When Pudding was first diagnosed with Hunter Syndrome (MPS) at three years old his future was laid out before us.
He would continue to gain skills for a few years, albeit slowly because of his learning disability. Then his ability to learn would plateau, with everything staying stable for a while before the learning curve started to fall in the other direction. Skills and abilities would be gradually lost until his body couldn’t take it any more and he would pass away.
It was all so very unreal at first.
In that first summer, when the sun was shining and I was surrounded by people enjoying their normal lives it just didn’t seem possible that I would some day have to say goodbye to my gorgeous boy. Watching him pull every book off the bookshelf in turn just to look at one page then discard it, or chasing after him when he made yet another bid for freedom from the playground, it didn’t make sense. Even when I was holding him down for another needle, or getting the results from the latest tests on his heart or airways (tests that already showed deterioration that I was previously unaware of) I still half-expected someone somewhere to tell me there’d been a mistake. The blood test had been checked again and everything was fine: he didn’t have MPS after all. A pipe-dream, of course. That call never came.
I’m not sure I can identify when his skills started to plateau. I do remember the joy of any small win. Watching him jump for the first time, hearing a new word spoken, seeing him respond to a simple command – these were all causes for celebration. Yet how can you truly tell when the little gains stop? It’s not something you can easily quantify. All you can do is suddenly realise that it’s been a long time since any new skill has been ticked off on the inevitable questionnaires.
Even then, as other parents saw me going from 0-60 to run after him yet again, I would joke that for a child with a life-limiting condition he was ridiculously healthy. Yeah, I know, inappropriate humour becomes a bit of a survival mechanism.
Now though… Now there’s no denying that we’re on that downward curve. Pudding no longer runs anywhere – he has to be persuaded to move from his chair. He still enjoys some food, but it’s taking him longer and longer to eat and his (much-delayed) gastrostomy is becoming more necessary. He is on medication for his bowels, for reflux and for joint pain and stiffness. Less mobility means more circulation problems and chilblains recently became an issue. Then just before Christmas, my poor boy started getting periods of gelastic seizures – bouts of laughing or crying for no true emotional reason. (And before you ask, the periods of laughing aren’t cute. After a few hours, they sound hollow and rather creepy.) Whereas before he would greet the whole world with open arms and a big grin, now we have to work hard to get even a brief smile.
As the medical issues stack up, all I can do is look out for the next lot of symptoms and hope that we can keep him as safe and happy as possible.
They say the lobster in the pot doesn’t know what’s coming, doesn’t notice that the water is getting hotter. Unfortunately, I’m not a lobster.