my eyeball instead. He’s a police officer now, which I hope you will find reassuring.

No, the kind of emotional immaturity I’m talking about is the PWS person’s inability to understand and control their emotions. 

The loss of control can take different forms: ‘temper tantrums’ more akin to a toddler’s; violent rages (a hefty teenage boy with PWS who has had testosterone supplements can be a frightening, unstoppable force); or sobbing meltdowns.

My girl’s speciality is the latter, a wave of crying that comes over her - sometimes with an obvious cause, sometimes not - that you have to sit and wait out, as while it happens she is oblivious to anything you are saying. You know, like how your mate got after she came round and drank two bottles of merlot after that terrible break-up and then just wouldn’t stop, despite you telling her she was better off without the bastard. The only difference with my girl is that when the tide rolls back and she stops crying, she suddenly clicks back to normal, as if nothing has happened. Not like your mate, who will spend the next half hour telling you she bloody loves you. And then puke.

The flip side of my daughter’s emotional meltdowns are that when you think she might have one, she won’t. When my mum, her beloved Nanna, died, she informed me that she felt like she should cry but she probably wouldn’t. She doesn’t understand emotions, even if she sometimes gets the theory of them, she can’t always recognise what she or others are feeling. 

Today, the feels are good, though. My daughter is curled up on the sofa watching a 1994 film adaptation of Black Beauty, happy and fascinated and not questioning the logic of a horse whose inner voice is narrated by Alan Cummings. My boy is near me, but not needing my immediate attention, as he is lost in the world of Minecraft. So I’ve been left alone long enough in daylight hours (for 42 minutes to be precise) to listen uninterrupted to an entire LP (a rather splendid pink vinyl copy of St. Vincent’s Masseducation). Although it may now have a scratch on it from where I was a bit clumsy leaping up to lift the needle when I remembered too late that the chorus to 'New York' contains not one, not two, but three perfectly enunciated ‘motherf***er’s.

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

Song is St. Vincent - New York. You're all right if your kids are listening: this version says 'you're the only other sucker'. I mean, I prefer the sweary one, but I thought I'd treat you to the catsuit.

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. All of you who have donated 26p, £2.60, £26 or any other amount are awesome motherf***ers, sorry, suckers.

Day Seven

As the Great Discombobulation of 2020™ continues its relentless progress, my erudite and philosophical* marathon musings (*brainfart blogs) continue apace.

Writing a blog a day for 26 days during the Covid-19 lockdown (to raise funds for the Prader-Willi Syndrome Association UK) is more taxing than you might think. My fingertips are vaselined up to prevent keyboard chafing, and I've done my nipples too, but that was just because it felt nice.

So as we reach Day Seven, I've decided to talk about the biggest cannon in the syndrome's artillery: FOOD. Or more specifically, the overwhelming and insatiable appetite that affects people with Prader-Willi, and their consequent obsession with food.

It's both physical (the trigger in their brain which should signal that their stomach is full doesn't work), and mental (imagine being hungry all the time and how much this would dominate your every thought).

Some families have to lock their fridges, kitchen cupboards, and even bins, to prevent their PWS child or adult food seeking and stealing (there have been cases of people eating frozen food when fridges but not freezers were locked). Not every person with PWS has such extreme behaviours, but almost all have a life-changing and challenging relationship with food.

So the current Covid-19 shituation (yes, I have spelled shituation correctly) is particularly effing excruciating for many PWS families. Someone with PWS finds coping with changes to routine difficult at the best of times, so many just cannot understand why the supermarkets have gone all weird, their favourite foods are often not available, and when it comes to shopping for food, their Mum or Dad are no longer preferring the 'little and often' technique, but instead are doing it 'on a bigger scale but less frequently'* (*coincidentally, this last phrase also applies - thanks to my husband's shift patterns and the kids being home all the bleeding time - to our sex life.  Oh, God, I seem to have typed that out loud).

Where was I? Oh yes, the food thing. In lockdown this is proving doubly difficult for everyone because normal distraction techniques often involve taking the PWS person away from the home and in particular away from the temptations contained within the kitchen (for us this means going to the library, heading to the cinema, visiting Grandad, or going for a walk, the first three of which we can't do, and the last of which we can only do once a day).

So if you're currently enjoying an 'interesting' relationship with food (aimless fridge foraging I believe is a current 'thing'), just subsitute raging hunger as your motivation to munch instead of merely just boredom, and spare a thought for someone with PWS.

You could also spare a quid or two, if you like.

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

Song is Spearhead - Food For The Masses 

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. I can't express my gratitude enough to everyone who has contributed so far. Well, I can't express it now, but I can when social distancing rules are relaxed. 
You guys who have given are great. You other guys who haven't given yet could achieve greatness. And OK, I promise not to thrust greatness upon you.

Day Six

I’m not saying things have gone feral here, but I have been reduced to grunting and burning stuff.

I had a pile of paperwork half as tall as me to dispose of, but once my ancient shredder died after three sheets, I decided fire was a valid option. Being all middle class, this resulted in me ordering a chiminea.  A cheap chiminea, cheap chiminea, chim chim chiminea (apologies, but I’ve been singing this all day). 

When the socially-distanced delivery driver dropped it and ran, I roped the kids into helping me with the Some Assembly Required. The only lesson anyone learned from this decision was ‘Don’t’. Don’t involve your kids in any form of required assembly.

So I’m sitting in the garden, eyeing the grey clouds and incinerating my way through the stack of papers. If you're wondering where the pile has come from, most of it is from my daughter, and is related to her Prader-Willi Syndrome (PWS). Because when someone has PWS they also automatically become a GOSOP (Generator Of Shitoads Of Paperwork). This monster pile included my girl’s old Independent Education Plans, out of date Statements of Special Education Needs, and hospital letters from long-retired consultants, much of it dating back to the early 2000s (I’ve got three boxes of more recent paperwork that are going to remain in the loft, probably for another decade or so!). 

For those of you who would like to paint a mental image of me, my rapidly greying hair is sticking up in wind-blown tufts and I have a 1000-yard stare from this morning's home schooling (which included a science experiment involving a bag of margarine and some ice cubes, plus a lesson with my maths-intolerant daughter on 3D shapes which left me wanting to make a human sacrifice of myself on this very chiminea). 

I am dressed, optimistically, in a T-shirt, shorts and flip flops, which is OK because this bonfire of the sanities is generating some proper heat, and I don’t care, it’s April, and I will now be wearing my shorts until October, because I’m hard, me.

My daughter has emerged from inside, asked if she can poke the flames, and announced that I ‘smell of smoke’. 

“Why do you think that is?” I asked.

“Oh, it’s because you’re using the chimpanzee-er.”

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

Song is Isobel Campbell & Mark Lanegan - The Flame That Burns

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. I've been overwhelmed by everyone's generosity in these uncertain times. THANK YOU to everyone who has already donated! 26p or £2.60 or any amount you care to give - it's all hugely appreciated, unlike my promise to hand out post social distancing sexual favours to those donating £26 or above, which has seen a number of less than enthusiastic responses. What's wrong with you all? If you could see me in my flip flops, standing by the chiminea, holding the fire tongs provocatively, you'd soon change your tune...

Day Five

So, what do you want to know about infamous conquistador Hernán Cortés? (Spoiler - he was a bit of a bastard). 

What about waterfalls? I can tell you all about how they’re formed (and have executive produced my son’s very informative video for you at the end of this blog. HARD ROOOOOCCCCKKK!). 

Essentially, this enforced home schooling lark means that I am smashing the Year 6 curriculum (as well as my head against the wall at my son’s avoidance tactics). 

I’m also carrying out my own science experiment: Does lockdown cause exponential grey hair growth? (initial examinations indicate yes, yes it bloody well does).

I’m doing less well with my daughter’s learning.

My 21-year-old should be at college, on her Steps To Independence course, learning life skills, doing work experience, participating in tutorials, and being helped and encouraged by experienced staff in a safe setting with her friends. 

It’s specialised and tailored to her needs, because she - like everyone with Prader-Willi Syndrome - has learning difficulties.

As with several of the characteristics of PWS, the level of learning difficulties varies from person to person. Some are only mildly affected and are even able to take some mainstream exams, others have a reading and writing age many years below their own.

My girl falls somewhere on the mild to medium spectrum, illustrated perfectly by the fact that the vast majority of my 11-year-old son’s work is too difficult for her.

It’s hard. It’s hard to keep both kids focused and happy. So I’m winging it a bit. I’m cherry-picking little bits of online college work and letting her loose on BBC Bitesize lessons for much younger children (one science quiz involved a cartoon pug, which sent her into paroxysms of joy. If you don’t know why, you have obviously never met her, because if you had she would have shown you her tattoo of a pug in a doughnut).

I’m including her in some of my boy’s ‘wellbeing’ exercises and discussions, and I’m getting her to write film ‘reviews’ (which turn out to be laborious retellings of the entire plot, in the manner of: ‘and then this happened, and then this happened, too’. (Ask me about every frame of the movie Hotel For Dogs, go on, ask me...). 

She's doing word searches, watching Emmerdale, and going to the park on our daily walk, where she throws the ball for our labrador to fetch, and where she points out - every single time she praises him - that she loves him, but not as much as a pug.

She may not be like other ‘typical’ 21-year-olds when it comes to academic performance. But I think back to the painstaking efforts of some of the many professionals who have helped her throughout her life: the portage nurse who taught us games to help us stimulate her as a baby; the speech therapist and Makaton specialist who taught us how to use simplified sign language to encourage her to make her first sounds and talk; the teachers who used visual aids to help her communicate her needs and make sense of her day; the TAs who learned how to circumnavigate her stubbornness and find a different angle to get her to engage with a lesson, the volunteers at the Prader-Willi-Syndrome Association UK who were on the end of the phone when I was at the end of my tether. I look back and wonder and feel all the feels and thank all the thanks, and love all the love.

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

Video is my boy's guide to how waterfalls are formed. HARD ROCK!!!!!!

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. THANK YOU to everyone who has already donated! If you can spare just 26p, it would be help. £2.60 would be brilliant. £26 earns you special 'personal' rewards after this crisis is over. And I'm mulling over doing something silly at the end of this blog challenge if I am incentivised by enough donations. Hopefully this thought is far enough down in the small print to be forgotten about if I chicken out...

Day Four

Today, I’m going to break down some of the characteristics of Prader-Willi Syndrome for you. With some pratting about, obviously.

If your reading device only shows you the first few words of a blog before you click on it, then you will have just read the phrase: ‘Today, I’m going to break down’. It’s always a possibility, of course, but don’t worry, I’m still standing. Or “I’m Dill Danding’, as Elton John inexplicably seemed to be singing on that ‘One World: Together In Our Enormous Celebrity Homes’ concert the other night. 

We’ll leave Mr Dwight Divving His Dife Like A Dandle In The Dind for now, and concentrate on one part of PWS that has one of the biggest effects: low muscle tone.

It’s actually called hypotonia (a word which always makes me picture someone manically feeding hundreds of sheets at breakneck speed into a photocopier, but then I’m an idiot).

It starts BEFORE birth, with Sunday-pub-football-level-instead-of-World-Cup-winning kicks in the womb. High numbers of PWS babies are in an abnormal position at delivery, needing assisted delivery or a cesarean (my girl needed the latter, which had one upside I enjoyed - amazing drugs). 

Then, as newborns, PWS babies are very weak, requiring special feeding and arousal techniques. (It’s a coincidence, because as an adult I also require special arousal techniques, but that’s a whole different thing...).

The low muscle tone has consequent motor developmental delays (this phrase makes me think of being stuck at a roundabout in my battered old Honda Jazz automatic cursing the ‘motor developmental delay’ of it having the pedal-to-the-metal acceleration of a roll-along lawnmower).

So a PWS baby might not sit up until they’re one. They might not walk until they’re two (my daughter was three and a half, but more widespread and earlier growth hormone treatment has brought the average age down since she was a tot).

And children with PWS continue to have problems with strength, co-ordination, and balance. They have delays in activities such as jumping, climbing, and catching. They also struggle with their fine motor skills of drawing and writing.

Physio and orthotics (specifically supportive shoes, splints, and braces) help. Complications, such as scoliosis, may require an operation. If you think you know what stress means, book your kid in for a spinal op or two and you will NEVER sweat the small stuff again.

This bastarding hypotonia is for life. Adaptive and modified physical education programmes are recommended to build and maintain strength, tone and function. That means I spend an inordinate amount of time trying to think of sneaky ways to get my daughter up off her arse and doing stuff without it seeming like exercise. It will not surprise you that we have a fit dog.

One interesting fact you might like to know is that my girl is on drugs favoured by bodybuilders - daily growth hormone injections. She has yet to express any desire to don swimwear, sheen herself up with baby oil, and clench her buns and guns. But, incidentally, that is what I ask my husband to do for me as one of my aforementioned special arousal techniques...

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

Song is British Sea Power - It Ended On An Oily Stage

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. THANK YOU to everyone who has already donated - we've already broken the £500 mark, which is amazing! If you can spare just 26p, it would be help. £2.60 would be brilliant. If you donate £26, I'm offering sexual favours to be arranged when social distancing is relaxed, which may or may not involve baby oil. If this lockdown goes on for much longer we could possibly improvise with a rubber glove on the end of a broom. What the hell, it's for a good cause. 

Day Three

Today’s entertainment has consisted of ‘coaxing’ my son to complete a science assignment on axolotl salamanders (his immediate thought, of course, upon learning how they regenerate amputated limbs, being: “What if you cut their WILLY off?”).

It has also included, ambitiously - and regrettably - reading a college-assigned sexual health resource pack with my girl, which was going reasonably well until I came to the sections on genital hygiene tips and anal sex* (*reader, I clicked onto another document faster than a novice nun being caught looking at NakedHotPriests.com by the Mother Superior.)

In other words, it’s just an average Tuesday in lockdown.

I didn’t expect to be home, social distancing, and *ahem* ‘teaching’. Covid-19 has forced us all, like the axolotl, to adapt. But hopefully not to grow a penis.

The thing is, I should be used to adapting. If you’re the parent of a person with PWS, it’s the only way to survive.

Most people, after all, have never heard of Prader-Willi Syndrome. Named after the Swiss doctors who discovered it (wouldn't it have been great if it had been Toblerone-Leerdammer Syndrome?), it is a rare, complex genetic disorder, affecting approximately one in 22,000 births. You don’t carry the gene for it - it just happens. I mean, I can give you the technical cause, if you don't mind some medical jargon. Ready? 

It's because of some random shit that goes on in a chromosome.

If you're interested, I did once explain it in slightly more detail in an earlier blog post [Genetics], in what amounted to a war crime against science teaching. Click on the link and prepare to be blinded by science. Or amused at my ineptitude. Take your pick.

When my daughter was born and we were given her diagnosis - after three weeks of not knowing what was causing her floppy muscles and her inability to feed without a tube - we were floored. No, not floored, bloody well steamrollered. We had to peel ourselves up from the tarmac and adapt. To her path through life being ‘off-track’. To the calendar crammed with medical appointments. To the constant vigilance around food. To the emotional meltdowns. To the future that was just...different.

We’re still adapting.

For example, home school/college lessons this afternoon have adapted into a showing of Hotel For Dogs on Netflix for the kids, and a stiff gin for me. Change is good.

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

Song is Ezra Furman - I Can Change

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. We've already beaten my modest target - and every donation counts. THANK YOU to everyone who has given, and also to those who are just reading and maybe learning a little bit about PWS. 
I'll repeat my scorecard: If you can spare just 26p, it would be help. £2.60 would be brilliant. £26 may earn you a sexual favour after social distancing is relaxed, please advise me if this will be required or not so I can adjust my training regime accordingly.