THE parents of the only two children in?Ireland with the rare Batten disease are determined to raise hundreds of thousands of euro in a bid to get both children on a revolutionary medical trial which could slow down their condition.

In the space of just a month, parents Tony and Mary Heffernan were told their five-year-old daughter Saoirse could die at any moment and that their 20-month-old son Liam had contracted the same fatal condition.

Batten disease is a rare, genetic, degenerative neurological disorder that usually becomes fatal in children by the age of eight to 12.

The family, who live near Castlemaine, Co Kerry, launched their charity Bee For Battens to raise awareness of the condition. They also made an emotional appearance on The Late Late Show last month.

The family said the reaction from their local community has been beyond expectations. An anonymous donor from Cork handed over a 27-foot yacht.

It is estimated that 200 children at any one time suffer from the condition around the world. The family hope Saoirse and Liam will qualify for clinical trials at New York's Cornell Hospital later this year. Gene therapy at the hospital, which involves injecting a harmless gene-bearing virus into the brain, has been found to significantly slow the progression of the disease.

Saoirse's first symptom was delayed speech development. Later she suffered spasms and was diagnosed with epilepsy.

"But it progressed and got worse and worse to the extent that we had to stop counting seizures at 200 a day. They take a lot out of you when you are four years old," said Tony Heffernan.

In May last year, Saoirse attended a consultant neurologist at Temple Street Children's Hospital and received an MRI scan in August. It showed up abnormalities and more tests were carried out. In September she was diagnosed with the disease.

"I can remember it like it was this morning. I broke first because I knew what Batten disease was.

"Mary didn't know what it was at first. But when she looked over at me and I had broken down, she asked the doctor if it was fatal and he said 'yes, I'm sorry, it is'. Mary broke down uncontrollably and Saoirse reached out and said: 'no sad, I'll get better'."

Today, the five-year-old has lost 70% of her eyesight. She is back in nappies and can't walk.

Earlier this month, Tony and Mary were told that Liam had developed the condition.

"We were sure that he was ok – that he had no symptoms and he was behaving very differently to Saoirse," said Heffernan.

"He still has no symptoms. He is just a happy-go-lucky 20-month-old boy. Saoirse has some idea that she is sick but he doesn't."

www.beeforbattens.org.