Month: October 2019

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 We have withdrawn Pudding from the clinical trial that he has been on.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.

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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

On By huntersmumIn family2 Comments

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

On By huntersmumIn medical12 Comments

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.