THE heartbroken mother of a three-year-old girl who died of a rare disease hopes to raise awareness of the condition that “stole” her little girl “piece by piece”.

Nikki Backus, of Kidderminster, is raising money for the two charities that helped her daughter Ruby Backus in her fight against the rare genetic condition Sandhoff Disease.

Ruby, who had a twin brother Sam, died on November 23 last year after battling the disease, which affects just one in 400,000 people.

A fun day and shopping event will be held at the Land Oak pub, in Kidderminster, on Saturday, from 1pm to 7pm, to help give something back to Acorns Children’s Hospice and Cure and Action for Tay-Sachs (CATS) Foundation, a UK charity that provided specialist equipment and paid for respite holidays and funeral costs.

Mrs Backus, 38, told The Shuttle: “There’s only a handful of cases of Sandhoff in the UK – most people don’t know about it, I wish I didn’t.

“There just isn’t the pushing [for research] because there’s not that many people that need it but it’s devastating. It’s terrible the way in which Ruby was taken, she suffered all her little life.”

Ruby began to suffer from the effects of Sandhoff, which causes a deterioration of the central nervous system, at the age of one when she started to have seizures “out of the blue” during a trip to America to see her father Jim Backus.

“Quite quickly, she deteriorated”, Mrs Backus added. “Doctors were testing for common things and those tests came back as normal.

"With every level of testing, you just know that the outcome would be worse. The prognoses were getting darker and darker.”

Ruby suffered with a lack of muscle control, digestion problems and the ability to see, hear or speak. Sandhoff eventually stopped her smiling and laughing and caused her airways to collapse a number of times.

“It just kind of steals them piece by piece,” Mrs Backus added.

“When she did smile, I can’t explain how wonderful that was.

“She never left my side – I always wanted her to know she wasn’t on her own.”

Last November, Ruby began to deteriorate further and the decision was made to move her to Acorns Children’s Hospice in Worcester, where end of life care had been arranged.

Ruby had been a day visitor at Acorns, where she enjoyed using the sensory room and the hydrotherapy pool, as “it was the one place that gravity was not against her”.

Mrs Backus added: “To be transferred, they had to take the ventilator away. That was probably the hardest thing I ever had to do.

“The doctor said she wouldn’t last half an hour. Being the tough little girl she was, she suffered another airway collapse but despite no medical intervention she breathed without the ventilator for 12 hours.”

Instead of a hymn, Ruby’s family sang Twinkle Twinkle Little Star at her funeral, as Mrs Backus had told Sam that his sister had gone to be a star.

Mrs Backus added: “We’ve always connected Ruby to a butterfly – they are graceful and beautiful but they’re not here for long.”

After the service, Ruby’s family released 41 pink balloons, each one representing a month of her life.

On Saturday, there will be face painting, a magic show and the opportunity for children to meet Spiderman as well as a number of stalls selling gifts for Mother’s Day.

Ruby’s aunt Leanne Christie will also be selling homemade items for children with special needs.

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