Addy's Story | Julia's House

Addy’s condition is a fast thief. Our daughter is fading in front of our eyes. It feels like the two-year-old girl in the photos on my wall is not my child any more. To be honest, it feels like we need to have a funeral for one child and a celebration of a different child.

The life we imagined for Addy is different – those feelings of ‘I can’t wait to take her to ballet lessons and see if she enjoys it; I can’t wait to see her hold hands with her best little friends.’ Now we have a different child and we have to come to terms with that.

It’s a whole bag of loss and as time goes on there’s another thing that you lose. This horrible disease has stolen the Addy we knew and it does feel like grief for us.

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Worse than the worst case scenario

Addy was four years old when we discovered she had an ultra-rare type of Batten disease, called CLN2. It is a genetic disorder known as ‘childhood dementia’ and only around five children are diagnosed with it each year. The condition is causing Addy’s brain to shrink over time. A lot of her brain is already missing at the back – that was really horrible to see when we were first shown on her MRI scan.

We could lose Addy at any time from now until 10 years old. We’re going to Great Ormond Street every fortnight to try a medication that we hope will slow the regression down, but it may not help her quality of life. Last year, Addy lost her ability to walk and eat independently, and in time she’ll go blind and lose all her mobility and cognitive skills.

When the doctors first gave us the diagnosis, it was like having the carpet ripped out from under our feet. It felt worse than the worst case scenario. You tell yourself that it can’t be anything like that. I remember my husband dissolving into tears and saying, ‘We’re going to have to bury our daughter.’

I’ve had to learn to live with the reality of losing my beautiful little girl.

Re-building a life with Batten’s

As a baby, Addy was happy and smiley and she hit every milestone early, especially the physical ones – she was pulling herself up to stand when she was six months old. But when she was a toddler, she started having these big meltdowns and getting really angry for no reason. I would get so cross with her and I had no idea how to cope with this behaviour. Looking back, it was probably her not being able to communicate that she had a headache or her brain didn’t feel right. It’s hard to think back on those times when I didn’t know why she was doing those things.

Over the past three years, Addy has gone from this very able, very physical child to being mostly in a wheelchair and needing a lot of assistance to walk and crawl. She’s changed so much, it’s a shock for everyone when they haven’t seen her for a while. Even this morning, I felt myself welling-up because I could see Addy struggling to move her legs.

There’s not one place of life for us that Batten’s hasn’t touched or changed, and that’s mainly for the negative. It’s like we’ve had to re-build our lives from the bottom up.

Friendships have been changed because you don’t feel free to go to the same places and do the same things – it might not be wheelchair accessible or Addy might not be feeling well enough. Even emotionally for friends, some people want to keep their distance so they don’t overwhelm you but actually it can be isolating. And you’re struggling physically because suddenly you’re lifting all the time. Like at the moment, my shoulder’s hurting. We’re in this hard, hodgepodge of life and as Addy changes, we have to change with it.

Addy is a deeply affectionate child. She loves to cuddle, she loves to kiss. She’s got this cheeky sense of humour and this grumpy and cute face that she does. There’s lots of giggling and laughter in our home, which is really precious. Cognitively she understands what a joke is and finds things funny, which is precious. You don’t want to see that aspect of your child fading and actually we’re not seeing that aspect fade, we’re seeing it develop.

Having a child with a life-limiting condition is a journey of grief. You’re always trying not to let grief overtake you; you’re trying to step into life and embrace every moment.

Julia’s House is our village

That phrase of ‘it takes a village to raise a child’ – it feels like it takes 10 villages to raise a special needs child.

We’re very thankful for the different pockets of support we have. And Julia’s House is a massive pocket for us.

We first met Julia’s House nurse Sam only a few months after we had Addy’s diagnosis. Their nurses and carers were the only ones we were able to leave Addy with when her seizures were out of control. We couldn’t leave her with anyone who wasn’t medically trained. And it still feels like that now as Addy has a peg in her tummy to help her feed. Even though you can learn how to use a peg, it feels scary to do that on someone else’s child.

With Julia’s House, they just sweep in and say ‘Okay, you go now and take some time for you’. And the fact that they come into our home has been such a huge blessing too. Sometimes, Dave and I even go out for a ‘dinner date’, which is the only time we get to do that.

There for big brother Samuel too

Addy’s big brother, Samuel, also gets his own special visits at home from the Julia’s House sibling support team. He really looks forward to Tracey’s visits and they’ve built up a lovely relationship. I was really thankful that Tracey was there for Samuel to talk to and spend time with after we told him about the life-limiting aspect of Addy’s condition. He used to say, ‘when Addy gets better…’ and we realised that he didn’t really understand that she wasn’t going to get better.

Samuel is a really happy-go-lucky, easy to laugh kid, but he’s recently been suffering with anxiety. I think death is on his radar now. That’s hard – it feels like we’ve robbed him of some of his childhood innocence, but at the same time there’s no getting away from the truth of it. The more we talk about things and how we feel, we’re just keeping the wound open. It’s tough to know what the right thing is to do.

Bringing life to families in grief

We love going all together to the whole family sessions at the Julia’s House hospice in Devizes. Recently we said, ‘We feel tired, could we just have a sleep?’ The Julia’s House team made up the bed for us and put chocolates out for us, and we felt so teary because it was so special and such a gift to receive. When you feel so worn thin from caring round-the-clock for your seriously ill child, just to have a few hours to relax and sleep is such a blessing.

Everyone at Julia’s House is so personable, warm and kind. When people hear ‘hospice’ it can bring up so many connotations in your head. But Julia’s House is so much more than that – really it’s about life and not about death. It’s about bringing life to families, who are living in a place of grief. And the hospice is such a beautiful place with the open set up and all the lights and equipment. Oh, and the massages! It’s just incredible.

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