Natalie's story: The life-prolonging surgery that became a 'death sentence'

0:00How does a banana trigger a CIA-backed coup? Do AirPods herald the arrival of a new global order? What do LED lights say about the future of humanity? I'm Ed Conway, and in each episode of my new podcast, Stuff Matters, I take an object, crack it open, and reveal the world-shaping forces hidden inside. This is economics told through the things we think we understand. Search Stuff Matters on your podcast app to listen and follow. Coming up on the Sky News Daily, the doctors said a graft of material from dead bodies would improve her life. Instead, she's now living with an incurable brain disease.

0:40Natalie's story is next. How does a banana trigger a CIA-backed coup? Do AirPods herald the arrival of a new global order? What do LED lights say about the future of humanity? I'm Ed Conway and in each episode of my new podcast Stuff Matters I take an object, crack it open and reveal the world-shaping forces hidden inside. This is economics told through the things we think we understand. Search Stuff Matters on your podcast app to listen and follow.

1:15How would you cope knowing that you could have a catastrophic stroke at any time? Well, that's the reality of life for Natalie Braley-Brett. Living with a death sentence is the only way I can subscribe it. She's suffering from a condition called iatrogenic cerebral amyloid angiopathy, or ICAA, and it's incurable. She only has ICAA because back in the 70s, doctors gave her a graft of material taken from dead bodies in what was then a pioneering procedure. It was meant to improve her life, not limit it. Our health correspondent Ashish Joshi has been following Natalie over the past year as she's been looking for answers.

1:55Hi Ashish, good to have you back. Look, let's start with you telling me just a little bit more about Natalie. Natalie was born with a condition known as spina bifida. It's a gap in your spine, which can be quite pronounced. It means you can't walk properly. It will affect your balance. Sometimes you are a wheelchair user for life because of that condition. and her parents were told at the time, this is 1975, that there was a new procedure which was being trialled. A pharmaceutical company had produced these patches. This is the 1970s. When you see doctors at Great Ormond Street Hospital and they tell you they've got a procedure to help your daughter who has spina bifida, you listen to the doctors.

2:38These are the people you respect, people you trust. They said that they had a procedure they would like to carry out on Natalie which would prolong her life and improve the quality of her life. And as a mother or a parent, that's the first thing you would say, that's exactly what I want for my child. Tell me more about this procedure. What does it involve? The procedure involved taking a graft from a cadaver, from a dead body, a donor. And this was a piece taken from the brain. It's called a duramater. It's a membrane which is found within the brain. This is then grafted onto the spine, in Natalie's case, during the operation.

3:21It's meant to protect the spine after surgery. It became, during the 70s and 80s, a very common procedure. Pharmaceutical companies, at least two in Germany, were producing these, hundreds of thousands, and they were used on patients across the world, including Natalie and her brother, Neil, who had the same condition, and then he had his surgery five years later. but Maureen was never told that her children would be receiving implants from cadavers. Not that it might have changed her mind, but at the time, again, this is the 1970s, patients just didn't have that much of an explanation about the procedures that were carried out.

4:01And at that point, no doubt, her mother would have been desperate for anything that could possibly help. And I'm wondering, in the short to medium term, whether that was the case. We thought it was working. For the first 10, 20 years, there were no side effects. But what was actually happening to Natalie was inside her brain, amyloid proteins were beginning to build up. Now, these amyloid proteins, as you are probably aware, can cause all sorts of problems. It leads to brain disease, neurodegenerative brain disease in all shapes and forms. So this was going on inside Natalie's body and that's what led her to where we are now.

4:42She sought treatment for her epilepsy. She had a brain tumour which was found when she was in her 30s. But the real truth about what was going on inside her body was known for 10 years later. And that only came about because of the advances in science. What problems was she experiencing as a result of this? And what was this, these advances in medical technology that allowed them to put their finger on exactly what was going on? She was experiencing debilitating headaches. She was having trouble with her vision. She'd have dizzy spells. And that was because of the brain tumour that was developing inside her brain.

5:19The epilepsy that was diagnosed. At the time, you were treated for the epilepsy and the surgery for the brain tumour. but it was only because she went back and she carried on having treatment 10 years later that because of the advances in MRI scanning, the micro bleeding that was going on inside her brain, that was found. She was asked a very important question at the time by her consultant who was clearly aware of these grafts and the problems they'd been causing in the 80s and 90s. He asked me, did I have surgery when I was a child? And I said, yeah, on my spine for spina bifida. Amazingly, they had my notes from 1975.

6:04And that confirmed that I'd received a cadaveric duromatograph. And that is how they found out that this graft, which had been transplanted into a body, had caused these problems in a brain. So very serious problems in a brain. So this is a condition known as ICAA. Iatrogenic cerebral amyloid angiopathy. Cerebral amyloid angiopathy is well known. Proteins on the brain happens naturally and is very, very dangerous. It leads to an incurable brain disease because of these plaques which build up inside your brain, can't be treated, only the symptoms can be managed. The important word here, and it's important for Natalie, is the iatrogenic.

6:51That means it was introduced. So that's introduced CAA. So these proteins weren't naturally occurring inside Natalie's body, but they were introduced through a procedure, either through infected surgical equipment or what is more likely is through that graft. And we know that these grafts, which are now banned, have been transmitting other forms of brain disease. In the 1980s, they were found to be carrying CJD, Creutzfeldt-Jakob disease. Some people might remember this because there were links at the time with BSC and mad cow disease, which is another form. But certainly that's when it entered into mainstream vocabulary.

7:33People would remember CJD and again, a fatal brain disease. And we know that over 200 people died of CJD because of these grafts. And that's why ultimately they were banned. The FDA in the United States first highlighted the problems in 1987. By the 1990s, they were phased out worldwide. We've had cases in Japan, Australia, New Zealand, Canada, the United States, and across Europe in the Netherlands. Ashish, you've been speaking to one of the world's leading experts on ICAA, Harvard's Professor Stephen Greenberg. And if you were one of the surgeons involved in this kind of procedure, knowing what they now know, I can only imagine how they're feeling.

8:15The argument is, and it's a fair argument, that the doctors and the surgeons were acting on the best available medical evidence at the time. And I found this investigation to be so difficult, Neil, because we promised Natalie over a year ago we would try and help her understand this condition. And we had to fly to the United States to go to Boston, to go to Harvard, because he was the only scientist prepared to speak to us on the record. We couldn't find scientists here in the UK or in Europe because ultimately, as a journalist, if you ask questions about a procedure that was used 30 years ago, their concern was that we were going to them to point the finger and say, ultimately, you're to blame because this is what's happened.

9:04And we have a woman now living with incurable brain disease who is afraid that she could die at any minute. We just wanted to find answers. And Professor Greenberg was excellent at explaining just how difficult this is as a condition to find answers.

9:20Natalie Bralee-Brett: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 CEA, 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. Ultimately, he accepts that what those practices were 30 years ago simply would not be acceptable now. The problem is, where did these cadavers come from? Who were these people? What diseases did they have? We just don't have the records to help Natalie find out where her duramater came from. And that for her is the difficulty.

10:04So how is all of this affecting Natalie, her day to day life? It's difficult. It's very, very difficult. Natalie is suffering from these microbleeds, which could lead to clots, which could lead to a seizure, a fatal seizure. At any minute, she has these headaches. She falls over all the time. She's losing her memory. She can't remember, you know, what she might have done an hour or two or three hours ago. And this is a woman in her 50s. This is a young woman. And for her, it's the not knowing. Every time I go for a scan, I'm then shown to have more bleeding. And the last scan showed that I also had inflammation.

10:48It's always hanging over you because you get a headache and you think, is this going to turn into something worse? She has this condition which if she hadn't had that surgery she wouldn't have this problem with her brain now. And she doesn't know how long she's going to live. And she is this incredibly resilient, stoic person who just gets on with the day-to-day. But every time you speak to her and you just scratch below the surface you know the tension that exists. And especially because she's seen what's happened to her brother. Her younger brother has now been diagnosed with ICAA. Imagine that, Neil.

11:26Two siblings, both born with spina bifida, both had these procedures at Great Ormond Street Hospital and both now living with life-limiting and probably life-ending brain disease. Tell me more about how Neil has suffered. Neil's situation has deteriorated so quickly and for Natalie, it's a constant reminder And it's distressing when she sees her brother who's had to give up work. He can't work anymore. He's in a very bad state at the moment. And Natalie is reminded constantly that that is what is coming down the line for her. Do we know how many more people might be out there suffering from this?

12:06And there could be people out there right now. Definitely. Walking around with this who just don't yet know it. Absolutely. To compound that, there will be people who have died without ever knowing. they would have had seizures or some other catastrophic strokes where they would have died and unless they'd had an autopsy or there would have been in an investigation into their death they would never have known because it takes 30 to 40 years for those amyloid proteins to start having an impact on your brain since our investigation we went back to great ormond street hospital and they have said yes of course we'll help you in any way we can they are going to set up a landing page on their website.

12:43Any patients who have been treated there who think that they need further help, they will be given access to their medical records and then they will be helped along that pathway. So anyone who is listening to us now, there are potentially hundreds, if not thousands of people who underwent this procedure who might, and we don't want to do any scaremongering, who might live a long life without any complications. But there are others who might not know what's going on inside their bodies. When you and I have conversations about scandals relating to health, generally by the end of our conversation, I can find a direction in which to point my finger of blame.

13:22I'm not sure I can in this story. Natalie can. Natalie can. She will tell you that had her parents been given more information at the time, they would have been able to make a better judgment call. Could there have been better checks and balances from the pharmaceutical companies involved in producing these grafts? Why isn't there a record of where the duramater was collected from? Who were these cadavers? And was there enough at the time, was there enough evidence to suggest that taking tissue from a cadaver and inserting it into another body might cause complications further down the line? I'll tell you another thing.

14:03Natalie, throughout this process, I said she always remains stoic and very resolute. She says she's had no help, right? The doctors who are looking after her are treating her symptoms. There has been no counselling. There has been no support. And this is an NHS patient who had an NHS procedure. Forget about finding out who's to blame, but surely now we know what's happened to her as a patient. She had a procedure which has had disastrous consequences. She should be given the care and support that she so rightfully deserves. She wants answers. She deserves answers. Why have I got this? Who gave it to me?

14:44Who is responsible? And somebody said to me, she deserves that. Because ultimately, accountability is not the same as culpability. How does she get through the day? I've no idea. I've no idea. Every time I speak to her, I'm blown away by her strength. but clearly being around her for the last year I also see how worried she is and I can't imagine what it's like for her children to know she's going through this. There is no cure, Neil. Scientists say they're working on a cure, they're working as quickly as they can but Natalie, ultimately, Natalie knows she won't live long enough to see that cure.

15:25Thanks Ashish. His full report is of course available on skynews.com and Great Ormond Street Hospital have put a page with help, advice and contact details up on their website. You can find the link in the episode notes. The Daily is back again tomorrow. How does a banana trigger a CIA-backed coup? Do AirPods herald the arrival of a new global order? What do LED lights say about the future of humanity? I'm Ed Conway and in each episode of my new podcast, Stuff Matters, I take an object, crack it open and reveal the world-shaping forces hidden inside. This is economics told through the things we think we understand.

16:07Search Stuff Matters on your podcast app to listen and follow.