A British woman infected with an incurable brain disease, resulting from an operation when she was three-years-old – and which has just 42 confirmed cases worldwide – told Sky News she is “living with a death sentence”.
Natalie Bralee-Brett (53) was born with the birth defect spina bifida, caused by a gap between the brain and spinal cord.
In 1975, her mother Maureen was told by doctors at London’s Great Ormond Street Hospital that her daughter would have an improved, prolonged life if they operated on her using a new procedure.
But this, unknown to them at the time, involved taking membrane from dead bodies and inserting it into her spinal cord. Now, nearly 40 years later, this treatment is the very thing that could kill Bralee-Brett.
It has caused microbleeding on her brain, leading to memory loss; she also falls over constantly, and suffers debilitating headaches. Additionally, she is at high risk of dementia and could suffer a catastrophic stroke at any time.
“I want to know why I've got this problem,” she said. “And that probably makes me angrier than actually having to deal with this condition.”
Search for answers
Bralee-Brett was given a dura mater graft in 1975. This is a piece of membrane collected from dead bodies, which was intended to protect the spinal cord and prevent fluid from leaking.
But over the years, proteins already in the membrane can build up and cause plaque, 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 thtn tens of thousands of patients worldwide might have had the procedure, but it is not known how many might be suffering the same potentially fatal consequences today.
Bralee-Brett was diagnosed with a brain tumour in 2009, at 37. A year later it was confirmed she had epilepsy. But it wasn’t until 2022 that she was diagnosed with iatrogenic cerebral amyloid angiopathy (iCAA).
Her family has now suffered another blow. Her brother Neil (45), who also had surgery for spina bifida in 1980, was diagnosed with iCAA in July. He’s no longer able to work and has Alzheimer’s.
The siblings were born with the same condition, underwent the same procedure, and are now infected with the same incurable brain disease.
They are the only siblings identified on the international iCAA register, which has just 52 confirmed cases.
‘A heartbreaking tragedy’
Cases have also been identified in the Netherlands, Australia, Japan, Croatia, Austria, Italy, Spain, Slovenia and the US.
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 affected, it is a heartbreaking tragedy.”
IiCAA is caused by the accidental transmission of amyloid-beta (Aβ) “seeds” during specific procedures. These Aβ seeds act in a prion-like manner, inducing the misfolding and aggregation of native Aβ proteins in the brain’s blood vessel walls, leading to the development of CAA decades later.
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.
Greenberg said: “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 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.
“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.”
Seeking answers
Bralee-Brett’s health is steadily deteriorating. The bleeding on her brain is causing memory loss, and she dreads what the next few months will bring.
“Every time I go for a scan, it shows more bleeding,” she said. “The last scan showed that I also had inflammation.” Her 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.
Diagnosing ICAA has only become possible due to advancements made in MRI scanning.
Specialists, including Greenberg, have set up the international register so any neurologist suspecting ICAA can flag cases to their peers.
History
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 have been used in more than 20 surgical procedures over 25 years, with the last known use in the UK in 1992.
Something seriously wrong
Simon Stratford was only 34 and a father of four children when he died in April 2003.
That was nearly 16 years after having surgery to remove a brain tumour, during which a Lyodura graft was inserted into his brain. The membrane was infected with CJD and a coroner’s inquest found that it was this procedure that caused Simon's death.
His widow Colleen said she warned doctors treating her husband that he was dying.
“I kept telling them he was deteriorating, that something was seriously wrong.
“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 killed him.”
Sky News contacted B. Braun, a major German manufacturer which made Lyodura, but they declined to comment on the link between dura mater grafts and iCAA.
Scientists in Europe declined to be interviewed. Bralee-Brett's own medical team in the UK also refused to be interviewed.
Some specialists told Sky News that surgeons who carried out operations using these grafts were acting on the best available medical evidence, at the time, to improve the length and quality of patients’ lives.
See more from MedicalBrief archives:
Woman dies of Creutzfeldt-Jakob 50 years after contaminated hormone
Alzheimer’s may pass between humans in rare cases – UK study
Deadly TB outbreak in US linked to tainted bone grafts