SAM'S STORY
To the world a little boy, to us the world
Samuel Liew (8th March 2016 – 8th May 2021)
Sam was such a very loved little boy. For five precious years, our beautiful boy graced this world with his presence. Sam was extraordinary - kind, gentle, and utterly beautiful inside and out. We always thought Sam was an old soul, he was wise beyond his years - even as a toddler. And he talked from such a young age - so when he was two years old it was like talking to a child five years older. He was simply the most perfect child anyone could ever wish for - and we are exceptionally proud of him.
Sam approached life with boundless curiosity and joy. His love for nature, animals, and outdoor adventures was infectious. He found magic in the simplest things - transforming ordinary sticks into anything his imagination wanted them to be, marveling at snails and ants with his Nana, or splashing in puddles wearing his batman wellies. At the beach, regardless of weather, he'd dig in the sand, splash in the water and collect stones. He found wonder in every moment.
Sam's bond with Daisy, his black Labrador, was extraordinary - they were inseparable companions who started each day with family cuddles on mummy's bed. Sam was always active, mastering his bike at just four and joining his daddy for swimming, walking, and camping adventures. His musical spirit shone through as he played his guitar, often making up songs with his mummy.
Like many children, Sam adored superheroes. Little did we know he would become one himself, inspiring our charity's name: Sam's Superheroes. Above all, Sam's defining quality was his kindness. His gentle, caring nature touched everyone he met.
Disclaimer: The following story is written by Sam’s mummy, sharing their personal experience with FIRES, including Sam’s final days. It is an honest and heartfelt account that some readers may find distressing. If you currently have a child experiencing FIRES, please only continue reading if you feel emotionally ready to do so.
The Day Our Lives Changed Forever
On Monday evening, 22 March 2021, at 8pm, every parent’s worst nightmare came true. Our lives, as we knew them, were irreversibly changed when Sam had his first-ever seizure. But the nightmare that unfolded was far far worse than anything I’d even considered could happen to us.
Sam was rarely ill, he was a very healthy and fit little boy. But during the weekend of 20 March Sam had a high fever, was feeling a bit sick, and seemed more tired than usual. We reached out to various out-of-hours services and were reassured that it was likely just a virus. On the morning of Monday, 22 March, as Sam had a very high temperature again during the night again I called our GP. Again, we were told it was probably a virus and that I should continue giving Sam Calpol and Ibuprofen.
By Monday afternoon, although Sam seemed better, he had been playing at his grandmas, I couldn’t shake the feeling that something wasn’t right. As his fever came back again at tea-time I wanted him to physically see a doctor, instead of telephone advice and so took him to Derby Walk-in Centre. Sam had an examination, but they couldn’t find anything of significant concern, and once again, it was suggested he had a virus. However, they did prescribe penicillin as a precaution, given his red throat and history of tonsillitis.
After stopping at the late-night chemist to pick up the prescription, we got home around 7:45pm. Sam had his first dose of penicillin and I asked if he would like a drink of water. He said, "No, mummy," which struck me as odd, after taking medicine. Those were the very last words I would ever hear my son speak.
As Sam sat at the kitchen table, I noticed he had started drooling and was blowing bubbles. He wasn’t responding to me when I asked if something was wrong. I called his dad, who had just left, and asked him to take us to A&E. I phoned my cousin to let her know how worried I was about Sam and that I was going to take him to A&E. Whilst talking to her Sam collapsed unconscious into my arms.
I screamed immediately called 999 and I scooped him up and rushed to my neighbour’s house, all the while shouting for help. The panic I felt in that moment cannot be put into words. Sam remained unconscious, having seizures for over 30 minutes.
East Midlands Ambulance (EMA) arrived within about 10 minutes. By 8:35 pm, Sam’s seizures finally stopped, and he was transferred to the ambulance. The paramedics blue-lighted us to hospital, giving a pre-alert to expect our arrival.
Whilst Sam was sitting up in the ambulance with his eyes open, but he wasn’t talking, wasn’t crying, and wasn’t reaching for me. He would have been terrified, but he wasn’t making a sound. I was utterly terrified, I knew deep down that something was horribly wrong with Sam.
Derby Royal A&E
We were rushed straight to Derby Children’s A&E, where my mind was fixated on getting Sam antibiotics. I was convinced that he had some sort of infection, though I had no idea what was happening. Within 90 minutes Sam went on to have two more seizures. I was utterly terrified watching on - I felt totally helpless.
During this time, although I didn't realise it at the time, the handover process between EMA and the A&E staff had gone terrible wrong. They thought Sam had only had a one febrile convulsion at home and not a 35 minute seizure and had assessed his GCS in accurately. Clearly they were the reasons I was being dismissed as an over anxious parent by the doctor, and this critical error delayed Sam being given anti-seizure medication, that he badly needed.
During the seizures, Sam’s airway became severely compromised, and his oxygen levels dropped dangerously low and a tube was inserted to help him maintain his airway. After the first seizure, he opened his eyes again, but he still wasn’t talking or reacting to anything around him. On his second seizure in A&E we were immediately moved to resus and finally Sam was given some anti-seizure medication and went into a very strange unnatural sleep.
I was told sleeping was common after a seizure, but something in my gut was telling me something was seriously wrong.
In A&E, Sam had a head scan and blood tests. The results showed nothing, and I was left with more questions than answers.
Derby HDU
In the early hours of the morning, Sam was admitted to the High Dependency Unit (HDU), and I didn't leave his side. Within an hour of being admitted, things took a horrific turn for the worse. Sam's seizures returned, and he started to have clusters of seizures one after the other and after another dose of anti-seizure medication he went into respiratory arrest - he couldn't maintain his airway. The medical team was starting to panic and they called for further support and for the crash team. The fear I felt in that moment is something I can’t adequately explain. It was a terror so raw and all-consuming I just wanted to scream at the team to save my son - I thought I was going to lose him.
It was clear that something catastrophic was happening to Sam. I wasn't fully aware at the time, but I’ve since learned that around 2:45 a.m. Sam went into respiratory arrest. In that moment, the medical team worked frantically, and more and more medical professionals began arriving to assist. I could see Sam surrounded by a large team, and I had no idea what was going on. All I could do was cling to the hope that they would save him.
I now know that they were preparing to place Sam into a medically induced coma in order to intubate him to help maintain his airway. But in those moments, I was terrified, in total disbelief at what was happening to my son.
Once Sam was in the induced coma, arrangements were immediately made for him to be transferred by the CoMET (Children's Medical Emergency Transport Team) to the Paediatric Critical Care Unit (PCCU) at Queen’s Medical Centre (QMC) in Nottingham. This was a specialist neurology centre about 20 miles away. When a child requires intubation, they must be moved to a hospital with the right expertise and equipment.
We didn’t know it then, but the team at QMC would become a huge part of Sam’s care over the next seven weeks. We are forever grateful to them.
Meeting the Super-Hero CoMET Team
The CoMET Team arrived around 5a.m. that morning. When they walked into the HDU, they brought a calmness and professionalism that instantly reassured me. I knew right away that Sam would be in safe hands, they were like real-life superheroes. It took them about five hours to stabilise Sam and prepare him for transfer.
When Sam was finally ready, seeing him surrounded by drips and machines to keep him alive was like an out-of-body experience. His transport trolley was a mobile intensive care unit. Sam's dad and I walked beside him as the transport team made their way to the waiting ambulance. We weren’t allowed to travel with him, as there wasn’t enough room for us. We were told not to follow the ambulance and the thought of my child being blue-lighted to another hospital without me was unbearable.
In shock and overwhelmed, we rushed home to pack overnight bags and head to Queen’s Medical Centre in Nottingham. Little did we know, heartbreaking and devastating times lay ahead for Sam.
Entering the New World of PCCU
We entered the unfamiliar world of PCCU around midday, terrified and in shock, with no idea what lay ahead. Sam was placed in isolation as it was unclear if COVID-19 was involved, especially since he and I had tested positive just before Christmas 2020.
We were incredibly fortunate to be given a parent’s room, just 30 seconds from Sam’s ward, for which we will always be grateful.
What Was Wrong with Sam?
The PCCU consultants believed Sam's seizures were likely caused by a virus or infection and there was a huge protocol of anti-seizure medication that could be given to Sam, and we felt optimistic. We felt he was in the best possible place, receiving antibiotics and antiviral meds to address any underlying causes.
Whirlwind of Activity
The first few days were a whirlwind of activity around Sam. A constant team of medical professionals worked tirelessly, preparing new IV bolus medications. Each treatment would initially stop his seizures, but then they would restart. It was utterly devastating, terrifying and heartbreaking.
I was permanently terrified, an emotional wreck, and in pieces. Alarms continuously sounded warning of changes in his vital signs like blood pressure, heart rate, or breathing, or signaling that medications needed replacing or adjusting. Sam’s tiny body was receiving an overwhelming number of medications, and I was gripped by a fear unlike anything I had ever known, continually terrified I was going to lose my son.
A CFAM (Cerebral Function Analysis Monitor) device was attached to his head to monitor his brain activity, alongside continuous visual recording to detect clinical seizures.
The medical team worked their way down the anti-seizure medication protocol. The further they went, the more toxic the treatments became. Sam had now entered Super-Refractory Status Epilepticus territory. Refractory status epilepticus occurs when seizures persist despite two anti-seizure medications. Super-Refractory Status Epilepticus is a life-threatening condition where seizures persist for more than 24 hours, even after anesthetic treatment, or recur upon weaning from anesthetic. Seizures lasting longer than 30 minutes can cause brain damage or worse.
Sam's seizures HAD to be stopped.
Stopping the Seizures... the Impossible Task
As the reality set in that stopping Sam's seizures was far from straightforward, I was overwhelmed with panic and fear.
The first few days in PCCU were a blur, filled with anxiety and emotional turmoil. I leaned heavily on Sam’s dad to communicate with the medical teams while I was struggling to cope with the enormity at was happening. I reminded myself that Sam was in the best place possible, and I had to trust the experts. Googling, second-guessing, or trying to micro-manage a condition I knew nothing about would only drive me more crazy and potentially harm Sam’s care.
But soon, it became apparent that I needed to step up a gear. Sam needed me to fight alongside him, not just as his mother but as an active member of his care team. There was no room for bystanders. He needed his mother to activate mama bear mode - to advocate fiercely, be someone who would not accept defeat. And so, I stepped up, determined to work with the medical team, be his voice, to research and fight alongside him.
One in a Million Chance
We were soon informed by Sam’s Neurology team that he was presenting with an exceptionally rare condition known as NORSE (New Onset Refractory Status Epilepticus). However, as Sam had experienced a febrile illness shortly before his seizures started it was given the category of FIRES - Febrile Infection Related Epilepsy Syndrome.
They handed us a printout from the National Organisation for Rare Disorders explaining that FIRES is a subcategory of NORSE. Rather than a diagnosis it describes the symptoms being presented. Hearing that your child has a rare disorder is every parent’s nightmare.
We learned that this syndrome was heartbreakingly rare, and there was no cure - with Sam having a one in a million chance of developing it. There were no standard protocols due to its rarity and we were entirely reliant on the expertise and knowledge of Sam’s exceptional neurology and critical care consultants.
The underlying cause of Sam's seizures remained unidentified, as it didn’t appear to stem from an infection like meningitis. It wasn’t genetic either, as all tests came back negative. Treating Sam was like fighting an invisible enemy, and we were navigating it blindfold.
Sam: A Real-Life Superhero
Sam was placed on a powerful mix of medications, including several anesthetic drugs reserved for critical care settings due to their extreme toxicity and short-term usage guidelines.
Remarkably, Sam endured these medications for weeks - doses that would have been fatal for an adult. His youth, fitness, health, and strong heart played a significant role in his survival. Sam is truly a real-life superhero, and saying I am proud of him is an understatement.
The anesthetic drugs were aimed at trying to suppress Sam’s brain activity to achieve what they called "burst suppression" for 48 hours. That means they wanted to stop almost all brain activity except for brief bursts every four seconds. This approach was intended to give his brain the necessary rest to reset, much like rebooting a malfunctioning computer.
In addition to this, Sam received innovative therapeutic treatments targeting his immune system as growing evidence suggests immune dysregulation and neuroinflammation may drive the aggressive, treatment resistant seizures. Some of these treatments had not been used in Nottingham PCCU before.
Other Complications
During the seven weeks Sam was in the hospital, he faced numerous complications and challenges, including brain swelling, cerebral salt wasting with elevated urine sodium, severe constipation leading to significant stomach aspirates and non-absorption of food through enteral tube feeding, high triglyceride levels, unstable body temperature, infections, pneumonia, fluctuating blood pressure, and an unstable heart rate. These were just a few of the hurdles Sam had to overcome.
Sam's medical team was exceptional, providing world-class care and collaborating with us as parents. They consulted with other medical centers in the UK such as Great Ormond Street hospital and experts around the world. It was clear to us there was absolutely no room for egos in his team, and we were incredibly grateful.
My family were frantically research and my sister-in-law Amy, based in Denver, discovered articles on Anakinra, a treatment that we tried.
Stressful MRI Scan Days
Sam underwent four MRI scans during his hospital stay, and those days were always incredibly stressful. Watching him being transported along with all the equipment that kept him alive was overwhelming. We were told that the brain, as the most complex and least understood organ, made predicting outcomes difficult.
A poor MRI scan didn't necessarily mean a poor outcome, and vice versa. There were cases where patients with concerning MRI results experienced minimal impact, while others with seemingly better scans had worse outcomes.
The only certainty in all of this was the uncertainty.
Best Care Scenario - Significant Disabilities
Sam's had his first MRI scan within 7 days and that had been delayed due to his instability. We received the results within hours and it was devastating. The best-case scenario was that Sam would have neurological disabilities. The scan showed damage to his brain from the seizures, including several lesions. His second MRI scan a week later revealed brain swelling, and this coincided with the fact his pupils had stopped reacting - we were utterly broken.
Through tears, we told the team that we didn’t care about disabilities - we needed the team to focus on keeping him alive - getting him through this.
As weeks passed, each MRI showed the progression of the lesions, where brain tissue had died. Eventually, these areas would fill with fluid, leaving behind catastrophic “holes” in his brain.
Daisy
We were incredibly fortunate to have an exceptional team of consultants caring for Sam. They were professional, calm, compassionate, innovative and dedicated. About four weeks in, Sam's wonderful consultant arranged for Daisy, Sam's dog, to visit. She was named "Treatment 18".
We all thought Daisy might just be the miracle Sam needed. And I think the consultants recognised that Daisy would offer emotional support to both me and Sam’s dad.
Daisy was phenomenal. She entered the hospital with the confidence of a superhero on a mission, determined to get to Sam. Despite the strange sights, sounds, and smells of PCCU, she wasn’t fazed. With a room full of doctors and nurses, Daisy hopped onto a chair, and with some help, we moved Sam's hand so he could stroke her. She then settled onto the rug on his bed.
Little did we know, this was just the beginning of Daisy’s journey as a therapy dog.
Reaching Out
We asked our Nottingham team to reach out to other neurology and PCCU consultants across the UK and globally - they unquestioningly did. Not that we were questioning their treatment of Sam, but in my mind the more manpower we had working on this, the better chance of success. They may have come across a treatment that we hadn’t heard of.
From this we tried a number of innovative treatments including super high levels of phenobarbital, isoflurane gas (used in theatre environment during operations), and cannabidiol, a compound from the cannabis plant known for its potential to reduce seizures.
Despair... Nothing was Working
The medical teams had reached a point, around six weeks into his treatment, where they needed to reduce Sam’s critical care drugs, as he could not remain on them indefinitely.
There was the realisation that we weren’t going to be able to stop his seizures, and we needed to see if Sam could sustain his life and have any quality of life. Whilst his anesthetic drugs would be stopped, he would continue receiving all multiple anti-seizure drugs. We could only treat the visible seizures, and only when they affected his vital functions such as breathing, heart rate or blood pressure.
Sam was then taken off his sedation which had been holding off the worst of his seizures. I was told by Sam's neurologist to be brave, as it would most likely be heart-breaking watching his seizures return.
They were right, it was the worst thing in the world, watching my darling, sweet, gorgeous boy have his seizures return with a vengeance. His near constant seizing, drooling, and twitching was pure hell and torture to watch, and it utterly broke me.
His seizures had to be timed but this was proving impossible, as they were continuous. The only time he had any respite was for around 4 hours in the afternoon after a certain medication called perampanel, which seemed to give him a few hours of respite.
I asked my mum to buy lots of baby muslins so I could put them around his face to try and catch as much drooling as I could, so it wouldn’t hurt the skin on his face; there was only so much invasive suction he could take.
Sam’s cooling blanket, which had been helping regulate his body temperature, was also removed. The hope was that he might be able to maintain his own temperature, but it quickly became apparent that he could not, as his body temperature began to fluctuate once again and he started to get high temperatures again.
I did my best to comfort him, to keep doing what I could to care for him such as reading books, helping bathe him, change his nappy, cover him in his familiar body lotion to try and prevent bed sores, and in effect be an early alert to any changes in his condition or bodily functions.
I was with him from morning to night, and felt I knew what the 'norm' observations were for him, without even looking at his 24-hour observation sheet.
The Final Few Days
As Sam’s condition continued to deteriorate, after weeks of intensive treatments, it became clear that we were reaching a turning point, and the next few days would be critical in determining the course of his journey.
Sam had a neurological exam which revealed devastating results. He showed no responses in his arms or legs, and worse, he lacked a gag reflex. Without a gag reflex, he risked choking. His pupils were still constantly dilated and unresponsive to light.
Sam’s final MRI scan took place on Wednesday, 5th May. When the results came in, they shattered what little hope remained. The brain damage had spread significantly, affecting both the center and outer parts of his brain. The extent of the damage was likened to rapid-onset dementia. Coupled with the results of the neurological exam, it was clear there could be no recovery. The damage was catastrophic and irreversible, and it felt as though Sam’s brain was being eaten away, with no end in sight.
The pain was unbearable. Watching our beautiful son, enduring unimaginable suffering at just five years old was more than any parent should ever have to endure.
Sam's medical team determined that his breathing tube should be removed - he would make the decision for himself - whether he could continue to fight or not.
As Sam's parents, who loved our son more than life itself, the thought of him living in a state where he had no quality of life, or where he was in pain but could not communicate it, or where he could not even breathe on his own, was something we just could not bear. And so we agreed with his doctors that as there were no more treatments left to try, it was time to let him make his own choice.
Sam’s Final Day
Sam's breathing tube was to be removed on Saturday 8 May. The night before, I stayed by Sam’s side, watching as his seizures relentlessly continued, despite all the medication he was receiving.
His body seemed to be losing its fight; his heart rate, blood pressure, and oxygen levels were all fluctuating wildly. Sam’s brilliant nurse and registrar worked tirelessly to keep him as comfortable as possible. The hours felt endless.
As the night wore on, Sam’s condition worsened. His oxygen levels were dropping more frequently, requiring constant suctioning and his heart rhythm had become erratic. Despite everything, I remained strangely calm. It may sound surreal, but I wasn’t overcome with hysteria. I had passed beyond the point of crying. My mind seemed to go numb, perhaps to protect me, as it knew if I didn't I wouldn't be able to look after Sam.
My role as his mother was to make sure that Sam was surrounded with as much love as I could possible could, making sure he wasn't feeling scared, and making sure he wasn't in any pain. I was whispering to him, that mummy had kept her promise and sorted out a new plan. He didn't need to feel scared, he didn't need to fight anymore, and there would be no more pain.
When morning came, Sam was given additional medications to try and make him as comfortable as possible. We took handprints and cut a lock of his hair. We knew we were preparing ourselves and Sam for the inevitable when we removed his breathing tube.
At 1 p.m. Sam's breathing tube was removed, and for 90 minutes, Sam fought to breathe on his own. But his brain, while capable of triggering a breath, could no longer control the muscles needed to sustain life. His oxygen levels dropped to dangerously low levels - 40%. Breathing is much more complex than simply inhaling and exhaling, and Sam’s body could no longer manage it.
In those 90 minutes we cuddled and kissed our boy. I sang Sam our special songs, recited our night time prayers and told him how loved he was by so many people - over and over again. We told Sam to go towards the bright light and not to be afraid. He would be met by baby Jesus and by those who loved him so very very much.
And so, at 2:30 p.m. on 8 May 2021, Sam, our beautiful boy, passed away in his parents arms. He was exactly just 5 years and 2 months old.
The moment Sam took his last breath, our world shattered. Life, as we knew it, was gone in an instant. We were thrust into a new reality of overwhelming grief, pain, and an aching emptiness. The realisation that we would never again hear Sam’s joyful laughter, his sweet voice calling us “mummy” and “daddy,” or feel his warm hugs and kisses. We would never again find a little handwritten note from Sam simply saying, “I love you.”
All of that was gone, and we were left with an unbearable silence.
To the world a little boy, to us the world.
Sam's mummy
SAM'S LEGACY
After Sam died, I knew I would not survive life without him unless I had somewhere to direct my pain - and all the love I still carry for him. I felt that as his mum, I still had a job to do and nothing was going to stop me.
In 2021, I founded Sam’s Superheroes Foundation (Registered Charity 1210458) to turn my grief into action and to ensure his light, his story, and his memory live on and prevent any other family from facing what we went through.
Our mission is simple: to improve outcomes for people affected by NORSE and FIRES by funding vital UK research, raising critical awareness, and supporting families facing the unimaginable.
Sam’s Superheroes is more than a charity - it is a movement built on love, hope, and the determination to create change. Every step we take is part of Sam’s lasting legacy.