During Children’s Hospice Week Together for Short Lives is working with MPs to raise awareness of the challenges facing children with life-shortening conditions across the UK. Today we held an event in Parliament to bring MPs together with families caring for children with life-shortening conditions, and professionals working in children’s palliative care to help them to better understand the obstacles facing these children. In this moving story Nikki Backus tells the story of her daughter Ruby, whom she lost to Sandhoff disease, and why the support she received from Acorns Children's Hospice and the CATS Foundation was invaluable.
Ruby was diagnosed with Sandhoff Disease when she was 16 months old. Sandhoff disease is regressive in nature and terminal in children – a devastating diagnosis to come to terms with for the whole family.
To begin with, once we found a suitable drug regimen Ruby’s symptoms were manageable, but over time as her symptoms became more complex, affecting more and more body functions everyday tasks became difficult and sometimes downright impossible. In particular social or even simple quality family time began to suffer, and this was especially difficult for Ruby’s twin brother Sam who was a typical, energetic toddler.
Until Ruby’s first hospital stay Sam, Ruby and I had never spent any time apart, this changed as Ruby’s needs did. When Ruby was taken to hospital, which unfortunately became more frequent as time went on, I of course went with her, which meant Sam would frequently wake up in the morning to find both his mummy and sister had disappeared and wouldn't return again for weeks. Sam and Daddy visited us as much as possible in hospital, but Ruby was mostly in PICU and small children often are not allowed to visit PICU and if they are it is only for a few moments. The situation was hard on Sam.
When Ruby was stable and we were home, I tried to make life as normal as possible for our family, but even our normal routine consisted of many appointments and check-ups for Ruby. Of course I didn't mind, I made sure Ruby had everything she needed, but it did concern me that Sam might be missing out.
When the twins were 2 ½ we were approached by the local Hospice – Acorns Children's Hospice. I was reluctant at first to meet with them as the word ‘hospice’ was frightening to me. When our hospice family worker made contact with us she was lovely and sweet and put me at ease, she engaged both Ruby and Sam equally and we arranged a visit just to see how we felt. When we walked through the Hospice doors we all felt immediately at home. The staff cared for Ruby and Sam with equal amounts of enthusiasm and support. Sam particularly came away that day with new found friends and a place he remembered with much fondness.
After that we visited Acorns Children’s Hospice a couple of times a month and I found it such a relief. We stayed for the day and were looked after as a family. I was secure in the knowledge that Ruby was having the care she needed, which meant amongst other things I had time to play with Sam, to spend some quality time with him. It also enabled all of us to spend time together enjoying activities instead of me worrying about feeding pumps, suction machines and drug schedules as the hospice staff took care of that for the day. My day was free to spend with my children….just my children, not Sandhoff Disease.
It also meant Sam was occupied for periods of time with the Hospice volunteers and I could spend some quiet time engaging Ruby, particularly in the Sensory Room which she loved. It was always difficult to juggle these types of activities for Ruby with an active, vocal little boy.
We came to look forward to our respite days at the hospice, it was always a relaxing day for me, an interesting, stimulating day for Ruby and a fun day for Sam. We would spend quality time together on these days and for a few hours the worries and medical support were not at the forefront of my mind.
As well as from the Hospice, the family also found support from the Cure & Action for Tay Sachs Foundation (CATS Foundation), a family support charity that specifically supports families affected by Tay-Sachs and Sandhoff disease and raises awareness of these conditions. Nikki got in touch with CATS when Ruby was around two and a half. Not only did she find great support in being able to talk to people who really understood Sandhoff disease, she found the charity could also supply equipment where needed. In Ruby's case they stepped in to provide a specially designed Vibro acoustic cushion for Ruby's home – Ruby really enjoyed the Hospice's cushion, and being able to buy this specialised equipment for her home was a great comfort.
End of life
The Hospice continued to support us and when we sadly had to face end of life care for Ruby we had no hesitation requesting we be transferred from the hospital to Acorns Children’s Hospice. In the darkest, most difficult hours of our lives the Hospice supported us with care and compassion. I honestly do not know how we would have got through that time without them.
We continue to be part of the Hospice Family and for this I am eternally grateful. Some of Sam’s clearest memories with Ruby are in the Hospice and he always talks of it with fondness, like part of our family – this is how he sees it. It helps me that we are still welcome at a place that I associate with good, positive memories…Ruby’s name is on a stone in the Hospice Remembrance Garden and I love to visit with her in this special place.