Mum's agony as daughter, 16, has half her brain permanently disconnected in radical operation

A mother today told how she made an agonising "life or death" decision to have her teenage daughter's brain cut in half in a radical operation. Fiona Williams watched helplessly as her daughter, Neive, 16, was plagued with TWELVE life-threatening seizures each day since the age of four. It took specialist medics nearly a decade to diagnose her with Rasmussen’s encephalitis, which is estimated to affect no more than 500 children globally. Surgeons told Fiona, 41, the only way to correct the rare inflammatory brain disease was an intricate eight-hour operation to permanently disconnect the two hemispheres of Neive's brain. Fiona was told the risky procedure might cause permanent vision or speech loss. But she knew that if she didn't give surgeons the green light, one of the violent seizures might end little Neive's life. Fiona, 41, said: "It was terrifying. At first I didn't want Neive to have the operation. I was scared she might not be the same person when she woke up, I was worried it would mess with her personality, or if it would cause further damage. It's serious surgery. "I knew there could be complications but it was also a life or death decision. But we knew we needed to do something about this because we couldn't just sit and watch it go on. It was hard because we were watching Neive suffer with seizures. they became so frequent Neive would get little 'warnings' and she knew a seizure was coming. She would take herself and lie on the floor so she didn't hurt herself." The only answer was for Neive to have rare hemisphere disconnection surgery to split the brain in half. The procedure disconnects one half of the brain from the other so seizure signals are unable to fire and spread. And after surgeons at Great Ormond Street Hospital carried out the intricate operation Neive is now seizure free. Fiona, who works in customer services at Audi, said: "It's changed our lives. Having the surgery was the hardest decision but Neive had her last seizure 10 minutes before the operation. She hasn't had one since. It's amazing." Speaking about having a metal plate in her head following the operation, bubbly Neive - who loves Harry Potter, books by the author David Walliams and shopping - said: "I'm part metal, half robot." Neive first started suffering daily seizures when she was just four years old, and her mum feared it was triggered by a bout of chickenpox. The Abbey Hill Academy-pupil was a healthy normal youngster before the relentless fits began damaging her health. Fiona said: “Neive was fine until she was four or five, when she started having brief vacant episodes at school, but for months people didn’t realise they were seizures. She was having focal seizures where she would just go blank. Then on Bonfire Night in 2015 she had a massive seizure, a tonic clonic. We had brain scans and they discovered a blur on her brain. It was frightening because we just didn't know what was going on." Doctors at Newcastle's RVI carried out an MRI which showed an abnormality on Neive’s brain. She underwent a string of operations before specialists identified inflammation affecting the left side of her brain. Over the past 10 years little Neive has been prescribed a cocktail of steroids, anti-epileptic drugs and immune suppressants to help stave off the seizures. But nothing worked and the relentless fits caused her health to deteriorate. She suffered epilepsia partialis continua (EPC), a jerking movement in her leg, and she became progressively weaker until she needed a wheelchair. It wasn't until 2021 that specialist doctors sent a sample of her brain to a specialist laboratory in the USA. She was diagnosed with Rasmussen's - thought to have affected just 12 children in the UK. Over time, patients begin to lose motor skills, speech and can become paralysed on one side of the body. Specialist surgeons at the world-leading Great Ormond Street Hospital said they could cure the illness by delicately disconnecting the links between the two hemispheres of her brain. It would mean the two halve would be unable to fire pulses between the two halves which was triggering the seizures. Fiona, who lives in Ingleby Barwick, Stockton-on-Tees, North Yorks., with her partner Alan Brett, 39, a shop fitter, said: "I was told the surgery was risky but none of the drugs we tried were working to stop the seizures. So it was something we had to try something. Neive had a poor quality of life. It was difficult to go out and we were constantly taking her to hospital in Newcastle." Neive went under the knife in 2022 at GOSH in London. Fiona said: "On the day of the operation we were told to leave the hospital. at 8am. They didn't want us there. It was awful. We were just kind of mulling round Leicester Square in a daze. It was horrendous. Awful. They said, 'We'll phone you as soon as she comes out'." Eventually medics rang Fiona and told her Neive was out of the operation. Fiona said: "Seeing Neive was awful. She was still coming around and the doctors were saying she'd done well. But we still didn't know if the surgery had affected her speech. "The doctors were absolutely amazing. Neive hasn't had a seizure since the surgery. It's changed her life. She's very brave. We're so proud of her. I wish we 'd have had the operation sooner. We've had a lot of speech therapy and she is talking again now." Despite being a success, since the operation in 2022 Neive suffers from scoliosis and weakness down the right side of her body. She has also lost her fine motor skills in her right hand. She has undergone surgery three further operations to correct scoliosis and hip problems cause by overcompensation on one side of the body. Fiona said: "She's had four operations in four years. It's more than some people will have in their entire lives. But now we're off the medicaiton and we can go on holiday together without taking a suitcase full of medication. I always refused to let the seizures rule our life so we went on holiday and went to places like Mexico. "But now I'm determined to do those things again without the seizures. Having to say yes to the surgery was very difficult but the operation was life-changing. " Professors are now staging pioneering research which aims to understand the underlying mechanisms driving Rasmussen’s encephalitis. Dr Eva Ioannidou, Action Medical Research Training Fellow and Doctoral Clinical Research Fellow at UCL Great Ormond Street Institute of Child Health, said: “Most children are healthy before developing Rasmussen’s encephalitis. "The seizures associated with Rasmussen’s encephalitis usually become more frequent over time and can’t be fully controlled with medications. Other symptoms include progressive learning difficulties and weakness of one side of the body and in many children, their speech and language abilities may also be severely impacted.” Dr Ioannidou's research is significant because early diagnosis is important. She will study brain tissue collected from children with Rasmussen’s encephalitis who have undergone surgery, along with cerebrospinal fluid - the liquid surrounding the brain and spinal cord - and blood samples. She said: “By the time most children are diagnosed with Rasmussen’s encephalitis, irreversible brain damage has often already occurred. Using a range of cutting-edge technologies, we will search for molecular clues – such as gene changes in brain tissues or the presence of specific proteins produced by immune cells – that could serve as early indicators of the disease." The study is being carried out at UCL Great Ormond Street Institute of Child Health and funded by children’s charity Action Medical Research and the British Paediatric Neurology Association (BPNA). Dr Caroline Johnston, Senior Research Manager at Action Medical Research, said: “Our commitment is to support groundbreaking research for rare conditions, like Rasmussen’s encephalitis, for which there are limited treatments and no cure. "Ultimately this research is one of the first steps which could lead to better tests, earlier diagnosis and effective treatments that can stop the progression of the condition and improve the lives of children with Rasmussen’s encephalitis." Fiona said: “Rasmussen’s encephalitis is so rare and there’s still so much doctors don’t know. Research is incredibly important because earlier diagnosis and better treatments could make such a difference for families like ours.”