Nine years ago as I turned 30 my life seemed pretty much perfect, happily married, two beautiful daughters, a lovely and supportive extended family, fabulous friends, a good job and a nice house. However in July 2007 our lives changed beyond all imagination when our elder daughter, Rebecca, was diagnosed with Late Infantile Batten’s disease. Rachel, our younger daughter, was diagnosed with the same disease seven weeks later.
Late infantile Batten’s disease is a childhood disease and over time, typically, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with the disease become blind, bedridden and unable to communicate, and, presently, it is always fatal.
At the point of diagnosis Rebecca was beginning to show some signs of deterioration, with a significant reduction in her fine motor skills, a reduction in some of her gross motor skills and her vision was variable, sometimes not being able to see at all. She had also begun to have seizure activity which irritated her.
Fairly quickly Rebecca completely lost her fine motor skills, she lost the ability to walk and had little control over the movement of her limbs. She lost her speech and her ability to swallow and had to be fed through a tube fed through her nose in to her stomach. Rebecca was completely reliant on us for all her personal care.
However, she never lost her personality, cheekiness or mischievous sense of humour. Despite not being able to speak she was still able to let people know what she was thinking or what she wanted, through her non-verbal communication. Unusually for this particular disease, Rebecca never lost her mental abilities.
Due to Rebecca’s inability to swallow, secretions began to drain in to her lungs and her lack of movement made it difficult for her to clear them and she started getting chest infections. Rebecca had a three week spell in hospital in spring 2008 with a bad chest infection.
In December 2008, she got another chest infection that she was unable to recover from.
At this time Rachel had shown no signs of the disease, however not long after Rebecca died, Rachel started having seizures and her speech began to deteriorate.
Over the next three years Rachel deteriorated to the state of having significantly variable sight, with periods of not being able to see more than when she could. Rachel lost all ability to speak and to swallow and also required a tube to be fed.
She lost her ability to walk and stand. She had no control of her limb movement and required aid to sit. Rachel lost the ability to hold her head up. Like Rebecca, she didn’t lose any of her personality or mental abilities and maintained the ability to assert her personality using non-verbal communication.
In spring 2012, Rachel’s seizures started increasing and the drugs became less effective. This led to Rachel being in and out of hospital for a number of months for uncontrollable seizures and chest infections. Early in September we were told that Rachel wouldn’t make it through the night. Typically, Rachel had other ideas, and continued fighting until the end of November when she decided it was time to die.
During the girls’ illness our family received invaluable help from Chestnut Tree House and The Sussex Snowdrop Trust, without which we couldn’t have coped. The two charities provided us with complementary services, supporting us through the girls’ illnesses and they continued to support us after the girls died.
Initially a couple of members of the community team from Chestnut Tree House came regularly to our house to provide us with a few hours respite to give us a break. As Rebecca became more unwell we started using the in-house services.
The house is beautifully designed and is very thoughtfully planned. There are spaces for all sorts of activities, our girls particularly liked the swimming pool and the art area. The bedrooms for the children are downstairs and are very light and airy and spacious even with all the equipment that the girls required.
Upstairs has a family area which meant that my husband and I were able to stay in a discrete area. We were able to catch up on sleep in the knowledge that we were there if the girls wanted or needed us. During the day we were able to stop being carers and just be parents, leaving the amazing staff to deal with the feeding, changing and caring responsibilities.
We all valued our time staying at Chestnut Tree House, it was like living in a bubble where all the day to day reality evaporated for that period of time.
When Rebecca died, Chestnut Tree House offered the use of their Stars suite to lay her body to rest until her funeral. We received more support than we will remember during those days, making sure that we all ate, hosting the various people who wanted to pay their respects, helping with the funeral arrangements and providing us with emotional support.
After Rebecca died, Rachel started seeing the play and creative arts therapist who not only helped Rachel through the death of her big sister, who she adored, but also through dealing with her own decreasing health and eventual death. This support was fundamental in helping Rachel deal with her situation but also helped us to help Rachel.
The last nine weeks of Rachel’s life were spent at Chestnut Tree House receiving end of life care. She was so ill that she could only move her eyes and the only noises made were involuntary noises when she was in pain. Despite this the staff understood Rachel’s communication and were able to meet her needs and understand what she wanted them to know.
The staff were phenomenal and there was nothing that Rachel, my husband or I wanted that wasn’t doable. They ensured that Rachel was virtually pain free and comfortable. Their understanding of Rachel allowed her personality to shine through to the end. They could not have done anything more for any of us. They enabled Rachel to die in the way that she wanted.
After Rachel’s death she was also laid to rest in the Stars suite. We were supported through the period until Rachel’s funeral whilst we stayed at the house. Since then we have continued to receive support from the staff, utilising their counselling and bereavement services.
The Sussex Snowdrop Trust provided Children’s Community Nurses who supported us in providing care to Rebecca and Rachel at home. They became involved at the point of Rebecca’s diagnosis helping us to come to terms with the bombshell we had been hit with. Rebecca’s named nurse became an invaluable support, helping us to cope with Rebecca’s decreasing health and liaising with other NHS professionals to ensure that Rebecca received the appropriate care.
Rebecca’s Snowdrop nurses taught us how to deliver care such as how to insert Rebecca’s feeding tube and how to feed her through it. They helped identify when Rebecca needed additional intervention and supported getting her into hospital when it was required. Rebecca’s named nurse routinely visited us when we were in hospital with her, irrespective of whether she was working or not.
When we were told that Rebecca was not going to make it, her Snowdrop nurse came in to the hospital to support us and to ensure that there was nothing that we needed, despite it being a leave day. She supported us after Rebecca died and ensured that we looked after ourselves as well as Rachel.
As Rachel started to deteriorate, our Snowdrop nurse was there every step of the way, providing care that was equal to that provided to Rebecca. As Rachel became more and more poorly the nurses visited more frequently. When we took Rachel home to die they supported us, visiting multiple times every day, helping with every element of Rachel’s care and providing a 24/7 on call service. They supported our transfer to Chestnut Tree House and visited Rachel there.
Snowdrop not only provided clinical support but also bought essential equipment such as a car seat, a changing table and a CCTV so we could keep an eye on Rachel overnight. They also supported us with the costs of both the girls’ funerals.
I want to do something to raise the funds and awareness of what The Sussex Snowdrop Trust and Chestnut Tree House do. Every child in Sussex who has a life limiting or terminal illness deserves the care that Rebecca and Rachel received. Every family deserves the care that we have received. However this costs and both charities are virtually totally reliant on charitable funding.