They will never know…

A while back, Pudding and I went to meet T at the school gate for the first time in ages. This is the mainstream primary that Pudding also attended for the first year and a bit of his school life. Loads of people noticed him and came out of their way to say hello to him – the lollipop man (probably not the right term, these days!), parents from T’s year, but also children who remembered Pudding from his time there.

I found that hard, as I knew how much he had changed in the last three years and felt that I needed to warn the children. That he might smile at them but wouldn’t interact in the way he used to. That he likes people saying hello to him but might not show it any more. I thought it would be hard for them to understand and reconcile their memories with the reality.

Later though, I thought more about it and realised that at least all these people do have memories of him, which will hopefully last. Much tougher for me to accept is that people who meet him now will never know the whole of him.

They will never know the little 5 year old who would thunder around school with a massive grin, making himself known to everyone. They’ll never have seen the Year 6’s queueing up to give him high fives. They missed him taking over the headmaster’s office and making himself comfortable in the spinning chair. They’d have to imagine him (with probably once of the worst attendence records in school) gatecrashing the ‘100% attendance breakfast’ and demanding juice.

Anyone seeing him now, passively watching the TV, wouldn’t know that once he pattered on stage at the travelling panto to ask where his favourite pantomime cow had gone. That at the donkey sanctuary he would run around telling everyone at top volume that there were ‘Dong-key!’ as if they might not have noticed. That everywhere he went, he greeted the whole world with joy and openness.

Boy with huge grin wearing Star Wars T-shirt running towards the camera. Man in baseball cap and glasses in background.

Those who don’t know him will see a child at the playground who needs to be persuaded to leave his wheelchair and clings to me for balance. They would never believe that I used to watch him like a hawk, that he would peer round at me with a cheeky grin and then make a beeline for the gate out to the road. That I would have to go from 0-60 in five seconds flat in order to catch him.

They won’t see the boy who would earnestly babble to his breakfast. Or wait at the window to watch for his Daddy to come home, then run to the door and catch him by the hand to lead him into the house. They’ve never known the delight of watching him hide behind a cushion to play peekaboo and then giggle so infectiously that you couldn’t help but join in.

Those who take the time to know him now still love him, and of course his family’s adoration has no bounds. I don’t really know what any random stranger meeting Pudding would make of him, but I do know that they have missed out on the most adorable child, more packed full of character in those few years than many people manage in a lifetime.

Middle Age

My son is as old as me.

Not chronologically of course – he’s eight and I’m 49. But the other day I realised that soon he’ll be nine, and in Hunter Syndrome terms that is getting closer to being old.

One of the blessings of being in lockdown again is not having to do the school run with T. Normally I do enjoy it; strolling along past the little lake, chatting about Minecraft or Pokemon or Last Avatar. Sometimes, I walk a different way which means I was more likely to see Pudding’s mainstream classmates – children he’d still have been in school with if MPS had not come into our lives. For a long time after he moved to his fabulous specialist school the obvious differences between them stopped bothering me. I stopped comparing him to them and wishing things were different. But last term the negatives began to creep in again. I would see them zooming along on scooters, so tall and growing more independent. And yes, it gave me a pang knowing that they would soon be walking to school by themselves, that so many possibilities stretch before them.

They are growing up but are far from old. Whilst Pudding is more like me.

I groan now when getting up off the floor. Pudding will no longer bend down to pick most things up off the floor (a tempting shoe to throw being one exception). I can hardly see to thread a fine needle like I used to. He is finding it more and more difficult to judge depth when stepping from one surface to another.

But while I know I’ve got years ahead of me before I am done with middle age, I don’t know about Pudding.

In the MPS world birthdays are scary. A birthday doesn’t just mean another year older. It means another year closer to old age. When a life can be cut off before adulthood, when is old age? 15? 13? 10?

I don’t know. And I don’t want to find out.

Seeing things differently

When Pudding was diagnosed with a rare progressive condition that I’d never heard of (I mean, who HAS heard of mucopolysaccharidosis in the course of normal life) I knew that nothing would ever be the same again.

I was right. It hasn’t been. But that doesn’t mean that life is over, that everything will always be bad.

When he was first diagnosed, other parents told me that the first six months to a year were the hardest. I tried to find that helpful but couldn’t really see myself going back to feeling normal again, not feeling all that fear and grief and anger. It just didn’t seem possible.

Five years on, and a friend, another MPS mum, recently messaged me with this photo that she’d just come across in a back copy of the MPS Society magazine. To her, it was just a lovely photo – Pudding reaching over to me as I leaned on his hospital bed.

To me, it was so much more than that. It was a reminder of the day our fears came true. The day, a few months after diagnosis, that we finally got the results from his DNA test, confirming a complete gene deletion and therefore the worst possible outcomes from his condition.

I mentioned that and she immediately apologised, wishing she hadn’t sent it. But as I told her, I truly didn’t mind. For despite the circumstances, I do now love that photo. Yes, it is bittersweet, but it doesn’t just make me think of the worst.

When I see it I also remember the consultant’s face as he told me, and I knew how much he cared. I remember the hug that our specialist nurse gave me as she wished she could do more to make things better. I remember the nurses on the ward not just giving Pudding his treatment, but loving him with all their hearts. I remember being so grateful that my mum was with us on that (as I thought) routine visit. I remember the beauty of the moors as we headed back home along the hated M62 in sunshine.

One photo. So many different ways of seeing it.

In fact, I actually find it hard now to truly remember my feelings from those first few months. Not that I have exactly welcomed MPS into our lives. But I do think I’ve come to much more of an understanding with it. An acceptance that what will be will be.

A lot of the reason I’m free to see things differently at the moment is the wonderful long break we’ve had from hospital. Leaving the clinical trial he was on was difficult certainly, but it has meant that normal life is more ‘normal’ – no more clinical visits, no more psychology tests where I’m hit again and again with the reality of what he can’t do. No more M62!

Pudding’s health continues to be mostly ok for the moment and he’s a lot easier to deal with, being so much quieter than he was. Whilst I know the things that are still to come for us, it’s like we’re in the golden days. The eye of the storm. And I’m liking it here.

I used to hate all those motivational/inspirational memes (still do actually). You know the sort of thing: ‘Special children are only given to special people’ or ‘What doesn’t break you, only makes you stronger’. But I guess one that does ring a bit more true for me now is ‘Whilst you can’t change what happens to you, you can change how you react to it’. Not that I’ve really made any attempt to change. Perhaps it’s more accurate to say that I have been changed.

The next stages in Pudding’s condition may come quicker than I think, or we may still have months or years to make the most of. Who knows, I may see things differently again tomorrow! But until then, I accept.

Fear

Fear. It creeps up on you, doesn’t it?

The whole situation with coronavirus has been a perfect breeding ground really.

I’ve written before about how my anxiety levels seem just naturally higher these days. It started when becoming a stay at home mum: my horizons narrowed and I was no longer pushed to do things that I found stressful. A few years later Pudding’s diagnosis came and things suddenly became that much more limited and worrisome.

And then a worldwide pandemic. Boom! Horizons reduced even further. We’ve been lucky: our home and garden have been very much a safe space for us. With the exception of sporadic trips out for exercise we’ve pretty much stayed put. Yet now shielding advice has been changing and I’ve known for a while that I’d need to venture out into the world again.

Scary.

Logically I know that the risk of me catching anything on a quick trip to the shops is minimal. I can tell myself that till I’m blue in the face. But of course brains don’t always work like that. Logic doesn’t always win over fear.

Last week I beat that fear in a small way. I stepped inside a building other than my house for the first time since the 21st March. It was only taking a parcel to the post office and I’d already paid the postage online. There was one other customer in the shop and it actually wasn’t at all scary.

We will definitely not be going wild and having a family trip to the pub anytime soon, but it felt like a positive step taken.

And then, this weekend the fear bubbled up again. Or should I call it anxiety? Sometimes I know exactly what has triggered it: an argument on social media, a TV programme I’ve watched that has touched a nerve. Sometimes it takes me a while to pinpoint what the cause was.

This time it was a letter received from the government on Friday – a letter that said Pudding is still considered to be in the ‘clinically extremely vulnerable’ group and should remain shielding until August.

cuddle

I know that those letters aren’t always accurate (see below for the explanation) and I know that his consultant was happy for us to start easing into normal life a little more. But I’m obviously well conditioned to respond to authority, despite my reservations about the current government, and that letter tickles all those ‘but what if…?’ nerves.

Logic taking another little holiday.

Well, the weather this weekend hasn’t been very conducive to getting out anywhere anyway. So I have been doing the next best thing – plenty of cuddles with Pudding. Very much like a therapy pet, it is impossible to stay stressed for too long with that soft, warm snuggly body pressed into you.

Fairly difficult to breathe too when he’s lying on top of you, but I’ll take that side effect any day!

 

Shielding letters: at the start of lockdown consultants helped identify groups of patients that could be most vulnerable to the effects of Covid19. All diseases/conditions are described by a code – a bit like the Dewey Decimal system for books. So Pudding’s condition would come under Inherited metabolic conditions, then lysosomal storage condition, then mucopolysaccharidosis, then MPS II Hunter Syndrome. (Don’t quote me on this by the way – I’m not sure of the exact breakdowns, but using this just as an example.) But when the NHS database was accessed in order to send out these letters the data was not able to be broken down in such detail. Therefore, rather than sending out letters to patients with Hunter Syndrome who also have particular risk due to airway issues, a much wider group of patients were targeted. If you have a shielding letter but are not certain whether it should apply to you or vice versa, I’d recommend talking to your consultant to get more individual advice.

Trapped

We can’t go anywhere or do anything that I want to. You can forget about lazy foreign holidays or trips around historical sites. Life will just carry on around us and we’re stuck. Stuck in a rut that will only end in the worst way.

No I’m not talking about Covid-19, but my state of mind a few weeks ago. Although I do bumble along quite happily most of the time, the lows are still there and seem to hit harder sometimes simply because they take me by surprise.

This time, although I knew I was feeling miserable, I just couldn’t see that I was being unreasonably so. I was in a hole and couldn’t see my way out and when that happens logic flies away and it’s impossible to reach out for help. What’s the point? Nobody can help. Nobody cares. 

I was worrying about some aspects of Pudding’s care, but not sharing concerns with Hubby – he’s got to keep working to support us and doesn’t need more pressure on him – and at the same time resenting him for not knowing. And I was losing sleep over little things that I had no control over.

Like I said, logic doesn’t hold much sway.

So what snapped me out of it? A blogger friend of mine, Gemma from Isla’s Voice checked in on me as she often does. Just a simple message asking how I was doing. Maybe it just came at the right time to find a way through my barriers, but I found myself letting some of it out and sobbing as I wrote back to her. That night I was still holding the world at bay, but Hubby came and gave me a hug. Often when I’m feeling emotional/angry I’ll escape from contact as soon as I can, but he held on and my walls came tumbling down. I cried. Messy crying.

We talked. And the world became infinitely better again.

But it wasn’t until the weekend and our walk in the woods that I twigged what one of the main contributing factors had been. (I’m supposed to be intelligent, but hey…) No wonder I was feeling trapped. It’s not my family that is the problem. It was the weather. We had been pretty much trapped in the house every weekend for the last month by regular storms. Not easy to wrap up and head into nature when you’ve got wheelchairs and poor balance to consider. And I DO really need a fix of nature every so often.

Of course with coronavirus complicating the world right now, social distancing and self-isolation are the key words being thrown around and we may end up having to stay in again. But spring is around the corner, the weather is improving, and the garden desperately needs some work. I am determined not to feel trapped by this.

I only ever really write about our own story. I’m no expert and don’t feel qualified to preach to others or give advice. All I can say is that reaching out to others really can make a difference. Maybe not every time. Maybe sometimes you’ll be pushed away. But just maybe you’ll be the right person at the right time and you can help them out of that hole.

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.

 

Aware?

May 15th – MPS Awareness Day

I did intend to plan something this year. Something to raise money and get noticed. Make some noise and increase people’s awareness of mucopolysaccharidosis. It’s not really in my nature to draw attention to myself, even so I felt it was something that I should do.

But I haven’t planned anything at all.

To be honest, the last couple of months I’ve wanted to stick my head in the sand and pretend that MPS just doesn’t exist. But of course, my son doesn’t have that luxury. The effects that MPS has had on his brain stop him from knowing what it is doing to him. But I am very aware.

I am aware that his breathing is louder than it was, and he needs to rest more often again.

I am aware that many of his hard-won skills, such as pointing to head, toes or eyes, have faded away.

I am aware that he hasn’t grown in the last six months and is now on the 9th centile for his age (up until he was four he was on or above the 75th centile).

I am aware that he has lost most of the words that he was using.

I am aware that the treatments that should be stopping all this from happening are prevented from doing so by his own amazing immune system.

And most of all I’m aware of what all that MEANS. It means that unless something else can be done we are watching our vital, happy little boy die very slowly in front of our eyes.

Since we moved to our new house six months ago, the practicalities of our situation have been a lot easier to deal with, but emotionally the reality of MPS does hit me hard at times. So although I haven’t planned any awareness events, what I can do – what I have always done – is write.

I write about our experience so that others can learn about MPS; so newly diagnosed families can feel a little less alone. I write to save my sanity and hope that I spread awareness that way.

All the current research points to the fact that the earlier MPS is diagnosed the better; the few existing treatments (and new ones coming through) are much more effective early on while the body is still developing and before too much damage is done. So, please, for Pudding’s sake this MPS Awareness Day wear blue, share a post or two, donate if you can, and spread the word.

I’ll be wearing my blue wig and MPS T-shirt all day, and spreading awareness where I can. Who’ll be joining me?

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