Ziggy loves coming to the Julia’s House hospice! When he first arrives, he’ll dart from one room to the other, exploring all the activities and toys. Often, he likes to sit and play Lego with another boy who struggles with his sight, and they have a lovely time together.
It’s so nice for Ziggy to be able to bond with other children his age at Julia’s House. I think at school he struggles a bit because they’re all so mobile. Ziggy can run but you’d think he was a toddler running, not an eight-year-old. He doesn’t play football or anything like that at school – he likes the quieter activities. And at Julia’s House, he can just sit and play, and he can be himself; he doesn’t have to worry about trying to be anybody else because everyone is accepted there.
Not ‘just a sicky baby’
Ziggy was born with an ultra-rare genetic condition, but we were unaware during pregnancy. We began to realise something was wrong when he was around three weeks old. He’d scream 24/7 and vomit after every feed. We had lots of people involved, and they all concluded that Ziggy had really bad reflux and was just a sicky baby.
Then when he was about four months old, Ziggy vomited blood. He was so dehydrated, doctors couldn’t get a good blood sample from him. We were sent home and told to come back if he got worse.
I managed to rehydrate him by syringing water into him, and he did seem to pick up. But just a few days later, Ziggy turned blue in my arms. He practically stopped breathing and went completely limp.
I felt sick. I don’t think we’ve ever driven to hospital quite as fast as we did. We hurtled through the main part of A&E and said ‘He’s dying, he’s dying!’ They just took us straight through to resuscitation. It was horrible.
Within 30 minutes, they concluded that Ziggy was in complete renal failure. His kidneys had stopped working.
Months of life support
For the next five months, Ziggy was on life support and constant dialysis in the intensive care unit while doctors tried to work out why his kidneys had failed. All the time, he was intubated and sedated, so we couldn’t hold him. He was just so poorly, he couldn’t breathe for himself and his blood was so poisoned from not having any kidney function for so long.
Until that point, I had been breastfeeding, and we’d always had that closeness. It was heart wrenching to hand over all the care of my baby to someone else, and not be able to cuddle him for that time. I would take his bed covers home to be washed, just so that I could do something for him. I wanted him to have that smell of home, not hospital detergent.
A shock diagnosis
Eventually, they called in a geneticist and she took a blood sample to be analysed for any genetic conditions. About six weeks later, we got called into a little room for the results. The geneticist said to us, ‘What I’m going to tell you has been a complete shock to us.’ She explained that Ziggy was born with an ultra-rare genetic condition called infantile primary hyperoxaluria type 1, which caused an enzyme to be missing in his liver. More tests revealed that I was unknowingly a carrier for the condition.
Without this enzyme, little oxalate crystals had built up in his tiny body, like limescale building up in a kettle. The crystals had got into his bones, his heart, his eyes, his kidneys, almost everywhere – causing devastating damage.
We were told that less than 20 years ago, Ziggy’s condition would be classed as terminal; palliative care would be our only option. But in more recent years, medicine had advanced, and they’d experimented with liver and kidney transplants.
The risks were high though. The majority of the children with Ziggy’s condition had not made it through both transplants. We had no choice. It was either take the risk, or accept palliative care from then on in.
To give Ziggy the best chance of survival, he needed a liver transplant from a living relative. It wasn’t possible for me to be a donor – it was too much of a risk as I had the faulty gene. Colin put his hand up straight away, but Ziggy needed to be bigger to take the adult liver lobe.
So, for the next year we travelled six days a week from our home in Dorset to Bristol Hospital to give Ziggy the dialysis he needed. That was our life; two hours in a taxi to hospital, four hours of dialysis, two hours in a taxi home. Our one day off was Sunday. It was horrendously tiring, but at least Ziggy was alive.
Julia’s House caught us before we hit the floor
It was around this time that we were first introduced to Julia’s House.
The nurses would come to our house, so I could take a break and have a cup of tea. And they would just sit with me and talk things through. Because when you feel like you’re fighting to keep your child alive, it’s very easy to get into a spiral where you think, ‘this is the end of the world, I can’t see a way out, it’s all too overwhelming.’
Julia's House is like a little net that catches you before you hit the floor.
The nurses understand what we’re going through. They help me to de-spiral and see that there is light, and they give me that little bit of strength to keep going.
To be honest, I don’t know where we’d be without Julia’s House.
Everything went horribly wrong
By 18 months old, Ziggy still wasn’t thriving. Despite his heart not being strong enough, we were told that he could be in a worse position for the transplant if we waited any longer. They said there was a 50:50 chance of him coming out of surgery alive, but that was our best option.
So, we travelled to Birmingham for the pioneering surgery – Colin at the Queen Elizabeth Hospital on one side of the city, and Ziggy in the children’s hospital three miles away. I had 24 hours when both people I loved the most were in theatre. And I couldn’t be with them at the same time. It was horrific; I felt so torn. I travelled by train to see Colin when he came out of theatre and then got back to the children’s hospital and Ziggy for when his liver arrived there.
They both had very long operations and went into ICU afterwards. It was a painful recovery for Colin, but for a while everything looked promising. It seemed like the surgery was a success.
And then two weeks after the operation, everything went horribly wrong. Ziggy caught sepsis and his Hepatic artery thrombosed; we were told he had 48 hours to live unless he got another liver.
It was probably worse than when we first received Ziggy’s diagnosis because we thought we were going forwards and then suddenly we were back down in this huge hole and everything was falling apart. For Colin, who was in a huge amount of pain still, it suddenly felt like he’d done it for no reason and the part of the liver that he donated had been wasted. Emotionally this was torture for us both.
An agonising wait for a new liver
We had to focus on Ziggy and getting him well. He went on the emergency cadaveric transplant list and we waited and we waited. And we suddenly got a phone call, and we were told that someone had very sadly passed away in an accident and a liver had become available that might be appropriate for Ziggy.
By this time Ziggy was very ill. He was fighting sepsis, his blood pressure was through the floor, he was a really poorly, sick little baby.
The first time he had the transplant, he had a 50:50 chance, so the odds this time were incredibly low. But what choice did we have? We were going to lose him either way. So we had to try, didn’t we?
So, Ziggy went back down to theatre and our incredibly amazing, strong, resilient little boy came back from theatre with a second liver transplant.
When he was well enough to come home, Colin and I became trained dialysis nurses so we could give him dialysis at home, rather than travelling to Bristol Hospital every day again. Ziggy was the smallest child ever in the UK to have gone onto home haemodialysis. We did that for a just over a year until he was big enough and strong enough to undergo a kidney transplant at the age of three.
Despite everything he’d been through last time, Colin incredibly put himself forward again to donate his kidney. And again, we headed to Birmingham. But this time it was in the middle of COVID, in 2020. So, I had to drop Colin off at the door with his bags, give him a kiss and say goodbye. It was a very scary and emotional moment – we had no idea when we would all see each other again. In the end, I was in hospital with Ziggy for six weeks, with just video calls for contact.
Beating the odds
Against all the odds, the kidney transplant went really well. Ziggy started weeing on the surgeons table, which showed that it was actually working! And since then, we’ve been dialysis free.
Shortly after his kidney transplant, at the age of four, Ziggy learnt to walk for the first time. It was an incredible moment! He even goes to a mainstream school now. But there are lasting effects from his condition, and he only has half the kidney function of a healthy person his age.
He’s more susceptible to infection, he has partial eyesight, and his bones are brittle so he can't move or play football like other children his age – he still runs like a toddler would. Because of his transplants, Ziggy is also vulnerable to serious and life-threatening complications and has to have regular tests.
Fun, play and peace of mind with Julia’s House
For a long time, I was really averse to Ziggy going to the hospice. I think because the word ‘hospice’ to me meant end of life, and I wasn’t in the right headspace to even think about that. So, for many years, all our support was community-based, and it was brilliant. Julia’s House would come to our home – always two nurses for a very long time because Ziggy’s classed as high risk – and they’d generally do a Friday afternoon for us.
Colin and I don’t work on a Friday, so when the Julia's House nurses come to look after Ziggy at our home, it means we can go for a cup of tea and a piece of cake, and just chat. It’s a bit of time just for us. And we know the whole time, Ziggy is getting the best care and a huge amount of fun and 1:1 play, which is what he really craves – having someone that’s focused entirely on him.
Now, Ziggy goes to the Julia’s House hospice once a month as well, and it's lovely because Colin and I spend a whole day out. We book ourselves a nice meal, we go off and I know that Ziggy’s having an absolute ball. After his first visit to the hospice, I picked him up and saw just how much fun he’d had, and that sealed it for me that I’d finally made the right decision to let him go.
Since then, Ziggy’s even had a couple of sleepovers at Julia’s House. And that’s the only place he’s ever slept away from us, other than hospital. We’ve got nobody else I can leave Ziggy with who would be trained to deal with a medical emergency. It’s the ‘what ifs’ - what if he takes a sudden turn in the night, you know? I know that at Julia’s House, there’s somebody with their eyes on Ziggy the whole time. He’s eight years old and I’ve still got a baby monitor in his room because I need to be able to hear him breathing and know he’s okay.
It’s hard when you’ve been a ‘medical mum’ - a nurse basically – to switch off and to trust anybody else with your child, who isn’t medically trained. But I do trust the Julia’s House nurses completely with Ziggy, and it feels like such a weight is lifted off when I can get that break.
Ziggy's been through so much – he's getting on with life and he’s happy now – but he could go downhill again very rapidly. We don’t know what lies ahead for him, and it feels like actually we’re just at the beginning of a whole new journey.