“I love going to Rangers and I’m now doing my Bronze Duke of Edinburgh Award…Batten disease will not stop me.”

Annabelle Hover, aged 14, tells of the difficulties of living with CLN3 as a teenager, and the amazing achievements she continues to take on.

My name is Annabelle and I am 14 years old. Here is my story.

All I wanted was answers

I failed the tests you get in your first year of school and got referred to Bournemouth hospital. I had routine eye tests and my parents pushed for more detailed tests. At the age of eight, they told us that I had macular degeneration and they referred me to Southampton hospital. My sight was quite stable but somewhere between the ages of nine and 10 years I lost all useful sight and nobody could find any answers. My last very clear visual memory is of my then baby brother Robbie and my little sister Georgie. I had so many appointments going from one doctor to another, carrying out lots of tests on me. I got fed up with going, all I wanted was answers.

In June 2016, Southampton sent me to Moorfields eye hospital and they took my blood and did more tests. At this time I had started to have problems sleeping, I felt tired a lot and I started to struggle with remembering things. In August 2016 they told my parents that I had Batten disease but my parents didn’t tell me until they had asked all the questions I would ask and tested my younger siblings to see if either of them had it.

My heart completely broke

Mum and dad told me that they had a name for what was wrong with me and they told me that Batten disease was causing all the problems I have. I asked lots of questions about what it actually meant for me. I found out that I would never see again, I will find walking harder, I will stop talking, have seizures, stop eating and be in a wheelchair all the time.

I also know I won’t have a long life.

My heart completely broke when my parents told me that my little brother Robbie also had the disease; that really upset me.

Teenage life with Battens

I have hard days where I am really tired from not having enough sleep and some nights, no sleep. I have days where I cannot remember anything.

I use a wheelchair so I can keep up with the family. I have seizures and my memory is not very good at all.

I don’t find Batten disease hard to deal with but Robbie having it as well is harder to deal with. I help him a lot and he also helps me.

As a family we wanted to raise awareness of Batten Disease. I chose the name of our website -Batten Fighters Forever or BFF, so it also means that everyone who joins us is a BFF – Best Friends Forever.

I’m still in mainstream school and I am enjoying it and I will hopefully get to sit my GCSEs. I have taken part in a Rare Revolution magazine editing team, where I got to meet a Paralympian called Sophie Wells.

I also go to a local hospice called Julia’s House. I am a massive Vamps fan and it turns out that James McVey is patron of the hospice. He made a personal visit to me at the house and I got to meet the whole band! I got to go on the radio and tell everyone about it!

The hospice is not a scary place, it makes me happy.

I love going to Rangers and I’m now doing my Bronze Duke of Edinburgh Award.

Batten Disease will not stop me.