Inclusion has been much on my mind this week.

A while back we made the decision to send Pudding to mainstream school when he starts full-time in September. Hopefully this will prove to be the right decision for us, but in order for it to work he would need full-time support in place. Without proper supervision he’d turn the place upside down in very short order. Oh, and I assume most people would be impressed with his throwing skills until the hard object hits them square in the face….

This week we have a meeting to discuss getting him onto an Education and Health Care Plan, which is the format that replaced the old Statement of Special Educational Needs. (This is after we sent off reams of information in December which was discussed by a panel to decide whether he can be assessed for the EHCP. Tortuous, eh?) So I’m girding my loins for another meeting where we have to focus on what he cannot do, and what the future may throw up.

On a much more positive note for this week, Pudding was invited to a birthday party for the first time. It’s a rite of passage for many children, but especially important to those with special needs. As a parent I’m sure many of us worry that our child will be included in these sort of events or overlooked. No worries there – with a bouncy castle, balls to kick around and plenty of pizza, Pudding had a whale of a time.

And during our ERT infusion last week there was another important step towards ensuring that he doesn’t always have to miss out on the fun things in life. He’s never been keen on using the shoulder bag that comes with the infusion pump; until now while it’s going we’ve had to keep a close eye out and leap to grab the bag if he makes a run for it. This time we tried a little rucksack that we’ve had for a while (bubble wrap and tape to try to stop it pressing on the port and gripper needle!). It worked brilliantly and meant that he could be far more independent and even enjoyed playing ball for a bit during the infusion. Although he’s not sensible enough to trust further afield for now, when the weather is better we will now be able to go out in the garden if we want. I may yet get it looking nice this summer. Hoorah!

Well, not quite, but something did make me very happy today.

I’ve found it difficult staying sane this last week. Being pretty much house-bound with two boys coughing through the night and whingey during the day is not designed to show me at my best. I freely admit, sometimes I think I am not cut out to be a mother. The image of a woman who tirelessly gets up in the middle of the night and is still smiling and patient through the day? Well…if she exists, it ain’t in this house.

Twiglet is mostly better. At least it’s just a cough hanging on now rather than a temperature, and is back at school. Pudding was off playgroup again yesterday but I decided he was ok for gym club today.

Yesterday I’d been very productive and while he was settled in front of the TV, managed to sort out all the piles of cardboard boxes that were threatening to take over the shed. So today’s job was to take them to the tip on the way to gym. That meant having Pudding’s car seat in the front as the back seat/boot were full of junk. I thought he’d be happy and excited to be there for this short drive. He was. Until he decided that what he really wanted to do was pull on the gearstick.

Not quite sure why I didn’t realise this would happen. Needless to say, he doesn’t deal very well with the word, ‘No!’ even when it is screamed at him while driving round a roundabout. It was not a fun trip. And he was still crying when we got to gym club, particularly as we were five minutes early so had to sit at the side and wait for our turn.

As soon as he was released he ran straight for the foam pit and bent his knees, arms back, looking like he was about to do a standing jump. Normally this is followed by him standing up, sticking one leg out in front of him and leaning forward, rather like a cartoon character walking off the edge of a cliff. Falling, with style. But this time he actually did it: an honest-to-goodness, two feet off the floor at the same time, full-on proper jump!

I was over the moon. I may possibly even have whooped (just a little one, I am English, after all)! I may not be a perfect mum, but my children can make me perfectly happy every so often…

OK, so it’s not the most inspiring title for a blog post ever, but bear with me!

Ever since Pudding was little I’ve had problems cutting his toenails. Admittedly I may not be the most expert nail cutter. I am, after all, the one who accidentally drew blood when trying to do Twiglet’s nails at only a few months. But Pudding’s were definitely a challenge; chubby toes seeming to overwhelm his tiny triangular nails. As he grew older, the big toes developed a tendency to grow in, and the rest curved under, moulding themselves to the shape of the toes such that it became difficult to even get the clippers under them.

Of course it wasn’t a huge problem; just one more little thing alongside his webbed toes, tongue tie, etc, that made me think he wasn’t put together quite right. We did get a referral to the podiatry service, but other than advice to ‘keep them short’ nothing more came of it.

Then last week, following a hunch, I posted about it on one of the MPS groups I’m a member of. Within a couple of hours, I had 13 comments from other parents all saying ‘Yes, we have this too!’

Of course, it’s not a scientific survey; probably not all MPS patients will have this issue and many without MPS will have. (Described as ‘clubbing’ it can be due to a reduction of oxygen in the blood, for instance because of heart or lung problems, and has also been reported in other conditions such as coeliac disease).

But for some reason it made me feel so lighthearted to have this interaction. Parents at the school gate quite often get to have those ‘Oh god, yes, me too!’ conversations about normal everyday kid things. For MPS parents though, that sort of thing just isn’t possible. The likelihood of living close to another family is fairly slim.

It is therefore one of the joys of this internet age to find a group like this online. All the bad things about the internet – the online bullying, trolling, and so on – can at least be balanced out a bit. Because there is nothing that says ‘I belong!’ quite like a group of parents and patients from all over the world bonding over a discussion about toenails.

The best laid schemes of mice and men…

Well I thought everything was sorted regarding our next visit to Manchester for Pudding’s trial medication. Hubby was going to come with us for this second time, to help me with the drive between hospital and hotel among other things. Someone would be picking Twiglet up from school tomorrow for gym club and then deliver him to Sister for the next two nights. After school club sorted.

Then last week, Hubby came down with a cold and I started praying that Pudding wouldn’t catch it. If he’s unwell, his sensitivity to the normal weekly infusions is heightened – but at least if his temperature goes up during ERT it can be switched off, as happened previously. With the injection of enzyme into the spinal fluid, once it’s in, it’s in, and any reaction would have to be managed with more medication.

Pudding has now succumbed to a cold, but seems generally ok apart from a runny nose. Still ok for Manchester?

But Hubby is now suffering from a nasty bug, and I doubt whether turning up at a hospital like that is a good idea. Twiglet’s cough has worsened and he had a temperature of 40 degrees earlier (not that I always believe the accuracy of my thermometer). Unless we see a miraculous recovery by morning, school is unlikely.

I know I’ve said it before, but I hate how since MPS reared its ugly head, the littlest things take on so much more significance. Ho hum!

I went out for a meal on Monday with a lovely friend. So nice to get out and have a good chat. She mentioned something about the difficulty of diagnosis and how you need to get to that stage where it isn’t the first thing you think about when you wake up and the last thing at night. Very true and insightful.

It made me realise how far we have come since those earliest dark days. Yes, I do obsess over it all more than I possibly should. And some days anxiety settles in my stomach for no discernible reason. But often life just continues.

The calendar is marked out with the usual routine of preschool, gym club and weekly ERT (enzyme infusions). Next week we will be back in Manchester for the next dose of trial medicine. Even that feels like it will be fairly routine now; we know the ward well, and what to expect from the day there.

We regularly have support from some wonderful people – our Healthcare at Home nurses, staff at preschool, our portage worker to name but a few – and we should be accessing some respite hours soon. Hopefully that will mean that time to concentrate on Twiglet’s needs will become part of our routine too.

We’re in that strange in-between-land; Pudding has a serious genetic condition but he is not a sick child. Spring is coming and I have so much to be grateful for.

I frequently struggle to articulate what Pudding is really like. How can  I describe such a gorgeous little lump adequately? So today’s post is a little bit different. I’ve let some other people describe him:

Twiglet (nearly 6) says,
He is very friendly and cute. He’s not like everyone else’s little brother because he’s got Hunters, and the Hunters makes him hit a lot. That makes me feel sad. He gives big cuddles a lot. He likes bananas.

Nephew (7) says,

Danny likes watching TV and eating. He has got Hunters so he’s different from other children. He acts like a baby but he’s a toddler. I like him because he’s cute.

Niece (9) says,

He’s fun, cute and lively. Whenever you’re sad he always puts a smile on your face. He loves food. He loves telly. He likes playing football in the garden. He does funny things like calling everyone Dave. In the future I think he’ll be just the same as he is now, only older and not quite as cute. When my Dad picks me up I like it because Pudding always gives him something random from around the room. I sometimes worry that his new medicine will stop him being like Pudding.

(I asked them all what they thought of Pudding at separate times so they didn’t hear or copy each other’s reply. I love that they all independently used the word ‘cute’!)

 

 

Who am I? Who am I?  Not Jean ValJean, that’s for sure. But when I was listening to Les Miserables the other day, I realised that he had a point. We all need to be defined by something, and that something is ourselves.

As with many mothers with young children I ended up subsuming my own identity a little into the daily grind of feeding and nappies and baby groups and weaning and nappies and …well, you get the picture. I was grateful that I didn’t have to juggle work and parenting, but I did lose something because of that. The world of work, although often stressful and with its own challenges can offer things too; a pay packet at the end of the month, a thank you, a simple acknowledgement of a task completed on time. .

When my youngest passed the age of two I expected to be looking outwards again, planning a return to work, finding myself again after the years of baby-fugged-brain. Then his development delay became increasingly obvious and just as things should have started becoming easier, I found myself struggling even more. Hunter Syndrome knocked a couple more punches in, and has dominated my life for the last nine months now.

Last week, at a writers’ group meeting I went to, we were asked to give our name and say something interesting about ourselves. It went round the table with people mentioning working in prisons, boxing for England and other fantastic things. Then it got to me. Who am I? There was so much I could have said, but instead my mouth opened and I blurted out that my son has a genetic disease.

I could have kicked myself. Yes, it’s highly important to me at the moment, and I find it hard to think about much else, let alone write about anything else. But Hunter Syndrome IS NOT ME! I am not defined by my son’s disease. I need to learn to step away from it sometimes, and find myself again.

So here is some of what I could have said. I have degrees in both Chinese and chiropractic. I love baking and made my brother’s wedding cake. I received a marriage proposal on a train in China. I had two home births. I was the only person in my class at school who answered yes to the question ‘Would you live on a desert island by yourself’. I once sliced the side of my finger off in a bacon slicer when I worked at a supermarket.

Wow, it’s a pretty hard exercise to try and think of something interesting. But it has taught me a useful lesson. I need to make more of an effort to be me. Do some yoga. Get back to writing the novel. Clear out my wardrobe. Anything.

Hold me to it?

It was Pudding’s birthday this week, and I’ve been surprised by some of the emotions it has thrown at me.

Facebook of course delivers memories to me of previous years and previous birthdays. I look back at them and wonder how this can be the same family; they have never heard of MPS or Hunters, not had weekly infusions or sat anxiously through another surgery. I looked at a photo taken of him at only a few hours old and simply could not comprehend that this was the same boy who has given us love and worry in equal measures.

For many MPS parents, I know birthdays and other events must be even more bittersweet; they can look back at previous years and remember ‘Back then, he could still walk/swallow/say ‘I love you’.

I at least have so much to be grateful for: we can still see progress rather than regression.

A year ago when Pudding had just turned 3, we had only just started appointments with Speech and Language. His understanding of even the simplest one stage commands was exceedingly hit and miss, whereas now I can ask him to do basic tasks, such as ‘get your shoes’, without having to repeat and repeat and use gestures. (Whether he chooses to do something is, of course, a different matter!)

Last year, he would mainly play on a very physical and sensory level. Now he still very much enjoys crashing his cars off the windowsill, or flinging objects at us very hard. Yet when I unwrapped (yes, we haven’t quite got that concept yet) one of his presents to find a remote control car, he immediately sat down on the floor with it and tried to work the buttons, looking between the controller and the car. He knew what it was meant to do!

He has come so far in a year, despite all the gunk clogging up his poor brain. Not as far as many other children will have done in the same time, but I am so proud of my little boy.

Hospitals are strange places.

When you spend longer than a few hours in one, time seems to stretch and contract in different ways. You enter a suspended state of animation where the hours are measured by the next set of obs (temperature, blood pressure and oxygen levels – every four hours), other tests or the regimented mealtimes.

A trip outside the ward, even if it’s just to go get a coffee, feels like you have escaped illegally. Breathing in some actual outside air full of invisible pollution from the surrounding city couldn’t be better, even if it was fresh from the purest mountain resort.

When Twiglet rings to speak to me on the phone before bedtime, I hardly recognise his voice. It seems impossible that he can be my son too; he exists on a different plane to me.

Staff are wonderful at trying to make us feel relaxed and at ease but it is odd to have no responsibilities. I’m not able to make my child a piece of toast but I can tell them which size gripper needle to use.

Freed from the restraints of tidying or washing up I find myself more able to sit and play with Pudding, truly enjoying being in the moment with him. Together we race cars down the ramp, and give Mr Potato Head an extra three arms.

But once he settles in front of a film and I have read a book for a while, I find myself a little at a loss and end up surreptitiously tidying up toys in the playroom. (The wonderful play specialist would tell me off for not taking it easy but I can’t help it!)

We’re of course in a privileged position – unlike many stuck in hospital Pudding isn’t unwell. Far from it. Apart from objecting to being pinned down for the dosing and many subsequent blood takings – 11 in 36 hours this time – he’s been having a whale of a time. Running around with the other boys on the trial, kicking a ball up and down the corridor, mesmerising all the nurses with his curly hair and lightning grin. He even got an early birthday cake and balloon octopus!

We did have a bit of a worry initially, as his temperature went a bit high just before the dosing. I didn’t think I could bear having to cancel and come back the next week, but after we stripped him to his nappy, his temperature came down again and we got the go-ahead.

The trial team had all the emergency medications ready in case of a serious reaction, but everything went smoothly. The new port worked just as it should; they were able to draw some CSF (the fluid that surrounds the brain and spinal cord) for testing and deliver the new drug. Unlike the weekly infusions, it was over so quickly; almost the longest part being the need to clean the area thoroughly three times to minimise the likelihood of any infection.

So that’s it until next month. And the next few times we don’t have to have the blood samples done, so it will just mean two nights in the hotel and not having to sleep on the narrow pull-down bed. Hoorah!

So this is it. We’re heading off to Manchester again later today.

This could be the first week of the rest of Pudding’s life. The first dose of a monthly enzyme that could potentially halt the progress of this horrible disease.

We’ve known about this trial ever since we first sat down to talk with the specialist consultant back in July. At that point we hoped to goodness that we would never need to learn any more about it, but of course that wasn’t to be.

Once it was confirmed that Pudding has the most severe form of Hunter Syndrome, we had to face it. Clinical trials of course have an element of risk. There was very sad news lately of a trial for Duchenne muscular dystrophy being suspended due to the death of one of the participants. A different drug and a different disease, and the death may not have been caused by the trial drug itself, but it’s enough to make anyone stop and think again.

Although other boys have been on the Hunters trial safely for a few years now, the numbers are still small. Could it be our son who reacts badly?

It’s a risk worth taking though; the one thing I couldn’t bear would be to look back in ten years time and think, ‘We had a chance, but we didn’t take it’.

So here we are, taking that chance. The dose will be given on Tuesday morning, but because of all the tests afterwards we don’t get to leave until first thing Thursday. Technology not always being my friend, I may not get a chance to update you until then.

For now, wish us luck!