A close-knit family has been told that their 10-year-old daughter may not make it through this winter.
Refsum’s disease sufferer Isabelle Hopkins is deaf, blind and has kidney and liver problems, a dislocated hip and problems with her muscles that stop her from living life like other children.
Issie was born with the degenerative genetic condition but has defied all odds after frequently coming in and out of hospital over the last two years.
But she could have just one year left to live after her devastated family were told to prepare for her death.
Her mother Miranda Hoogewerf said: “Over the last 10 years Issie has been the most wonderful, happy girl but she has become very ill now.
"She still smiles and laughs. And if she doesn’t like something it’s like having a strop.
"But they [doctors] will be shocked if she gets through the winter.
"It has taken time to understand that she is not going to be here but she is suffering so much.
"I don’t want to see her continue to suffer."
Children with refsum’s disease can live up to about 13 years but as Isabelle’s condition has continued to worsen her treatment has been stopped.
Mrs Hoogewerf who has two other young children – Giles, 2 and Pippa, 1, with her husband Bevan, said the family who live in Berrylands, and own Langley’s restaurant in Surbiton, were having a difficult time.
She said: “It is getting a lot harder now. She used to smile and play but now she is tired a lot of the time so she does not want to communicate.
"We are having a difficult time because we don’t know how to prepare for it. We are struggling."
Isabelle with mum and dad, Miranda and Bevan Hoogewerf
She added: "It’s hard because the children will never remember her and they are at a stage where they are so much fun.
"Issie is just normal to Giles. He has been born into it. If we go past the hospital he always asks ‘is Issie in there?’
"But he does not understand enough to know there is something wrong with her."
Even though Isabelle spends a lot of time in bed, she still enjoys music therapy with the sounds of Beethoven on the piano providing vibrations for her to take in.
She also continues to swim regularly at her hospice school in Putney and sees her birth father every week.
Mrs Hoogewerf said: “I have looked after her for 10 years all day and night so it will be a painful hole inside.
"All of the rooms in the house are especially adapted for her so it would be massively hard.
"We just need to treat her like normal – make sure she is happy and comfortable.”
The Hoogewerf’s spoke about Issie to ask for support for children with genetic conditions through charity Genetic Disorders UK.
Genetic Disorders UK is calling on people to celebrate the 18th birthday of Jeans for Genes day on Friday, September 20 by donning some denim and donating money at jeansforgenesday.org.
To find out more about Genetic Disorders UK click here. To donate to jeans for genes day click here.
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