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”.
Police appeal for information regarding ‘serious assault’ around Old Trafford before United game in October
Danny Jones
Greater Manchester Police (GMP) are appealing for fresh information on a “serious assault” committed around the Old Trafford area earlier this year.
GMP issued a press release on Friday, 9 December, asking for members of the public to come forward and share any details they might have on the attack carried along Seymour Grove in Trafford around 1:30pm on 16 October 2022.
They go on to explain that “a man in his 20s was assaulted by a group of unknown males” on the way to the game at Old Trafford, adding that “it is believed that the suspects were also attending the Manchester United vs Newcastle United match.”
It remains unclear whether or not the assailants were travelling fans in town to support the away side but GMP are looking to speak to the men pictured as they may be able to help with enquiries.
Credit: GMP
As detailed in the statement, “the victim suffered a severely broken jaw, a broken nose, numerous cuts to the face, and damage to the nerves of four teeth which he is likely to need further medical treatment for.” Terrible stuff.
The authorities are continuing to investigate the Seymour Grove assault after having followed up on a number of lines of enquiry since the incident took place and are now asking for the public’s help.
Anyone with information, or anyone who may have footage/images, or can identify the men in the photos, can contact Greater Manchester Police on 101, quoting log number 1589 on 16/10/22; or, alternatively use the Live Chat service HERE.
Alternatively, information can be shared anonymously via the independent charity Crimestoppers on 0800 555 111.
Featured Image — Greater Manchester Police/David Dixon (via Geograph)
News
Football fans call out ‘tone-deaf’ FA TikTok for mocking ‘life-threatening’ head injury
Danny Jones
Football fans are calling out the official FA Cup TikTok account for mocking a serious head injury suffered by a Stockport County player earlier this week.
The Hatters beat Charlton Athletic in fine fashion with a 3-1 victory on Wednesday, 7 December, with Will Collar’s hattrick (the club’s first since 2019) sending them through to the next round of the cup.
However, another notable moment from the night was right back Macauley Southam-Hales’ collision with advertising hoarders after a shove from Charlton captain George Dobson.
As the likes of County fan Joel Ward wrote on Twitter, many online were quick to slam the clip for joking about an injury that hospitalised the player, the consensus being that it isn’t a “great look” for the FA.
Don’t think it’s a great look that the official @EmiratesFACup account is making joke tik toks about a head injury that sent our player to the hospital just a few weeks after a player nearly lost his life to a similar incident pic.twitter.com/7r9Stm8Vnr
While many reacting in the comments recognised that Dobson didn’t intend to hurt Southam-Hales by shepherding the ball out of play, the Stockport defender does hit the predominantly metal structure with some force and was left in clear discomfort following the smash.
The video itself has since been removed from the official account following the backlash but people are still reacting as the clip continues to be reshared on social media.
The clip was branded as everything from “disgusting” and “disgraceful” to “simply unbelievable”.
Considering it’s the FA cup and I’m assuming it’s an FA ran account, it should be someone pretty senior and stuff is checked thoroughly first before posting so this one’s gone under the radar I reckon.
What’s most concerning is that despite the recent push to promote awareness surrounding concussions, brain trauma and even heading the ball – which, historically, haven’t been properly monitored in football – whoever posted the video on behalf of the FA didn’t consider the very serious nature of the incident.
As alluded to above, perhaps what makes the post even more shocking and tone-deaf is that it comes just weeks after Bath City player forward Alex Fletcher was placed in intensive care following an almost identical accident.
The 23-year-old underwent life-saving brain surgery and although he has since been discharged from the hospital, he is said to have a “long road to recovery ahead of him”.
County confirmed that despite being the all-clear pitchside, Southam-Hales was taken to the hospital as a precaution after he suffered significant swelling around the neck area. Nevertheless, as we know all too well, it could have been much worse.
As for the FA, they have now apologised for the inappropriate attempt at humour, confirming that they have removed the video from all official accounts, admitting that “it should never have been published and assuring that they will “review [their] processes to ensure this never happens again.”
You can watch the full highlights from Stockport County vs Charlton Athletic HERE but we’d recommend you watch Alan Shearer reminding everyone who plays football to be more vigilant when it comes to head injuries.
Emily Sergeant -
14th January 2022 
