A BRIDGWATER grandfather donated £3,100 of winnings to a charity close to his heart earlier this month.

John Slee won the money from a raffle at The Bunch Of Grapes pub in Bridgwater, and chose to donate the funds to the Batten Disease Family Association (BDFA).

The charity is important to John, after his grandson, Isaac Tilley, was diagnosed with CLN2 Batten Disease in August 2021, also known as childhood dementia.

Isaac, five, was diagnosed after suffering from a range of symptoms, including seizures, loss of mobility and delayed speech.

Isaac's mum, Aimee, said raising awareness of Batten Disease is 'so important'.

"This charity is extremely close to our hearts as my son was diagnosed with CLN2 Batten Disease back in August," she said.

"Since then the BDFA have been a part of my life, whether it’s been a voice for us as parents in meetings with NICE or just a quick call in which they’d check in to see how I am doing.

"They also fund research into Batten Disease which is needed to find the cure my little warrior so desperately needs. Without the cure, Isaac will lose his precious life far too soon.

"Batten Disease may be rare but it’s here and it’s killing our children, the earlier children are diagnosed (specifically with CLN2) the earlier they can get access to treatment and enjoy an improved quality of life.

"If Isaac was diagnosed earlier he would still be running around playing football not relying on a walker to just take a few steps.

"I wanted to be here today to show the local people the sort of family the BDFA is supporting, also to say a huge thank you. Without the BDFA us Batten Families would be lost."

Bridgwater Mercury: Bunch Of Grapes cheque presentation, Bridgwater ; Isaac, Eva and Aimee Tilley

Batten disease is an inherited condition, only passed down if both parents have the faulty gene. Isaac's sister, Eva, does not have the condition.

Licensee of the Bunch of Grapes, Jim Cavanagh, added: “I am humbled that our pub is able to support this amazing charity and the incredibly important work that they do. I hope that money will help little Isaac, and many others.”

To keep up to date with Isaac's journey, or to donate to the family's JustGiving page, visit their Facebook page at facebook.com/isaacfightingbattens.