Tragedy of brother and sister with cruel genetic disease that will rob them of their lives

Ellie Mae, six, and her four-year-old brother Caleb want to experience as much as they can before the devastating disease claims their speech, eyesight and mobility.

Late Infantile Batten Disease - which is inherited through an extremely rare recessive gene - only affects one to three children in the UK each year. 

Most of those do not live to see their 20s. 

After being handed the heartbreaking diagnosis the siblings' parents, Lynsey and Duncan, created the ultimate bucket list to make every second count before it is too late.

Full-time mum Lynsey, 35, said: "I try not to think about the future because it's just too hard.

"The doctors can't really tell us how it will progress so we're just trying to make it all count.

"We do have some big activities planned, but some days we're happy to just to be at home as a family.

"Duncan and I just want to make memories for the children while they still can."

The family, from Leeds, have already been to Disney World in Florida, on a safari day trip and for an excursion into London.

Their heartbreaking ordeal began when Ellie Mae, three at the time, suffered an absence seizure at pre-school and was diagnosed with epilepsy.

However, when the seizures continued to get worse, until she was having up to three a week, doctors became increasingly concerned there was something more serious behind them. 

Lynsey, said: "The doctors told us that they might never know what was wrong with her. A lot of children have seizures and they can only treat the symptoms, not the cause.

"It was a very worrying time. We had a lot of tests - blood tests, MRIs - but we just weren't finding out the cause of the seizures.

"She was falling behind at pre-school and the staff said she was struggling with counting and shapes."

Then, in March 2013, tragedy struck again when her baby brother Caleb had his first seizure. 

Recalling the heart-stopping moment, Lynsey said: "I can remember it like it was yesterday. He was sat in the back of the car and I saw him in the mirror. I knew straight away what it was.

"He was diagnosed with epilepsy too, but Ellie Mae was getting worse.

"Not only was she struggling at pre-school, but she had started falling over a lot.

"We were sent back to the doctors for degenerative tests.

"The results of the MRI found that her brain was smaller than it should have, so the cells are dying.

"Although we didn't have a definite positive test result, they told us that LIBD is the only condition that can cause the results." 

Two months later the family were handed the devastating confirmation that the siblings had the fatal illness. 

It means that the pair will both slowly lose their speech mobility and sight and, while the decline in their condition is expected to slow as they get older, they are not expected to live past their teenage years.

Lynsey said: "I have never really accepted that it is a terminal disease.

"We just try to make the best of it. Duncan works for Vodafone and his colleagues helped us go to Disney World last year.

"We also went on a safari day and to Disney Land Paris thanks to the Make a Wish Foundation.

"Ellie Mae and Caleb are both happy children - they can still laugh and play. They have kid's tablets that they use, which we got through Duncan's work.

"It does get me down when they lose something, like Caleb recently stopped walking, but they just seem to accept it and learn to adapt.

"I know I will have bad patches, but I usually pick back up.

"We just got back from a trip to London where we went to see the Lion King, the Natural History Museum and London Zoo.

"I would love to take them to Peppa Pig World. They are both huge fans and I'm sure they would love it." 

Her husband Duncan is planning a tandem cycle ride from Norway to the Arctic Circle to help a charity fund research into the illness - even though it is unlikely to yield results for a decade.

The 39-year-old said: "With our kids we understand that the way research works. It's not a quick process. 

"There's one clinical test going on at the moment and that will not end in time to help our kids unfortunately and that's something we have to accept.

"It's a 10 year trial and they don't believe our kids have got 10 years left. I understand and accept that but it doesn't make me want to stop supporting further research." 

To donate go towww.justgiving.com/Duncan-Brownnutt4