When George was two years old he could walk, talk and play. His mum couldn’t have predicted that just two years later he would start to forget how to do all those things and have to use a wheelchair to get about.
“He used to run everywhere, he would get out of the car and just take off. I thought that was just him being funny, but now I think, on some level, he knew he only had a certain amount of time to do all of that,” his mum Claire Young, 35, from Kenilworth, tells HuffPost UK. “He was trying to pack in as much as possible.”
George has Batten Disease, a neurodegenerative condition, which causes children to suffer from dementia. It’s estimated there are between 100-150 children and young people currently living with this diagnosis in the UK.
George’s life skills will slowly deteriorate, yet the rate at which this will happen is unknown as it varies from child to child. Claire also doesn’t know how much time she has left with her son, as she has been told his life expectancy is around nine or 10. Maybe 12 at a push.
“It’s very difficult so we are led by George,” she says. “I feel it has been incredibly quick to see such a deterioration in him. It’s very much a case of taking each day as it comes and making the most of those days, because everything could change in a second.”
George started off developing like most other children his age. At his peak, he had around 20-30 words in his vocabulary and could interact with his family.
The first signal that there might be something wrong came when George was three. He had a seizure at nursery and was immediately taken to hospital where tests were carried out. He was diagnosed with epilepsy, with the caution that it was likely to be part of a bigger condition.
After more testing, Claire was told in February 2017 that George had Batten Disease and that one of the main symptoms was dementia. She hadn’t heard of the condition before. “Dementia is instantly associated with the elderly and it’s not something you think younger people will develop, so when we were told about it and what the future would hold, it was devastating,” she says. “No parent should have to hear that news.”
At the time, Claire racked her brains to try and think of any symptoms George displayed when he was younger, but says it would have been “impossible” to put two and two together.
She does recall times when he got overly confused or distressed, which she had put down to his age. When they used to drive past his nursery, George would often look at it and cry. “I thought he loved his nursery so much so he wanted to go,” she says. “But with hindsight, he was distressed about the feeling of going somewhere new and having a change of routine, as with dementia he can get confused very easily, which upsets him.”
Since the diagnosis, George’s abilities have dramatically reduced. He can no longer speak and instead communicates through showing emotion. He started to struggle with eating, so recently had a feeding tube inserted into his tummy to make sure he gets enough food, “That’s been really difficult,” adds Claire. “He loves his food and gets so much enjoyment from it.”
One thing Claire says is promising is that George hasn’t lost his memory of who the people around him are. “Thankfully he isn’t showing any sign of that at the moment, but it can be a symptom as the condition develops,” she says.
There is no cure for Batten Disease and treatment options are limited. On a day-to-day basis, Claire says managing George’s condition involves therapies that keep him comfortable and palliative care.
He has quite a busy schedule of appointments: He sees a community nurse weekly, has a therapist and regularly sees an ophthalmologist to keep an eye on his sight. He also sees a nurse who monitors his swallowing and sees a senior consultant every few months at hospital. While at school he has physio every week to keep his muscles strong and mobile.
But despite the difficulties George faces, Claire says he still has the same desire for fun as other children his age. She calls George a “happy, bubbly and brave boy” who thrives off adventure and excitement. He gets excited every morning when Claire tells him he’s going on a bus to school his special needs school, Ridgeway in Warwick, where there is a soft play room, light sensory room, hydrotherapy pool and a huge trampoline. “It gives me such comfort to know he’s in such an amazing place,” she adds.
George also absolutely loves to swim. Claire thinks it’s because of the freedom he gets in the water, as he’s able to move more freely. “He’s such a cute little boy,” she says. “He loves being outside, getting dirty and making a mess.
“He loves cars, anything with wheels, trains, and Peppa Pig, too. He’s still able to enjoy all of those things and experience those sensory elements of being outside. He is able to find joy in the things that he does and the things around him.”
Claire says the national Batten Disease charity, BDFA, has been “instrumental” in connecting her family with others who have children with the same condition. “That sort of insight is invaluable to us,” she says. “It’s incredible to have.”
The family have also been in touch with the Make A Wish charity, which aims to grant wishes to children who have long-term illnesses. “Obviously I can’t get a wish out of him, so I had to think of something that would get emotion out of him and make him giggle,” she says.
As George is an adrenaline junkie who “loves anything fast”, they’ve decided to go tobogganing in Austria. “I’m so excited, I can’t wait to see his reaction,” Claire says.” I hope it’s as exciting for him as I think it’ll be.”
If there’s one thing Claire wants people to take away from her story, it’s to learn about what Batten Disease is and how it affects families. “It’s so unknown, but if one more person knows about it from me speaking about it, then that’s a job done really,” she adds.
Claire and her family are raising money for the Battens Disease Foundation on JustGiving. Find out more about the condition here. Claire is also backing the Dementia Strikes Children Too campaign. Visit childhooddementia.co.uk to find out more.