Henry (4) has the incredibly rare, inherited blood condition, Diamond-Blackfan Anaemia. Since diagnosis, Henry’s parents have been on an emotional rollercoaster having to take him for blood transfusions every three weeks and then a bone marrow transplant. Henry’s Roald Dahl Specialist Nurse, Clare, has been by the family’s side throughout. We caught up with Henry’s mum, Zoe to find out more about the impact of having a seriously ill child and why having the support of a Roald Dahl Specialist Nurse is so important:
“We’d just be completely lost without Clare. She’s our person and we go to her when we need anything. I don’t know what we would do without her. She’s an instrumental part of our hospital lives. There’s no one else who could take her place that does the job she does.”
pregnancy scan. They could see that Henry (now 4) was quite small and had fluid accumulating around his body. The consultant said that Henry was either going to have a genetic condition or a severe heart condition and that he had just a 20% chance of survival over the next two weeks of the pregnancy. We were given the option of having a termination or to wait for the worst – it was very stressful. They then offered a placenta biopsy to try and figure out what was wrong, but the genetic results came back clear. We were referred to UCL and I had fortnightly scans. Somehow, Henry managed to hand in on there even though he was looking poorly on the scans and no-one could say what was wrong. We made it to 36 weeks and then Henry was born by emergency C-Section.
As soon as he was born, he did cry but he was extremely pale, so they whisked him away to test his blood and found that he had an extremely low haemoglobin level of 42 (the normal level is 200). He was so anaemic that he was going into heart failure, so they gave him three blood transfusions straight away. They put him in an induced coma and on a ventilator. That’s how it all began, and it hasn’t got any less stressful since then!
After three weeks in intensive care, we took him home but were still none the wiser as to what was going on. We were told to bring him back to hospital if he looked poorly which put a lot of pressure on us to keep a close eye on him. It was when he was four months old that he was referred from our local hospital in Colchester to Addenbrookes in Cambridge, which is where our Roald Dahl Specialist Nurse, Clare is based. Our consultant, Anne did a bone biopsy on Henry and it came back that he had the extremely rare, inherited blood condition, Diamond-Blackfan Anaemia (DBA), which affects the
Henry remained blood transfusion dependent and had to have one every three weeks. When he was 18 months old, they realised that he had built up a dangerous amount of iron from his blood transfusions, so they needed to start treatment to strip the iron out of his organs. We had to insert a needle into his leg or tummy daily to hook him up to medication overnight. Even though he was little, he was fully aware of what was going on and he hated it. It was awful. We did that for a year and a half and then they said he would need to go on an iron chelation pump 24 hours a day, seven days a week. We said, we can’t do that to him as his quality of life would be severely affected. The only other potential treatment available was a bone marrow transplant.
Henry had the bone marrow transplant last year when he was three and a half. The transplant went ok but watching him go through it was hard. He had to have a high dose of chemo and seeing him lose his hair wasn’t nice. We had to be in isolation for such a long time. Our Roald Dahl Specialist Nurse, Clare really came into her own once Henry had had the transplant. We were at Addenbrookes where Clare is based twice a week and have now seen an awful lot of Clare! She’s been a godsend, amazing. I don’t know what we would have done without her.”
Photo: Henry's Roald Dahl Specialist Nurse, Clare with her fellow Roald Dahl Specialist Nurses, Zoya and Katrina at Addenbrookes Hospital in Cambridge.
Tell us about Henry? “Henry has an eclectic taste! He has always been into Halloween and anything horror related. I’m not sure if it’s because he’s endured so much trauma. His favourite film is Tim Burton’s Nightmare Before Christmas. He also really loves Willy Wonka and Charlie and the Chocolate Factory. He has a golden ticket pillow and wants to work in a chocolate factory when he’s older. He’s even made up some chocolate concoctions that he wants to create in real life. Now that he’s feeling a bit better, he is so active and very outdoorsy. We are lucky to live 10 mins from the beach and he loves the woods and playing outside. He also loves water but wasn’t able to go in for year and a half due to having a central line fitted (catheter placed into vein to administer medication). It’s been taken out now, so he is in the sea, bath, paddling pool as much as possible. He’s a happy little boy. He’s always been happy despite everything that’s happened – he’s remarkably resilient and happy.”
What impact has having a child with a lifelong health condition had on you and your family? “It’s been lifechanging for us. There’s not a single aspect of our life that it hasn’t affected. I had to quit my job to look after Henry and I never intended to be a stay at home mum. I’m sort of ok with it now but it’s taken a while. It has impacted me mentally, emotionally and financially. It’s been the same for my husband, Craig. He’s had to be sole earner and we’ve had to make a lot of sacrifices financially. Emotionally it’s so hard. We had to stay in isolation for so long and we weren’t able to see people. Nothing has been how we had hoped it to be or how we intended it be. It’s been difficult but it’s given us a new perspective on life that I’m grateful for. As hard as it’s been with Henry being poorly and watching him suffer, given they wrote him off and gave him such a low chance of survival, seeing him defy the odds and watching his resilience and his will to survive has been amazing.”
How has your Roald Dahl Specialist Nurse, Clare helped you cope with these challenges? “Henry absolutely loves Clare! Despite all of the horrid things that happen to him, he loves going to Addenbrookes hospital and seeing Clare. He has an absolute blast when he goes there. His face lights up when he sees Clare and vice versa and that’s really lovely. It’s so important – I couldn’t wish for better care. I would find it really difficult taking him to hospital if he didn’t have such a good relationship with Clare and the team. They’ve had to do some really horrid things to him and knowing he is in safe hands is so reassuring. They treat him like their own. They put so much effort in and make it seem so effortless. They keep him going. Sometimes, there are days when he wakes up crying and he doesn’t want to get out of bed because he feels poorly. He hasn’t been able to see other children, and the nurses, especially Clare have become his friends and extended family. They encourage him and jolly him along when we can’t get through to him. It’s amazing. We are so grateful for that. You can’t underestimate how important that is for us. Having a poorly child and knowing you can’t fix them and having to entrust your baby to others is such a huge deal but the fact that our Roald Dahl Specialist Nurse Clare and the team treat him with such kindness is lovely.
Whatever I need and whenever I need it, I can phone our Roald Dahl Specialist Nurse, Clare and she’ll get it done. 90% of the time, she can answer my questions herself but if not, she gets hold of a consultant and finds out for us. Knowing she is there and is our person of contact is like having someone as a security blanket. She is always there, and she always knows the answer. Anything we need in terms of medication or half the time when we need reassurance, we can phone up and say ‘this is happening; is this ok? Or should I bring him in?’ It was particularly hard when he’d had the bone marrow transplant and we had Henry at home and had full responsibility for him. Having access to Clare to ask when we didn’t know about something and having her be able to tell us it was ok was so important.
Before we had Clare’s support, it felt like we were drifting a little bit. Obviously, we knew the doctors were there for Henry’s care, but it felt like we were having to jump through hundreds of hoops to try and speak to them. Now we have Clare, we have someone we can get in touch with easily who has immediate and direct access to the doctors and that’s amazing. It’s massively reassuring. We know that she’s our safety net.
For example, I have phoned Clare so many times over the last year when I was worried that Henry’s blood counts weren’t ok. Henry had complications where his donor cells started attacking his cells and it seemed like the bone marrow transplant was in jeopardy. I could ask her what she thought, and she would take a look and say, ‘if I’m not worried, you don’t need to be worried.’ Henry has to go to a number of different hospitals and specialist teams as the transplant was done at St Marys Hospital in Paddington and we are still going to our local hospital in Colchester as well as Addenbrookes for some appointments. He has urology issues so is due for surgery for that and we are also under Royal Brompton for a heart condition that Henry has as part of his DBA and we were also going to Great Ormond Street at one point for genetic testing. Having to work with all of the different hospitals and teams was a nightmare before we had Clare. She organised for scans to be done at Addenbrookes rather than Royal Brompton during COVID and she will pull everything together and book all his appointments under one roof, which is massively helpful. Clare has done so well to manage me and my anxieties. I don’t know what we would have done without that especially when Henry’s blood counts dropped. Clare would say, ‘don’t worry, we are keeping an eye on him. We’ve got your back’ and that’s all I need to hear. She takes a lot of the stress away.”
How would you feel if you didn’t have your Roald Dahl Specialist Nurse? “We’d just be completely lost. She’s our person and we go to her when we need anything. I don’t know what we would do without her. She’s an instrumental part of our hospital lives. There’s no one else who could take her place that does the job she does.”
What would you say to the people who have donated to Roald Dahl’s Marvellous Children’s Charity and helped fund your Roald Dahl Specialist Nurse? “Thank you isn’t enough. Unless you’ve been in a similar position to us, it’s really hard to understand how difficult having a really poorly child is, especially a poorly child with a really rare condition that no one has heard of. Having someone like our Roald Dahl Specialist Nurse, Clare to guide us through all of it, stand with us and fight for what we need is amazing. I don’t know what we’d do without her. I would say a massive thank you. I don’t think people understand how important it is that they have donated.”
If you would like to help us help more seriously ill children like Henry, please donate here: www.roalddahl.com/donate