Thursday, 22 May 2014


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 Adults. Does your son or daughter live at home or in supported living or residential care? How is it working out?

Today’s topic is the one. The one that gets me. If I had to plot a graph of my fear levels regarding PWS, it would be creeping up and up with every birthday my daughter celebrates.

When your Prader-Willi child is a baby, all floppy and helpless and ‘other’, you’re shocked and terrified. Your life has taken a wrong turn. It takes a while for you to realise that the wrong turn, unlike the ones in films, doesn’t lead you over the edge of a cliff or into cannibal-infested woods - it takes you pretty much where you wanted to go, just the long way round. 

So from initially stratospheric fear levels, my graph dropped to low grade background worrying once I got to know my daughter and her charm and grace made her one wonky chromosome seem less important.

There have been spikes along the way: I’ve never known real terror until those hours I sat waiting to find out if a 10 hour spinal fusion operation had been successful and safe; I’ve never felt as frightened and helpless as when she raged and swore in the middle of the night as her then undiagnosed mood disorder briefly changed her into an almost unrecognisable cuckoo in the nest.

But life, for the most part, has been lighter on the jitters and heavier on the jollies than I ever expected. 

And yet. And yet. Adulthood is looming. I’ve just read the stories shared by parents of PWS adults today, as part of the awareness week, and I’m crying. At the honesty, bravery, resilience, and often the sadness portrayed in their tales of the difficulties that their families have had to face, are facing, and are yet to face. Problems with food and weight, mental health, frustrations over jobs, routines, friends, a social life, with living. 

An adult with Prader-Willi can find living in residential care hugely difficult. They can also find supported living hugely difficult. Living at home can also be, you guessed it, hugely difficult. They are a person, with hopes, and dreams, and tumultuous emotions which they cannot control. They need structure, they need stimulation, they need a proper, satisfying, fulfilling shot at life. And it is so hard to find a place for them in the world, where they can be happy and healthy.  

My girl is only 15. I believe staying on at her school until she is 19 will work well for her. I believe this, but I don’t know it. After that, she may go to college, but finding the right course, the right supervision, the right help, won’t be easy. If it goes well, that will take her up to 22, and then after that? What then? Where then? How is it going to work?

I have to think about these things. The line on the fear graph is looking mighty steep, but I have to scale it. I’m going to need some grappling hooks, some thermal undercrackers, and, hell, maybe some Sherpas.

Video is Eels - The Sound Of Fear


  1. I too had the same reaction when reading the stories-in fact I wanted to throw my pesky phone out the window and bury my head in the sand-and considering my baby is only 1 I find this to be the only sensible option. We all just have to keep hoping that things will continue to improve and help can be accessed. By the by-any advice on wills for people with pws? Ours is a bog standard one but I feel this now needs amending.

    1. Feel a lot better about it all today. I went back and re-read the stories, and picked up on a positive one that I'd missed. Sometimes, the future rears its head and frightens us, but that's OK, because for the majority of the rest of the time we can just get on with living life with our rather damn splendid children.

      As for wills, we set up a trust for Josie, with my brothers down to administer it, as it would be better if someone else was in charge of the money, otherwise I suspect it might all be spent on cakes. It was a fairly simple thing to do, as far as I can remember. But we've got to update it, as her brother isn't on it. And I haven't actually purposely cut him out of the will, although this is obviously a possibility...

    2. Well indeed-only good behaviour earns a place in the will!