Ringwood family on experiences with juvenile Batten Disease

Sister and brother Annabelle and Robbie Hover have both been diagnosed with juvenile Batten Disease – a rare, genetic disorder of the nervous system with a limited life expectancy.

Sixteen-year-old Annabelle, who lives in Ringwood, was born in 2005 to parents Lisa and Andy. “She was a healthy, happy baby,” said Lisa. “Then, aged seven, she started to lose her sight.”

Annabelle was registered blind in 2013 and the family spent years seeking to understand the cause of her vision loss. In 2016 she underwent blood tests to rule out Batten Disease.

Annabelle Hover

Lisa said: “We’d never heard of it before. After the tests my husband Googled it and I watched the colour drain from his face. Looking down the list of symptoms, it was everything that Annabelle had.”

Six weeks later, they travelled to London for the results. Lisa recalled: “When we were in the waiting room, I said to Andy ‘if there’s more than one chair, it’s bad news’. There were five chairs.

“It was horrendous. Getting on the train back, surrounded by everybody living normal lives when your life has come crashing down.”

Juvenile Batten Disease is a neurodegenerative disease of the nervous system which inhibits the body’s ability to recycle cells. It begins in the brain and eventually impacts the whole body. There is no cure.

Devastatingly, Annabelle’s younger brother Robbie was also confirmed to have the disease in 2017.

Annabelle and Robbie Hover

Lisa said: “She was so upset, of course, but it made her more determined. Robbie was only four, so had very little understanding. We told him he was the same as Annabelle – he was just happy that he was like his big sister. He loves her so much.”

The family have since set up their own charity, Batten Fighters Forever (BFF), to raise awareness of the condition and campaign for further research.

Robbie and Annabelle were referred to the charity Make-A-Wish UK in 2019, when Robbie’s eyesight had started to deteriorate. His wish, to see Santa before he lost his eyesight, was granted that year with the family travelling to Lapland.

Annabelle’s wish for a new bed with massage function was granted by Make-A-Wish in March 2022.

Annabelle Hover with her new bed thanks to Make-A-Wish UK

Since November, she has suffered serious cognitive decline and is now displaying signs of dementia. Loving mum Lisa added: “That bed has helped her so much. It makes her happy – and all I want now is for her to be happy.”

Visit make-a-wish.org.uk/south-east and battenfightersforever.com for more.