Kyran's story Batten disease No Cure - Jeans for Genes

Kyran's story

Eight-year-old Kyran lives in County Durham. In March 2011 he was diagnosed with Batten disease, a genetic condition for which there is no cure. Children are born apparently healthy but later develop epilepsy and lose their sight, speech, mental and motor abilities. Life expectancy for people with Batten disease is between 5 and 30 years old.

In many ways Kyran is like any other eight-year-old boy. He loves messing about with his brothers Cameron, 13, and Hayden, 6, and he can tell you everything you need to know about dinosaurs. He goes to a mainstream school and has lots of friends. But ever since being diagnosed with Batten disease, life for him and his family has changed forever.

There are three main types of Batten disease, affecting children at different ages depending on which defective gene has been inherited. Kyran has juvenile Batten disease. His sight is deteriorating; he has to turn his head to the left to use the peripheral vision of his right eye. He also has balance issues, bumps into things more and more, and suffers from night terrors. But he’s a very determined little boy. It’s why he won an award from the local council for his achievements and perseverance at drama last year. ‘If you ask him “Can you see such and such?” he won’t tell you that he can’t. He knows he has poorly eyes but he doesn’t mention it,’ says mum Debbie. ‘He just gets on with it. We have good and bad days and we take each as it comes.’

On 15 March 2011, Debbie and her partner Carl met with doctors who suspected that Kyran had retinitis pigmentosa, an eye disease in which there is damage to the retina, because his sight was deteriorating. When doctors told the couple that, in fact, Kyran had Batten, they were heartbroken.

‘It was awful. A big shock,’ says Debbie. ‘We’d never heard of it and our first question was “Is there a cure?” And they said “No. Life expectancy for children with juvenile Batten disease is late teens.” It felt like someone had torn my world in two. You never expect to be told that about your child. We couldn’t stop crying.’

Doctors didn’t have any booklets or NHS information that they could give the family. There are only around 200 children and adults with the condition in the UK. So, Debbie and Carl were directed towards the Batten Disease Family Association (BDFA), a charity that supports families, raises awareness and organises research into Batten disease.

Since then they’ve attended the BDFA’s biennial conference, where they’ve been able to make contact with experts and other families affected by the terminal disease.