An eight-year-old Cheshire girl with a rare degenerative disease has become one of the first to receive a pioneering new treatment that will attempt to save her eyesight.
Amelia Carroll, from Poynton near Stockport, was diagnosed with CLN2 Batten disease – an incurable and fatal condition that affects roughly around 30-50 children in the UK – when she was just two.
The disease is caused by a change in a gene that is responsible for making a specific enzyme in the nervous system, and it results in seizures, a gradual decline in a child’s ability to walk, speak and see, as well as progressive dementia.
Amelia’s 11-year-old brother Ollie also has the same condition, yet has sadly already lost his eyesight.
“We have watched our son Ollie go blind, and now the same is starting to happen to Amelia,” mother Lucy Carroll explained, which is why her and her husband Mike decided to contribute to a fundraising campaign together with families of other children affected by the disease alongside the Battens Disease Family Association (BDFA) to help fund a world-first trail of a groundbreaking new treatment at Great Ormond Street Hospital (GOSH).
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Over £200,000 was raised through the campaign, and clinicians and researchers at GOSH and ICH say the treatment programme has the potential to transform the quality of life for children living with CLN2 Batten disease.
Amelia became one of the first children in the world to receive the treatment, and is said to already be responding to it “perfectly”, CheshireLive reports.
For the past two years, enzyme replacement therapy has been used to help prevent neurological deterioration in children with CLN2 disease. The drug, called Brineura, was first approved for use on the NHS in 2019 and is administered directly into the brain by a regular infusion.
It has shown to restore enzyme activity and slow the onset of disability.
However, this infusion into the brain does not prevent children going blind, as the enzyme cannot cross the blood-retina barrier, and this means that the nerves in the eye cannot function and vision is lost, which is why clinicians and researchers at GOSH and ICH are trying a the treatment to prevent the onset of blindness instead.
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To do this, GOSH said they are using the tiny amount of the drug leftover from the brain infusion and injecting it directly into the back of the eye.
The 18-month treatment trail – which is said to be being given on a compassionate use basis – hopes to prevent eyesight deterioration in children by providing the enzyme that the nerves in the back of the eye need to function.
Ms Carroll said “to save a child’s sight would be incredible”, and if it does indeed work, it would “make such a big difference to Amelia’s quality of life”.
“We just pray it works,” she added.
Speaking on the groundbreaking treatment trail, Professor Paul Gissen, from GOSH and the UCL GOS Institute of Child Health, said: “If successful, we hope our work can pave the way to saving the sight of more children with this disease to preserve their quality of life for as long as possible.”
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Amelia’s father, Mike Carroll, said the “amazing” nurses and doctors treating his daughter were “incredible”.
Sara Cox tipped to replace Scott Mills for Eurovision 2026
Danny Jones
Greater Manchester’s very own Sara Cox is reportedly set to replace Scott Mills at the 2026 Eurovision Song Contest following his sacking from the BBC.
Mills was axed by the news and entertainment corporation on Monday, 1 April, for ‘misconduct’, which has now been revealed to involve a historic relationship with a boy aged under 16.
While the fallout surrounding Scott Mills is only just beginning, the task of finding his replacement in multiple roles has already begun – though it looks like one may have already been decided.
Now reported by multiple outlets, it appears as though Bolton-born Sara Cox is first in line to pick up Mills’ co-hosting duties for this year’s Eurovision show.
Having already been involved with coverage of the event on multiple ocassions in the past, it now appears that she’ll be in post for the Grand Final too.
As a long-standing radio and TV staple, it only takes a quick look at social media to see how much backing she has already received in the past, with a post from last year reading, “Great to have Sara Cox back commentating on Eurovision“, and others from over a decade ago praising her past presenting.
In fact, it might not end just here either, as many are already calling for her to replace the 53-year-old breakfast show host.
From her days as a Radio 1 favourite to becoming a BBC mainstay across the board, her popularity only seems to have grown over the last decade, especially thanks to her charity fundraising efforts.
While an official announcement is yet to be made, the feeling is that it is all but confirmed for her to take over from Mills as part of the Eurovision commentary team.
As for the now vacant early morning slot on BBC Radio 2, the likes of Claudio Winkleman, Vernon Kay, fellow Manc Nick Grimshaw, the aforementioned Rylan, and several others have all been floated as potential stand-ins/future full-time hosts.
Do you think both should be given to Sara Cox, and if not, who would you like to see replace Scott Mills?
Millions of UK workers to get pay rises from today as National Living and Minimum Wage increases
Emily Sergeant
Millions of workers across the UK are set to begin receiving substantial pay rises from today.
After the Government announced back in November that it would take the recommendations made by the Low Pay Commission, and increase both the National Minimum Wage and National Living Wage, those changes have now come into force in a bid to ensure people on lower incomes are ‘properly rewarded’ for their work.
If you’re unfamiliar with the Low Pay Commission, it’s an independent body made up of employers, trade unions, and experts whose role is to advise the Government on the minimum wage.
As mentioned, the rate recommendations introduced today were agreed unanimously by the Commission.
This means that the living wage, for eligible workers who are aged 21 and over, has now risen by 4.1% from today to £12.71 an hour.
For a full-time worker, that means a pay increase of £900 a year.
Millions of workers in the UK are getting pay rises from today / Credit: John Kakuk (via Unsplash) | Pexels
The National Minimum Wage rate for workers aged 18 to 20-year-olds has also increased today by 8.5% to £10.85 an hour, and then for 16 to 17-year-olds, and those on apprenticeships, the rate has increased by 6% to £8 an hour.
“The recommendations we made last autumn sought to balance the need to protect the economy and labour market, whilst providing a real-terms increase for the lowest-paid members of society,” commented Baroness Philippa Stroud, who is Chair of the Low Pay Commission.
“A lot has changed since we gave our advice to the Government last autumn, and we are now beginning to gather evidence for recommendations later this year.
“The current economic uncertainty makes it essential that the Commission hears from those affected by the minimum wage and builds consensus for evidence-based recommendations.
Workers aged 21 and over are now legally entitled to the National Living Wage after the age threshold for the highest rate was lowered from 23 in 2024.
National Minimum Wage rates are available to workers aged 16 upwards.
Emily Sergeant -
14th January 2022 
