Connect with us

Published

on

A “life-changing” new treatment for people living with sickle cell disease is to be made available on the NHS. 

Around 4,000 people living with the potentially fatal blood disorder could see their lives transformed by the drug Voxelotor.

Hazel Attua, 33, has been taking the medication for nearly two years. The former finance worker said the chronic pain forced her to quit her career.

She said: “One of the most significant changes I’ve experienced is in my energy levels. Previously, the exhaustion from sickle cell made it challenging to function, impacting my ability to work and enjoy time with loved ones. I wasn’t living the life of a 20-something by any means.

“Since starting Voxelotor, my energy has soared, making a massive change to my daily routine. This has not only improved my physical well-being but also my mental health.”

Sickle cell disorder causes red blood cells to take on a “sickle” shape, break and die early, which vastly reduces the amount of oxygen they can carry.

It leaves those affected with chronic anaemia and episodes of crippling pain, called crises, which can be life-threatening and frequently require hospital care.

Sickle cells and normal red blood cells. Pic: Dr Graham Beards/CCv3
Image:
Sickle cells and normal red blood cells. Pic: Dr Graham Beards/CCv3

The inherited condition also gives rise to a range of life-altering complications such as stroke, chronic fatigue, delayed growth, infections, progressive tissue and organ damage.

The drug works by helping haemoglobin – a protein in red blood cells – hold on to more oxygen and prevent red blood cells from becoming misshapen.

It was initially rejected for widespread NHS use but the National Institute for Health and Care Excellence (NICE) has approved the drug in final draft guidance after a reduction in its price.

New treatment will help bridge widening health inequalities

Sickle cell disease disproportionately affects Black African and Caribbean populations: a community that suffers discrimination in access to health care.

And for people from this community who live with the inherited blood disorder, that health inequality is compounded.

The disease needs constant medical attention – a sickle cell crisis causes crippling pain and requires emergency intervention.

But most A&E staff will not have knowledge of the condition.

One senior researcher found that the same clinical staff can make dangerous assumptions about the need to access pain-relieving drugs based on race.

Joni Jabbal from the King’s Fund warned: “Getting good care for sickle cell disorder is a postcode lottery.”

This substandard care can lead patients to stop accessing healthcare and fall out of the system.

Despite being the fastest-growing genetic condition in the UK, there has been only one licensed treatment for the disorder over the past twenty years.

In contrast, 511 drugs have been approved for cystic fibrosis since 1988. 

John James, chief executive of the Sickle Cell Society, said: “Today’s approval by NICE marks a significant milestone in sickle cell care, addressing decades of limited access to disease-modifying treatments.

“We look forward to this being the first in a series of new treatment options for people living with sickle cell disorder.”

Read more from Sky News:
NASA and China’s space race explained
Football star hit with new betting ban
Black cab drivers file Uber lawsuit

Helen Knight, director of medicines evaluation at NICE, said: “This is great news for people with sickle cell disease, particularly given the health inequalities experienced by people with the condition.”

The drug, which is taken as a once-a-day tablet, will be available straight away.

Follow Sky News on WhatsApp
Follow Sky News on WhatsApp

Keep up with all the latest news from the UK and around the world by following Sky News

Tap here

Amanda Pritchard, chief executive of NHS England, said the service had “worked hard to make this life-changing treatment available”.

She added: “Sickle cell disease can have a huge impact on people’s lives, and this is one of the biggest breakthroughs in treatment in recent decades, so I know how much this announcement will mean for thousands of people across the country.”

Around 17,500 people are living with sickle cell disease in the UK and the condition is more common among people with an African or Caribbean family background. Of those 17,500, about 4,000 are believed to be eligible for the new treatment.

Voxelotor is also known as Oxbryta and is made by Pfizer.

Continue Reading

UK

Storm Bert: Father rescues son from sinking car as floods wreak havoc

Published

on

By

Storm Bert: Father rescues son from sinking car as floods wreak havoc

Tragedy almost struck a family in West Yorkshire after a father had to suddenly rescue his 11-month-old son from their flooded car.

Andre Randles, 22, was driving with baby Luca from Hebden Bridge to his father’s home in Todmorden to watch a football match on Saturday afternoon.

He was diverted away from his main route when he hit a dip and went “straight into a puddle of water”.

Speaking to Sky’s Shingi Mararike, Mr Randles said he thought it was a shallow puddle that he could drive through but soon his car began to float.

Storm Bert live: Follow latest updates

Paige and Andre
Image:
Andre Randles’ partner Paige Newsome said the incident was ‘really scary’

He called emergency services but soon “water started seeping in”.

“I thought I’m going to have to get out, I’m going to have to smash a window,” Mr Randles said.

More on Weather

He wound down his and his son’s windows, and climbed out before rescuing his son.

Please use Chrome browser for a more accessible video player

‘Devastating’ flooding in Wales

“The water was chest high, I held him up as high as I could to keep him out of the water.”

“It wasn’t raining so heavily, I’ve driven in much worse rain,” he added.

Mr Randles, a self-employed roofer who relies on the car for work, said he remained calm during the ordeal and was helped by the fact that Luca was asleep during the rescue.

Mr Randles’ partner Paige Newsome – who was not in the car at the time – said the incident was “really scary”.

“To think I could have actually lost them both – I don’t know how I would’ve lived,” she said.

Please use Chrome browser for a more accessible video player

Resident feels ‘abandoned’ in floods

Check the weather forecast in your area

The road has been flooding for at least two decades, the couple said.

“What is it going to take for the council to sort it out? Does a fatal incident have to happen? It’s been going on for years,” Ms Newsome said.

The couple are worried about affording another car as well as Christmas celebrations.

But Mr Randles said: “I’m grateful that we got out safely and that we can spend his first birthday and Christmas as a family.”

Storm Bert has brought more than 80% of November’s average monthly rainfall in less than 48 hours to some parts, the Met Office said.

Around 300 flood warnings and alerts are in place in England, with another 100 in Wales and nine in Scotland, as heavy rain and thawing snow bring more disruption across the UK.

A major incident was declared by Rhondda Cynon Taf County Borough Council in South Wales after homes and cars were submerged in water.

‘It is devastating’

Gareth Davies, who owns a garage in Pontypridd, a town in Rhondda Cynon Taf, told Sky’s Dan Whitehead that flooding has put his small business “back to square one”.

As the River Taff burst its banks, the majority of the vehicles in Mr Davis’s garage were so damaged he says they will have to be written off.

Garage in wales destroyed by Storm Bert
Image:
Mr Davies speaking to Sky’s Dan Whitehead in his flooded garage

Garage in wales destroyed by Storm Bert

“I am gutted,” he said, standing in his flooded garage, most of which is also covered in oil after a drum tipped over.

“How long is it going to take to sort out? I am going to lose money either way. I can’t work on people’s cars when I am trying to sort all of this out.

“It is devastating.”

Mr Davies said he has never had an issue with water coming into his garage until now.

Garage in wales destroyed by Storm Bert

Pointing to one car that had been hoisted into the air before water reached it, he said: “Lucky enough, I did come in this morning just to get that car up in the air.

“I don’t know what to say, I have been working flat out for two years to build this up and something like this happens, and it just squashes it all.

“This has put me back to square one.”

At least two to three hundred properties in South Wales have been affected by flooding, Councillor Andrew Morgan, leader of Rhondda Cynon Taf Borough Council, said on Sunday.

He said the affected buildings are a mixture of residential and commercial properties, after the weather turned out to be worse than what was forecast.

Continue Reading

UK

MP behind assisted dying bill says she has ‘no doubts’ – as she rejects minister’s ‘slippery slope’ claim

Published

on

By

MP behind assisted dying bill says she has 'no doubts' - as she rejects minister's 'slippery slope' claim

The Labour MP behind the assisted dying bill said she has “no doubts” about its safeguards after a minister warned it would lead to a “slippery slope” of “death on demand”.

Kim Leadbeater told Sky’s Sunday Morning With Trevor Phillips that she has “huge respect” for Justice Secretary Shabana Mahmood, but that she doesn’t agree with her opinion.

In a strongly worded intervention ahead of Friday’s House of Commons vote, Ms Mahmood said the state should “never offer death as a service”.

She said she was “profoundly concerned” by the legislation, not just for religious reasons, which she has previously expressed, but because it could create a “slippery slope towards death on demand”.

Asked about the criticism, Ms Leadbeater said: “I have got a huge amount of respect for Shabana. She’s a very good colleague and a good friend.

“In terms of the concept of a slippery slope, the title of the bill is very, very clear.

“It is called the Terminally Ill Adults (End of Life) Bill. It cannot include anybody other than people who are terminally ill, with a number of months of their life left to live. It very clearly states that the bill will not cover anybody else other than people in that category.”

More on Assisted Dying

Ms Leadbeater’s bill proposes legalising assisted dying for people with six months left to live, on the approval of two doctors and a High Court judge.

She wants people who are in immense pain to be given a choice to end their lives, and has included a provision in the legislation to make coercion a criminal offence.

The matter will be debated for the first time in almost 10 years on Friday, with MPs given a free vote, meaning they can side with their conscience and not party lines.

As a result, the government is meant to remain neutral, so the intervention of cabinet ministers has provoked some criticism from within party ranks.

Labour peer Charlie Falconer told Sky News Ms Mahmood’s remarks were “completely wrong” and suggested she was seeking to impose her religious beliefs on other people.

Please use Chrome browser for a more accessible video player

Kevin Hollinrake says he will be in favour of the assisted dying bill

Read more:
Mum diagnosed with cancer tells of the day her life changed ahead of assisted dying vote

Why is assisted dying so controversial and where is it legal?

Asked about his comments, Ms Leadbeater said it was important to remain “respectful and compassionate throughout the debate” and “for the main part, that has been the case”.

She added: “The point about religion does come into this debate, we have to be honest about that. There are people who would never support a change in the law because of their religious beliefs.”

Ms Leadbeater went on to say she had “no doubts whatsoever” about the bill, which has also been objected by the likes of Health Secretary Wes Streeting and former Labour prime minister Gordon Brown.

Asked if she has ever worried about people who don’t want to die taking their own lives because of the legislation, Ms Leadbeater said: “No, I don’t have any doubts whatsoever. I wouldn’t have put the bill forward if I did.

“The safeguards in this bill will be the most robust in the world, and the layers and layers of safeguarding within the bill will make coercion a criminal offence.”

Continue Reading

UK

Mum diagnosed with cancer tells of the day her life changed ahead of assisted dying vote

Published

on

By

Mum diagnosed with cancer tells of the day her life changed ahead of assisted dying vote

There is a lot at stake this week for Sophie Blake, a 52-year-old mother to a young adult, who was diagnosed with stage four cancer in May 2023.

As MPs vote on whether to change the law to allow assisted dying, Sophie tells Sky News of the day her life changed.

“One night I woke up and as I turned I felt a sensation of something in my breast actually move, and it was deep,” she says, speaking from her home in Brighton.

“Something fluidy, a very odd sensation. I woke up and made a doctor’s appointment.”

Sophie underwent an ultrasound followed by a biopsy before she was taken to a room in the clinic and offered water.

“They said, ‘a hundred percent, we believe you have breast cancer’.”

But it was the phone call with her mother that made it feel real.

More on Assisted Dying

“My mum had been waiting at home. She phoned me and said ‘How is it darling?’ and I said ‘I’ve got breast cancer,’ and it was just that moment of having to say it out loud for the first time and that’s when that part of my life suddenly changed.”

Sophie says terminal cancers can leave patients dreading the thought of suffering at the end of their lives.

“What I don’t want to be is in pain,” she says. “If I am facing an earlier death than I wanted then I want to be able to take control at the end.”

Assisted dying, she believes, gives her control: “It’s an insurance policy to have that there.”

Read more:
Why is assisted dying so controversial and where is it legal?
UK on ‘slippery slope’ Justice Minister says ahead of vote

On Friday, the government is set to debate the issue before voting on it. Sophie hopes they’ll back the proposal.

“It should be my choice to be able to have a compassionate death,” she says.

There has been much debate about the bill since details about how it would work were published earlier this month.

On Friday, former prime minister Gordon Brown became the latest senior political figure to share his opinion on the matter, coming out as against the legalisation of assisted dying, based on his experience of his own daughter’s death.

Disability rights advocate Lucy Webster warns that for people like Sophie to have that choice, others could face pressure to die.

Lucy Webster, disability rights advocate
Image:
Lucy Webster

“All around the world, if you look at places where the bill has been introduced, they’ve been broadened and broadened and broadened,” she tells Sky News.

Lucy is referring to countries like Canada and Netherlands, where eligibility for assisted deaths have widened since laws allowing it were first passed.

Lucy, who is a wheelchair user and requires a lot of care, says society still sees disabled people as burdens which places them at particular risk.

“I don’t know a single disabled person who has not at some point had a stranger come up to us and say, ‘if I were you, I’d kill myself’,” she says.

The assisted dying bill, she says, reinforces the view that disabled lives aren’t worth living.

“I’ve definitely had doctors and healthcare professionals assume that my quality of life is inherently worse than other people’s. That’s a horrible assumption to be faced with when [for example] you’ve just gone to get antibiotics for a chest infection. There are some really deep-seated medical views on disability that are wrong.”

Under the plans, a person would need to be terminally ill and in the final six months of their life, and would have to take the fatal drugs themselves.

Among the safeguards are that two independent doctors must confirm a patient is eligible for assisted dying and that a High Court judge must give their approval. But the bill does not make clear if that is a rubber-stamping exercise or if judges will have to investigate cases including risks of coercion.

Julian Hughes, honorary professor at Bristol Medical School, says there’s a very big question about whether courts have the room to take on such a task.

Julian Hughes, honorary professor at Bristol Medical School
Image:
Julian Hughes

“At the moment in the family division I understand there are 19 judges and they supply 19,000 hours of court hearing in a year, but you’d have to have an extra 34,000,” he explains.

“We shouldn’t fool ourselves and think that there wouldn’t be some families who would be interested in getting the inheritance rather than spending the inheritance on care for their elderly family members. We could quickly become a society in which suicide becomes normalised.”

Continue Reading

Trending