Christmas light switch on for terminally ill boy

Robert and Lydia Kent, of 22 Raven Way, Leighton Buzzard, are switching on over 3,000 Christmas lights outside their house tomorrow (December 1) at 7pm to help support Roman James, five, who has type C Niemann-Pick disease, a fatal condition known as childhood Alzheimer’s.

There will be music, mulled wine and mince pies, and most importantly, a banner displaying a Niemann-Pick disease website address for fundraising.

Robert said: “I’m friends with Roman’s uncle, Joe Rojek-Smith, and we wanted to help support their family and raise awareness.

“My wife and I will be running the festive event, which will get everyone in the Christmas spirit.

“People can donate and pass by the banner outside our house.”

Parents Ben James and Scarlet Rojek-Smith, from Cheddington, were given the devastating news in October 2014 that son Roman had type C Niemann-Pick disease, a fatal condition known as childhood Alzheimer’s.

After enjoying a completely normal babyhood, Roman – now aged five – has fast deteriorated since his initial diagnosis.

Speaking to the LBO in August, Mum Scarlet said: “When Roman was first diagnosed, I was eight months pregnant with my second son Gabriel and panic-stricken. I didn’t know how severe the disease was going to be.

“In a sense, having Gabriel so soon afterwards was a blessing because it kept us going. He was hardly going to wait for us to pull it together.”

The LBO also interviewed the couple in December 2014, as they nervously awaited the results of Gabriel’s blood tests.

Scarlet remembered: “At that point we were waiting to find out if Gabriel had the disease. He doesn’t, he’s just a carrier.

“I think it was a shock to everyone including the professionals how quickly Roman has deteriorated. His condition is quite aggressive. He’s now completely tube-fed, he suffers from seizures and he’s completely immobile.”

Dad Ben added: “Within eight months, he lost the ability to eat, walk and talk.”

Ben is Roman’s registered carer, while Scarlet is a registered carer for her own mum.

She said: “We’re both here 24/7 around the clock.

“In terms of prognosis, initially they gave him 10 years but his condition has been so aggressive it’s now more like two years.

“He’s having periods of dips where he loses a lot of ability and then he stabilises for a couple of months.”

The family are supported by community nurses, and Roman also receives respite care at Keech Hospice.

“He loves it there,” said his mum.

It is estimated there are around 1,000 cases of type C Niemann-Pick disease around the world, with about 100 in the UK.

Scarlet and Ben have already met three families affected by the illness in Milton Keynes.

With relatively few cases around the world, more research is now going into type C Niemann-Pick disease – whose patients suffer different levels of severity.

Scarlet added: “Even after Roman is no longer here, we’ll make it our life’s work to find a treatment, and hopefully one day a cure.

Visit: http://www.npuk.org/