Pitched

Today was one of those community days.

I don’t mean I had to don an orange jumpsuit and paint over graffiti - that’s every other Sunday, and I still insist I was provoked, officer.

No, it was one of those days spent with other members of the PWS community. This PWSA UK (Prader-Willi Syndrome Association UK) family day was at Thetford Forest, and proved as much of a grin-generator as previous events we’ve attended. And, as you may know, we’ve attended a lot. Once my daughter has got wind of a Prader-Willi meet-up - no matter whether the location is The New Forest, Manchester, London or The Lake District - I have to have a cast iron excuse not to take her. By cast iron, I mean basically a death in the family. Hell, no, immediate family.

So we punched in the postcode in the sat nav, entered into the usual Brexit-complication-level negotiations over song choices on the stereo along the way, arrived, parked up, disembarked, registered, and got our name stickers. (Incidentally, I’m thinking of standing as an independent candidate in the forthcoming General Election on the single issue of it being compulsory for everyone, everywhere, at all times, to wear a name sticker. I’ve essentially got to that age where my brain just has no more face-name neurotransmitters left. I've pondered over the cause of their destruction, and I've narrowed it down to over-consumption of gin). 

We spotted a few old friends from previous events, and made a few new ones. There was, as always, a mix of tiny babies (with parents with a tell-tale, slightly shell-shocked demeanour), wobbly toddlers, cute children, my own diminutive teenager, and a lovely 21-year-old chap with a charming heavy facepaint/light beard combo, who introduced himself to everyone at least twice, and was utterly thrilled as this was the first time he’d met more than one person with Prader-Willi ‘like him’.

My girl was adored at by adoring Polly, who has a strong track record of previous adoration. They wandered around hand in hand, stopping off for my daughter to throw a little adoration of her own at Polly’s new baby brother (see picture). And my PWS girl - you know, the one with the insatiable appetite - insisted on not starting her picnic like everyone else until Polly had finished having her face painted and could sit next to her.

My boy behaved impeccably (by his standards), which meant he only burst half a dozen balloons, which wouldn’t have been so bad if they hadn’t sounded for some reason as though they were filled with gunpowder as well as helium. (Actually, in all seriousness, he was remarkable, playing a mini football match with a PWS boy, which awakened in him hitherto dormant traits of patience and magnaminity. Unlike when I play him, and he rugby tackles me, feigns injury, and bursts into tears if he doesn't beat me by ten goals).

It was a sun-kissed day, and a meeting of families who are all on the PWS map. We may camp out most days in very different topographic areas, from the beginners field to the SAGA cruise ship harbour, but today we pitched up together. And it was pitch perfect.

Song is: Yeah Yeah Yeahs - Maps

Curve

My girl's struggle with pneumonia continues slowly. My struggle to present my best bright and breezy game-face to her also continues apace.

Yesterday there was talk of ITU and intubation. There's been less talk of it today, and my heart rate has gone down accordingly.

Antiobiotics have been switched. The ward has echoed to the sound of a succession of sinewy female physios drumming out a rhythm on my daughter's side and back with resounding cupped hand pats and manipulations. My girl has been blowing through a straw to make bubbles in a jug of soapy water. Her observations are being taken frequently and assessed carefully.

Despite the stubborn infection not wanting to shift, she's been more like herself  today. Typical trademark random conversations have occurred, when she previously wasn't saying much at all. The one that amused the nurses the most was a sudden worry on my daughter's part that Michael Jackson's doctor might be charge of measuring out her medicine (what the actual fuck?). Also, out of the blue, she told one doctor that "the matron was horrible to children and beat them". He seemed somewhat relieved to hear my quick explanation that my girl was talking about a character in Hetty Feather, a Jacqueline Wilson novel about a Victorian foundling hospital which I'd been reading to her at her bedside. Not referring the sister in charge of Ward 5 at Milton Keynes Hospital, who seems very nice.

Friends have helped keep little brother entertained with playmates. My husband has helped with shuttle runs, provisions, and general superhuman steadfastyness. Yes, that is now a word, I just wrote it.

Steadfastyman* and I are doing a tag-team switcheroo tomorrow. (*Autocorrect turned this into Steadfarty man and I was tempted to leave it, as it's actually very accurate). I need my bed for a night. I need the next couple of days to continue the tiny upward curve they've taken today. And I definitely need them be curvier, and more upward.

Bounce

I had that warm, tingly, sun on your face feeling.

I’d just watched my girl running - yes, technically, definitely, sort of running - down the track for the sprint race at her special school sports day. She was at the back of the pack but she was having a real go.

Minutes earlier, I’d stood back, amazed at her bouncing along in the sack race, her cheeks reddening from the effort. I got flashes of her beautiful grin as she shot sidelong glances and giggles at her dad: a middle-aged man with sticky-up hair, wearing flowery shorts, shouting out "Boing! Boing! Boing!" as he pogoed along beside her.

Special school sports day is always fun. One of my daughter's classmates, having been told he could dress as his favourite sporting star, came as Kevin Pietersen in full cricket gear, pads and helmet and all, which I can attest makes the long jump a challenge.

I even got involved myself, as part of an impromptu and rather hefty parents team in the Parents v Staff Tug Of War. Victory tasted sweet. An estimated 30 stone team weight advantage had nothing to do with it, I tell you. It was all technique.

It was my second sporting foray of the day, having already taken part in the mums' race at my boy’s sports day earlier the same day. I’d seen my opportunity to cause him burning shame, and I’d taken it, because he’d been a little sod all week.

He watched as I had galumphed up the track, trailing in the wake of several mums who were younger, fitter, and - as my own mum would quaintly put it - less ‘booby’ than me. I’d adopted an unusual running style: the ‘right hand clutching right nork, left hand clutching left nork’ technique* (*first pioneered by Dolly Parton when she forgot her sports bra for track practice. Probably). It had seemed apt to protect my assets (coincidentally encased in a Tony The Tiger ‘They’re GGGGGGRRRREAT!’ T-shirt) as I needed way more jiggle-proof scaffolding than that provided by my standard M & S bra.

I walked back over to see my boy sitting on the ground next to his dad with his hands over his face. “Brilliant!” I thought. “I’ve really embarrassed him.” But no, the ‘hand-bra’ style had passed him by, possibly because he was watching from behind the start line and had only been able to see the back of me. No, his hands were not hiding his shame, they were suppressing derisive laughter. “You came last, Mum. You’re RUBBISH.”

Yes. Yes I was. But at least I didn't have sore jugs and two black eyes, and I consider that a victory, even if nobody else does. 

Song is Lonnie Mack - The Bounce

Genetics

This week is PWSA UK Awareness Week. The Prader-Willi Syndrome Association UK want to spread the word about the rare chromosome disorder. They want more people to know about the syndrome, in an effort to allow children and adults with PWS to be understood and accepted, and to make more people across the country aware of what the condition means for PWS people and their families, friends, and carers.

Genetics

Genetics is complicated. There is no medical explanation, for example, for the existence of Katie Hopkins. We do, however, know a bit about the genetic causes of Prader-Willi Syndrome.

I am now, foolishly, going to try to explain it. I’m taking a big breath, and diving in. Any scientists reading, feel free to pull me out when I start to panic...

Most of our body’s cells contain 46 chromosomes (23 from our mother and 23 from our father). These tiny structures are packages of genes which contain the instructions our bodies need to grow, develop and function properly. (The ‘saying no to a glass of wine’, ‘ever wanting to vote Tory’  and ‘dancing without looking like a numpty’, packages in my DNA are missing, obviously).

Egg and sperm cells normally contain just 23 chromosomes, so that they join up in conception to provide the baby with the 46 needed. The joining up bit is where changes or errors in genes and chromosomes can occur. I am a big fan of the joining up; not so keen on the errors.

Many genetic disorders are caused by a change in a single gene and can be passed down from parent to child. But Prader-Willi, apart from some exceptional cases (see below), is not an inherited condition; it just happens. More than one gene is involved, but they are all near to eachother in an area of one particular chromosome: chromosome 15. Every case of PWS is due to the baby not receiving active genes from this particular area of the father’s copy of chromosome 15. The mother’s genes in this area on her copy are ‘turned off’ because of a rare process called ‘genomic imprinting’. (Anyone else think this sounds like a phrase for a typo on a Snow White And The Seven Dwarves poster? Oh, just me then).

There are at least three different chromosome errors that can keep the key PWS group of genes from working normally: 

1) Paternal deletion
About 70% of all cases of PWS are where - with no known explanation - the critical PWS section of chromosome 15 (or The Bastard Bit Of The Bastarding Bastard, as I like to call it) is missing. The section has disappeared, and there’s no rhyme or reason to it. (Although a rhyme would be pretty rubbish: ‘It’s a paternal deletion, our blinds are Venetian’. See?). The deletion is inexplicable, and there is a less than 1% chance of it happening in another pregnancy. This is the genetic reason for my daughter’s PWS.

2) Maternal Uniparental Disomy (UPD).
Yes, this sounds like a Peel Session. “A great live track from Maternal Uniparental Disomy there - three more from them later...”. UPD is a less common form of PWS (about 25% of cases are caused by it), and it is where the baby inherits both copies of chromosome Bastarding Bastard from the mother. Even though there are two complete copies of the chromosome, the key genes are imprinted - ‘turned off’ - resulting in PWS. This has the same effect as a deletion. Again, there is very little chance of this happening again in a further pregnancy (in fact, it never has).

3) Imprinting Defect 
In very rare cases (less than 5%), the PWS genes on the father’s chromosome are there, but do not work because his imprinting process is faulty. Chromosome 15 has a tiny little control centre (imagine the bridge of the Starship Enterprise), which is responsible for ‘switching on’ all its genes. When there is a tiny defect in the imprinting control centre, the PWS section may not be switched on correctly. (Imagine if Captain Kirk accidentally knocked out a power lead when having sex with a green alien woman while his shipmates were in the canteen. That sort of thing). An imprinting defect can appear suddenly, or can be present in the father’s chromosome he received from his mother. If the latter is the case, then there is a 50-50 chance that any child he has will receive the defective chromosome, because, crucially, the defect has now crossed to the father’s side. Further testing and genetic counselling are hugely important for families with a child whose PWS is caused in this way.

So, I hope I’ve made that all clear. References to Snow White and Star Trek may or may not have helped. I don’t think a quick burst of Heigh Ho! or a panic about the reoccuring shortage of dilithium crystals has anything to do with Prader-Willi. Although an urge to be beamed to another planet does occasionally cross my mind. 

Please note: most* (*all) of the facts on this page have been heavily influenced* (*stolen) from the Genetics Of Prader-Willi Syndrome section of the Prader-Willi Syndrome Association USA's website. To read the original, more detailed, and much more sensible article, click here.

Video is Mull Historical Society - Minister For Genetics And Insurance MP

Pirates

She had no idea what his name was. But she could see he was a pig. And a pirate. And that deserved a hug.

My daughter was feeling friendly yesterday, which was more than could be said for me, having attended a hen do the day before. A family trip to Gulliver’s Land - a theme park with numerous spinning rides - was not an ideal destination for a mildly-hungover, middle-aged woman, but I went with the flow. Queasily.

Pirate Pig was a cuddly highlight for my girl, as was the Pirate Ship, which made me turn a whiter shade of pale even when I was merely adopting the get out guise of Official Photographer.

The day was dizzying in more ways than one. A large part of the afternoon was spent painstakingly going through the reasons why the rides had notices on them saying they weren’t suitable for people with back conditions. And my daughter's circular conversation revolved more reliably than Gulliver’s Giant Teacups:

“Do the notices mean I can’t go on because of my back operation?” 
“Well, you have had a back operation because of your scoliosis, but what did Dr Gavin say? No rollercoasters or rides that were very jerky, but smooth ones are OK, aren’t they?” 
“But what about the notices?” 
“Well, it’s up to Mummy, because I know what Dr Gavin said, so you’re fine to go on this one.”
“So it’s OK?”
“Yes.”
“But what about the notices?”
“That’s so people don’t sue.”
“What’s sue?”
“Blame the park for hurting their back.”
“Will it hurt my back?”
“No.”
“But what about the notices?”

I managed to snap her out of it with the timely distraction of drink and snack time in Lilliput Land Castle (otherwise known as ‘the café’). I, for one, felt much better after a cuppa. Not so good after sitting in an unidentified sticky foodstuff on the café chair. And definitely not great after a go on the Flying Texan Boots. 

As for the Twist 'n' Joust, I don’t want to talk about it.

Video is Alela Diane - The Pirate's Gospel 

Matilda

"How perfectly composed this photo is," I thought.
Until I realised the back of the programme makes
it look like they went to see The Lion King.

It’s been a perfect day.

Apart from the start of the train journey, when I took off my daughter’s coat as she sat down, and she flapped her flappy hands a little too flappily, knocking my coffee cup over and sending it flying up in the air. I stretched my arms out to catch the now plummeting coffee missile, showing the reflexes of a cat. A dyspraxic one. I ended up juggling the java, with some of it going up my sleeve, and the rest splashing onto the trousers of the man sitting next to the coffee-bomber.

“I’m so sorry,” I spluttered. 

“It could be worse,” my daughter told him, nodding philosophically. 

We were on our way to London to see Matilda The Musical. Thankfully, there were no more dangerous incidents with hot liquid. We met up with Prader-Willi Syndrome Best Friend Forever (PWSBFF) and her mum (PWSBFFM...no, now that’s getting silly) for a lunchtime salad in a Pret A Manger (tuna nicoise, 174 calories, don’t add the dressing, that’s 231 more). 

She won't be smiling when I tell her she's paying that bloke's dry-cleaning bill.

Next we headed to the theatre, stopping off at Covent Garden on the way to see a woman street performer hang upside down by her feet from some wobbly scaffolding, juggling knives. As you do.

We ensconced ourselves into our spectacularly good seats armed with programmes, diet coke, and low calorie popcorn for the girls, plus ridiculously-expensive-but-bloody-lovely G & Ts for the mums. The tickets had been my daughter’s main Christmas present - secured at half price with a phone call asking for ‘access tickets’ for children with special needs. (WHY did no-one tell me about this before?). 

The show was fantastic. Imaginatively staged, with a brilliant cast, swinging high on composer Tim Minchin’s smart, inventive lyrics and catchy melodies. The gin was pretty good, too.

I alternated watching the stage with looking at my daughter and her pal: wide-eyed at the actors; gasping as Miss Trunchbull swung a girl round by her pigtails (that took some explaining about stage trickery afterwards); and giggling with glee as Mr Wormwood’s hat was superglued to his head.

An aftershow Mexican feast at Wahacas was shared out in suitably small portions for the Prader-Willi pair, with my spice-loving daughter adding the extra hot sauce to the already sizzling platters, and the adults partaking in some major mojito action (can you see a pattern emerging?)

And all too soon it was time to catch the train home, wave goodbye to our girly compadres, and head home to see what havoc had been wreaked by little brother and dad in our absence.

“Mum, that was the best Christmas present ever,” my girl beamed at me as we sat on the train, elbowing me in the ribs every now and again to check whether I was still awake.

I beamed back. We’re looking into Charlie & The Chocolate Factory for her birthday.

When I Grow Up - Matilda The Musical

Even if you're little you can do a lot, you

Mustn't let a little thing like 'little' stop you.

If you sit around and let them get on top, you

Won’t change a thing…

Just because you find that life's not fair, it

Doesn't mean that you just have to grin and bear it.

If you always take it on the chin and wear it, nothing will change.

Just because I find myself in this story,

It doesn't mean that everything is written for me.

If I think the ending is fixed already,

I might as well be saying I think that it's OK,

And that's not right!

Video is Mexican Institute Of Sound - Alocatel. Because songs from musicals always sound and look naff out of context. Actually, a lot of them sound naff in context. I'd rather share something different with you. Yes, it also happens to be naff. What of it?

Frenzy

The phrase ‘feeding frenzy’ is an interesting one. It usually comes up in two contexts: the first is when the tabloid media goes bananas over the latest celebrity scandal; the second involves sharks or piranhas. Just imagine if there was scandal about a celebrity who got eaten by piranhas - there’d be a feeding frenzy over a feeding frenzy...

Our life has included long, difficult, feeding frenzies that have nothing to do with Sun reporters or Great Whites. Feeding frenzies in a Prader-Willi household are a whole different thing.

For some PWS parents, they can be literal. They’re when a child gives in to their uncontrollable hunger and sneaks downstairs in the middle of night, and eats and eats and eats. The discovery of which ends in a frenzy of bolted doors, locked fridges, rage and tears.

For our family, the frenzy part has been more about us, the parents, than it has been about our daughter, the hungry one, who as yet has not discovered the illicit joys of night-time foraging.* (*Dear God, I mis-typed that and it came up with ‘the illicit joys of night-time Farage-ing’, which is possibly the most disturbing thing I’ve ever written).  

From the moment your child is diagnosed with a disorder that is so wrapped up in the issue of hunger and diet and overeating and satiety, you become frenzied about feeding. It’s fear, a fear that you will provide the wrong food, that you’ll fail to foresee temptations, that you’ll give them too much food, or too little, you’ll serve up things that are too fattening, or not fattening enough, that they’re missing out because you’re too strict, or that you’re making it tougher because you’re too soft.

You’re surrounded by it, enveloped by it, obsessed by it. And all the while you know that however much thoughts of food dominate your life (for eminently practical reasons), you’re a mere amateur compared to your PWS child. They really take the biscuit, if you’ll pardon the expression.

Your feeding frenzy isn’t constant, though. Just like the Government escalates and de-escalates terror threat levels from ‘substantial’ to ‘severe’ and back down to ‘chillax’* (*possibly), you learn to live with the idea that you have to supervise your child’s diet carefully and continually. You become so used to it, it becomes second nature, and doesn’t sit at the forefront of your mind all the blummin’ time. 

We’re lucky: our daughter, at the moment, despite being 4 foot 10 and half inches of stubborn, pretty much accepts the food rules. 

We appreciate this isn’t the same for everyone, and we know our frenzy levels may need to be raised to ‘ohmygodohmygodsweetmarymotherofjesusonaunicycle’ at any point.

But frenzies aren’t good for you, believe me. Whatever your circumstances, if there’s any way you can de-frenz, try your best. Food is a big issue. A huge, calorific, mountain of an issue. But it is only part of your life. And like all parts of your life, you will get it wrong sometimes, and other people will get it wrong sometimes, and do you know what? That’s OK. You’ll get by, with a little help, and less frenz.

Video is The Fall - Victoria (from the album The Frenz Experiment, you see). Thought I was going for The Beatles or Joe Cocker, dint'cha?

This post first appeared in the October edition of PWS News, the newsletter of the Prader-Willi Syndrome Association UK. They have a very informative website here.

Three

The girls are tucked up in their beds, in a bouncy sea of airbeds, mattresses, and duvet sets featuring every known shade of pink.  Three teddy bear sleeping masks, the cause of much giggling when the girls tried them on earlier, are hanging up on the bedpost.

My daughter was 16 today. 

I swear there’s a sizzle in the air, from some of the moments burned into my memory today, like the ranch brand on the rump of a prize steer.

My daughter Skyping her cousin in Australia this morning, wringing her hands with excitement and pride that she was using her very own tablet computer.

Her first two emails on the device:

Hello Eve I got a tablet bit like a I pad I can email you more now. I can’t wait to see you later 

Hello manna [sic] it my birthday day eve and bethany coming later for going out for curry hut and film night and sleepover

Her arm, encased up the elbow in bangles, bracelets, and friendship bands, courtesy of her friend’s mum’s all you can grab trolley-dash raid in Claire’s Accessories.

My mum’s amazing Treasure Island birthday cake, complete with treasure chests made out of Milky Ways, and luminous blue jelly for the sea. And her low fat no-sugar fruit cake alternative for my girl. And the hours of work that went into them.

One Direction board game dares, including barking, moonwalking, robot dancing, roly polys, opera singing, and chicken impressions.

My girl, her PWS BFF (Prader-Willi Syndrome Best Friend Forever) and her AFC BB (After School Club Best Buddy) nattering on at the Indian restaurant; my daughter and her pal with the same unusual syndrome, her other mate with learning difficulties; all three of them together for the first time, talking to eachother, talking over eachother, all smiles.

Watching The Witches film, and being hula-hooped to a dizzying state by the circular conversations of: how witches aren’t real despite the characters insisting that they are; how if mice get squashed in a film, they’ve not really been squashed; and how Rowan Atkinson is an actor and is not actually Mr Bean.

The three amigas, jim-jammed up, squashed into the bathroom, cleaning their teeth. 

Oh yeah, and the guilt of thinking my seemingly malingering son’s ‘tummy-ache’ was rooted in jealousy. Said guilt kicked in like a mule at 3pm, when he was sick four times and had to retire to bed for the rest of the day.

I wouldn’t have changed a momentous moment. Well, apart from the sick bit.

Song is The Charlatans - Opportunity Three

Flotilla

I  wrapped my arms around her and squeezed, gently.  A little wave of emotion had drenched her, and she was sobbing, into my chest. To be fair, my chest is large enough to comfortably accommodate several sobbers at once. Anyone’s welcome, I don’t discriminate. 

But I wasn’t concerned. My daughter needed just a minute or two for the wave to subside, for the tide to go out, and for her feelings to dry off. Her emotions had overwhelmed her. A fact that was hardly surprising, considering the weekend she’d had: two nights in a hotel, two days of activities with other PWS children and young adults, two PWS picnic dinners, two evening meals out, a bagful of cuddly toys won on the pub’s ‘grabber' machine, oodles of crafts, a game of bingo, two lots of dance workshops, and an entire weekend buddying up with her Prader-Willi Syndrome Best Friend Forever.

It did subside, quickly, and easily. She was right as rain by the time we drove out of the conference centre gates. Three hours later, after a tactical Maccy D tea stop (grilled chicken salad wrap - 330 calories, and pineapple stick - 37 calories), we were home. Low-fat hot choc slurped, tablet swallowed, teeth cleaned, retainers in, jim jams on, bed.
___

The packed weekend was the PWSA UK's first national conference for seven years. The event, in Derby, was spread out over two days, concentrating on under 16s yesterday and focusing on over 16s today. The Association subsidised conference fees to try to make it more affordable for parents and carers, although as a charity, its funds are limited. 

I really hope they make it a regular event.

It can be daunting, especially for new parents, to hear some of the tougher topics and scarier stories from professionals, carers, and other mums and dads, even when it's emphasised that symptoms and behaviours can vary wildly from child to child. It can be repetitive, when speakers tell you things you’re already very familiar with. Not every talk turns out to address the issues you were hoping they’d cover. At certain points, you might even begin to understand what a PWS emotional overload might feel like.

But there is always something that resonates. There’s always something that surprises. I found Medical And Dietary Issues advice from a team from a PWS multi-disciplinary clinic (God, how I wish there were more of those) to give me plenty of *ahem* 'food for thought'. PWS BFF’s mum said the Changes to SEN Education And The Law session was hugely useful. I’m sure everyone there took away something; nuggets of information, little treasures, lightbulb moments, ideas, practices, realisations, recognition, revelations. 

And there's always something funny: the mum who went into the adjacent bible conference by mistake, and dashed out pretty sharpish when they started talking about Psalm 24 instead of Chromosome 15; our theory that said bible class (99% female) looked remarkably like the evil WI convention from the film of Roald Dahl's The Witches; and the idea that the catering staff, decked out in old-fashioned black and white uniforms, were actually dinner ladies from the 1950s transported through time to serve us up our gravy. 
___

As the day came to an end, a few parents and other Prader-Willi teenagers and young adults, walking past us on their way out, noticed my daughter finishing up with her aforementioned 'it'salltoomuchforme' weeping session. One lad picked up her Froggy soft toy, and patted her back. A couple of people caught my eye and smiled back at me when I smiled at them, as my daughter’s tears wet my shirt, and her face continued to crumple and leak. They didn't think me callous for seemingly not looking overly concerned at my distraught child. I knew that they knew that it would pass and be forgotten about as quickly as it had started. They got it. They got her. They got us.

I know I always bang on about it, but I’m more convinced of it with every PWSA event that I attend: being there, being together, seeing so many people sailing the same ocean on the same wonky, wobbly raft as you, is special. Some of them will encounter choppier waters, some calmer. But knowing they’re there, bobbing along beside you, makes a huge difference. We can set off flare guns and chuck each other life-belts if needed. 

Knowledge arms us. We’re a goddamn flotilla.

Song is The Waterboys - Strange Boat

Conference

I am anticipating a good night's sleep. It's my second night at the Alfreton Travelodge, a hotel that thankfully has rooms far nicer than the desolate car park environs, which look the kind of litter-strewn wastelands regularly invaded by hoards of zombies in The Walking Dead. (Put it this way, if Egg from This Life had turned up with a trooper's hat on and told me to bash any approaching people in the head with a blunt instrument, I would not have been fazed).

My daughter is fast asleep next to me. Our girly road trip to the Prader-Willi Syndrome Association UK National Conference is proving as enjoyable and exhausting as I envisaged. 

Today was the first day of the conference, which consisted of a whirlwind of quick chats with old friends and new faces, a small amount of cooing over apple-cheeked babies, and a portion of teary wonder at the sight of an amazing PWS girl talking about her job working with a disability charity. In between were workshops and lectures on research, behaviour, medical and dietary issues, feeelings of guilt along the lines of: "Oh God, I should be doing THIS!", alleviated by feelings of relief along the lines of: "Actually, it's perfectly OK that we're doing THIS!" While all this was going on, my girl was shyly proud of herself for 'helping with the little ones' during the day's programme of events for PWS children. She and her Prader-Willi Syndrome Best Friend Forever (PWS BFF) were honorary volunteers for the day (which as far as I can gather consisted of joining in all the activities whilst wearing an association T-shirt, but hey, they were 'working', OK?).

Co-ordinating adjacent hotel rooms with PWS BFF and family proved to be a minor stroke of genius, as it allowed the two special-peas-in-a-pod teenagers to play Hello Kitty Yahtzee yesterday, and to watch Eastenders on the iPlayer tonight. And beam at eachother.

Two observations about Eastenders: 
1) The last time I watched it, about 25 years ago, there was some skinny, ginger girl shouting a lot. I found out tonight that the same, skinny ginger girl, now a woman, was still shouting. No sign of Mary the punk, though. 
2) Me and PWS BFF's mum, after drinking a couple of mugs full of Prosecco that had been nicely chilled in a Travelodge bathful of cold water, were pontificating about why the programme seems to fascinate our girls. I think I actually used the pretentious phrase: "It's a kind of a pantomime of social interaction." This amateur psychologists' babble balloon was swiftly burst by asking the girls directly why they liked it. One said: "Because of the shouting," and the other: "It's the swearing."

Tired, but happy, before retiring to her room for the night, PWS BFF checked 15 times with me that we would definitely be meeting up at 8am tomorrow in the Little Chef for porridge, just like we did today. My daughter then checked 16 times that my confirmations of the confirmation were confirmed.

Hopefully PWS BFF's dad won't lock her hotel key inside the room like he did this morning. And hopefully I won't be put at the front of a convoy of cars going to the restaurant for tea, blindly follow the sat nav instructions, and end up taking everyone needlessly up to the next motorway junction, only to drive practically all the way back to where we started, like I did this evening. That would be stupid.

Video is CW McCall - Convoy. I will pick a decent tune for the next post, I promise. First Duran Duran, and now this. What am I thinking?

Spacebats

I’m not saying I’ve won the summer holidays, but, well, I am, I am saying I’ve won the summer holidays. On the first day. 

Admittedly, I may be crowing a little early. After all, there are 40 days to go. Yes, I may possibly be counting.

So just how did I come to be temporarily wearing the Tour De Vacances’s Maillot Jaune?
It’s simple: it was down to a giant metal man, a teenage girl with an infectious giggle, and a woman who is making a sacrifice beyond comprehension.

The 16ft tall silver fellow was the star of a brilliant outdoor theatre show I took the kids to today: Graeae Theatre’s adaptation of Ted Hughes’ ‘The Iron Man’. We sat on our picnic rugs in the glorious sunshine in a lakeside park, and watched as with the aid of pedals, pulleys, wheelchair power, and imagination, the clanking, charming giant was brought to life.

[Graeae Theatre is an amazing company, by the way. Their aim is to provide a platform for the talents of deaf and disabled actors and musicians (which they did to brilliant effect with the London Paralympics opening ceremony, which was co-produced by Graeae’s artistic director and included a riotous and rousing version of Ian Dury’s Spasticus Autisticus from their theatre show Reasons To Be Cheerful). Graeae take their productions out into communities and perform great spectacles that just happen to make the idea of a lad in a wheelchair operating a giant robot, and a deaf chap getting a crowd to learn the sign for ‘Spacebat’, normal, entrancing, and fun. As a consequence of today’s entertainment, if just one of the kids watching in pyromaniac awe when some Real Fire was used in the production thinks twice in the future about patronising, teasing, or ignoring someone with a disability, then Graeae's work here was worth it. (Yes, of course, my boy was one of the pocket-sized flame-worshippers, and no, League Of Gentlemen fans, Graeae are absolutely NOTHING like Legs Akimbo)].

The teenage girl was my daughter’s PWSBFF (Prader-Willi Syndrome Best Friend Forever - come on, you should know this by now). I did what I usually do when they get together: watch them watching. There’s something amazing about these two teenagers; these unique, rare, unusual, idiosyncratic, original, out of the ordinary girls. Who despite being unique, rare, unusual, idiosyncratic, original, and out of the ordinary - or perhaps because of it - are like peas in pod. An odd pod. But our pod. 

“And what about the woman making the sacrifice?”, I hear you ask, or perhaps I don’t, because I mentioned that ages ago before I Led Zepped this post and rambled on.

Well, that’s PWSBFF’s mum, who took both girls home for a sleepover, and, more importantly, is taking them to see the film Pudsey tomorrow morning. You know, the one starring the dancing dog that won Britain’s Got Talent (no, me neither). All I know about it is that it is supposed to be truly, monumentally, mind-bogglingly awful. 

And I DON’T HAVE TO GO.

Like I said, I've won. Well, peaked, at least. 

______

Click on link for today's song: The Primevals - Hit The Peaks

Graeae's The Iron Man production is part of the International Festival Milton Keynes, which continues until Sunday. There are two more free performances on Friday July 25.

Graeae is a dangerous name, you know. Try and write it down: you'll be lost in A & E before you know it. Thangyouverymuch, try the veal, I'm here all week.

Polling

You’ve heard of Gallup Polls? Well, my daughter is slowly perfecting her own similar method of gathering data. Mind you, with her day-dreamy, meandering walk, it’s not so much a Gallup as a Lollop.

We’ve been attending a few PWS events lately, organised by parents, volunteers, or staff from the Prader-Willi Syndrome Association UK. And my girl’s Lollop Polls are stepping up a gear.

The questions at today’s sunny, sociable PWS picnic at Coram's Fields in Central London, weren’t just the usual “What’s your name?”, “Have you got Prader-Willi?”, “How old are you?” and the slightly random “How many teeth do you have?”. Oh no, a new, astonishingly scientifically accurate, belter of a question has been added to the repertoire...

[Before I repeat it for you, I should check if you’re familiar with the genetic doobriewotsits of PWS. Wrap your noggin around this explanation:

Three PWS girls, and Trevor (Professional Photobomber)

PWS is caused by a baby not receiving active paternal genes from a specific part of one chromosome (chromosome 15). Around 70% of all cases of PWS are caused by a deletion, which means a tiny part of chromosome 15 inherited from the father is missing. Around 25% of cases are caused by maternal uniparental disomy (UPD), which means that instead of taking one copy of chromosome 15 from their dad and one from their mum, the child has ended up with two maternal copies. The remaining 5% of cases are caused by an imprinting defect.

OK? So far, so clear? I’ll let you into a little secret, I wasn’t aware of the third cause, the imprinting defect one. (It sounds a bit like the daily battle I have with my Canon inkjet, to be honest). Consequently, my daughter hasn’t heard of this either, so it didn’t figure in today’s super duper question. If it’s OK with you, let’s not mention it to her. I’d feel a bit like the confused cardinal from the Spanish Inquisition: “The chief cause is deletion...and UPD...the TWO causes are deletion and UPD....and imprinting defect...the THREE causes are deletion, UPD, and imprinting defect....AMONGST the causes are...I’ll come in again shall I?” ]

Her question to PWS children today (or to their parents to answer on their behalf) was this: “Have you got the Prader-Willi where it’s a little bit missing from the chromosome like me, or have you got the rarer one where its a double one from your mummy?”

That’s a cracking query, isn’t it? (The results of the poll were pretty much in line with the statistics mentioned above, by the way). She’s been quizzing her dad about PWS and its causes lately, and to be fair to him, he sounds like he came up with a simplified summary that the bird from Nina And The Neurons would be proud of. Thinking about it, he does take a rather special ‘one for the dads’-type interest in Nina. Mr Drake, if you’re reading this, no, it would not be an anniversary treat if you buy me a lab coat, tell me to put on a Scottish accent, and inform me of a perplexing problem that can only be fixed by me using my sense of touch.* (*I realise this makes little or no sense to non CBeebies viewers, because you have a life).

Where was I? Oh yes, the picnic. So I was proud of my daughter today. Coping with train delays. Asking her questions. Shyly answering other people’s questions. Holding a lovely PWS baby boy, who we kidnapped and held cuddle hostage until he had to be prised from our arms (see photo, top right). 

And the good news is, we’ve got not one but two more PWS get-togethers this month: a picnic in a park in Manchester, which is nicely timed to coincide with a visit to relatives oop north, and a party in a Mormon church hall in Enfield where in order not to offend the Salt Lake City brethren we can’t drink tea or coffee because they think caffeine is the seed of the devil, or sumfink like that. I am, of course, planning to mainline Red Bull before I go.

Song is Black Heat - Questions And Conclusions

Click here for full details of the PWS family events in June

Subterfuge

This week is PWSA (UK) Awareness Week. The Prader-Willi Syndrome Association (UK) want to spread the word about the rare chromosome disorder. They want more people to know about the syndrome, in an effort to allow children and adults with PWS to be understood and accepted, and to make more people across the country aware of what the condition means for PWS people and their families. “Talk about PWS” is the strapline. 

Today the PWSA (UK) is talking about healthy living. Do parents have any tips about helping a PWS person eat healthily?

Well, in our case, it’s all to do with subterfuge.

Don't get me wrong, you have to be honest with a person with Prader-Willi. They have to know that they need to try their best to abide by some pretty strict rules when it comes to food, despite their constant hunger, or actually because of their constant hunger. My daughter knows she will become ill if she was allowed to eat whatever she wanted, because when it comes down to it, people with PWS want everything.

So whilst I am brutally honest with her about the whys and wherefores, I use a shitload of subterfuge when it comes to the hows. I employ deceit, tricks, manipulation, and all kinds of underhand shenanigans to help my daughter eat healthily. (Plus my magic handbag, containing emergency rations for those thankfully rare, but potentially explosive, times when circumstances mug you and suddenly find yourself not where you are supposed to be at an allotted mealtime). 

In summary, it pays to be sneaky mother.

Here are my Top Five Sneaky Tips for keeping a PWS person on the straight and narrow:

1) Don’t be like Alan Partridge at the Linton Travel Tavern’s All You Can Eat Buffet - downsize their plate

My daughter’s main meals are served up on a side plate. The portion is a good size and fills the small plate right to the edges. I’m convinced this makes her feel as if she is getting a bigger portion.* (*Note to self regarding bigger portions: perhaps I should get my husband to wear smaller underpants).

2) Strength in depth - have a substitute warming up

There have been occasions when my daughter has dropped a spoonful of rice onto the floor. There have also been occasions when my son has done a fusilli smash and grab raid from his sister's plate and nicked a single piece of pasta. My daughter calmly and methodically devours every last crumb of every meal. If she feels diddled out of just one flipping fusilli, then meltdown can ensue. Hold a spoonful back and have it ready on the bench, for when a replacement is needed quickly. 

3) Packaging is there to be tampered with

You know those little boxes of raisins? You can split the contents in half and stick one lot in an empty box. That way two ‘boxes’ of raisins have the same amount as one. Also, Easter egg packaging can be sliced open and the chocolate replaced with a plastic egg filled with no-sugar, low fat treats. (Bonus: the slicer gets to dispose of the chocolate egg in whatever way they see fit).

4) Embrace the word ‘instead’

If they can’t have a Magnum ice cream, they can have a mini-milk lolly instead. If they can’t have a Mars Bar, they can have a Jaffa cake instead. Yes, the important stuff is the healthy, low fat meals, but PWS people can still have treats - they just need to be planned and accounted for in your daily food routine, and you just need to learn what’s low calorie and low fat and can be given to your child instead. 

5) Maybe it’s not that bad if they don’t understand fractions very well

Especially if you’ve spent several years explaining that when you cut something in half, yes, there’s TWICE as much. 

I probably shouldn't have told you that last one. 

Video is Imelda May - Sneaky Freak