Swindon family say Christmas could be their last with sick daughter

“We are acutely aware this Christmas is probably going to be the last one Addy can see,” said mum Hayley Clarke. “Our daughter is fading in front of our eyes.”

Hayley and her husband Dave have spoken out at the launch of a special Christmas Appeal for a Wiltshire children’s hospice, Julia’s House.

Their daughter Addy has an ultra-rare type of Batten disease (CLN2), which only affects approximately 30 people in the UK, with around five children diagnosed each year.

The genetic condition is a devastating and debilitating brain disorder known as ‘childhood dementia’ because it steals every skill a child has ever learned. The disease is terminal and without medical advances, Addy isn’t expected to reach her 10th birthday.

Addy's condition often leaves her very poorly and needing to spend time in hospital (Image: Julia's House)

Dave and Hayley only discovered that Addy had Batten disease when she was four years old after she began to have seizures.

“When the doctors first gave us the diagnosis, it was like having the carpet ripped out from under our feet because it felt worse than the worst-case scenario,” said Hayley from the family’s home in Walcot, Swindon. “I remember my husband dissolving into tears and saying, ‘We’re going to have to bury our daughter.’”

Before then the youngster, who attends the specialist Brimble Hill School in Redhouse, had been like any other healthy little girl her age – full of energy and happiest whizzing around on her scooter or building sandcastles.

Now, she has lost the ability to eat and walk independently and is beginning to go blind. While she still loves to laugh, in a short time Addy will completely lose all her mobility and cognitive skills.

“This horrible disease has stolen the Addy we knew,” said Hayley. “It feels like the little girl in the photos on my wall is not my child anymore."

Addy before her health declined (Image: Julia's House)She said, “The life we imagined for Addy is different – those feelings of, ‘I can’t wait to take her to ballet lessons and see if she enjoys it; I can’t wait to see her hold hands with her best little friends’ – now we have a different child and we have to come to terms with that.

“We don’t really know what the next month or the next year will hold - so we are making the most of the time we have together.”

This Christmas, Wiltshire’s children’s hospice, Julia’s House, is appealing for donations to help make magical memories and provide vital respite to families like Addy’s.

Julia's House is taking part in the Big Give Christmas Challenge which will see donations made between November 28 and Tuesday December 5 doubled. 

The specialist nurses and carers at Julia’s House are a lifeline to local families, providing clinical, respite, emergency and practical care, however and whenever they need it – both in families’ homes and in the charity’s hospice building in Devizes.

But with just eight per cent ongoing government funding, the charity relies almost entirely on donations to raise the £2.2m needed each year to provide this vital service to Wiltshire families.

Hayley said, “Julia’s House has been with us since almost the beginning. They’ve been there as Addy has stopped walking by herself and stopped eating. It’s such a relief to know that we have Julia’s House nurses and carers who are medically trained to look after Addy, and who will sweep in and say, ‘Now, go and take some time for you.’

“When you feel so worn thin from caring round-the-clock for your seriously ill child, just to have a few hours to sleep or have time as a couple is such a blessing.”

“Addy’s big brother, Samuel, also gets his own special visits from the Julia’s House sibling support team and he loves the devoted time that’s especially for him,” said Hayley. “He used to say, ‘When Addy gets better…’ and we’re thankful that they’ve been there to talk to Samuel.”

She said, “Having a child with a life-limiting condition is a journey of grief. You’re always trying not to let grief overtake you; you’re trying to step into life and embrace every moment.”

The Clarke family with Julia's House nurse Sam (Image: Julia's House)Julia’s House Nurse, Sam Roberts, who is a named nurse for the family, said, “Children like Addy are extremely vulnerable – they have medically complex needs and the pressure parents like Addy’s feel to help their children survive each day is immense.

“This Christmas, we have lots of plans to make Addy’s care sessions magical. We’ll have princess-themed crafting, baking and dressing up, plus sensory games and stories that all the children can enjoy with her,” said Sam.

“At home, Addy needs two carers at all times because of her complex needs and while she is in the safety of our care, mum and dad can complete their Christmas shopping and have time for all those seasonal preparations that will make their time together extra special.”

Hayley added, “Addy loves Christmas so much. She loves the presents, and she loves the lights, and she loves Father Christmas – so we go for it! And that’s amazing because looking back we will know that we have done all we can to make her happy and enjoy what she can, when she can.”

If you would like to make a donation to Julia’s House this Christmas you can donate to www.juliashouse.org/appeal