Appeal life for John and Bridie

01 November 2007

THE relatives of two children with a fatal genetic disease have been overwhelmed by offers of support since the Recorder highlighted their story.

We reported the efforts of Joanne and Daniel Richardson, of Rusper Road, Dagenham, to find a cure for the strain of fatal Batten's disease that affects their nephew and niece John and Bridie Philpott, 11 and nine, respectively.

After the story appeared last week, the couple appeared on ITV news programme, London Tonight.

They were interviewed on on Friday with researcher Sarah Mole, who is studying variant late infantile Batten's disease at University College, London.

John and Bridie, who live in Chadwell St Mary, are the only children in England with this strain of the disease, and are two of just 800 worldwide. They are only expected to live until their early teens.

Mrs Richardson said the couple had been contacted by Les Burons carp fishing farm in France, and given a holiday for four at the farm to raffle for their fundraising efforts, and Cycles UK had donated a bicycle for them to auction.

The Richardsons are in discussions with a company considering sponsoring John and Bridie's Batten Disease Charity, the organisation they have set up to fund research into the disease.

Mrs Richardson said: "I'm knackered, but apart from that very happy and very confident that the story's getting out and people are taking notice of us now, so I'm very pleased."

Watching her niece and nephew deteriorate, losing their ability to speak, feed themselves and control their bodily functions was "devastating", she said.

"We have two children the same sort of age as well," she said.

"Shelley is only five days younger than John and they have grown up so close and done everything together, from laying on the carpet staring at the ceiling to playing in the garden.

"So to see Shelley growing up and John deteriorating the way he is, is awful."

Batten's disease affects the nerve cells in the brain, eyes and other parts of the body, causing a progressive loss of vision and physical and mental skills.

It is caused by an abnormal build-up of substances in nerve cells in the brain and body, which prevents them working properly.