Tag: emotions

A letter to Hunter Syndrome

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We’ve been together a while, you and I. And like any relationship we’ve had our ups and downs. Well, quite a lot of downs if I’m being honest. I’ve written enough about your bad side for you to have got the message by now.

I suppose you could call it a toxic relationship. In a situation like this, the thing people are supposed to say is ‘You should just leave!’ But I can’t. We’re stuck together for now.

It’s our ten year anniversary today. Did you know? People celebrate all sorts of dates for their anniversary. It’s not going to be a wedding in our case. But is it the day I first heard your name? The day we were informally introduced? Because of course, you’ve been around much longer. From the day Pudding, my youngest son, was born. The day he was conceived even – when a faulty egg wrote that error into his DNA. So there you were in our lives – uninvited, unwanted, unknown. I’d like to have met you sooner, had the chance to end our relationship before it took too much hold. But so far, I don’t have the power to change history. So I count today as our anniversary. The day that we sat in the consultant’s office and it was made official that we were now an MPS family. The first of July 2015.

Like I said, I’ve written so much about you. About how much I hate you, because there is oh so much to hate. You’ve taken so much from us – the ability to lead a ‘normal’ family life and the prospect of watching our son grow up and leave home to name but two. My 13 year old should be upstairs playing computer games with his friends, demanding endless snacks to fuel his growing body. But he can’t play computer games, he doesn’t have friends, he can’t communicate what he wants and he hasn’t grown since he was six and a half. Instead he is on the sofa next to me complaining about something that I can’t help him with because as usual once I’ve tried all the things that I can help with, I’ve no idea what it is. Maybe he’s just complaining about you. I don’t blame him.

I have tried to be fair, of course. I’ve also written about what you have given us. It was you that sculpted those features of his – the high forehead, his adorable nose – every inch of this body that I adore. You gave him that mouth that was so ready to grin and shout hello to the world (before you stole his smile away). You gave him those eyes that look into my soul and his soft curled fingers that grab mine to use as his fiddle toy.

What I’ve not really thought about much before is what you’ve done to me. Oh, of course there was the tsunami of emotions – the anger, grief, anxiety, and so on. You’re probably fed up of me going on about them. But without you, I would not be the person I am today. Ten years of knowing you has changed me in so many ways (and it’s taken a year on a counselling course with lots of self-reflection to make me realise that!).

I have my faults, I know. But there are the good things too. You have taught me to parent differently, for both my boys. You have taught me patience, with myself and others. Oh gosh, it’s such a cliche – you have made me stronger. Resilient, even. I know now that I can face the worst and make it through. I share my pain so that others might feel less alone. These days I even dare to admit I can be brave when I need to be. Most of all, I have become comfortable with who I am – the whole me, flaws and all. Someone who is never going to break the mould or change the world, but who can sit with pain and take pleasure in simplicity.

I really quite like this version of me. So when it comes down to it, I guess what I’m trying to say is… thank you?

Happiness

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I am happy.

It feels weird to say that on this blog, and also slightly wrong. Like I shouldn’t be.

When my son was diagnosed with a life-limiting condition back in July 2015, I was thrown into a maelstrom of emotions. Most of all, I couldn’t imagine ever laughing again, let alone feeling the deep contentment that I’ve settled into lately. But I guess that’s the thing about emotions – they don’t hang around forever.

Now, I have so much to be happy about: I have a lovely house and garden; strong family support around me; T is growing into an interesting and studious young man who frequently makes me laugh out loud with his quirky take on life; I’ve been able to get into more acting again; meditation has changed my outlook quite a lot; and going on HRT has also made a huge difference in eliminating some of the anxiety that I’d come to think of as normal for me. After two years of writing a gratitude diary every night I certainly don’t struggle to find three things to put down each day.

And then there’s Pudding…

When we got his diagnosis I was already struggling to deal with his whirlwind antics on a day to day basis. And now came the punch punch punch that MPS brings. Heart problems, general anesthetics, weekly trips to Manchester for enzyme treatments, issues with airways, joints…. Through all this there was the knowledge that he might be one of the unlucky ones, one of the ones with progressive Hunter Syndrome who will lose all his hard-won skills and die in his teenage years. That was confirmed in October 2015.

Then we were onto the next roller coaster of a clinical trial – the shaky hopes as we were hauled to the top and plunging despair as the reality of his decline kicked in, the impossible decisions that we were forced into making.

A close up. Boy with features of Hunter Syndrome, reclining in a disability buggy looking off to the side.

Now, all the hope has gone. He no longer has any treatment, only symptom management. We know that the inevitable will come to pass. Short of a miracle happening we will continue to lose him bit by inexorable bit. Anyone looking in from the outside might expect our situation to be much bleaker than the times I have described above. But with the hope gone we’ve also lost the deep lows. With the loss of Pudding’s abilities we’ve also lost the challenging behaviour. We’ve not necessarily stepped off the roller-coaster – I know there will be further ups and downs ahead – but most days we coast along fairly happily. Back then, I saw the future as a constant bleak decline, but in reality progress is more step-like with a sudden change followed by months of stability where we can settle into peace. After many years of saying I need to learn from him, to live in the moment, I’m pretty much there. And we have many beautiful moments.

Of course, it’s not all sweetness and light. Even Pollyanna had her down days. Ironically, reading the posts that I’ve linked to above I’ve just been crying again, but they’re tears for the me that was writing then. The me who was having to deal with all that. There are days that I cry for the future – a conversation with a doctor at Martin House triggered a vision of saying our final goodbyes to my baby. And there are other days when I cry for the now – when Pudding is unhappy and I don’t know why and can’t fix it, no matter how much I want to. Those times will undoubtedly become more common as we get further on.

But until then I am happy. In the moment.

Arrival

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Warning: contains plot spoilers for the film Arrival

For a couple of years now we’ve had a family tradition – every Friday once Pudding is in bed, we settle down to watch a film together, taking it in turns to make the choice.

A few weeks ago we had a visitor from another MPS family staying with us. Evan is travelling the world on a fellowship, finding out how a diagnosis of mucopolysaccharidosis affects families like ours and learning from clinicians and policy makers who are trying to make a difference.

Although I was a little nervous about having a complete stranger to visit, he slotted right in to our lives – playing chess with T, discussing music with Hubby and having some beautiful moments with Pudding. He wanted to be treated just like one of the family, so of course we gave him the choice of film that week. This was a responsibiliy he took very seriously, considering what we’d seen previously and what would suit us all. A film about aliens arriving on earth and the difficulties of communicating with them seemed perfect, so we sat down to enjoy Arrival.

The opening scenes were a bit of a reality-check for us all. The protagonist, a linguistics professor, separates from her husband and watches as her 12 year old daughter dies from an incurable condition. A little hard-hitting when we’re facing the same situation ourselves. (Not the separation, don’t worry!)

Our guest was mortified and immediately suggested that we could watch something else, but we figured that it was just a back story and wouldn’t feature again. We were wrong. Towards the end, visions of her daughter return and it becomes apparent that contact with the aliens has enabled her to experience time differently. She is actually seeing visions of the future and now faces the choice of whether to go ahead, knowing what will happen. Evan groaned that he couldn’t have picked a worse film for us. But I don’t think that’s true.

The choice faced in the film has stayed with me over the weeks since. What would I do….?

I’ve written in a post previously that if I’d known Pudding’s diagnosis during pregnancy, I might instead have chosen to have an abortion. That I’d rather spare us all the heartache and loss that his MPS will mean. How could I bring a child into the world only to also give him suffering? How could I do that to us and other family members?

Pudding peering around a tree with a huge cheeky grin.

The point of the film though was that she could see the loss and the heartache that was coming. And that was almost too unbearable to contemplate. But she could experience all of the rest of it too. The love. The joy. The wonder. And the chance of those was too much to turn down.

And I’ve been torn. What would I do…?

When it comes down to it, there could only be one answer. I’d choose the tears. I’d choose the moments of contentment. The joy. The smiles. The curly-headed whirlwind. And the snuggly boy who purrs warm breath on my shoulder. I’d choose heartache that only comes from love. I’d choose life.

I would choose you, Pudding. Every time.

They will never know…

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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

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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

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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

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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

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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

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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

On By huntersmumIn medical12 Comments

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.

OLYMPUS DIGITAL CAMERA

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.