Maidstone mum creates charity Hope for Hermione after daughter diagnosed with rare brain disorder BPAN

A mum has set up a charity in honour of her daughter who has an extremely rare brain disorder.

Caryn de Nocker was devastated after finding out nine-year-old Hermione will be left with dementia, Parkinson's and a reduced life expectancy.

She said: “It was almost a relief because we'd waited nearly a year-and-a-half to find out what was wrong.

“This was after having test after test and nothing was coming up.

“Now we can make a plan for her future. It could have been pretty grim, but actually, we can make the best out of it for her.

“She'll never have a job and be able to do what normal people do.

“However, I don't want her disability to define her. She can still make a difference in society with a charity that has her name on it.”

Caryn, who is originally from South Africa, moved to Downswood near Maidstone in 2011.

She told KentOnline that Hermione was just 17 months old when she had her first seizure.

Sadly, it was set to be one of many.

“Once she had been seen by doctors it was confirmed that she had a seizure which had lasted well over two hours,” the 46-year-old said.

“This was obviously very distressing to us as parents and so we began to keep a close eye on her."

In October 2019, Hermione was diagnosed with epilepsy and had severe speech and language difficulties.

By December of the following year, she was also diagnosed with autism, which left the family with more questions.

Caryn works as a higher level teaching assistant (HLTA) at Snowfields Academy near Bearsted.

Despite having experience with students who have special educational needs, she still knew something else was wrong with Hermione.

After pushing for further tests, in May it was discovered the youngster had Beta Propeller Accumulation Neurodegeneration (BPAN).

It is caused by a mutation of a gene called WDR45, which leads to a build up of iron in the brain.

There is currently no cure and it is understood to affect around 500 people worldwide.

Most children with BPAN have already developed epilepsy or an intellectual disability, as well as delayed gross and fine motor skills.

They may also experience hand wringing, teeth grinding and sleep disturbances.

Hermione’s condition will gradually decline over time.

Dementia and Parkinson’s-like symptoms, such as slowed movements and difficulty walking, typically begin during early adulthood.

Caryn added: “My mind went straight to when I die – what is going to happen to my child who has severe and complex needs?

“She will need looking after for the rest of her life.

“I don't ever want it to be my son's burden to carry because when he was born that's not what he signed up for.”

As a result, Caryn along with her husband Rory, decided to set up a charity called Hope for Hermione.

It aims to raise awareness and money for researching a cure or treatment to halt the condition through gene therapy.

Caryn added: “I never saw this coming. A year ago, I would have laughed and said ‘yeah right’ but now we have a fully-fledged charity.

“For many of those with BPAN, some of them never walk or talk and they need constant support.

“Although we have received the worst news possible, we still can make a change and have something good come out of all the tears."

You can donate to the JustGiving page by clicking here.

If you’d like to support the charity, please get in touch via info@hopeforhermione.com