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”.
New £60m fund launched to fill potholes across Greater Manchester
Emily Sergeant
A new £60 million Pothole Fund has been created to repair more of the city region’s roads and pavements.
The fund – which has been set up by new Mayor Bev Craig, and is to be spent across every borough in Greater Manchester – is set to be a ‘major boost’ to efforts to tackle the pothole problem, as it will be enough to repair and maintain over a million square metres, or around 125 kilometres, of road across the wider region.
Around 2.5 billion trips are made on Greater Manchester‘s roads every year – not only by motorists, but also by buses, trams, cyclists, and pedestrians. So the fund will be used to help keep the highways network ‘safe and reliable’.
The fund will enable transport teams to repair hotspots where surfaces are in poor condition, as well as resurface roads and pavements to prevent them from deteriorating in the first place.
A new £60m fund has been launched to fill potholes across Greater Manchester / Credit: TfGM
Cold and wet winters, more traffic, and heavier vehicles using the roads are just some of the contributing factors to the pothole problem, which is why the fund is being introduced before autumn and winter properly arrives.
The amount each of the 10 boroughs will receive is based on an agreed funding formula, with the allocations being:
Bolton: £6,552,000
Bury: £4,374,000
Manchester: £8,155,000
Oldham: £5,258,000
Rochdale: £5,243,000
Salford: £5,388,000
Stockport: £6,820,000
Tameside: £4,980,000
Trafford: £4,992,000
Wigan: £8,241,000
The extra cash comes from the region’s transport budget, with leaders agreeing to reallocate funding towards highways maintenance, and it fulfils a commitment Bev made in her Mayoral Manifesto to repair more roads and make sure pavements are safe.
“Keeping our roads and pavements safe and well maintained is essential for a growing city region like Greater Manchester,” explained Mayor Bev Craig.
“That’s why I promised to create this new Pothole Fund that will benefit drivers and everyone who uses our roads to get around. Properly maintaining our roads will save motorists money on costly repairs, and by preventing potholes forming in the first place, councils won’t have to spend as much money on emergency road repairs.
“This issue came up repeatedly on the doorstep during my election campaign so I’m pleased that I can do something about it so quickly, building on the significant work already planned by councils across Greater Manchester.”
Featured Image – TfGM
News
Six GMP officers dismissed following racial discrimination allegations
Emily Sergeant
A number of police officers have been dismissed following an internal investigation, Greater Manchester Police (GMP) has confirmed.
Following the internal investigation into alleged racial discrimination against an officer on the Bury district – which was launched by GMP‘s Professional Standards Directorate in July 2024 – six officers and two former officers were found to have committed gross misconduct, and a ninth officer was found to have committed misconduct.
The investigation came after a report was made that an officer had been subjected to ‘racist behaviour’ by colleagues in December 2023.
The panel heard how PC Y – who has African heritage and has been granted anonymity – made a complaint after her accent was mimicked by a group of officers. She was also compared to a suspect with similar skin colour, and one of the dismissed PCs made a derogatory comment about her hair.
PC Y was supported and kept updated throughout the investigation, GMP assured, as well as providing evidence at the hearing.
The misconduct hearing lasted just over two weeks, with the panel concluding that the officers breached the Standards of Professional Behaviour relating to Equality and Diversity, Authority, Respect, and Courtesy.
The six officers who have been dismissed are PC Demi Green, PC Heena Rani, PC Lucy Granby, PC Matthew Barlow, PC Matthew Hill, and PC Mohammed Abid, while the former officers who were found to have committed gross misconduct are PC Jake Holt, and former Officer A – who has been granted anonymity.
The panel also found that Sergeant Adrian Bick’s actions amounted to misconduct, as there was no breach of the standard of equality and diversity.
As a result, the six serving officers have been sacked without notice, while the two former officers would have been dismissed had they not already left the force, and Sergeant Bick was issued a written warning.
“No officer or member of staff should have to come to work and experience discriminatory behaviour from the people they serve alongside,” declared Deputy Chief Constable, Terry Woods.
“Everyone has the right to feel respected, valued and part of the same team with a shared commitment to serving our communities and keeping people safe.”
Emily Sergeant -
14th January 2022 
