The family of an 11-year-old with a life-limiting illness have paid tribute to their "beautiful brown eyed girl" who has died.
Nicole Rich, from Throckley, Newcastle, had a rare and incurable neurodegenerative condition called Batten disease which causes seizures, childhood dementia, sight loss and mobility issues.
On Tuesday 24 October, her parents Gail and Matthew Rich, posted on social media that Nicole had died following complications from a chest infection.
The post read: "It is with the heaviest of broken hearts that we share our beautiful brown eyed girl, our darling Coley passed away peacefully yesterday afternoon in our arms, snuggled into Mammy & Daddy.
"Surrounded by love from the second she was born until her final moments, capturing the hearts of everyone she met.
"The past two weeks have been incredibly hard and traumatic and lots of difficult conversations were had. To preserve Nicole’s dignity we will just share that she fought severe complications resulting from a chest infection and she showed her strength like the princess warrior she has always been.
"Small but mighty - that was our Coley pops."
Nicole was diagnosed with Batten disease in 2016 at the age of four. At the time, no treatment was available on the NHS.
Nicole was placed on a pioneering medical trial at Great Ormond Street Hospital, in London, funded by a pharmaceutical company.
Three months later, there was further heartbreak for her parents Gail and Matthew when it was confirmed their youngest child Jessica also had Batten disease.
In 2017, at the age of two, Jessica went on to become one of the youngest in the world to have a pioneering brain operation to insert an implant that allows her to receive enzyme replacement therapy to help treat Batten disease.
As a result of early intervention, Jessica is making very good progress, showing little to no Batten symptoms at all. It has been a bittersweet reality for the family - that their eldest daughter's diagnosis could potentially save their youngest child's life.
As there were few treatment options available, the sisters were forced to travel hundreds of miles to two different countries, to receive treatment at two different hospitals.
Jessica was accepted onto a trial in Hamburg, while Nicole continued to be treated at Great Ormond Street.
Here started her family's campaign for enzyme replacement therapy to be provided on the NHS to allow the family to be reunited and the sisters to receive treatment together, in the UK.
Gail and Matthew also have a son Louis who does not have Batten disease, meaning the family needed support from family and friends to look after him while they travelled for treatment.
Two years later, Jessica was granted permission to receive enzyme replacement therapy at Great Ormond Street, alongside Nicole, but the family feared there was no guarantee the pharmaceutical company would continue to fund the trial.
The Rich family then campaigned tirelessly for treatment to be made available on the NHS, to secure their daughters' futures.
After months of lobbying, the National Institute for Health and Care Excellence approved Brineura for use in the NHS, allowing their children to continue to receive specialist infusions to delay the effects of Batten.
During the pandemic the Rich family had no option but to continue to travel to London for vital life-prolonging treatment for their daughters, despite the risk of coronavirus.
In September 2023, the family welcomed the news the girls will be able to receive their bi-weekly enzyme replacement therapy at the RVI in Newcastle instead of London.
What is Batten disease?
Batten disease is a rare, fatal, inherited disorder of the nervous system that typically begins in childhood.
Those diagnosed with the neurodegenerative condition aren't expected to live past the age of eight to 12.
It is estimated that only 30 people in the UK have the disease. There is no known cure for the disease.
What are the symptoms?
Poor eyesight which may result in blindness
Changes in personality and behaviour
Speech and motor skills problems that get worse over time
Short life-expectancy if not treated
How common is Batten disease?
Approximately 1-3 children are diagnosed with an infantile form of the disease each year, meaning there are probably between 15 and 30 affected children in the UK.
Jessica has the late infantile form of the disease - the same as Nicole's.
What causes Batten disease?Batten disease is an inherited genetic disorder that appears to affect the function of tiny bodies within cells called lysosomes.
Lysosomes are the “recycle bin” of the cell. They regularly break down waste, proteins, and naturally occurring fatty compounds called lipids into smaller components that can be discarded out of the cell or recycled.
Is there a cure for the condition?
There is no known cure for Batten Disease, however there are a number of treatments that can delay the effects of the rare condition.
Jessica and Nicole Rich have CLN2 Batten Disease. In 2019 an enzyme replacement therapy called Brineura was approved for use on the NHS. The therapy is proven to slow, or in some cases halt the progression of Batten Disease symptoms.
This is the treatment that the Rich sisters are currently receiving at Great Ormond Street Hospital.
Thanks in part to early diagnosis and treatment, Jessica Rich has not yet started to display any symptoms.
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