Over the Wall

On a Tuesday afternoon a few weeks ago, I stood in Leeds coach station anxiously watching the bus in front of me. I was waiting for one face. And there suddenly he was, in unfamiliar clothes, tall, dark and with striking eyebrows. I soon realised the last two were from badly-wiped-off face paint (much of which seemed to be all over his trousers), but after four days away my 9-year old did look somehow taller and more confident.

I had been told about Over The Wall Sibling camps ages ago, not long after Pudding was diagnosed but at that point T was too young (the camps are for 8-17 year olds). This year I thought he was ready but that didn’t stop me worrying about him going off without knowing a single other person there. I needn’t have been concerned – the first question he asked me as we walked out the coach station was ‘When’s the next one?’

I could write a whole blog post about this myself but what’s the point? I wasn’t there experiencing it, so I’ll turn this one over to T. (I interviewed him on your behalf!)

A tie-dyed T-shirt and some trousers covered in face-paint

So tell me a bit about camp. What’s it like? It’s fun cos you do lots of different activities. Some outdoor things like climbing and abseiling. We also did indoor activities like picking a partner and going round a course blindfolded.

Was it just physical activities? No, we made a film where I was the evil genius with a side-kick. And we made tie-dye T-shirts and origami. And after most meals we had a disco and did camp songs. My favourite was one where you’d sing the start of it and then challenge someone to ‘shake your booty’.

How was camp organised? We were in different teams and we all had a T-shirt. I was in orange team which was the youngest. And a few of us shared a bedroom. There were grown up volunteers who took us to the activities and cheered us on.

Did the staff and volunteers do things to help you make friends and boost your confidence? Yes, there were trust things like the blindfold course and holding ropes when someone else was climbing. And we did one thing where we had to write nice things on some clothes pegs and then sneak around and try and pin them on someone without them noticing. I was very good at that! Another thing was when we had to draw round our hand and then everyone in our team wrote what they thought about you.

Origami, a little wooden chest with a friendship bracelet in, a toy monkey, and a picture of a hand with lots of nice comments written on.What was the thing you enjoyed most? Climbing because you had to climb up a log onto a rope onto another log and then there was a ladder and a big net and some tyres to get up. It was really cool.

What was the hardest thing you did?  Abseiling! I hated it at the top cos you have to go backwards and it feels like you’re about to plummet to your death. But unlike some of the others I actually did it and it made me a feel a bit braver.

Were you all very sensible and well-behaved all the time? Er…no. Well, sort of. One morning we woke up at 4am but we stayed in bed till a better time.

What would you say to someone who was a bit nervous about going to camp? Don’t worry about going away. Just do it. It’s fun. I will guarantee you’ll like it!

Did you miss us at all? No! I didn’t even miss Pudding cos I was just too busy the whole time having fun!

 

I think you get the picture. He loved it, and even though I was going down with that horrible tummy bug when I picked him up, I couldn’t keep a smile off my face as he talked non-stop about all the things he’d done. And yes, he had a VERY long lie-in the next morning!

Over the Wall run camps for children with serious health challenges, their families and siblings. Applications are now open for 2020 (T is on the reserve list now, as they quite rightly prioritise people who haven’t been before). The camps themselves, and transport to them from various big cities, are free to all campers. Therefore any donations would be very welcome!

 

Today I cry

Today I cry.

This morning I should have got in a taxi to the station with Pudding, caught a train to Manchester and arrived at the hospital. We would have had a lovely greeting from his fan club of nurses and other staff. He would have had a dose of the potentially life-saving drug, just as he has done every four weeks for the last three and a half years. And we’d have whiled away the next four hours with playing and TV in between medical observations.

I am sure that we have made the right decision in withdrawing Pudding from the clinical trial. But that doesn’t stop it being hard. And so I cry.

I cry for the loss of hope. I cry for the loss of his future. 

Pudding with a massive smile, laughing at someone just off camera.

I cry that after three and a half years one of the boys on the trial finally learnt my name and now we won’t see him again.

I cry for the skills he has lost and those he has still to lose.

I cry for the other boys with Hunter Syndrome that never even had the chance of this hope. And I cry for families that have been on trials before that were ended with no choice or input from them.

I cry for the strength I will need to deal with things still to come.

Today I cry. But not for ever.

I don’t have the time. For one thing, it’s production week for our play right now and I’m too busy to spend long in the emotional depths. But also, I think of the reasons that we actually made this decision. One of the huge positives of this choice is to give us more time to enjoy away from the clinical hospital side of things and I am determined not to waste this.

Every time over the last few weeks that I have looked at my gorgeous Pudding, my heart melts even more. And every time I cuddle him, I hold on just a little bit longer. Every minute has become that much more precious.

Less than a week after we saw the consultant for that life-changing discussion I did a talk at Martin House Hospice during their Open Day. I used a talk that I’d written for them on a previous occasion and hadn’t got round to updating it much. Reading through it just before, I knew there was one paragraph that I would struggle to get out without being too emotional and that’s because it had taken on a particular poignancy since I had written it months before.

And most of all Martin House has taught me that a hospice isn’t just a place about dying. Coming here is definitely about having the space and energy to live life to the full and celebrate what we have. And whether we have days, weeks, months or years to spend with our life-limited children, it’s important that we spend them living, not dying.’

And that is what I intend to do.

This is it

It is done.

I have sent the email setting in stone my son’s future. We have withdrawn Pudding from the clinical trial that he has been on.

I’ve said before that it was a horrible decision, but the answers were partly in my heart anyway. As I’ve watched Pudding lose skills over the past few years, despite being on a treatment that has the potential to save boys with Hunter Syndrome, I had come closer to accepting that we would lose him. But it still felt wrong to actually articulate it, to say that this is what was going to happen. As if by articulating it I’d have given up on him.

While we’ve been talking it through, I have wondered if selfishness was creeping in. That I’d be relieved to have less trips to Manchester and less stress over hospital procedures. That I’d be making this decision for the wrong reasons.

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But when it’s come down to it, Pudding himself has made that decision for us. I asked myself what he would want. He lives in the moment. He likes football and TV and food and going his own way. He doesn’t like needles. He doesn’t like feeling sick from anaesthetic. He doesn’t like being kept on a bed for infusions. He doesn’t understand why he feels so rotten if he gets ill.

He wants to run and enjoy life. And we want him to have that while he can.

At the moment he is still so ridiculously healthy, and all that could change in an instant if we wipe out his immune system. He has lost so much already and I don’t think we could take that away from him.

For now we are going to keep on with his weekly enzyme replacement therapy just in case there is still even the slightest hope that it is doing something to keep him more comfortable. It feels a little less stark than stopping everything at once.

We have sat with these thoughts for a while now, and it feels as right as it can do. It helped that we had a visit to Martin House Hospice at the weekend, a chance to talk with other parents and healthcare professionals away from all the normal household routines. And it has helped having messages from so many of you telling us that whatever we do will be right. Maybe that’s the benefit of blogging about all this – our support network is much bigger than I might otherwise have expected.

We’re under no illusions what our decision means. We will lose our beautiful boy to Hunter Syndrome. But not yet.

Circles

At this difficult time, my mind has been turning circles: reading up on options, moving towards a decision, learning more, wavering again. Yet strangely, I am finding it easier to deal with than the period just after diagnosis.

After diagnosis, when we first heard the word mucopolysaccharidosis, the bottom dropped out of our world. Learning that our little boy had a progressive, life-limiting condition left us reeling with shock, anger, grief, guilt…the lot.

You may have seen that diagram of a line showing what we think grief looks like, and then a tangled scribble saying this is what it is actually like. (Sorry, I don’t have a copy and wouldn’t know who to credit if I redrew it myself.) Well, that’s what it also feels like going through this journey. Emotions really aren’t linear. They’re complicated; overlapping and folding back in on themselves, circling round, revisiting you when you think you’re already done with that one. Ebbing and flowing. And over time, I’ve found them easier to deal with.

So even though we’re making this life and death decision for Pudding, it is with four years of experience behind us and without that paralysing shock punching into me every morning when I wake up unawares.

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My brain is full of ‘what if’s and consequences but I can still laugh with my friends and have funny conversations with T on the school run. Seemingly simple tasks like booking a taxi are sending my underlying anxiety into stomach-churning levels, but yet I still managed to sort out my hoe insurance ten days before the deadline. I may find it hard to concentrate on planning meals, but can still enjoy eating. I need a hot water bottle at night to ease those tense muscles, but my mind has mostly let me sleep.

The world keeps on turning and another aspect of my life has circled round to bring home how far I have come. I’m rehearsing for a play at the moment, and it’s being directed by the same person as the play I was in at diagnosis time. I have always loved acting – the chance to step away from real life and be someone else – but back then it was tough. Everyone was very supportive and there were numerous times when I had to leave the rehearsal room to go and cry by myself. But now? Well the play is pretty challenging, dealing with family relationships, love and loss and I am finding it quite cathartic in a way – I may not be able to shout and scream at MPS in real life, but I can channel that into my character.  And though they may know I have a disabled son, I doubt most of the cast have any idea of what we’re currently facing and that’s the way I’m happy for it to be. I would do anything for my Pudding, but I also need to be just me sometimes. An ordinary person doing ordinary things.

And as for the future…? We have probably made our decision, but we’re sitting with it for a while, to check that it feels right. All your messages of support and love for Pudding really has helped, so thank you.

Decisions

This is a decision no parent should have to make.

This is a decision that I always knew we might face sometime in the future. But not yet. Not when Pudding is only seven and a half years old.

I knew it wasn’t going to be an easy meeting in Manchester last week. Not when the consultant asked to see both Hubby and I. That’s obviously not a routine appointment; it’s decision time.

Essentially, the treatment we tried in June to reduce the antibodies Pudding has towards his treatment has not made any difference. The stronger a body’s reaction to the treatment and the longer one has antibodies for, the harder they are to get rid of. Pudding has a complete gene deletion, so the enzyme is completely foreign to his body. And he has had antibodies since at least February 2017, probably longer. So there’s a double whammy.

Some families in America that I know of have, even with a complete gene deletion, successfully eliminated antibodies. So I have of course been reading up and learning as much as I can about the options out there. It seems to boil down to a long course of more toxic drugs or daily/twice-daily infusions. Both of which could potentially be for years. Or for ever.

The problem is, as always when talking about rare disease, that the numbers are small. I can’t look at the figures and say 100 people tried this and 75% were successful. We’re talking ones and twos.

Pudding sitting on the floor by hospital ward doors.Pudding has already been on a clinical trial for three and a half years. It seemed like the right decision at the time to put him through more medical interventions even though there were no guarantees. Given the hope that it offered, it was worth the time and the risks.

Of course it hasn’t turned out to be an entirely positive experience for us as we have watched him gradually lose skills, known about these antibodies since last year and yet been unable to treat them.

And now…

We have to decide whether to put him through more. Or to say enough is enough. Quality over quantity. More treatment over living life as it is now. Knowing that the choice of doing nothing will mean accepting the inevitable course of Hunter Syndrome – decline and death.

To be honest, it’s a pretty shit choice.

There are so many factors to consider – risks, benefits, side effects, damage already done, family life. My head is spinning with information and every night when I go to bed I realise quite how tense my body is. I just don’t know how to make a decision like this. How to know we’re doing the right thing. None of the options feel like the right thing. Whatever we choose there will be somebody who says, ‘You chose wrong.’

And the thing I am most scared of, is that it will be me saying that.

 

Control

I’ve not written a ‘proper’ blog post in ages, and it’s not for lack of stuff to write: I’ve started this particular post a number of times but it never quite comes out how I want. When I started this blog in 2015 soon after Pudding was diagnosed, the words poured out of me. All the fear and guilt and anger and devastation just had to make it onto the screen in any which way. I barely even had to think about what I was writing, whereas now…things feel more complicated.

This year so far has been one of contrasts. After a very stressful first few months we had a great summer, and I think it can be summed up with one word. Control.

I remember reading an article many moons ago about stressful jobs. It may seem counter-intuitive, but it was saying that the most stressful work wasn’t what you might think. It wasn’t necessarily those in high-powered careers who suffered the most, but those people who had the least control over their work environment. The people who have to react to what’s thrown at them with little or no control over their situation.

Over the last few months I’ve come to realise that this what many of us go through when parenting a disabled child. Before anyone gets upset with me, I don’t mean it’s the child themselves that is the problem. It’s all the other things that impact on our lives  – the lack of accessibility, the fight for support, the forms, the waiting for school places and the never knowing what the future will bring. The control that politicians, budget-holders, pharmaceutical companies have over our children’s lives. That knowledge that your precious wonderful unique child is, to them, a figure in a spreadsheet or just another service user.

When I was writing one of my updates on Facebook recently (come find us here if you haven’t already!) I used the word trauma and then wondered if I was being guilty of over-exaggerating. But actually I don’t think I was – consistent lack of control over your situation IS traumatic.

I never know much about what Pudding is thinking, but it’s pretty clear that the same is true for him. When he can decide where he’s going, or what he does – when he is in control – he is happy.

OLYMPUS DIGITAL CAMERAFor us, the main issue has been Pudding’s health. Not his health now (anyone who sees our photos and videos knows that despite his life-limiting diagnosis he still remains ridiculously healthy) but what is to come. We’ve known since February last year that his body was producing antibodies against the enzyme replacement treatment. Yes, antibodies against that very treatment that is meant to be keeping him alive.

We had to make a difficult decision about whether to go ahead with immune modulation drugs which could result in side effects or increased risk of serious infections. There were also other considerations that had a huge bearing on our decision that I still don’t feel able to talk about. All I can say is that it was a time of agonising changes of mind. How to make important choices on not much information? How to keep going when other people hold all the cards? How to know that we’re doing the right thing when any impact could take years to show what the true benefits have been?

In June we went ahead with the immune treatment – four visits to Manchester over two weeks (four injections and two infusions). Pudding was of course a star, taking even more medical interventions in his stride despite not liking them. Then the waiting started.

The summer has been almost a honeymoon period. With plenty of respite in place for Pudding allowing us to concentrate on fun things with T, and with hope that Pudding’s future was a bit more assured, I felt like I was back in control.

But that assurance is starting to wobble again. Latest results from blood and urine tests have not been very positive and more decisions will be needed soon.

 

Holiday challenges

Trying something new can be difficult. Challenging.

And when you’re making decisions on someone else’s behalf, it’s even easier to stick with the same old things. I know I’m guilty of that with Pudding. I know what he likes and often don’t branch away from the tried and tested. After all, I can’t ask him what he thinks.

CPWe’ve recently come back from a holiday at Center Parcs at Sherwood. A tried and tested formula for us. We go with the whole of my family (parents, Sister, Brother and their children) so there are lots of people around to chat with or do activities. I loved going before Pudding was diagnosed with MPS, and I’ve come to appreciate it even more since. Holidays can be challenging for us in many ways, but I know what we’re getting at Center Parcs. I’ve always found the staff to be really helpful and responsive regarding Pudding’s accessibility needs and they even have a Changing Places toilet in the Sports Centre with another one planned in their pool revamp.

While we’re there, all the other kids get to try out new activities, stretch themselves and their abilities. We find things they like and things they don’t, and that’s ok. And I need to remember that Pudding deserves that chance too.

This holiday I challenged myself and booked him on the Mini Captains Adventure – a boat ride for young children with one accompanying adult. I had suggested that we could draw straws to see who went with him, but no-one else was brave enough!

To be honest, I had no idea what he’d make of it. The success of an outing or activity can depend very much on his mood at the time (and whether he’s had something to eat). But I was prepared for pretty much anything short of being capsized.

It’s often the people we encounter that can make or break an experience for us. I often think Center Parcs have good customer care and the chap running this session was no exception. He was very happy to accept us on our terms and didn’t bat an eyelid when Pudding sat in his wheelchair watching his tablet throughout the safety briefing and instructions.

Grandma, Brother and Sister had come along with us for moral and physical support (very useful when it came to getting the chair across a short stretch of beach), but found it terribly funny when the instructions told us to visit each buoy in turn and note down which picture was on them. I very sensibly decided to ignore them and the instructions!

Pudding looking through the front window of a boat holding onto the steering wheel. I am sitting by him, arm just visible guiding the wheel too.Once the other three boats had set out (again, my decision) we got Pudding out of his chair and took him down onto the jetty. The massive smile on his face made it worthwhile even just for those few seconds. Manhandling him into the right place on the boat was a bit difficult but we did it. And then we set out onto the lake.

He loved turning the wheel, but wasn’t quite so keen on me actually steering so our progress on the water was somewhat erratic! He also loved the occasional person zooming past overhead on the zipwire. I had a feeling though that a good mood wouldn’t last too long, so after a while negotiated back to shore. Seeing his fan club there brought more massive smiles and we even got a picture of him holding his Captain’s graduation certificate.

It was probably the most expensive ten minutes he’s ever spent, but I don’t care. We tried something new!

(PS. I am not being paid for this post and haven’t been asked to promote it, so any advertising is entirely coincidental – just my opinion! Other holiday companies do exist, etc, etc)