Spending Christmas at GOSH

GOSH patient Evan with his mum Becky

This year, five-year-old Evan will be spending Christmas at Great Ormond Street Hospital (GOSH) with his family as he recovers from a bone marrow transplant to help treat a rare immune disorder. 

As part of our final day of the 12 Days of GOSH Xmas – sharing some of the hospital’s festive moments – Evan’s mum, Becky, tells us about their experience and what they have planned to celebrate Christmas at GOSH.

“In February 2017, Evan came down with a very nasty cold and temperature. The doctors thought it was a viral infection, but after a few weeks, he started coughing up blood and had a severe rash. He was prescribed some high-dose antibiotics, and that weekend, he started coming around. 

“But by Monday, he was coughing up blood again and had a temperature of 42˚C. We rushed Evan to A&E and he was admitted to our hospital’s children’s ward, where he received platelet and blood transfusions. 

“Blood tests revealed it was much more than a bad cold – Evan had a condition called HLH, where his white blood cells (T-cells) had become overactive and started attacking each other and the body’s organs, shutting them down. 

“We went to St George’s Hospital, where they noticed some other problems. They decided to send his blood tests to GOSH, which revealed that he had XIAP deficiency, a genetic condition where the body lacks protein to help fight infections. The original infection in February had triggered the XIAP deficiency in Evan’s body, which then caused the HLH.” 

Coming to GOSH 

“Evan began chemotherapy, but over the next two weeks he deteriorated rapidly. So we were admitted to GOSH, where we met Consultant Paediatric Immunologist Dr Austen Worth and his team. He knew a lot about Evan’s condition and told us that Evan was the ninth patient in the UK to be diagnosed with the XIAP deficiency. 

“We were in and out of GOSH a lot for the next few months. Initially, Evan responded well to steroid medication, but he relapsed after being discharged. Around his fourth birthday in October 2017, he was admitted to intensive care with sepsis – he had a line infection and his HLH had returned. That’s when we started looking for a bone marrow transplant (BMT) donor. 

“This year, we’ve been at GOSH almost constantly since May with other complications.” 

Having a bone marrow transplant 

“On 30 August 2018, Evan had his BMT. Just before the transplant, people at the hospital, including Evan’s Immunology Consultant, Claire Booth, and the Play team, helped to organise a party for him! Unfortunately, the actual transplant was followed by more complications, including more bleeding in his gut, a popped artery under his bowel, and a nasty virus that could have been fatal. 

“But, after a week, Evan started to respond to his medication, and we discovered that his T-cells (immune cells) had come through! Now he’s in Robin Ward (for children with immune problem who need protection from infection), sitting up like nothing has happened! He scared us – we really thought we were going to lose him.” 

Evan’s sisters 

“Evan is the baby of the family: he has two sisters – nine-year-old Brooke and 11-year-old Nadine. They’re amazing – they’ve had a few upsetting moments, but they generally just deal with it as best they can, by helping with Evan’s medication and asking questions about his condition. They know he’s in the right place. 

“We’ve been honest with them about Evan. We’ve said that he might not make it through, and that his XIAP deficiency will never be cured – it will require monitoring throughout the rest of his life. There’s no case the same, so we don’t know how it will pan out.” 

GOSH patient Evan in his hospital room

Support of GOSH staff 

“The staff at GOSH are wonderful. The doctors, the play specialists, the nurses – they’re so good at explaining Evan’s condition and treatment to him. 

“With the nurses, if I want to go outside and have bit of a cry, they’ll take me into a room and we can talk about it, or they’ll give me a cuddle. But you can also have a laugh with them. 

“It’s not just about the medical care that the staff give Evan – they’ll come and sit, socialise and play with him. That’s why we’ve got them all Christmas presents – we feel part of their family. At Evan’s pre-transplant party, a photographer snapped a picture of Evan and Austen together. Evan wanted to give that to Austen in a frame as a Christmas gift.” 

Christmas at GOSH 

“Evan loves Christmas, particularly decorating the tree and his Elf on a Shelf! He looks forward to the elf because he’s so naughty. Evan also loves all the food, although, sadly, he’s nil by mouth this year, so he won’t be able to eat. 

“The families on the ward come together – we all support each other. For Christmas Day, we’re all planning to go to the hospital’s restaurant – The Lagoon – for a meal, open our gifts, and in the evening, I’ve arranged a gathering for all the parents, and the nurses are welcome to pop in too if they have chance to. We’re going to have our own little party! 

“When we’ve spoken about the presents this year, Evan’s sisters have said: 'We don’t want any, we just want you and Evan home.' Evan has said the same. He’s excited about presents from Santa and spending it with the nurses on the ward, but he’d also like to be at home with his family.” 

The future for Evan 

“Evan can leave his isolation room for about an hour a day now because his neutrophil levels (a type of white blood cell that helps to fight infection) are really good, so he’s not high risk anymore. He’s been walking around the corridors, being cheeky to the nurses, and visiting the Disney Reef. 

“Beyond Christmas, it’s a ‘wait and see’ game. Come January, the doctors want Evan to start eating, but that will be a slow process to not make him sick. We’re currently looking at four to six months, but we don’t know. We don’t plan ahead now – we don’t want everyone to feel disappointed when we end up having to stay in hospital. We just take each day as it comes.” 

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You can share your own GOSH Christmas experiences with the hashtag #GOSHXmas.