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.


16 thoughts on “Decisions

    1. Kevin willetts

      You will choose wrong. It is inevitable. There is not a correct decision. My boy will decline whichever method is used so it is easy for me given the choice to choose quality over quantity because everyone can be taken at anytime without notice. Lots of of love and kisses. xxx

      Liked by 1 person

  1. Malin Sjöblom-Dormer

    Oh no, this is devastating to read, let alone going through… I can only imagine. I don’t know what to say really… Many hugs xxx


  2. Lisa Patrick

    I had to make the same choice with my son and it was the hardest decision I’ve ever had to make. The treatment just wasn’t working for him so we took the decision to stop and made the most of every single day that we had with him. Sending my love to you x


  3. Carole Irwin

    How brave you are to share your feelings so openly with us. I have nothing but admiration for you.
    You may just choose the option that is right for Pudding.
    No one can walk in your shoes to advise you.
    I wish all your family all the best and know you will make the correct decision for your gorgeous son.
    Sending love and gentle hugs to your family


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