Surgery this evening.

After finding out in March that the port that delivers Pudding’s trial medication to his brain is no longer working properly, he is having it replaced today.

This morning I chased him and let him climb on my back  and tickled him until we were both in helpless giggles, as I knew we wouldn’t be able to do that for a while.  I watched him eat a very early lunch knowing that in a few hours he would be looking at me with those accusing eyes and repeatedly asking for ‘bibit’ (biscuit). He kept running away with the bag I was trying to pack and for once I didn’t get cross or frustrated because I knew that he’s going to feel reluctant to move at all for the next week or so.

Pudding was very excited when we got back to the trial ward in  Manchester. We’ve not been for three months now so he was obviously keen to make up for lost time, running up and down the corridor and shouting ‘Found you!’ at all the nurses. Very cute, but it was difficult to share his enthusiasm knowing that in a few hours I had to sign a consent form for surgery listing ‘permanent nerve damage’ as a potential side effect.

The anesthetists here are fantastic, and experienced with the short neck and difficult airways that Hunter Syndrome produces. I trust them with my son’s life but I wish I didn’t have to.

At 6pm we walked down to surgery with him complaining all the way. It was nearly his bedtime. He was tired and hungry and had had enough. I watched his eyes roll back into his head as the anesthetic took effect. Now we wait for three hours until we can see him in recovery and listen to those pitiful cries as he tries to tell us that he feels rotten and he hurts and he doesn’t know why. And I’ll feel helpless because I can’t explain to him why he needs to go through this.

We’ll stand ready with the sick bowl and obsessively watch the SATS monitor over the next few hours. The TV will stay on for a week and we’ll check his dressings for any leakage of spinal fluid.

It’s his sixth general anaesthetic since diagnosis almost 2 years ago, and we’ve got used to the routine but it never gets any easier.

Clinical trial – a year on

Monday and Tuesday we were in Manchester again. It is the end of Pudding’s first year on the clinical trial that aims to halt the progression of Hunter Syndrome in his brain. So he needed to go for an MRI scan and lumbar puncture under general anesthetic.

It’s IMG_20170109_180120been an emotional journey of course. First there was the decision to enter the trial when the potential good had to be weighed against the negative implications. There was the major surgery itself meaning the placement of a portacath into his spinal cord. The first few months when he reacted with sickness, and then the gradual easing of tension as the reactions were brought under control. The waiting game as we wondered whether it would help him at all. And the slowly dawning hope that as he approaches his fifth birthday there is no sign yet of him losing skills. Indeed he seems to be gaining them.

It is little things. Things that other parents would hardly notice. Like me asking him to turn on a light that was beside him and him doing it. Like me saying ‘good building!’ as he played with his blocks and him responding ‘da dyu!’ (his version of thank you).

So although the results of this end of year MRI scan won’t be available to us, I’m pretty sure it will be positive. Enough boys with Hunter Syndrome have now been recruited onto this study and while we wait for them all to complete the trial year, Pudding will join others on the extension study. They continue to receive the monthly doses but with less regular blood tests, ECGs and so on – something both Pudding and I will be very happy about.

At the end of this calendar year, Shire (the pharmaceutical company who developed this drug) will be putting the numbers together and starting the process of getting it approved as a treatment.

I should be feeling positive.

Yet my newsfeed has been filled this week with stories of an NHS in crisis. Not enough beds, not enough doctors, not enough money to treat an increasing number of patients. With these sort of pressures who is going to sign off an eye-wateringly expensive new treatment?

Right now I should be filled with campaigning zeal. I should be writing to my MP and demanding to know what can be done to save the organisation that could save my child’s life. But I know what the response will be. The NHS is ‘very important to us’. ‘We are doing all that is necessary’. Health funding is at ‘record levels’.

It shouldn’t stop me, I know. I shouldn’t let the sense of powerlessness take over. But I just…right now, I just…can’t…

Hospitals (and play)

I have a little luxury that I indulge in when we’re in Manchester if it’s a long day or we’re there two days running. Our visit last week was a long long day. There was a problem in Pharmacy making up the drug so Pudding wasn’t given his dose until three hours later than usual.

(Because the drug is injected into the spinal cord it means that there would be severe consequences if any infection was introduced with it. The drug is made up in the aseptic lab – think protective clothing, disinfection, working in sterile environment – and if anything goes even slightly wrong they have to start again.)

If I’m stuck anywhere for any length of time I start to go stir crazy and hospitals seem to exacerbate that. There is always a lot of waiting around – waiting for the next set of observations, waiting for the dose, waiting to see if he reacts badly – and we’re powerless to really do anything much. So what I do is take a walk to the M&S Food Shop for a nice sandwich and maybe even a pudding. It is a definite improvement on the canteen food, but that’s not the luxury. No, it’s the short walk there, and more importantly leaving the building. I go the long way round and cut across the grass. Even though there are no signs saying you shouldn’t I still feel guilty but it’s worth it. The chance to walk outside and gaze at lush green nature, even if it is only a tiny patch, is priceless and is my healing.

Not that Manchester Children’s Hospital is a bad place. I’ve not got much to compare it to having never stayed in one myself, but if you have to spend a lot of time in any hospital I recommend it. It’s pretty bloody brilliant. It was purpose-built in 2009 and obviously has the needs of children very much at its heart. There are toys and cartoon characters everywhere, clown doctors doing their rounds, visitingSmiling lady with a birthday hat on playing with a transformer celebrities and amazing staff…

The most important people though, at least as far as the kids are concerned, are the play specialists. Nope, I hadn’t heard of them either until recently. These wonderful people have a background in childcare but now work in the hospital to offer all sorts of opportunities for play – distracting children from the procedures they are facing and supporting families. And oh my word, they are worth their weight in gold!

The first thing most kids ask when they get onto the research ward is ‘Where’s Emma?’. Non-verbal children like my Pudding rush to give her a hug. She makes each and every one of them feel special and loved. Of course, the nurses do a brilliant job too, but they have to focus on the clinical side and have loads of admin to do as well, so Emma gets to be the fun one. She provides DVDs, spare batteries and wifi, arranges the chill-out room for the teenagers and messy activities for the young ones. She has an enormous smile that never disappears. (And she’s even been known to clean up vomit when the need arises.) Pudding has one T-shirt that says ‘Mad as a box of frogs’ and we have always joked that she should have one too. Madcap indeed, but exactly what we need to keep us all sane in stressful circumstances.