Reverie

We've just spent a splendid weekend with family up in Yorkshire.

The highlight for my daughter, of course, was today's Sunday roast, the sight and smell of which elicited a huge gasp of delight as it was served up. "That looks LOVELY!" she uttered, her eyes gleaming at the sight of the carved chicken. We should hire her out to chefs undergoing a crisis of confidence, because praise never sounded so heartfelt.

Her Sunday highlight followed her Saturday highlight, which was an Indian takeaway (heavy on the tikka, light on sauces and rice).  When she eats this treat, her eyes close, and she goes into a kind of religious reverie.

When someone has Prader-Willi Syndrome it's only natural that the focus is on the dangerous and upsetting aspects of their physical, all-encompassing hunger. Their body does not send them the signals that say they are full up, and once diagnosed life quickly becomes about control and limitation of food.

But sometimes it's good to step back and take a fresh look at mealtimes. My daughter is truly happy when she's tucking in to food. An uncomplicated, natural joy. I see it flooding through her, as she savours every mouthful. Putting everything else to one side, it's a wonderful sight. It always has been. Probably even more so when her face is painted with tiger stripes (see the picture above).

My husband, on the other hand, mint sauce drooling down his chin after too much red wine? Not so much.

While we were up north, we went to a fabulous gig by Geordie folk band The Unthanks, at Holmfirth Picturedrome. They did a wonderful version of the song in this video - Here's The Tender Coming. 

Clean

The food thing.

It's a huge issue. I mean, it still stops me in my tracks when I actually think about it, even after all these years of living with my daughter's Prader-Willi Syndrome.

She never feels full up. We have to be rigid and regimented about mealtimes and portions and punctuality, and she courageously sticks to the rules. But her brain just does not tell her that she's had enough. We have to do that for her.

But sometimes it's good to turn the issue on its head. There is a flip side: By God, she enjoys her food.

Watching my daughter eat is a sight to behold. She doesn't dive in. She gets herself steady and comfortable, grips her knife and fork, and starts the slow, methodical, demolition of her meal.

Every spoonful is savoured. Head down, her hair flopping over her face, she will leisurely hoover up every last pasta shell or grain of rice. She'll scrape the lining off the bottom of every yoghurt pot. She is the Founder, the Secretary and the goddamn President of the Clean Plate Club.

She'll try ANYTHING. Recent favourites are a spoonful of lime pickle alongside a rogan gosh. She'll have crab, cockles, and sardines. Brussels, cauliflower, beetroot, radishes, and spinach. Muesli. Flaming hot Thai green curry. Pickled onions. Salads, casseroles, tagines, stir-frys and gumbo. Separately, obviously. Although, technically, I suppose, she would quite happily eat all of these together...

She's a chef's dream.

The toddler is another story.

Video is Dr John - Let The Good Times Roll (from the album Dr John's Gumbo)

Picture

I love this picture. I absolutely love it.

It's a group photo from a chaotic, funny, fabulous, family holiday spent with my parents and my two brothers and our children in a ramshackle old Norfolk farmhouse in 2005.

I love it because of its imperfections, not despite them.

I love the way most people aren’t looking at the camera. I love my niece Kate having a tantrum, and Aunty Angela trying to talk her down, while her dad tells her to get a grip, and her big sister Jess looks on in disdain. I love my Mum and me (far left) looking like little chubby peas in a pod. I love my Dad on the other end, completely oblivious to the mayhem going on behind him.

And I really love the sight of my girl, wriggling out of my grasp, because she didn’t want to line up. All she wanted to do was go and find some more peacock feathers to add to the few she's already collected and is clutching tightly.

The reason I love that so much is because she’s in the thick of it. Getting fed up of posing. Doing normal kid stuff.

When she was diagnosed with Prader-Willi Syndrome, I never dreamed this would be achieveable. I thought the issues with food would make extended big family gatherings impossible.

But we all spent a week living, snacking and eating together. And watched in amazement as our hungry little girl insisted on waiting way past her normal tea-time in order to eat with her cousins. 

It was such good fun, we did it again the next year. But because we’ve now got a bigger clan, different jobs, and most of the kids are now teenagers and twenty-somethings, logistics mean we probably won’t all go on holiday together again.

Maybe that’s the real reason why I love this picture so much. 

To everyone else, it might just look like a bit of a rubbish line-up. An out-take, even. For me, it bottles a bit of the magic from a magical time that's now out of reach.

Video is McFly - All About You (one of my daughter's favourites from this year.)

List

I stumbled across this list yesterday*. It was created by a young man with Prader-Willi Syndrome who had a New Year’s Wish ‘for people to know what it feels like to have PWS’.

When asked how he could get his wish, he said: “People could do some of the things that I feel every day, just for one day and maybe they would understand.”

It's a real eye-opener.

Here’s his list. If you like - and in the spirit of New Year's Resolutions -  you could pick one or two of the challenges to undertake. Just for a day. See how you cope. And then think about living your life like this. Every day.

1. In the summer, turn up the heat and wear extra clothing or in the winter, wear very little clothing and turn the heat down.

2. Only eat ½ of your meal, breakfast , lunch or dinner and leave the table hungry.

3. Go to an office or school party with all the extra food and goodies before eating lunch and DO NOT EAT ANYTHING! 

4. Put cookies, cupcakes, or other goodies that you like on the counter and be sure to walk by them every time you go in the room but you can NOT eat any!

5. When you are really hungry watch someone else eat something that you LOVE, knowing you cannot have it.

6. Do something out of the ordinary or different in public and look around to see someone stare, laugh or tease you because you are different. 

7. Lock your cabinets or refrigerators and give someone else the key. Tell them to only let you eat when it is meal time or time for a snack. DO NOT eat in between meals. 

8. Restrict your calories to only 1100 calories per day including all meals and snacks. Count all calories including drinks. ONLY eat 1100 calories!

9. Drink only sugar free, diet drinks or water do not drink liquids that contain calories.

10. Never go into the kitchen, pantry or cabinets unsupervised. Only enter the kitchen or dining room if someone else is with you and can be watching what you are doing.

Video is Eels - Guest List

*The list came from an entry by Janis Tull Williams on the Prader-Willi Problem Solving And Solutions Exchange page on Facebook

Genius

We finished our tea last night and, as usual, my daughter had eaten every last scrap.

When your child has Prader-Willi Syndrome, their insatiable appetite means you don’t have to worry about anything being left on the plate. They will scoff the lot. Without exception. Even Brussels Sprouts, the weirdos.

She always has three courses for her lunch and dinner: main, low-fat pudding, and fruit. The Holy Trinity.

So she had polished off the lot. She clonked down her knife and fork. She rubbed her finger up and down on each side of her mouth in turn. She pushed her glasses up from the end of her nose, and flicked back a stray strand of fringe.

She looked me straight in the eye. And declared:

“I’m a bloomin’ genius."
 

Video is Ian McNabb - I'm A Genius

Panic

My daughter’s face was stricken.

I could see the panic starting in her toes and rising up through her body. I needed to stop it before it reached her head, at which time there would be NO REASONING WITH HER.

“It’s OK, we can sort it out. It’s not a problem. Everything will be fine. Don’t worry. Listen to what I’m saying: it’s all fine. It’s all right,” I insisted, trying to keep my voice firm, and get her to actually listen to what I was saying.

So what had happened to put her in a tailspin?

The Asda delivery truck had been. We were on a tight timescale to have Sunday roast ready in time, and they’d missed one vital item from our order: the chicken.

You need to understand how DISASTROUS this is. It’s why I don’t often order groceries online. Meals have to be precision-planned and to happen at set times in our household - it’s part of the regime needed to keep our hungry daughter happy and calm. There is no margin for error.

“Listen, listen. I ordered chicken breasts too, and they've brought them, so we can have those instead of the roast chicken,” I told my worried girl.

I had to repeat this four or five times before she accepted it. But she wasn’t happy. The menu and method had changed. Food that had been promised had not been delivered. 

And so, despite tucking in to a good, hearty meal, with breast fillets instead of roast chicken, (but piled up, as usual, with plenty of veg), we had the full scale “I’m so hungry!” routine in the evening. It doesn’t happen too often. But the minor mishap had lodged in her brain and food was all she could think of.

So, I’d just like to send a message to the very polite and friendly delivery driver, who whistled a jaunty little tune as he sauntered back to his lorry.

You’re an utter b**stard.

Video is Alan Partridge - Knowing Me, Knowing Yule clip. It's not actually a chicken, but it's how I felt.

Video is The Smiths - Panic

Toast

It’s the simple things that make it all right. The little things other people probably don’t think twice about.

Like our breakfast routine.

Having a daughter with Prader-Willi Syndrome, who’s always physically hungry but has to be on a strict diet, means you have to keep a close eye on her in the kitchen.

But being obsessed with food means she wants to help with the cooking and preparing of meals. So I've started letting her make her own breakfast.

This doesn’t sound amazing, but it is, it really is.

I open the cupboard and lift down the cereal boxes and line them up in a row. My daughter gets her bowl, a spoon and the milk. And makes herself a ‘mix-up’, which is her name for having a little bit of everything. 

I keep out of the way, until she presents me with her bowl for inspection. “Is that too much, Mum?” she asks, anxiously, worrying I’ll take some away. I’ve never had to, so far. She has a knack for judging just the right amount.

Then it’s time for toast. And, incredibly, she’ll stick a slice on for herself, and one for her brother. She’ll spread some marmite on hers, and some chocolate spread on his. And so far, she’s not even sneaked in a crafty lick of the knife. Her desperate need to feel grown-up and independent is so important to her she chooses not to give in to her hunger. Although she knows I'm watching, so I suppose it's like forcing yourself not to kick the centre forward right in front of the ref.

She’s very nearly a teenager. I don’t know how many other mums feel quite so happy at the sight of their awkward, spotty offspring sticking some bread in a toaster. Mostly I think they want to swear at them and their mates for making yet another loaf disappear. 

I bloody love it.

Video is Morecambe & Wise's Breakfast Sketch

Video is Streetband - Toast. This really is a terrible, terrible, song. But it's about toast. And features an unfeasibly young-looking Paul Young, bless him.

Forget

I sometimes forget my daughter has it.

I sometimes snap at her when she asks me for the 10th time when tea is ready.

I sometimes fail to remember that she’s not just being impatient. She’s not just tired or bored (although if she is either of these things then the intensity of her chef-bothering increases).

Having Prader-Willi Syndrome means my daughter is hungry. Physically hungry. All the time.

I could tell you about the science, and the tiny missing stripe* on one tiny chromosome that causes this. (*Stripe may not be the accepted medical terminology here).

But all you need to know is that the little switch in my daughter’s brain that should click on to tell her she’s full up doesn’t work. 

(Incidentally, the same little switch in my brain seems to develop some kind of fault within 20 feet of a cake. It must be an as yet undiagnosed syndrome. If any scientists are out there, I’m perfectly willing to help with research. As long as I get to eat lots of cakes).

She really can’t help it. She’s not being greedy. And amazingly, for the most part, she doesn’t plague me all the time for food. When her days are structured, and she has her set meal and snack times (a rigid school timetable helps here), she probably begs for grub less often than my toddler, who I believe is starting a new world religion which involves worshipping at the altar of the biscuit tin and praying to a God made entirely of Custard Creams.

So although sometimes I do forget, I try really hard not to. I attempt - not always successfully - to keep a lid on my irritation if she does get stuck in a food loop.

Because when I imagine what it must feel like to always be hungry, my heart aches for her.

Video is Bob Marley - Them Belly Full

Bananas

When my daughter was about three years old, a friend of mine invited me on her hen night.

This involved a group of us going to the local theatre to a performance of Sing-Along-A-Sound Of Music. Before you say it, I know it’s not exactly the height of sophistication, but compared to my hen weekend at Butlins in Bognor it was high class culture, believe me.

As part of the prescribed fun, we had to dress up as characters from the film. 

Not for me, the easy option of a generic nun. Oh no. I had to be different. I decided to go as ‘The Hills’. If I’m honest this was purely for cheap gag purposes. It meant if anyone asked me what I was, I could reply: “I’m alive, with the sound of music.”

But where to get the materials needed for my makeshift costume? Easy, I thought. I’ll pop into the greengrocers, and they’ll give me a spare bit of that plastic astroturf they use to display the fruit boxes on.

However, when I wandered in with my daughter (who I was still pushing everywhere in an oversized buggy), the fella behind the counter said: “Sorry, luv, no can do. It’s expensive stuff. What do you want it for anyway?”

It was at this point, I lied. Not wanting to wheel my girl round a fancy dress shop at the last minute, and thinking on my feet, I looked downcast.

“Oh, don't worry. It’s just my daughter. She’s disabled. She just loves this plastic turf. It’s very tactile and she loves to feel it. But it doesn’t matter. Never mind.”

The greengrocer looked slightly guilty at his earlier refusal, and took a small piece out from on the counter, placing it on my daughter’s lap, looking expectantly at her face. Of course, as her astroturf adoration was entirely fictional, she wasn’t remotely interested.

So I leaned forward and whispered in her ear: “Bananas!”

Instantly, she grinned from ear to ear, and the lovely man behind the counter ran, literally ran, to the back of the shop and returned with armfuls of the stuff for me. (Enough to transform me into The Hills, I noted, with satisfaction).

It was wrong of me. I know that. I invoked her disability, played on a stranger’s pity, and then used the hunger caused by her Prader-Willi Syndrome for my own selfish ends.

But I’m not a complete bitch. I did buy her a banana.

Video is The Dickies - Banana Splits

Bones

Question: How does someone know that they shouldn’t eat the bone on a chicken drumstick, if they’ve never had a chicken drumstick before?

Answer: They don’t.

If cooking chicken for my daughter, in accordance with my husband’s motto in life - "Always go for the breast" -  I’ll pick healthier, skinless cuts.

But there was this one occasion, running late, frazzled, and faced with a poorly-stocked Co-op shelf, when I grabbed a pack of chicken drumsticks.

Having cooked them, I plonked one down in front of my girl, along with some new potatoes and a mountain of salad, and then proceeded to knock over a glass of orange juice all over the table, chair, floor, radiator and wall. My attention was elsewhere for a good few minutes.

When I looked back, my daughter’s plate was clean, apart from a few splinters of bone.

Five hours later, the A & E doctor finally saw us, felt her throat and belly, and announced that it must have been a reasonably soft bone, and my daughter must have “munched the bejaysus out of it”, because there were no bits stuck anywhere they shouldn’t have been.

Prader-Willi Syndrome means you’re constantly, overwhelmingly hungry. And when someone is controlling your portion sizes, you’re damn well going to eat every last bit.

So she did.

Video is The Bees - Chicken Payback

Video is Elbow - The Bones Of You. 

Gillian

I really should get a T-shirt.

When I went to the supermarket when my daughter was little, I couldn’t take her with me. Rows and rows of food lined up, and her being hungry all the time. Not a good mix, really.

Oddly enough, as she’s got older, it’s not so bad. Although food is still her all-consuming passion, now she can tell me what she wants, so there's less screaming and more choosing. 

She’ll grab something off the shelf, pore over the label, and hold it up, shouting triumphantly, “I can have this, it’s only 65 calories, Mummy!”

You can just imagine the amount of evil looks this kind of phrase elicits from passing shoppers. I call it ‘the Gillian McKeith’. Their eyes narrow, giving chubby mum a disdainful look from head to toe. 

I know what they’re thinking. “That awful mother has got her poor little girl on a diet!” Then they probably even want to look at my poo, to see what rubbish I’ve been eating.

So I really should get a T-shirt.

Maybe this:

Nah. This is better:

Perhaps Bo Diddley put it more politely:

Video is Bo Diddley - You Can't Judge A Book By Looking At The Cover

Grandad

“She’ll be OK. I don’t think she’s got it that badly.”

My dad displayed all of his years of medical knowledge trying to convince me, and himself, that his granddaughter’s life wasn’t really going to be affected by Prader-Willi Syndrome.  When I say medical knowledge, I actually mean none. This is a man who has to be at death's door to drag himself to the doctors. If he chopped his arm off in a threshing accident, he’d probably try to use superglue and string to fix it.

But he had to learn that when someone is obsessed with food and never physically feels full up, you can’t do the following:

• Wander to the kitchen cupboards every now and again, and saunter around the house grazing on liquorice sticks.
• Mention the words breakfast, dinner, tea or snack when it isn’t breakfast, dinner, tea or snack-time.
• Share a piece of your chocolate bar, using the phrase “Oh, go on, a little bit won’t hurt.”

What you can do is this:

• Keep a few bits of pasta on your plate, ready to replace any your granddaughter drops on the floor, thus avoiding a Big Drama.
• Have a box of raisins in your pocket, to dish out as an alternative in case you’re out somewhere and sweets or cake are offered by the well-meaning uninformed.
• Allow your granddaughter to take great delight in plucking your chest hairs, however painful (see picture, top of page).

It took a while for him to accept and understand that where PWS is concerned everyone has to change their behaviour to make life easier. But he did, and I love him for it.

Happy Father’s Day, Dad.

Video is Ian Dury & The Blockheads - My Old Man