Frank was born in 2008 and for the first three years of his life he was, as mum Rachel describes, “a regular little boy, very active”. Then in July 2011 he had his first known seizure. A brain scan showed no significant irregularities but Rachel and Bob (Frank’s dad), became increasingly concerned as the seizures continued. Twelve months later a further MRI scan showed a level of deterioration and Frank was admitted to hospital in September 2012. Within two weeks he could no longer walk unaided.
“So much changed and so quickly,” says Rachel. “As Frank’s condition got worse we did some research and had an inkling he may have late-infantile Batten disease, but after reading the prognosis we desperately hoped it was something else.”
Their fears were confirmed in October 2012.
“I just wailed. It was devastating. Even though we were half expecting it, it was such an awful thing to be told because we knew what that meant. Frank would lose the ability to do most things. He would lose the ability to walk, to move his arms and legs, to eat properly, to swallow, to see, and would eventually become bedridden.”
A rare disease
Frank is one of only five or six children a year diagnosed with late-infantile Batten disease and it is estimated there are between 30 and 50 children affected in the UK. The condition is life-limiting. “Life-expectancy is about 5 years of age to 12 years of age, but things have changed so quickly for Frank I’d be surprised if we have that much time with him,” says Rachel, “I think we’ll be lucky to have a few years. Maybe it’s not helpful to think like that but we have to prepare ourselves,” she added.
“Even when we first heard about Shooting Star CHASE my initial thought was to only use the hospice for end of life care when that time arrived. I have a background in nursing and I know how important it is to manage pain at the end and Shooting Star CHASE is really good at that. Knowing that is a huge comfort for me because I want those days to be as best as they possible can be for Frank and the family. But I’m so glad we changed our minds because the hospice has become so much more than looking ahead to end of life care.”
The family changed their minds after being shown around Shooting Star House in Hampton.
“Our key worker, Trish, explained everything they could offer, not just to Frank, but to the rest of the family. They were very welcoming and handled everything so sensitively. The first time we stayed it enabled us to have proper family time for the first time in a long time, because we were able to be mum and dad, and not Frank’s carers, as the nurses could safely take care of his medical needs.
“We used the hydrotherapy pool as a family and we watched a DVD together. Frank loved the sensory room and playing in the garden with the nurses. Our other children, Mae and Kirk, particularly loved the music room and the games room, and Bob and I got a full night’s sleep. That doesn’t happen very often anymore.”
Support for the whole family
Rachel says the support provided to the wider family has been crucial.
“The siblings activities are great for Mae and Kirk and they’ve been very enthusiastic about them. Mae got chickenpox in between visits and the first thing she said was she was really pleased to have got it when she did because she would be better in time for the next siblings day! The interaction with children in similar situations is vital because it helps Mae and Kirk to explore their feelings and frustrations – they are often left on the sidelines with Frank needing so much attention.
“But the support goes even further. My parents are heavily involved in this as well because they worry about us and they worry about their grandchildren. The care team showed my mum around Shooting Star House to show them all the great facilities and that really helped her.”
“Shooting Star House is anything but morbid, but at the same time talking about death is not ignored. On one of our first visits I met a grieving parent who was being helped with funeral arrangements. We know death is part of life but for us and the other families it is going to come sooner. It could easily be a sad place but it’s not – there’s a lovely mix of joyfulness and sensitivity.
Rejuvenate and recharge
“We get an allocation of 12 nights a year and we will definitely use every single one. Our entire calendar is planned around our visits to Shooting Star House – that’s how vital they are to us as a family. Life would be even more difficult without that support. It gives us a chance to rejuvenate, recharge our batteries and have some family time. We all look forward to it. I can’t imagine how we would have managed without the support we’ve had from the hospice.”
To find out more about Shooting Star CHASE Children’s Hospice visit their website.
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