“In my heart I know it was her time and asked to take my girl home… Bee taught us so much during our time together and those lessons will be there for the rest of our life.”
Blanie White’s family are facing their first awareness day since Blainie passed away of CLN6 Batten disease aged 12. Her mum Valerie writes movingly about her life, the incredible bond she had with her siblings and the moments of joy amidst the grief. The family live in Beauly in Scotland.
Blainie lit up our lives when she was born on the 29th October 2008. Surprising us all with her chubby cheeks and mass of hair, she met all her milestones and was growing beautifully. She loved food, dancing, swimming and exploring outdoors in all weathers. Most of all she loved her brother, Kion, and would follow him everywhere and her face lit up whenever he called her name.
Early symptoms
Our life changed when Bee, as we called her, was four. She didn’t follow the typical path of Batten disease and seizures weren’t the first symptom. Blainie suddenly started falling while running. In some ways it wasn’t a big deal as all kids fall but from the very first time it happened I knew something wasn’t right. Our fantastic GP sent us straight to hospital for tests that same day and our story was set in motion. Initially Bee had multiple tests and appointments and an MRI scan just a month later showed an imbalance in Bee’s brain. Unfortunately Bee was already deteriorating she couldn’t run anymore and her walking gait had changed. We had no answer as to what was going on or where her regression was going to next.
So we carried on as our family enjoying every moment with Bee 🐝 How could we not when she smiled and laughed no matter what! Bees speech started to regress very quickly and a year later when she had her first seizure she only had two words left which were “Kion” and “no”. I missed hearing her say mama but those two words stuck around for many years and we treasure them. Bee’s seizures were very subtle at first, with absence seizures throughout the day. The seizures soon changed for us this was indeed the worst part of battens. They took so much out of Bee.
Years passed by with no answers
The seizures were uncontrollable from early on and medication never got them truly under control. Just when we thought we were on top of them they would “up the anti” and become even worse. But still, who smiled and laughed and enjoyed life no matter what? Our little girl did so we decided we had no time to dwell and be sad or worry. We were on this journey and we were going to laugh and smile with her. Four long years passed with many tests and appointments with no answers but we were determined to give our all to our special girl and enjoyed every minute with her lots of trips out, doing the things she loved. One day I had a call from a close friend who happened to have seen something about Batten disease in her work and it had so many similarities to our story. I shouldn’t have but I read up on it and I knew straight away that is what we were dealing with.
A friend mentions Batten disease…
I called Blainie’s nurse straight away. Two weeks later, in January 2017, we had the confirmation in the doctor’s office she did indeed have Batten disease, CLN6. I cried in that office but when I got home and saw my happy girl smile from ear to ear as soon as I walked into the room, I made a choice never to cry again. We weren’t going to be sad, we were going to love deeply smile lots and giggle and laugh every single day living life to the extreme best we could and that is what we did. We adjusted things and made sure everything was accessible and work for her we didn’t matter if it put us out if it made her life a little bit better. Bee’s little sister Kaia was born in 2015 and she was absolutely smitten, Bee would lay next to her and just smile. These two had the most amazing caring bond you couldn’t see one without the other and Kaia seemed to understand Bee on a completely different level. I am blessed that they got to have so much time together and have these memories to grow with. Bee loved our thrill-seeking trip to Disneyland. Even though she could no longer talk the pure joy on her face was the confirmation that it was worth every bit of hassle and red tape to get her there. She was such a thrill seeker and we are so glad we got to share this memory with her, we treasure it deeply.
Batten disease ‘takes over’
Shortly after her official diagnosis, the disease started progressing rapidly. Batten started to take over and every week we were losing a little bit of Bee. Her eyesight rapidly deteriorated and she could no longer stand or walk or eat. She had a gastrostomy fitted and
She had reverted to crawling which lasted a while before one morning she woke up and could no longer move freely by herself. This hit me very hard but still we made the adjustments and Bee, of course, still smiled. Life continued on this roller coaster of ups and downs and multiple medications but we enjoyed our time so dearly with every little walk we took, every bumpy ride with seizures, every single shoulder jiggle and belly laugh from Bee lots and lots of road trips and beach days. We choose not to have a lot of carers or help from professionals apart from what was immediately required. We carried on as a ‘normal’ family. I can truly say we enjoyed every minute of life with Blainie, no matter how hard it got she was such a joy.
The disease progresses and Blainie goes to hospital
In September 2021, Bee took a sudden turn and was admitted to hospital with choking and secretion issues. The seizures really started taking over and we got home for a weekend but ended up back in hospital. In my heart I know it was her time and asked to take my girl home so we did just that and quickly sorted her room so it was cosy and comforting. We brought our girl home where we loved and hugged her, laughed and read stories, singing her favourite songs and seeing lots of visitors.
Blainie passed away on the 21st September with everyone she loved so dearly around her. For the second time since the diagnosis we cried but she was finally pain free and able to fly freely. We were so extremely sad and Blainie has left such a massive hole in our lives. We find ourselves with so much time we never knew we had before and some days pass by in a complete daze. We look up and we smile because we know that’s exactly what you will be doing, we carry on and hope we are making you proud.
I have started a new job in care which I would never have done had it not been for my girl. We spread Bee’s ashes at her favourite place and visit often and Kaia enjoys her playtime with her sister throwing small rocks in the sea and watching the waves bring them back to her. Bee taught us so much during our time together and those lessons will be there for the rest of our life. Love truly and deeply every second however small and meaningless moments may seem, they mean so much more…