Connect with us

Published

on

Sophie Lewis knows one day she will face the most devastating moment for any parent – her daughter will die.

But when Isabel is gone the family will be left with not just overwhelming grief – but also a mountain of debt.

Isabel has Batten disease, a fatal disease attacking her nervous system. Children have a life expectancy of up to ten years. She is now 12.

But while the mum-of-four should be caring for her child, she is also fighting another battle: the rising cost of living.

“All we have ever done is fight – you fight for everything, but you don’t want to because that word ‘fight’ feels quite gross,” she told Sky News.

“Really what you are trying to do is give your child a good death and give them a good quality of life and comfort in the meantime.”

The Lewis family runs a “mini-intensive care unit” for Isabel, who now requires two-to-one care around the clock.

More on Cost Of Living

Three years ago, at the same time of year, it cost them £4.60 a day. This has now risen to £16.06, and the family has no idea how it is going to pay its soaring bills.

Ms Lewis, from Guildford, said: “In my deepest, darkest moments I have thought, I can’t go on like this, and the only thing that will stop it is if our daughter dies. And that is a terrible way to think, it’s heartbreaking.”

“We are only just surviving [the cost of living crisis],” she added.

“I don’t know what things will look like in a year or two if things continue as they are.”

‘There is nothing we can do’

Isabel experienced a few minor health issues before she was three and then, shortly before the birth of Ms Lewis’ second child, she was diagnosed with Battens disease.

“We just got a phone call one day telling us that she was going to die and there was nothing we could do about it,” she said.

Battens is a recessive disease, and Isabel “very rapidly began to lose her skills”.

She went from walking to crawling, to not being able to sit up anymore. Previously a chatty toddler, she was soon unable to say certain words and eventually stopped speaking altogether.

Isabel then lost her ability to swallow and eat, and eventually went blind.

By the time she was three, she needed full-time care.

The one place she is not in pain

Isabel is constantly in pain, except for when she is in the family’s hydrotherapy pool – a hot tub in the back garden.

But with their bills now topping £600 a month, of which they can barely afford to pay half, the family feel it can no longer turn this on.

“I feel guilty for talking about removing what some people might think is a luxury item in our home,” said Ms Lewis.

“But Isabel is bedbound, housebound, hasn’t left the house for over a year, hasn’t been to school for five years – her childhood and her life have been taken away from her.

“Suddenly I was in a position where I was thinking we can’t actually afford to heat the pool, the one place she is pain-free, and the one place where I can still hold her.

“And that is really hard to talk about because people lead you to a place over the years where they make you think having these things is your choice.”

She said parents are saving the NHS money, as Isabel’s hospital care would cost anywhere between £1,500 and £3,000 a day.

“We are doing that for her at home – as we should do, and we want to do that,” she said.

“She has only been in this situation for a few years and she won’t be here in a few years’ time.”

 Isabel on her 11th birthday
Image:
Isabel on her 11th birthday

Growing mountain of debt

The family can no longer afford to keep up with its escalating energy direct debit and every day falls further into debt.

“It’s insulting hearing people say, put on a jumper or use an air fryer,” Ms Lewis said.

“Yeah, we could do all of that, but it still wouldn’t take away from the fact our energy bills are high, because we are at home all the time, we are running equipment and the heating is on to help my daughter regulate her temperature.”

The Lewis family is not alone.

Together For Short Lives is fundraising to provide grants to children receiving palliative care.

One family the charity supports recently hit the headlines after Kate Winslet donated £17,000 to help cover their rising energy bills.

Freya and Carolynne - cost of living disabilities
Image:
Freya and Carolynne were supported by actress Kate Winslet

Every parent’s worst nightmare

Andy Fletcher, its chief executive, said there are about 99,000 children living in the UK with a life-limiting or life-threatening condition.

Of these, around 3,000 are children who need ventilators to keep them alive.

“Parents already facing the emotional turmoil of the potential of their child dying in childhood, which is every parent’s worst nightmare,” he told Sky News.

“And on top of that, they’re trying to make as many special memories as possible with their children and family.

“And these are memories that will last them a lifetime, but at the same time, they’ve got these external pressures of rising costs.

“And that’s the real challenge when you are making choices at this time of year of what to prioritise because the number of Christmases they have may very well be short.”

A heart rate of four beats per minute

Hand in hand with soaring energy costs is the risk of blackouts in the UK, as rising demand puts pressure on supplies.

For 10-year-old George, a power cut at night is a matter of life or death.

With a resting heart rate of four beats per minute, he requires a ventilator at night to help him breathe.

Grandmother, Nicola Gatbutt, helps with his care as his mother, her daughter Holly, faces her own health issues.

After collapsing at work, Holly is now partially sighted.

George, Holly and Harvey
Image:
George, Holly and Harvey

But she has been told she does not qualify for personal independent payments – given to those who have a “long term physical or mental health condition” – despite not being able to drive, and having collapsed twice in the last two months and broken two joints.

Meanwhile, her electricity bill has tripled, taking it from £200 to £600.

“I am dreading my next bill,” Ms Gatbutt, from Skipton, said.

She goes to school with George five times a week as his carer and looks after him on some evenings, weekdays, and during the school holidays.

Energy companies, the grandmother and mother-of-three said, need to reduce their costs for children on long-term ventilation and provide them with more concrete advice on what to do in the face of a blackout.

‘He will never outgrow it’

The family also faces increased petrol costs – what used to cost £30 to and from the hospital now costs £55 – and Ms Gatbutt’s own mortgage has tripled, and now costs £305.

George has been ventilated since he was one year old and “he will never outgrow it”.

George in hospital (left) and pictured with his older brother Harvey (right)
Image:
George in hospital (left) and pictured with his older brother Harvey (right)

Despite his complex medical problems, Ms Gatbutt said: “If you see him in person it’s a different picture. He does cross country, he has just run a big race.

“He lives life to the full.”

Continue Reading

UK

Big Issue founder on the ‘great distraction’ stopping Britain tackling ’emergency’ crisis

Published

on

By

Big Issue founder on the 'great distraction' stopping Britain tackling 'emergency' crisis

“Chauvinistic” debates on immigration are distracting ministers from tackling the child poverty “emergency”, the founder of the Big Issue has told Sky News.

Lord John Bird, a crossbench peer, said there is “no evidence” the government is trying to “stop the growth or the propagation” of generational poverty, and the best thing they can do is admit they “haven’t got this right” and change course.

It comes amid a delay to Labour’s child poverty strategy, which is looking at whether to lift the controversial two-child benefit cap, among other measures.

While not affiliated to any political party, Lord Bird warned Labour will not hold back the rise of Reform UK unless they get a grip on the issue – calling debates on immigration a “great distraction”.

Lord John Bird is a lifelong poverty campaigner
Image:
Lord John Bird is a lifelong poverty campaigner

“They’re largely there because of the problems in the country,” he said of Nigel Farage’s party.

“There’s a kind of rightward move in the country and a lot of that has to do with the way the immigration is going.

“It’s all about, in my opinion, chauvinism – and patriotism has become a new value. I am particularly concerned about that.”

Lord Bird is proposing an amendment to the Children’s Wellbeing and School’s Bill next month that would impose a statutory duty on the government to reduce child poverty in England.

Education minister Baroness Jacqui Smith has previously rejected the idea, saying targets “would not in themselves drive reductions in poverty”.

But according to analysis by the Big Issue, Scotland has seen a 12% drop in relative child poverty since passing legally binding targets in 2018, whereas England and Wales has seen a 15% rise.

Lord Bird’s amendment has the support of Labour peer Ruth Lister, the former director of the Child Poverty Action Group, who argues targets “galvanise” governments and local authorities into action.

Please use Chrome browser for a more accessible video player

Parents struggle to feed children

Manifesto pledge at risk

Labour is under pressure as its manifesto promised an “ambitious strategy” to bring down child poverty, but the taskforce set up to deliver it after the general election missed its deadline in May.

The delay followed cost concerns around lifting the two-child benefit cap, which multiple charities and Labour MPs argue is the most immediate thing the government can do to help the record 4.5 million children living in poverty in the UK.

That figure is projected to rise to 4.8 million children by the end of this parliament without further action – putting the manifesto pledge in jeopardy.

The cap is likely to be a significant issued at Labour’s annual party conference kicking off this weekend, against the backdrop of a deputy leadership contest in which both contenders have pledged to make child poverty a priority.

Education Secretary Bridget Philipson, who is standing in the race and co-chairs the poverty taskforce, said this week that “everything is on the table, including removing the two-child limit”.

Work and Pensions Secretary Pat McFadden, who co-chairs the taskforce, has not ruled out an announcement by the prime minister at the conference, but stressed: “Everything has to be paid for, everything has to be budgeted.”

Read more politics news:
Trump is ‘racist’, says Sadiq Khan
Royal Parks dismisses Farage’s swans claim

Lord Bird said removing the two-child cap, estimated to cost £3.4bn a year, would alleviate an “emergency”.

However, he said a longer-term strategy was needed to prevent poverty, warning it is more entrenched now than during his own “terrible” childhood.

The 79-year-old was born in a Notting Hill slum to a poor Irish family in 1946, becoming homeless at age five and learning to read and write through the prison system as a teen.

Back then “no one was giving you a handout” whereas there is “institutional poverty now”, Lord Bird said, blaming recent governments for “trying to make the poor slightly a bit more comfortable” rather than “turning off the tap”.

Lord John Bird escaped poverty and founded The Big Issue in 1991
Image:
Lord John Bird escaped poverty and founded The Big Issue in 1991

‘Aim for the impossible’

He urged Labour to challenge the radicalism of Nye Bevan, the founder of the NHS, and “aim for the impossible” in eradicating child poverty, with investments in education and social development.

“There’s no evidence that the government is trying to stop the growth or the propagation from one generation to another of poverty,” he said.

“The cheapest but most efficient thing this government could do is stop pretending they’ve got it right, stop pretending they got the answers. The most important thing they could do is say, whatever we’re doing, it’s not working.”

Continue Reading

UK

UK boosts pandemic readiness with new vaccine factory

Published

on

By

UK boosts pandemic readiness with new vaccine factory

A US vaccine firm has opened the first mRNA manufacturing plant in the UK, against a backdrop of increasing anti-jab rhetoric back home.

The new facility outside Oxford is part of a £1bn investment in the UK by Moderna, which specialises in mRNA.

The novel vaccine technology delivered some of the most effective and fastest-to-develop jabs during the COVID pandemic.

Several pharma companies, including Germany’s leading mRNA pioneer BioNTech, are now racing to develop new therapies.

Moderna says the plant will produce up to 100 million doses of its existing vaccine products each year. It has also been designed to scale-up production to 250 million doses a year in the event of a new disease outbreak.

“God-forbid, if there is another pandemic, we can switch the facility any day,” said Moderna CEO Stephane Bancel.

The UK investment deal was agreed by the previous government, but the plant’s opening is welcome relief for the current one.

In recent weeks, four major pharmaceutical companies have halted planned investments in the UK following disputes over drug pricing and profitability in the UK.

‘A great statement’

It also promises to restore domestic vaccine manufacturing capability in the UK, the lack of which was exposed when dangerous supply interruptions threatened the early COVID response.

“It’s a really fast way of getting new vaccines discovered,” said Lord Patrick Vallance, former chief scientist and now science minister.

“It’s also a great statement of confidence in the UK that [Moderna has] chosen to base themselves here.”

Health Secretary Wes Streeting attended the opening
Image:
Health Secretary Wes Streeting attended the opening

The Trump effect

The mRNA molecule is the same used by our cells to order the production of new proteins, and allows vaccines to be produced using just the genetic code of a virus or other biological target.

Moderna’s investment decision pre-dated Donald Trump’s return to the White House, but the Moderna CEO said its operation in the UK – a country that “still believes in vaccination” – may pay dividends if anti-vaccine rhetoric translates into a lack of demand for its products in the US.

“If there is less appetite by governments around the world, including in the US, to use vaccines, we might invest less in vaccines,” said Mr Bancel.

“We have to invest where there’s a demand for our products.”

Read more: All health claims made by Trump – and what experts say

Please use Chrome browser for a more accessible video player

Is US politics fuelling a deadly measles outbreak?

The UK presents other attractions for the company which has suffered substantial losses as demand for its COVID vaccine has fallen.

It’s betting that leading UK universities and a large patient population will make for successful clinical trials.

The company has ongoing NHS trials of new jabs against seasonal flu, a combination COVID and flu vaccine, cancer vaccines and mRNA therapies for two inherited childhood diseases.

Moderna says it is now the largest private commercial sponsor of clinical trials in the UK.

Continue Reading

UK

How one of the world’s rarest diseases left Sky’s Deborah Haynes critically ill

Published

on

By

How one of the world's rarest diseases left Sky's Deborah Haynes critically ill

Paddling in a bay on the tiny Channel Island of Sark, I suddenly felt very sick and cold.

Less than 48 hours later, I was being emergency evacuated to the intensive care unit of a London hospital via cart, tractor, lifeboat, private jet and ambulance.

Ultimately, an incredible team of doctors, nurses, and volunteers saved my life – for a second time, though falling ill with one of the rarest diseases in the world while in one of the remotest corners of the British Isles was an unfortunate first.

Deborah Haynes covers some of the biggest foreign stories around the world. She also hosts The Wargame podcast
Image:
Deborah Haynes covers some of the biggest foreign stories around the world. She also hosts The Wargame podcast

I have something called atypical Haemolytic Uremic Syndrome (aHUS) that – when triggered – affects my immune system, destroying blood cells and harming other vital bodily functions.

Classed as “ultra-rare”, there is only around one new incident of aHUS per two million people every year. And an attack can be fatal, so the speed of diagnosis is key.

In my case, I already knew about the condition as I first fell ill with it eight years ago.

When it happened a second time, the heroic efforts of Sark’s only doctor, a group of volunteer rescuers and the medics at University College Hospital (UCH) meant I was raced from the middle of the English Channel to an intensive care bed in just over 11 hours – enabling rapid and effective treatment.

Now back home and expected to make a full recovery, I thought I would share my experience to help raise awareness about this little-known disease as today is aHUS Awareness Day.

Deborah Haynes in ICU after falling ill with atypical Haemolytic Uremic Syndrome (aHUS)
Image:
Deborah Haynes in ICU after falling ill with atypical Haemolytic Uremic Syndrome (aHUS)

‘I was feeling increasingly wretched’

My husband and I had planned to spend a few days in August on Sark – a beautiful island in between the UK and France that is a designated “dark sky” area because of an absence of light pollution.

There are no public streetlights on the territory.

More relevant to this story, cars and regular ambulances are also banned.

Instead of driving, Sark’s just over 500 residents and ferry-loads of tourists either walk, cycle or sit on the back of carts towed by horses – and on occasion tractors – to visit beaches, coves and other attractions.

A some 100-metre-high ridge connects the rock islands on Sark. File pic: AP
Image:
A some 100-metre-high ridge connects the rock islands on Sark. File pic: AP

I started to feel queasy on the ferry that took us to Sark.

We initially thought it was seasickness.

But the nausea lingered as we walked from the port to our hotel to dump our bags.

Thinking a swim might make me feel better, we trekked down a steep path to the beach and ventured into the sea, which is when my body decided to break.

I came out of the water, shivering uncontrollably and thought I was going to faint. After getting myself dry, we tried to return to the hotel, but I started vomiting violently on the side of the path – much to the disgust of a family that was trying to overtake us.

Once back at the hotel, I collapsed into bed, only leaving it to be sick.

We speculated that it must be food poisoning and hoped it would pass within a day.

But 24 hours later, while I had stopped vomiting, I was feeling increasingly wretched and beginning to wonder whether it could be aHUS again.

The 'ultra-rare' condition is caused by part of the immune system becoming overactive
Image:
The ‘ultra-rare’ condition is caused by part of the immune system becoming overactive

‘The onslaught is like an invisible storm’

The only other time I have been struck down by the disease was in January 2017, while working as the defence editor at The Times.

On that occasion, I took myself to my local hospital in Kent to be told that I had acute kidney failure and my bloods were “deranged”.

Fortunately, the haematologist on duty had been aware of aHUS – then a new acronym for me – and had me rushed to University College Hospital in London, which has a specialist team that can treat the condition led by Professor Marie Scully, a world-renowned expert.

I soon learnt that aHUS is caused by part of my immune system – called the complement system – becoming overactive and attacking my body rather than targeting bugs.

This “friendly fire” – likely linked to a genetic glitch that, in my case, had thankfully lain dormant for the first 40 years of my life – can be activated by infection, pregnancy or food poisoning, though sometimes the cause is unknown.

The subsequent onslaught is like an invisible storm that destroys a patient from the inside, shattering red blood cells, damaging small blood vessels and causing tiny clots.

The clots clog up kidneys and trigger acute renal failure.

If left untreated, other organs can also collapse, while the risk of a stroke or heart attack rises.

Without intervention, the prognosis is dire.

Between 10 to 15% of patients die during the initial illness, while up to 70% of patients develop end-stage renal failure, requiring a lifetime of dialysis.

Since 2013, however, patients in the UK have had access to a drug called eculizumab, which effectively turns off the malfunctioning part of the immune system. It is expensive – at many thousands of pounds a shot – but it saves lives, including mine.

An aHUS attack can be fatal, so the speed of diagnosis is key
Image:
An aHUS attack can be fatal, so the speed of diagnosis is key

‘My protein levels were off the scale’

Lying in bed in Sark more than eight years on from the first episode, I did not want to believe my body had turned on itself again.

But after a little over 36 hours, with no improvement, my husband decided to get help.

His action likely saved me from even graver kidney damage or worse.

He set out to find Sark’s only GP, Dr Bruce Jenkins.

Blood tests are the best way to diagnose aHUS, but they were not an option on the island.

Instead, Dr Jenkins did a urine test, which is a good alternative.

Any trace of blood or protein in the urine is a sign that a person’s kidneys are in trouble.

My protein levels were off the scale of the test.

Upon seeing the result, Dr Jenkins instructed my husband to go back to our hotel and pack our bags – I was still floundering in bed – while he coordinated an emergency evacuation.

On Sark, given the lack of vehicles, this meant mobilising an ambulance cart towed by a tractor, which is operated by a team of Community First Responders – all volunteers.

The Sark medical team who helped save Deborah's life
Image:
The Sark medical team who helped save Deborah’s life

The GP also contacted the main hospital on Guernsey, a larger Channel Island, which provides a marine ambulance service to rescue anyone with a medical emergency on Sark.

While all this was happening, I called an emergency number for the aHUS medics at University College Hospital to warn them I was likely suffering a relapse.

By chance, Professor Scully was on duty that day – a Friday – and over the weekend. She and her team sprang into action, contacting Sark and Guernsey to help.

Speed was key as my condition was worsening.

‘I asked a doctor if I was going to die’

Within minutes of the alert going out from Dr Jenkins, a tractor, pulling a white ambulance cart, arrived at our hotel, and the first responders guided me and my husband onboard.

They took us to the port to wait for the “Flying Christine”, an ambulance boat carrying two paramedics and operated by St John’s Ambulance and Rescue Service.

That team transported us via sea to Guernsey hospital before we were transferred to a specialist medical plane to be flown to Luton airport.

The last leg was a more conventional ambulance drive to UCH, where critical care doctors, as well as Professor Scully and her colleagues, were poised to start the treatment.

Read more from Sky News:
New dinosaur discovered
Tributes to legendary umpire
NASA accelerates moon mission plans

This quick response by the NHS and a network of volunteers meant I was taken from my sickbed in Sark to life-saving treatment in London in barely 11 hours.

By this point, the aHUS attack had caused my haemoglobin and platelet levels to drop, and my kidneys were failing.

The main treatment was the eculizumab drug – administered intravenously – but I also needed blood transfusions and to be put on a machine that acts as a form of dialysis.

The relief of being in the best possible place for my condition was immense, but the next few days were still frightening as my body took time to respond.

At one point, I asked a doctor if I was going to die – she assured me I was not.

Deborah has now been discharged and says she is 'on the mend'
Image:
Deborah has now been discharged and says she is ‘on the mend’

On another occasion, pumped full of medication, hooked up to various machines and drifting in and out of sleep, I dreamt I was under missile fire – an occupational hazard of being a journalist who covers war – and tried to leap out of bed to an imaginary shelter, prompting the nurse who was looking after me to spring into action and make sure I stayed put.

Gradually, though, the treatment started to work.

After nearly a fortnight in hospital, including one week in intensive care, I was well enough to be discharged.

Today, I am on the mend and incredibly grateful to everyone who helped to save me from myself.

Deborah Haynes and Professor Marie Scully will be on Sky News from 8.30am to speak about aHUS Awareness Day.

What is aHUS?

Atypical haemolytic uraemic syndrome is an ultra-rare disease that affects between 2.7 and 5.5 people per million population worldwide. It has an incidence rate of about 0.4 cases per million people a year and can occur at any age.

The disease affects part of the immune system called the complement system. It starts to destroy the body’s own cells, especially those that line blood vessels. This leads to clots forming within small vessels. The kidney is most commonly impacted, but all organs can be harmed.

AHUS is typically linked to a genetic fault in the complement system or a group of proteins meant to regulate it.

The trigger for an attack can be infection, pregnancy or food poisoning.

Symptoms can include feeling unwell or tired, becoming confused, blurred vision, shortness of breath, high blood pressure, nausea and vomiting, diarrhoea and abdominal pain.

Continue Reading

Trending