George has forgotten dozens of words and lost the ability to walk
George Young is one of Britain’s youngest sufferers of the disease, which has already began to rob him of his childhood and will eventually stop him growing up.
George was a happy, healthy child who had just started to walk and feed himself when he began having seizures 18 months ago.
Doctors initially thought he had developed epilepsy, but further tests showed he was suffering from Batten disease – an extremely rare form of childhood dementia.
His condition rapidly deteriorated. George has now forgotten dozens of words and lost the ability to walk.
There is currently no treatment for George’s strain of the condition. He will eventually have to be fed through a tube in his tummy and is unlikely to live past his late teens.
Fighting back tears, Claire, 35, said: “He was just at the age where he had mastered all of those skills that we spent time teaching him – his walking and his eating and his language. To watch those all unravel for him was really difficult.
“It’s horrible to see him being robbed of his childhood.”
While dementia typically affects people aged over 65, Batten disease is an incurable inherited disorder of the nervous system that typically begins in childhood.
It leaves children suffering cognitive impairment, seizures, and progressive loss of sight and motor skills.
It’s horrible to see him being robbed of his childhood
Early symptoms of the terminal illness appear between the ages of five and 10 years and parents have to endure the agony of helplessly watching their child’s rapid decline.
Claire, a solicitor who is separated from George’s father, does not know how much time she has left with her son.
Yesterday she told the Daily Express how she is determined to cherish every moment with him. She has devised a “bucket and spade list” to give him as many experiences as possible before his condition deteriorates further.
Claire, of Kenilworth, Warwickshire, said: “I wasn’t aware of childhood dementia. You especially don’t expect it to happen to one of your own.
“Apart from the symptoms you can already see, I can’t believe what’s going to happen to my little boy.”
Claire is backing the Dementia Strikes Children Too campaign. Visit childhooddementia.co.uk to find out more.
Claire does not know how much time she has left with her son
CLAIRE YOUNG…A MOTHER’S STORY
I WOULD never not have George in my life in whatever capacity, whether he was well or he had the condition. In that sense I don’t care about the condition.
But it is ravaging my son and it completely destroys families. Since the seizures started 18 months ago his deterioration has felt incredibly quick.
The hardest thing for me is the unpredictability. Because it’s a rare condition the professionals don’t have the information available to be able to predict a pattern or a prognosis.
When you are faced with that fate, all you can do is make the best of it and make the most of the time that we have with him, because otherwise I think you would just lose your mind.
‘He’s so brave and determined’ says Claire
Nights are very unpredictable. It might take him hours to get to sleep or he might wake up in the middle of the night.
He has seizures every day. Some days he has very few, then on other days it could be upwards of 20.
To begin with his seizures were very distressing. You then become used to it – and it’s good to feel that in a sense, because then if he does have a particularly big seizure you are more prepared to cope.
He’s so brave and determined, it hardly seems to bother him. He has it and then he gets on with whatever it was he was doing before.
He’s amazing in that sense. I wouldn’t ever give up on him – no parent would. All he has to do is look at me and he’s got me and that’s it.
The life expectancy of a sufferer is around 13
GENE THAT KILLS BY 13
SEVERAL groups of neurological conditions cause dementia in children, including Batten disease.
Sufferers born apparently healthy develop seizures and dementia.
They lose vision and the ability to walk, talk and eat, becoming wholly dependent on their carers for the rest of their short lives.
The life expectancy of a sufferer is around 13. The gene CLN8 is passed on from two unaffected parents.
The Batten Disease Family Association estimates about 150 UK children and young people have the condition.
Professor Paul Gissen, of Great Ormond Street Hospital, said: “It is much less well known that dementia can also affect young people. Often these children have rare neurological conditions caused by genetic faults, such as Batten disease.”