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”.
Body found in search for missing Stockport postman Steven Caulfield
Emily Sergeant
A body has been found in the search for a 70-year-old former postman from Stockport who went missing a couple of months ago.
Greater Manchester Police (GMP) issued a public appeal after to help find Steven Caulfield, who was last seen in the vicinity of the golf course next to Middlewood Way, in the Marple area of Stockport, at around 4pm Tuesday 1 October, after he failed to return home from a walk.
Since Steven was reported missing, an extensive missing person investigation was launched by Stockport detectives, alongside a widespread search by specialist GMP officers, and a number of missing appeals were also published on social media and shared widely – not just in Greater Manchester, but all across the UK.
#UPDATE | At around 12pm today (Wednesday 11th December 2024), an officer sadly located a body in woodland near to Middlewood Way in Stockport.
At this time, we believe this is likely to be Steven Caulfield who went missing on Tuesday 1 October.
But sadly, at around 12pm yesterday (Wednesday 11 December 2024), a GMP officer has sadly located a body in a woodland area near to Middlewood Way in Stockport, and at this time, police believe this is likely to be the body of Steven Caulfield.
Steven’s next of kin have been made aware and are currently being supported by officers.
There are not believed to be any suspicious circumstances surrounding Steven’s death, according to GMP.
Speaking on the discovery of the body, Chief Superintendent Lewis Hughes, Stockport District Commander and GMP lead for Disaster Victim Identification, said: “Our deepest condolences remain with Steven’s family and friends, who have been devastated by this traumatic news, we have officers supporting them through this difficult time.
“It has been a challenging couple of months since the missing report came to us in GMP and our Tactical Aid Unit, Specialist Search, Canine and Underwater Units were all utilised to search for Steven in challenging conditions and locations around the Middlewood Way.
“We hope today’s discovery provides some answers for Steven’s loved ones.”
Featured Image – GMP
News
Plans submitted for new £250 million mixed residential neighbourhood in Stockport
Danny Jones
Plans have officially been submitted for a brand-new £250 million neighbourhood in the heart of Stockport town centre.
The new mixed residential mini-village simply dubbed, ‘Stockport 8’, has been put forward by the local LLP of the same name, set up as part of a partnership between Stockport Council and ECF (English Cities Fund – itself a joint venture between Homes England, Legal & General, and Muse).
Set to cost a quarter of a million, the proposed neighbourhood is just the latest step of the ever-growing Stockport Mayor Development Corporation (MDC) masterplan, a wider £500m scheme to transform the specific Stockport Town Centre West area into the most sustainable, liveable town centre in the UK.
With a planning application now submitted, we’ve also been given another look at what the new Stockport neighbourhood would look like if green-lit.
We first heard reports of the new Stockport 8 neighbourhood back in January after CGIs of what the developers envisioned were shared with the public, alongside how it fit into the West blueprints.
The overall goal of the development is to create a high-quality, green neighbourhood featuring a mix of housing tenures to suit existing and new homeowners and tenants.
Aiming to build a grand total of 1,300 high-quality, energy-efficient homes all told as part of a whipping £1bn investment in Stockport town centre, there will also be room for businesses and community spaces on the ground floor level to knit into the existing centre and its community.
There’s also an insistence from those behind the project to create different atmospheres, creating new environments for people to meet, socialise, relax and enjoy, such as residential courtyards and green roofs, as well as the usual modern amenities re: parking, vehicle charging, bike storage etc.
All told, the council and their development partners – which include chief designers, shedkm, and landscape architects, Planit – are hoping to contribute nearly a third of the homes in Stockport MDC masterplan, which is hoping to surpass 4,000 homes over a total of 130 acres.
As well as helping contribute to the busy and vital road network that is continuing to be redeveloped, the goal is to create a safe and sustainable travel environment too, with a pedestrian-only street running through the centre of the scheme.
With one big road (Cook Street) running throughout the neighbourhood, the new builds will also look to knit into Stockport’s existing structure, celebrating the area’s rich heritage and character by even reintroducing historic street patterns and street names.
If you’re interested in the skyline-changing new project, you can find out more HERE.
Planning permission applications in Manchester can sometimes take upwards of 13 weeks due to the various individual stages and processes, so we’re still a while of way from knowing if/when Stockport 8 will begin work but with a summer 2027 completion date, it could be a huge moment for the town.
Emily Sergeant -
14th January 2022 
