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).
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.
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.”
Amelia’s father, Mike Carroll, said the “amazing” nurses and doctors treating his daughter were “incredible”.
‘Eyesore’ Northern Quarter car park to be redeveloped as ambitious new neighbourhood
Emily Sergeant
An underused multi-storey car park in the heart of the city centre is set to be sold and redeveloped as an ‘ambitious’ new neighbourhood.
The ‘eyesore’ car park situated in Manchester’s iconic Northern Quarter will be transformed into what Manchester City Council is hoping will become a green and sustainable neighbourhood set to complement the ‘unique and independent’ ethos of the area.
It comes after developers CBRE were appointed by the Council last year to market the Church Street site for disposal, ahead of seeking formal planning permission.
The new scheme will deliver more than 300 new homes – including 60 affordable homes – alongside new commercial opportunities and high-quality public spaces.
A before and proposed after of Church Street Car Park / Credit: Manchester City Council
It’s expected that the development will respect the heritage and architecture of the historic city centre neighbourhood and help to enhance the wider area, all while improving a car park site that has long fallen out of favour.
The new neighbourhood will also feature four new public squares and green spaces, along with opportunities for pedestrianising the surrounding streets as part of the public realm.
As mentioned, commercial space is also part of the plans, as the ground floor of the building will offer a mix of smaller, more affordable units to ensure local independent businesses can access the neighbourhood, alongside units for food and beverage outlets.
“For too long, the Church Street car park has been an eyesore and a barrier to the ongoing success of the Northern Quarter,” commented Cllr Bev Craig, who is the Leader of Manchester City Council.
“We want to bring forward a world-class development that has the potential to completely transform this part of the neighbourhood, together with the newest city centre public squares and green spaces.”
The final legal negotiations will conclude over the summer, and it’s expected that a public consultation will then take place to inform a future planning application.
Featured Image – Manchester City Council
News
‘Major milestone’ as planning permission secured to transform Oldham town centre
Emily Sergeant
Planning permission has been granted for six transformational sites in Oldham town centre, with developers calling it a ‘major milestone’.
This approval marks a key step forward in Muse Places and Oldham Council‘s long-term partnership with a shared ambition to deliver a ‘vibrant, inclusive, and sustainable’ town centre that not only reflects Oldham’s heritage but also meets the needs of the people who live there.
Following ‘extensive consultation’ with the local community earlier this year, where hundreds of people took park and shared valuable feedback to help shape the proposals, a total of 1,619 new homes will be delivered as part of the plans, offering a mix of high-quality, energy-efficient apartments designed for families, older people, and young professionals.
The plans also include social and affordable housing provision too in a bid to help more local people find a home that suits their needs and circumstances.
According to the Council and developers, the approved plans will bring underused sites back into ‘productive’ use and support a ‘cleaner, greener’ future for Oldham, opening the door to new opportunities for local businesses and communities alike.
“This decision marks a key milestone in delivering on our promises for Oldham,” commented Cllr Arooj Shah, who is the Leader of Oldham Council.
Planning permission has been secured to transform Oldham town centre / Credit: Muse Places
“Oldham Council and Muse have a shared, ambitious vision to create a quality place that people genuinely want to spend time in.
“I am thrilled that we can continue moving forward with our plans, delivering high-quality homes, including the provision of social and affordable homes to meet local needs. We will also support local businesses and create opportunities for everyone, as part of a clean, green and sustainable future.
“Together, we are creating a place that we can all be proud of.”
Site investigation works are now complete at the Civic Centre, and works at Prince’s Gate will begin shortly, followed by the former Magistrates’ Court later this year.
With planning consent secured and preparatory works underway, construction at Prince’s Gate is expected to begin this autumn.