We are pleased to share this blog, written by Evan, in memory of his cousins, Ellie Mae and Caleb who both had CLN2 Batten disease:

“Whoever said, “Never meet your heroes” obviously had the wrong ones.

Because I’ve met my heroes: My cousins, Ellie Mae and Caleb. 

You’re probably thinking “why are they your heroes?” And I can’t really put it into words, which is obviously a bit of a problem when writing. But I’ll try my best.

Ellie Mae was always the happiest person in the room. My lasting memory of her is during a family holiday being sat beaming in the corner of the room. All you had to do was go over to her to get the biggest smile and a squeal of delight. Knowing that she recognised you was amazing. One of the most difficult things for me is how quickly Batten disease can take away a child’s mobility, speech and ultimately life. All while you sit there helpless, not knowing what will happen next. One week she was chasing us round the room, the next she could no longer sit in a chair without help. 

Even through all this, she never stopped being her happy self, a true superhero.

Ellie Mae lost her battle with Batten disease aged just six. We all miss her dearly. 

I could write volumes on the complexities of Caleb. He was a world expert on diesel locomotives and single handedly kept several trainspotting YouTubers in a job with his iPad. He also holds the record for most views of ‘Hot Dog’ in one sitting. And he definitely knew all the words to Hakuna Matata. 

But for me, nothing sums up Caleb more than his own strange but very logical language, like his names for his favourite DVDs like Buzz, The Wuzz or Wo-Wohts (full marks if you get all 3). 

To him it all made perfect sense and he would find it hilarious if you dared to question him.

One Sunday he came over to me and said, “Bye Bye Cheese” then ran off laughing to himself. I had absolutely no idea what he was on about. Who is cheese? Am I cheese? Is he making fun of me? What does he mean? I would ask him what he was saying, just to get another “Bye Bye Cheese” followed by a mocking laugh. Rinse and repeat. 

This then went round in circles for weeks, months, years. Caleb never let us in on the joke and I still sit and think about what he meant to this day. It was his world and how fun it was to live in it. Caleb lost his battle with Batten disease aged only 9. I’d do anything to sit and watch Buzz with him again. 

Batten disease took Ellie Mae and Caleb from us, and it continues to do the same to other families across the country. The BDFA offer support and guidance to families at what can be an extremely confusing and distressing time when they receive a diagnosis and have no idea what to do next. Donations allow the BDFA to continue this vital work, to raise awareness of the disease.”

Evan and the corporate graduate group, Atomic Aesthetics who he was involved with last year, raised over £5,000 for the BDFA through the sale of their nuclear themed prints and various other initiatives.