Understanding Noah's rare condition

Six-year old Noah has a rare genetic condition called Alagille Syndrome, which affects around one in 100,000 children from birth. It affects lots of different parts of the body, including the heart and liver, but the biggest concern for Noah is how the condition is impacting his kidneys. His mum Gemma shares their story. 

Bringing joy to Eagle ward staff

“We were referred to Great Ormond Street Hospital earlier this year to meet the consultants and experts for the next part of Noah’s journey. He was seen by cardiac, renal and liver doctors, and all of that preparation was building up to the removal of Noah’s non-functioning kidney, which took place while the Little Heroes series was being filmed. 

“We’re from Leicester, but the surgery Noah needed was very specialist and complex – his right kidney is attached to his liver via a collateral blood vessel, so we came to GOSH for this procedure.” 

Despite living with such a complex medical condition, Noah managed to keep the staff on Eagle ward and his mum Gemma entertained while he was at GOSH. He spent lots of time with the GOSH play team, and even hosted an afternoon tea in the ward’s playroom for Paul O’Grady during filming! 

It was in character for Noah to help keep everyone’s spirits up, as his mum Gemma describes: “Noah’s absolutely crackers! You don’t know what he’s going to come out with next, he’s so unpredictable, he’s a funny little boy. He brings lots of joy to our family. On dark days it’s always Noah that I go back to. He puts a smile on my face. 

“I did feel nervous about him having this operation, as it was such a big one. We knew it would affect our whole family with Noah being in hospital for so long. My two older children stayed at home with their dad because of school, and Noah missed them all a lot.” 

Raising awareness of Alagille Syndrome

Getting Noah ready for his angiogram - so his clinical team could learn more about his complex blood vessels - and preparing for his subsequent operation to remove his kidney was a multi-disciplinary team effort as his consultant Dr Jelena Stojanovic explains: “Our usual practice for this kind of complex patient is to admit the child a couple of weeks before the planned surgery and to have all necessary teams meet the family and do timely assessments to enhance the safety of procedures. 

“Noah had lot of blood tests and scans to give us a better picture of his complex blood vessels, and he was also seen by our cardiac specialists, anaesthetists and neurosurgeons ahead of the surgery. 

“The final decision is made in a multidisciplinary team meeting with all the different teams who are involved in Noah’s care, and in consultation with his parents too.” 

After recovering from surgery, Noah is now back at home in Leicester, and has returned to school which he loves, as Gemma explains: “He’s very bright and loves maths and reading, often when he gets home he’ll write down all his numbers and sums. The condition doesn’t seem to affect him in the sense that it’s all he knows so he just carries on with life as normal. 

“My hopes for Noah’s future are a happy, pain free life, and to have fewer hospital visits. I don’t want him to have the burden of doctor’s appointments and medications weighing on his shoulders. 

“I also hope that appearing in Little Heroes will help raise awareness of Alagille Syndrome. So many people have never heard of Noah’s condition, and if it prompts people to do some research or be more aware of rare conditions, that would mean a lot.”

Noah in wizard costume