Sunday, 31 January 2016


I’m in training. Not for a marathon - I did a half marathon years ago and cemented my belief that there is absolutely no need to run a step further in a world where public transport is available.

No, the regime I’ve adopted is mental, not physical. And my brain-training doesn’t involve forward motion; it’s about deliberately, consciously, taking a step back.

It’s in response to a talk given by my daughter's teacher, Mrs D. My girl, who is now 17, is at a special school satellite class at a mainstream school. She’s adapted well to some of the changes that come with being a sixth-former, after initially finding the timetable tiring. 

“We’re usually up on our feet, walking to the town to go shopping, working on the farm, or catching the bus to college or work experience,” Mrs D explained. “And what we’re really working hard on is independence.”

Now, independence is a frightening word. We all know that people with PWS have hugely varying capabilities, but that supervision around food is almost always required. So the idea of independence always comes with alarm bells and caveats and provisos. But Mrs D’s talk got me thinking - hard - about how we as parents can be too protective. She urged us not to do things for our children, but instead to help them gain the skills, where possible, to do things for themselves.

“I know it’s quicker and usually easier and tidier for you to do it,” she said. “But try to think about what they could do that you automatically do for them, unthinkingly, and perhaps unnecessarily. And then - change your habits!”

So I am. I’m trying. (Very trying, according to my husband). I’m involving my daughter more in doing things around the house. I’m involving her more in choosing, buying and preparing food (I know this isn’t an option for many PWS people). I’m even working on a plan which will allow her to walk a short distance to school on her own *breaks out into a sweat, forgets to breathe, sits down until dizziness subsides*. It’s hard to overcome my instincts, they’ve become so entrenched over the years. But I think it’ll be worth it. And if it means we start getting Kevin The Teenager-style “It’s so unfair!” complaints when she moans about having to tidy her bedroom, then so be it. 

Actually, her bedroom is way tidier than mine. That’s one part of the independence regime that I’m going to excel at - as soon as Mrs D said: “I know we all like our houses to be perfect and tidy, but you should try to let them help more and let go of perfection,” I had to stifle a guffaw. Me and perfection let go of eachother a very long time ago. If I’m honest, we were never even nodding acquaintances. 

So, this is good. Seeing my girl do things for herself that I never imagined she’d be able to is heart-stoppingly, head-swimmingly amazing. It makes me proud in the best sense - not preening, but smiling. A warm-like-a-hug-style pride. 

And, as an added bonus, I can pretend that working on improving my daughter’s independence is all about her, when in fact there is one big advantage for me: I now have a great excuse for the state of my house.

Song is The Tears - Imperfection

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

Friday, 29 January 2016


I’ve never worn ribbons in my hair, or displayed a penchant for gingham dresses. I also swear like a trouper, so you may be surprised when I say that I consider myself to be a bit of a Pollyanna. But that thing the eponymous heroine of the Pollyanna stories has - about seeing the good side of a situation - I’ve got that. It’s part of my nature to scramble to find something funny in even the worst of times, and it’s my bleedin’ superpower, I tell you.

All superpowers falter, though. Kryptonite weakened Superman; becoming a floppy-fringed, disco-dancing Emo boy turned Spiderman into an idiot (Sam Raimi, what were you thinking?); and this week there has been a concerted attack on my Superpollyannaness.

It’s not been one thing. It wasn’t an alien mineral-powered laser cannon expertly aimed at me by my nemesis. It’s cumulative crap.

It’s the fourth working day of my husband’s four on, four off shifts (a time when the end of my tether is a familiar sight).

My boy has not only been testing the boundaries, he’s been climbing up the boundary walls, jumping up and down on them whilst moonying, and then leaping so far over the boundaries I need a pair of binoculars to spot him. (The moonying is a metaphor, although he does have real world form in this area).

My girl’s anxieties have been at full frazzle setting: the doctors still haven’t had their meeting to decide whether her back op will go ahead; a reading comprehension test at school has set off a million questions; and to top it all, she’s perfected a brand new addition to the list of things she repeats over and over and over and over again. (I’ll tell you about that one another day, when I get my mojo back).

Finally, marvellously, after 30 odd years of pretty much pain-free menstruating (*sounds ‘wimmins things’ klaxon*), I seem to have suddenly developed awful lower-back ache and atrocious stomach cramps to go with the whole bleedin’ bleeding kerfuffle. The wince-inducing, teeth-gritting, doubled-up in pain-causing, impossible to sleep kind of stomach cramps.

Bah!, grrr! and, indeed, ow! 

*retires to bed with a hot water bottle*

Video is The Cramps - Bikini Girls With Machine Guns

Saturday, 16 January 2016


"Well, I was playing the clarinet, the phone rang, I jumped, and..."
My daughter's interesting interior.
I have a new favourite person.

It doesn’t take much for someone to get into my good books. Cake and alcohol usually works, although a new abstemious regime for 2016 means other ways are now needed to win my heart.

My new love is one of the back specialists at the Royal Orthopaedic Hospital, in Stanmore, and he wooed me with his words. Or more specifically, the words he used when he talked to my daughter, after she walked into her consultant appointment yesterday to discover he was a different doctor to the one she was expecting, and began to gently lose the plot.

This chap immediately ignored me, and talked directly to my girl, explaining how he was on the same team as her usual doctor, who he’d be working very closely with. Over the course of several minutes, in a calm, simple, and non-patronising manner - and without any prompting from me - he talked her down.

One of the offenders (left hand bolt)
He then explained to us both and showed us on the computer screen exactly what was going on with her back, answered my questions, answered her slightly more random questions, soothed her anxiety a few more times, and most importantly gave us both his full attention, respect and time.

For the first time I could properly see and understand how some bolts anchoring the titanium rods that helped her spine fuse in a straightened position eight years ago are pressing against the ‘membrane’ which protects the spinal cord. That’s what’s causing pain, and that’s why they need to be removed.

As I suspected (gggrrrrr!), the results of the SPECT scan (carried out a a different hospital a few days before) weren’t yet in, but my new best friend told us that he’d be surprised if they showed anything that could be behind my daughter’s discomfort.

“So, I'm going to tell you what will happen now,” he said, again addressing my girl, who returned his gaze solemnly. 

He broke his explanation down into short sentences, pausing between each to check if she'd taken things in:

“I think before very long we will be sorting out this operation for you."
"I know you were supposed to have it before and our plans changed, so I'm sorry about that."
"I cannot say yet that you will DEFINITELY have the op, which I know you would like me to."
"But don’t worry - your mum will be able to call us when the scans come in."
"And after we’ve had a special meeting all about you, we can tell you what will happen."
"We’ll do that as soon as we can."
“And you need to know that your spine has fused so well that you will stay lovely and straight if we take the metal out.”

And then the killer finish.

“You don’t need to worry. I know it’s hard, but will you try?”

She gave her biggest, most forceful nod.

It wasn't the black and white decision she needed. But it was a damn fine interim explanation.

We both shook his hand as we left. I was this close to kissing him. 

Song is Frankie Beverly & The Butlers - She Tried To Kiss Me

Monday, 11 January 2016


Example photo of a SPECT scan
Today was just the average kind of Monday. You know, one of those where you catch a train to take your kid down to London to have radioactive dye injected into her. That sort.

Yes, today was the much-anticipated SPECT scan. (I know it sounds like a truncated version of the latest James Bond film - even more so when the section of the hospital it’s taking place in is called ‘nuclear medicine’) but SPECT stands for Single-Photon Emission Computed Tomography. 

It’s a very particular and specialist kind of scan (yeah, I know, you can tell I’m not totally up on the science), and she was having it to try to establish the cause of pain she’s been having in her back. Just before Christmas, doctors were due to operate on my daughter’s spine and remove her titanium rods (inserted during a spinal fusion operation seven years ago), because they believed the metal was pressing on her nerves. But right at the last minute, they changed their minds and decided she should have this scan before any decisions about surgery should take place. This was the genesis of today’s strange adventure. 

At midday, we reported to University College Hospital, London, for her to have the dye injected. The syringe came in a futuristic-looking pod, presumably designed not to break and spill dangerous Hulk-creating gamma rays if dropped. I expected it to be green, or at least to glow, but the dye was disappointingly indistinguishable from the water they injected with it to flush it into her bloodstream.

My daughter, of course, was blasĂ© about the needle. What was causing the persistent, overriding worry today (and there is usually a persistent, overriding worry), was the instruction to report back to the hospital three hours later for the actual scan, having drunk ‘around two pints’ of fluids in the meantime. 

I bunged her a couple of tomato juices and a cup of tea along with her less than 500 calories ‘leggero’ pizza lunch at Pizza Express, then insisted she take a sip of a drink every time she played a card in our after-dinner game of Top Trumps at a cafĂ© near the hospital. All the while she was fretting: concerned she’d not drunk enough, and then anxious about having drunk too much. It didn’t help that she had no clue whatsoever about how big a pint was, despite my repeated attempts to impart my expert knowledge about the quantity of this particular measurement of liquid. At least she was allowed to go to the loo, because if bladder control had been added to the equation, we both might have had some sort of breakdown.

So it was that we returned to the hospital, lightly frazzled, and ready for the fray.

The chap who dealt with her was lovely. He talked her through what was going to happen, and helped get her comfortable, lying her down on a flat stretcher-like bed in front of what I can only inadequately describe as a ‘giant photocopier in the shape of a sideways claw thing’.

And here was where she came into her own. She had her feet tied together, a strap put across her stomach to hold her arms against her side, and she had to lie still for 17 minutes, whilst the camera slowly scanned her up and down, just millimetres from her face. Then after a quick readjustment of her position and the machine’s ‘camera claw’, she had to do the same again.

My girl, my little bundle of anxieties, completed the task perfectly. She didn’t move a muscle for each 17-minute pass. I praised her all the way home on the train. This was in amongst a word for word, worry for worry repeat of the drink shenanigans (she'd been told to imbibe another two pints after the scan, which meant that there were at least 20 people in our train carriage treated to a practical demonstration of just how many times a teenager with Prader-Willi Syndrome can repeat themselves in a 50-minute journey). 

On Friday we see her consultant at the Royal Orthopaedic Hospital in Stanmore. Whether he’ll actually have seen the scan from UCLH before then is another matter. I’m going be persistently pestering his secretary on the phone between now and then to try to convince them that this is a sensible idea. Wish me luck.

Had to pick a Bowie track today, after the sad news of his passing. I’ve gone for New Killer Star. (‘Nuclear’, you see).