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.

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.

‘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.

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 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.

‘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 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.

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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.

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.

A woman infected with an incurable brain disease due to an NHS operation when she was three years old has told Sky News she is “living with a death sentence”.

Natalie Bralee-Brett was born with spina bifida, a birth defect caused by a gap between the brain and spinal cord.

In 1975, her mother Maureen was told by doctors at Great Ormond Street Hospital that Natalie would have an improved and prolonged life if they operated on her using a new procedure. 

But this, unknown to Maureen at the time, involved taking membrane taken from dead bodies and inserting it into Natalie’s spinal cord. Now nearly 40 years later this treatment is the very thing that could kill her.

It has caused microbleeding on her brain, leading to memory loss. Natalie, 53, is also falling over constantly and suffers debilitating headaches. 

It also means Natalie is at high risk of dementia and could suffer a catastrophic stroke at any time. 

“Living with a death sentence, this is the only way I could describe it,” Natalie told Sky News. 

“Because every day, if you get a headache, you’re thinking ‘is this a sign of an episode that is linked to this problem? Is it a stroke?'” 

“I want to know why I’ve got this problem. And that probably makes me more angry than actually having to deal with this condition.”

Search for answers

Sky News has been following Natalie’s case for more than a year, and we have spoken to experts around the world to try to help Natalie get answers.

Natalie was given a dura mater graft in 1975. This is a piece of membrane collected from dead bodies. Its use is intended to protect the spinal cord and prevent fluid from leaking.

But over years, proteins already in the membrane can build up and cause plaques, leaving patients at high risk of diseases like Alzheimer’s. It can also make them vulnerable to strokes and brain haemorrhages. 

The procedure was common in the 1970s in the UK, across Europe and the US.

It’s thought tens of thousands of patients around the world might have undergone the procedure, but it is not known how many might be suffering the same life-limiting and potentially fatal consequences today. 

Natalie was diagnosed with a brain tumour in 2009, aged 37. A year later it was confirmed she had epilepsy. But it wasn’t until 2022 that Natalie was diagnosed with iatrogenic cerebral amyloid angiopathy (ICAA). 

Her family have now suffered another devastating blow.

Natalie’s brother Neil, who also had surgery for spina bifida in 1980, was diagnosed with ICAA in July. He’s no longer able to work and is suffering from Alzheimer’s.

The siblings were born with the same condition, underwent the same procedure, and are now infected with the same incurable brain disease. 

Natalie and Neil, 45, are the only siblings identified on the international ICAA register, which has just 52 confirmed cases.   

Natalie says her mother Maureen is “heartbroken”. 

ICAA cases ‘a heartbreaking tragedy’

Cases of ICAA have been identified in the Netherlands, Australia, Japan, Croatia, Austria, Italy, Spain, Slovenia, and the US.

Explained: Why cadaver implants are causing brain disease

Harvard Professor Steven Greenberg is one of the world’s leading ICAA experts. 

“We hope and believe the numbers will be limited,” he said. “The hope is that we’re talking hundreds.”

But for those who are impacted, “it is a heartbreaking tragedy, and we feel it very much as the healthcare providers taking care of them”.

ICAA is caused by a medical examination or treatment, relates to the brain, involves amyloid proteins that can stick together and cause a range of problems for major organs – and that is a disease of the blood vessels. 

Doctors believe there are three possible causes of the illness: cadaveric material introduced into a body during surgery, the use of human growth hormones containing cadaveric material and surgical tools not sufficiently sterilised. 

Professor Greenberg explained: ”When I was in medical school, one of my professors said ‘the I stands for I, the doctor caused the problem’.

“And in the case of iatrogenic CAA, this is kind of a heartbreaking echo of an era when it appeared that a good neurosurgical procedure was to use tissue from human cadavers to close defects in the nervous system.

“And I am not a surgeon and I certainly wasn’t practising at the time. But my understanding is that it appeared to be good natural biological material for closing up areas and then had this unexpected and tragic effect of introducing some kind of protein that would later cause disease in the brain.”

‘I deserve answers’

Natalie’s health is steadily deteriorating. The bleeding on her brain is causing memory loss, and she dreads what the next few months will bring. 

“The pattern seems to be every time I go for a scan, I’m being shown to have more bleeding,” she said.

“And the last scan showed that I also had inflammation. It’s always hanging over you because you get a headache, and you think ‘is this going to turn into something worse?’

“And because of being at risk of a stroke it’s constantly there, and it’s a condition that is going to get worse.”

Natalie’s agony is further compounded by the lack of information.

She wants to know more about the donor of the diseased membrane implanted into her brain. But there is no record of where the dura mater came from.

And most of the time these grafts were made up from more than one body.

Natalie said: “I think I deserve answers. And if you’ve got something wrong with you, you want to know it’s human nature. You want to know why you’ve got this problem.”

Diagnosing ICAA has only become possible due to advancements made in MRI scanning.

Specialists including Professor Greenberg have set up the international register so any neurologist suspecting ICAA can flag cases to their peers.

This isn’t the first time cadaveric dura mater grafts have infected patients.

The World Health Organisation advised against their use in 1997 after it was discovered they had been giving patients Creutzfeldt-Jakob disease (CJD) which at the time was also being spread by eating cattle infected with BSE or ‘mad cow disease’.

There were 228 of these confirmed cases worldwide.

Dura mater grafts were used in more than 20 surgical procedures over 25 years, with the last known use in the UK in 1992.

‘Something is seriously wrong’

Simon Stratford was only 34 and a father of four young children when he died in April 2003.

That was nearly 16 years after having surgery to remove a brain tumour. During the operation a Lyodura graft was inserted into Simon’s brain. The membrane was infected with CJD and a coroner’s inquest found that it was this procedure that caused Simon’s death.

Speaking exclusively to Sky News, his widow Colleen said she warned doctors treating her husband that she knew he was dying.

“I kept saying to the doctors he’s getting worse and something seriously is wrong, and you know, Simon did say to me at that point he thought he was dying.

“He had a brain tumour removed which saved his life and then was given a life sentence. They said it was the Lyodura dura mater that took his life.”

Sky News asked B. Braun, a major German manufacturer which made Lyodura, to help with our investigation. They declined to comment on the link between dura mater grafts and ICAA.

For more than a year we have been speaking to experts around the world. Scientists in Europe declined to be interviewed. Natalie’s own medical team in the UK also refused to be interviewed.

The risks posed by the grafts are being better understood but the scale of the damage they have caused is not.

Some specialists who spoke to Sky News but did not want to be named are keen to stress that surgeons who carried out operations using these grafts were acting on the best available medical evidence to improve the length and quality of patients’ lives.

Natalie had her operation at Great Ormond Street Hospital. It’s likely, at least hundreds of patients would have undergone the same procedure.

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Following our investigation, its medical director Dr Sophie Varadkar told Sky News: “We understand that this situation may be deeply worrying for former patients who believe they could have been affected.

“At the time, this type of product was commonly used in surgical procedures worldwide, and sadly, the risks associated with it were not yet understood.”

“For any of our former patients who think they may be affected, we will support you with getting access to your medical records to allow you to discuss your concerns with your current doctors.”

The Department of Health told us concerns around ICAA – including new forms of transmission – were being investigated.

“The Advisory Committee on the Safety of Blood, Tissues and Organs is currently considering this issue with a view to providing further advice to the Government,” a spokesperson said.

“We encourage anyone with concerns about historical treatment to speak with their GP or specialist healthcare team for appropriate support and guidance.”

Do you have a story to share with us? Contact NHSstories@sky.uk

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.

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.

‘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.

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 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.

‘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 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.

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.