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”.
Two Trafford towns are set to see dozens of new EV charging points installed imminently
Danny Jones
Two big towns on the border of Greater Manchester and Cheshire are set to see a fresh batch of electric vehicle (EV) charging points installed throughout their streets by Trafford Council very soon.
Local authorities have teamed up with engineering and infrastructure company Amey to roll out a series of new EV charging stations across Trafford, starting with Altrincham and Hale.
Dating all the way back to 2020, the collaboration with Transport for Greater Manchester (TfGM), the brand was tasked with helping improve sustainable travel across the area as the government body’s electric vehicle charging suppliers.
Steady improvements have been made across the 10 boroughs, but this particular update marks one of the biggest network upgrades that the likes of Alty and Hale have seen for some time.
Good news – we are thrilled to announce the installation of our first public EV charge points in collaboration with @TraffordCouncil.
Issuing a statement on Wednesday, 17 September, the firm – which specialises in managing, designing and maintaining complex facilities and transport infrastructure across the country – announced that they will “start the installation of EV charging points in Trafford in the coming weeks.”
It is expected that “up to 100 new public charges” will be integrated throughout the respective town centres and residential streets as the suburbs continues to push towards its sustainability goals.
As per Altrincham Today, Amey account director Anna Gornall said: “We’re excited to launch our first EV charge points in Trafford, working in partnership with Trafford Council (TC) to make electric vehicle charging more accessible to local communities.
“As the UK’s leading provider of energy transition and decarbonisation solutions, we’re well placed to use our existing expertise and resources to support TC in delivering a holistic public EV charging network for local communities.
“We’re helping residents make the switch, so everyone can plug in and power a greener Trafford.”
The country at large has various carbon-free initiatives, including the aspiration of achieving net-zero emissions by 2050; the electoral ward of Trafford itself continues to thrive in this field, having recently won environmental accolades, including 12 ‘Green Flag Awards’ this past July.
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Peter Anderson, Managing Director of Transport Infrastructure at Amey, commented: “This is a pivotal moment in Amey’s energy transition strategy. By leveraging our extensive experience in whole lifecycle asset management, strong partnerships, and innovative solutions, we can deliver value for both our clients and members of the public who will use Amey’s electric vehicle charge points.
“Working with Trafford Council, we are making electric vehicle charging more accessible to local communities and helping residents make the switch to EVs.
“Amey is well-positioned to support emerging opportunities within this landscape, and we are delighted to be working with Trafford and other local authorities to provide the public EV infrastructure needed to achieve the government’s transition to net zero.”
As for Trafford Council, Corporate Director of Place, Richard Roe, went on to add: “We are delighted to be working with Amey on this project to bring more and better charging options to the people of Trafford.
“This is an extension to the current EV charging options in the borough and is great news for committed EV owners and those who are thinking about going electric.”
Featured Images — Publicity pictures (via Amey Ltd)
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Further disruption expected as more bus strikes announced across Greater Manchester
Emily Sergeant
Further disruption is expected as even more bus strikes have been announced across Greater Manchester this autumn.
It comes after the strikes set to place this month from 19 to 22 September were announced a couple of weeks back, and now a second round will take place towards the end of this month and going into early October.
In case this is the first you’re hearing of the upcoming industrial action, 2,000 workers who are employed by Stagecoach, Metroline Manchester, and First Bus Rochdale – all of which are firms among those that make up the bus part of the Bee Network – are due to walk out in a co-ordinated strike amid an ongoing pay dispute.
Unite the Union says all the firms are ‘highly profitable’ and it’s therefore ‘disappointing’ that workers are being denied a fair wage.
More strikes have been announced on the Bee Network this month / Credit: TfGM
At Stagecoach, around 1,000 drivers based across the Oldham, Stockport, and Middleton depots have rejected a pay offer of 3.5%, and 1,000 Metroline Manchester members will also do the same after turning down an ‘unsatisfactory’ below-inflation pay offer.
Workers at both Metroline and Stagecoach believe the offer doesn’t address years of low pay they’ve recieved, especially given the ongoing cost of living crisis.
Then, over at First Bus Rochdale, 110 members have rejected this year’s pay offer of 6%, as they feel this does not go far enough to address the fact they’ve had years of being paid less than their counterparts at other companies, and are still the lowest paid in the region.
Stagecoach, Metroline, and First Bus Rochdale, part of First Group PLC, are all firms which have seen a rise in profits in recent years.
2,000 drivers are set to stage strike action over two different periods / Credit: TfGM
The second round of strikes will now take place from from 30 September to 2 October.
Speaking ahead of both sets of upcoming strikes, Unite General Secretary, Sharon Graham, said: “These companies are very profitable but are putting greed over their hardworking members of staff.
“Further strike action will be extremely disruptive, however this is a dispute entirely of the bus companies’ making and they could solve it easily by coming back with a better deal.
“Our members involved in the dispute have Unite’s complete support.”
Unite Regional Officer, Colin Hayden, added: “The strikes this week as well as the further action we have called will cause travel chaos in Greater Manchester. However, it is entirely the fault of the employers involved, who have failed to address the issue of low pay and reward their staff accordingly.
Emily Sergeant -
14th January 2022 
