“Our children found it heart-breaking, as we all did, but to see them being brave on each visit, not knowing if it was the last, was just so tough.”
Liz Brownnutt, auntie to Ellie Mae and Caleb, who died from CLN2 aged 6 and 9, describes the lasting impact losing her niece and nephew to Batten disease has had on the wider family.
We’ve always been lucky to have family living close by. Having grandparents and an extended family, a brother, sister-in-law, niece, nephew, and cousins for our three children has always been a huge blessing to us as a family. Our eldest child was ten and youngest two when Ellie Mae was born and Caleb followed two years later. Having little cousins was such a joy and our family gatherings were always energetic and noisy!
They continued to be that way, even after Ellie Mae and Caleb were both diagnosed with CLN2 Batten disease in early 2014.
In the preceding couple of years, it was so hard to see Ellie Mae go from running around confidently, to falling and becoming so unsteady on her feet. We knew that something was very wrong and we will not forget that time, watching their parents go through the uncertainty, all the investigations and tests, and then to be told that Caleb had his first seizure in the back of the car and that he too was being investigated.
Enjoying the good times
The initial months after Ellie Mae and Caleb’s diagnosis were so hard for us all as a family. We often felt so helpless, as it seemed that we could only do little things like make meals for the family so that they didn’t have to cook. And trying to be supportive to our children’s grandparents too. But looking back, I think the most important thing we as a family could do for them was to just love them, be there for them and make precious times count.
We spent some amazing times together; trips to the zoo, Wildlife Park, Flamingo Land and an unforgettable holiday at Center Parcs with even more extended family! One very fond memory is of Ellie Mae sitting in her special ‘pink’ chair and the look of delight on her face as all the girls (grandmas, aunties and her two girl cousins) gathered round her to watch her favourite movie ‘Frozen.’ That will always be ‘Ellie Mae’s movie.’
Caleb was still running around at that point and took the most delight in making up his own little phrases to simply just confuse his older cousins, such as ‘bye bye cheese!’ My son would say, “What does he mean?” Caleb would say it again and on it would go.
The happiest girl in the room
The few months after that holiday saw Ellie Mae’s condition deteriorate fast and the family were told in mid-April 2015 that she didn’t have long left. She was admitted to Martin House children’s hospice in Wetherby near Leeds, a wonderful place and we shall always be grateful to them for their expert care and kindness.
The final ten days that Ellie Mae was there were so hard, again we felt so helpless and all we could do was visit as often as we could and take supplies to Ellie Mae and Caleb’s parents as they stayed by her side. Our children found it heart-breaking, as we all did, but to see them being brave on each visit, not knowing if it was the last, was just so tough for us all. Our youngest daughter was just nine years old and she was Ellie Mae’s ultimate hero.
Ellie Mae lost her fight with Batten disease on 7 May 2015, aged just six. She will be remembered as being the happiest girl in the room, always full of joy and delight at being with the people who she loved so much. We miss her so very much.
Strength and resilience
We enjoyed another four years with Caleb and tried to make each precious time that we had with him count. He continued to confuse us all with “nye bye cheese!” and he delighted in all the attention he got from his cousins. Our youngest daughter’s name was one of the last words he could say before he lost all his speech.
In early October 2019, Caleb went to stay at Martin House with his parents, because the doctors said he was near the end. Caleb continued to amaze us all with his strength and resilience as he battled on, refusing to give in to Battens. Each visit we thought would be the last until the next week would come around and we visited again.
Though that was the most horrendous time, it was also peaceful as we were allowed time to just sit with Caleb, hold his hand and talk to him. We always said “bye bye cheese!” when we left him. Caleb lasted six weeks at Martin House, but sadly lost his battle with Batten disease on 15 November 2019 aged nine. Oh how we missed him, his strength, his delight at being with us all and his love of all things Disney and Pixar.
Ellie Mae and Caleb will always be our much-loved niece, nephew and cousins. We remain deeply impacted by their capacity to love ‘big’ and the resilience and strength that they both showed. We watched Batten disease take away their mobility, their speech and ultimately their lives, but it never once took away their ability to love. Ellie Mae and Caleb have made a lasting impact on our children, whose experience of loss at such young ages is something that you would never want for them. They were Ellie Mae and Caleb’s heroes and what a privilege that is for them always!
Ellie Mae and Caleb have taught each one of us so much about love and strength. They have also influenced some of our life choices, as my own experience of being their auntie led me to becoming a member of the BDFA staff team. They inspire me every single day in my role as Head of Fundraising and my desire to see the BDFA go from strength to strength for the precious families we support.
We will always miss them, but remember them with joy and love.
Two very special children.