The family of an 11-year-old girl was told she may not reach her teenage years after a rare fatal disease devastated her health.

Ava-Bella Hartigan, from Bushey, suffered seizures around March 2020 before tests eventually revealed she had a genetic Batten disease.

Her auntie Amy Hartigan said the news was “like a cut to the throat”, adding: “It didn’t really sink in at first.

“It just didn’t seem real. But then once we saw her regressing and going downhill it was just more about making things better for her.”

Watford Observer: Ava-Bella Hartigan, from Bushey, was diagnosed with Batten disease.Ava-Bella Hartigan, from Bushey, was diagnosed with Batten disease. (Image: Amy Hartigan)

Neuronal Ceroid Lipofuscinosis (NCL), commonly referred to as Batten disease, denotes several neurodegenerative diseases with similar features.

They are incurable and cause symtoms such as vision loss, problems with movement and thinking, and eventually death. 

Amy, 22, said Ava’s condition deteriorated rapidly after being diagnosed aged 10.

“It’s gone downhill a crazy amount," she said. "This time last year she was able to run, dance and do everything like a normal 10-year-old would do.

“And now she’s wheelchair-bound and pretty much non-verbal.”

READ MORE

Ava went to Hartsbourne Primary School in Bushey but is set to start at a new school in Hemel Hempstead that can better cater for her needs.

The family has “banded together” and learnt to care for her while making as many "precious" memories as possible, particularly with her brother.

They have also been on an awareness and fundraising drive by organising events – one of which was at Teddies Tots Nursery Watford, where Amy works as a practitioner.

Watford Observer: Ava, her mum Kirsty and auntie Amy.Ava, her mum Kirsty and auntie Amy. (Image: Amy Hartigan)

In an online blog outlining Ava’s story, her mother Kirsty says: “Ava absolutely loves visiting her hospice, Keech Hospice Care.

“She loves music and one of her favourite things at the moment is kinetic sand.

“Ava also loves going on holiday, and we have spent time visiting local beach resorts and staying in caravans, as this is what Ava loves, and makes her happy.

Watford Observer: Ava-Bella.Ava-Bella. (Image: Amy Hartigan)

“We hope to make more precious memories during our family holiday to Spain next year.”

All money raised is going to the charity Batten Disease Family Association. To donate visit: https://www.justgiving.com/fundraising/Amyleigh-Hartigan

For more information on Batten disease visit: http://www.bdfa-uk.org.uk/about-batten-disease/.

Have you got a story for us? You can contact us here.

Follow us on FacebookTwitter and Instagram to keep up with all the latest news.

To receive breaking news alerts or newsletters sign up here.