One in a Million - Sam's Fundraising & Story

'One in Million' Story

Sam, our darling, precious, much loved little five-year old super-hero, was always a very healthy little boy, he had little in the way of illnesses, he was very fit. Sam loved nothing better than running around in the outdoors or cycling for miles on his bike with his daddy. Along with his mummy, daddy and Daisy (his beloved dog) Sam enjoyed going on long walks, playing outdoors in mud and streams, finding sticks to play with so he could turn them into anything he wanted them to be, collecting stones and finding the best puddles to jump in. Sam was a dream little boy. He didn’t really have tantrums, he loved to please people, loved to make people laugh, loved to play and use his imagination, and had a real thirst for learning.

The Day Our Lives Changed Forever

On Monday evening 22 March 2021 at 8pm, our lives irreversibly changed forever, and every parent’s worst nightmare came true when Sam had his first ever seizure. In fact, this nightmare was far worse than one anyone could even imagine, it was one that had not even entered my conscious thought process.

Over the weekend of the 20 March Sam had a high temperature, slight sickness and lethargy. I had spoken to 111 on a number of occasions over the weekend, and as he was still up and down in himself, I made an appointment with this GP on Monday morning. Both 111 and the GP put it down to a routine a virus and to continue what I was doing of giving Sam Calpol and ibuprofen. Late Monday afternoon he still had a high temperature (though he had been relatively fine in himself on Monday afternoon and had been playing etc) so I was beginning to really worry it was something more serious. I couldn’t get an appointment at his doctors, so I took Sam to Derby Walk in Centre for an assessment. They did not identify anything and again felt he had a virus but gave Sam penicillin as a precaution as his throat was red and he had a history of tonsillitis.

Minutes after returning home Sam had his first seizure just minutes, whilst sitting on the kitchen table, where I had put him after I had given him his first lot of penicillin. I noticed he had started blowing bubbles and was drooling. When I asked him what he was doing, he didn’t reply and just fell into my arms. I immediately phoned 999, all whilst scooping Sam up into my arms, running out of my house to my neighbour, screaming for help and shouting my address out over the phone. I was utterly panic stricken. At my neighbours we lay Sam on their sofa, and he had a full body tonic clonic seizure (arms and legs jerking rapidly and rhythmically) for around 30 mins. After around 30mins he stopped seizing and he got transferred to the ambulance. Sam sat up in the ambulance with his eyes open, but he was not talking, not even to me.

Derby Royal A&E

We got rushed to Derby children’s A&E and I was obsessed at this point about Sam having antibiotics as I was thinking it was in infection. Sam preceded to have further two seizures and after his third seizure he didn’t wake up but seemed to be in a very strange deep sleep, but I was told that this apparently was commonplace after having seizures. Sam had a head scan whilst we were there, which showed nothing significant. To say I was panic stricken and beside myself with fear and worry about my little boy would be an understatement.

Derby HDU

Sam got admitted to HDU around midnight and I stayed with him. Then during the night, Sam’s stats plummeted, he started having further seizures. He was having trouble maintaining his airway and was becoming hypoxic (lack of oxygen). I could see the nursing team was panicking, and this is by no means critical of them. It was clear something was catastrophically wrong with Sam. More and more medical people were coming to Sam, and he ended up with a huge team around him. I was simply terrified I didn’t have a clue what was happening. I now know that they were arranging for specialist teams to help him maintain his airway, put him in an induced coma and onto a ventilator.

Whilst all this was going on the Derby team were also seeking advice from the on Duty Paediatric PCCU Consultant at Nottingham Queens Medical Centre, Paediatric Critical Care Unit (PCCU), a specialist neurology centre around 20 miles away. The Duty Consultant is someone who we would grow to enormously respect and trust and would prove instrumental in Sam’s care over the coming seven weeks.

Meeting the Super-Hero CoMET Team

The team at Nottingham felt that Sam should be admitted to their PCCU for specialist treatment. The amazing CoMET (Children’s Medical Emergency Transport) team were sent to transfer him, and they arrived around 5am that day. When they walked in to HDU, they had this calmness and utter professionalism that I was blown away by. Straight away I knew that Sam would be in safe hands, they were like a team of real-life superheroes. It took them around six hours to prepare him for transfer.

When Sam was ready for transfer, it was like an out of body experience to see my precious boy surrounded with drips and electronic equipment to keep him alive. Myself and his dad walked with Sam and his transport team to the waiting ambulance, we then went quickly home to pack an overnight bag and make our way to Nottingham’s Queens Medical Centre. Little did we know just what truly heart-breaking and devastating times lay ahead for Sam and us.

Entering the New World of PCCU

We entered the alien world of PCCU around midday, and it was terrifying, we were distraught, we had no idea what to expect and what lay before us. Sam had been placed into isolation, as it was still uncertain whether COVID-19 had a part to play in this, or indeed if he was still testing positive for COVID from when he and myself had it just before Christmas.

We were extremely fortunate that we were given a room to stay in, which was just 30 seconds away from Sam’s ward, and we feel forever grateful for this.

What was Wrong with Sam?

From our initial chat with the PCCU consultant team, we were positive that these seizures could be treated and stopped. It was thought they may be caused by a virus, or infection. Apparently, there was a huge protocol of anti-seizure medication that could be given to Sam, and we felt optimistic. He was in the best possible place; he was on antibiotics and anti-viral meds to treat underlying causes.

Whirl Wind of Activity

The first couple of days there was a whirl wind of activity around Sam. There was a constant team of people making up new IV bolus medications. Medications would initially work, but then his brain would find a way round them and he would start to seize again. It was devastating.

A CFAM (Cerebral Function Analysis Monitor) device was attached to his head, to monitor his brain activity, alongside continuous visual recording of his body.

The medical team were making their way down the anti-seizure medication protocol, the further they got down it, the more toxic the medication was. Sam was now in Super-Refractory Status Epilepticus territory. Refractory status epilepticus is seizures that persist despite administering two anti-seizure medications. Super-Refractory Status epilepticus is seizures that persist for greater than 24 hours, despite anaesthetic treatment, or it recurs on an attempted wean of the anaesthetic regimen. It is a life-threatening condition, his seizures needed to be stopped.

Stopping the Seizures........ the Impossible Task

It soon became clear that stopping the seizures was not going to be straightforward. The first few days of Sam being admitted to PCCU are a blur. I was too panic stricken and emotional to even think straight, let only remember anything. I was relying on his dad listening and communicating with the medical teams. I just kept thinking he is in the best place possible, you need to leave it to them Rachel, don’t start googling, trying to input into his treatment, trying to second guess, trying to micro-manage a subject that you know nothing about. You will send yourself crazy, it won’t be helpful and may even impact on Sam’s care.

No Room for Bystanders

As the realisation became apparent that stopping the seizures was proving difficult, I knew that Sam needed his mummy to fight alongside him. There was no room for bystanders. He needed a mummy who would work alongside the medical team, be his voice, who would fight with every breath and bone in her body to help find a cure for this catastrophic condition. And alongside my son and his daddy, that is what we did, we FOUGHT, FOUGHT, FOUGHT, FOUGHT FOUGHT. We were not giving up, and I would not allow anyone else to give up. I was like a lioness fighting to the death for her cub. I have never felt more protective of Sam in my life. Anyone who dared utter any negativity I was all over it. I needed to know and feel that everyone was 100% committed to Team Sam, to finding a solution.

One in Million Chance

We were soon informed by Sam’s Neurology team that what he was presenting with was an exceptionally rare condition called NORSE (New Onset Refractory Status Epilepticus) and FIRES (Febrile Infection Related Epilepsy Syndrome). We were given a printout from the Rare Disease Organisation explaining it. A rare disease is something that no parent wants to hear their child has got. FIRES is a subcategory of NORES, and it is not a diagnosis but describes the symptoms that are being presented by the patient; it is a condition.

https://rarediseases.org/rare-diseases/new-onset-refractory-status-epilepticus-norse/#:~:text=Febrile%20infection%2Drelated%20epilepsy%20syndrome%20(FIRES)%20is%20a%20subtype,of%20onset%20of%20status%20epilepticus

This syndrome is exceptionally rare, and heart-breakingly Sam had a one in a million chance of developing this condition. It was extremely challenging to treat, and it was yet to be understood what was causing these seizures. It didn’t appear to be an infection such as meningitis.

Sam a Real-Life Super-Hero

Sam was placed on a huge mix of medications including a number of anaesthetic drugs. Some of these drugs were only for use in a critical care setting, were exceptionally toxic, and therefore should only be used for a short time. Sam was so amazingly strong, he ended up being on some of these medications for weeks, which could have proved deadly for an adult. He was surviving as he was young, so fit, healthy and his heart was so strong. Sam is truly a real-life superhero, to say we were proud of him, would be an understatement.

The anaesthetic drugs were aimed at suppressing his brain activity, so we could achieve what is called burst suppression for a period of 48 hours, i.e., stop all brain activity apart from an occasional burst approx. every 4 seconds. It was hoped that this would be sufficient to rest his brain, and allow it to reset, so we could then slowly restart it. Similar to a reset on a computer when it goes haywire.

Other innovative therapeutic treatments were given which were generally aimed at suppressing Sam’s immune system as it was felt this was a probable cause of his seizures i.e., his immune system was attacking itself. Some of these treatments had not been used in Nottingham PCCU before to treat these type of clinic symptoms.

Other Complications

During the seven weeks Sam was in hospital, he many other complications and challenges, too numerous to mention here. But included brain swelling, cerebral salt wasting with elevated urine sodium, severe constipation resulting in significant stomach aspirates and non-absorption of food through his enteral tub feeding, significantly high triglyceride levels (fat in the blood), unstable body temperature, infection, pneumonia, low blood pressure (which went too high towards the end), low heart rate (which went too high towards the end) etc.

He had an amazing team around him, and we felt he really was receiving world leading care. The teams were very engaging and so very open to input from us as parents, and with other medical centers and colleagues from around the UK and globe. There was no room for egos and the teamwork was first class. We as parents were viewed as a very active part of the team caring for Sam. My fantastic sister-in-law, Amy, who lives in Denver was frantically researching possible treatments, and she found articles on the use of Anakinra as a possible cure.

Stressful MRI Scan Days

Sam had four MRI scans throughout his stay, I hated these days, they were so stressful. From seeing him being transported with all his equipment keeping him alive, to receiving the results. The brain is the most complex organ of the body, and the least understood even in 2021. The one certain outlook, outcome or prognosis in all of this, was uncertainty.

A bad MRI scan did not necessarily correlate into a poor outcome. There were patients who had poor MRI scans which had minimal impact on them. Then there were generally not to bad MRI scans where patients had poor outcomes.

Best Care Scenario - Significant Disabilities

After our first MRI scan on Wednesday, just 28 hours after his very first seizure, we were informed that the best-case scenario for Sam was that he was going to have significant disabilities. He had a number of legions (damage) on his brain caused from the seizures and brain swelling. We determinedly but tearfully told the team, we didn’t care what disabilities our boy had, we would give him the best life we possibly could. He would have a full-time mummy caring for him, who would make sure he had the best quality of life regardless of disability. They just needed to focus on keeping him alive.

At each MRI scan we could see the progression of the legions, which is where the brain tissue had died. It would eventually evolve and leave catastrophic 'holes' in his brains which would fill with pockets of fluid.

Reaching Out

We asked our Nottingham teams to reach out to other neurology and PICCU consultants across the UK, and they unquestioningly did. Not that I was questioning their treatment of Sam, but in my mind the more brain power we had working on this, the better chance we had. 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, using isoflurane gas to achieve burst suppression, and cannabidiol.

Nothing was Working

The medical teams had reached a point where they needed to reduce Sam’s critical care drugs, as he could not remain on them indefinitely. The new strategy was that were not going to be able to stop his seizures and we needed to see if Sam could have any quality of life with them. So, we were just going to treat the clinical seizures, the ones we could see.

So, Sam was taken off his Isoflurane gas, which had been sedating him and holding off the worst of his seizures. I was told 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. His constant drooling, his constant twitching. It was pure hell and torture to watch and broke me. We had to document and time his seizures, but this was proving impossible as they were coming thick and fast, one after another. I asked my mum to buy lots of baby muslin's 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 face; there was only so much suction he could take.

The Final Few Days

His cooling therapy blanket was removed, but his temperature started to become erratic again, clearly, he was not able to maintain his own body temperature.

His CFAM was removed as we were only monitoring those clinical seizures we could see. He had a neurological exam by this neuro team, and the outcome of this wasn’t good. He had no reactions in his legs or arms, and he didn’t have a gag reflex which you need to sustain any form of breathing on your own. If you can’t gag you can choke. He pupils were constantly dilated, and not reacting to light. In fairness they had been like since he was first admitted.

He had his final MRI scan on the Wednesday 5 May, which had been delayed as he had an infection. The results were completely life shattering news. His brain damage had evolved and was catastrophic. It was everywhere, in the middle of his brain, around the outside. It was likened to having super-fast dementia. When considering the neurological exam, all hope had been blasted away of Sam recovering, or having any quality of life. The FIRES had simply eaten away at his brain, it wouldn't stop until there was nothing left of it.

Words are insufficient to explain how we felt. But all I could see was the suffering my darling brave baby boy was going through at five years old. It was just too much to bear.

A decision was made by the medical teams that Sam’s breathing tube, which was maintaining an airway for him, would be removed. Sam would make his own decision on whether he could survive or not. As parents we agreed with this, as there were simply no other treatments left to try.

His Final Day

I stayed up with him through the night on the Friday evening, and it was heart wrenching. His seizures were just relentless despite all his anti-seizure meds. Our brilliant registrar was trying to keep him as comfortable as possible, and I was still bathing him in all his lotions that night to try and keep his skin soft and prevent bed sores.

Sam was given additional meds to try and calm down some of his seizure activity and make him as comfortable as possible. We took handprints, in case the inevitable happened and he wouldn’t be able to sustain his breathing when his breathing tube was removed.

Time that morning seemed to stand still whilst we prepared both Sam, and ourselves, to take out his breathing tube. It was removed around 1pm and for 90 minutes Sam tried to sustain his own breathing, but it just wasn’t possible. Whilst Sam's brain could trigger a breath, it could not control the muscles in his airway or maintain efficient breathing to sustain his life. Your brain does so much more than trigger a breath to breath, breathing is so much complex. Sam passed away in his mummy and daddy’s arms, with his mummy singing to him our special song that we made up just for us, and one that he knew so well.

When Sam took his last breath, our world and life that we knew so well catastrophically shattered into a million pieces all around us. We then entered yet another new world of overwhelming grief, emptiness, numbness, desperation, and heart wrenching pain with the realisation that we would never again see our beautiful son, or hear his joyful voice his laugh and his giggles, or hear him call the words mummy and daddy, or feel his loving kisses and hugs, or see him write a card with I love you in, just because……just because he felt like doing it.

Our Fundraising

Following Sam’s devastating passing, as his mummy and daddy, we wish to express our sincere appreciation to our beautiful friends, family, school (Silverhill Primary School, Mickleover) and Nursery (The Orange Tree Day Nursery Mickleover), Funeral Directors (Coop, Mickleover) the local Mickleover community and the local business community, for the love, care, compassion and support we have been given. The care and compassion we have also been shown from people whom we may not personally know, but who have taken the time to reach out to us, has given us more comfort and support than you could possibly know.

Thank you also to those of you who have donated to the separate Just Giving fundraising page which Sam’s dad, Jim, initially set up for the Pediatric Critical Care Unit (PCCU) at Nottingham Queens Medical Centre.

Our primary focus has now turned to creating an ever-lasting sustainable legacy for Sam under the name of “Sam’s Superheroes” www.samssuperheroes.co.uk. We will raise funds, to support initiatives and experiences that have kindness, children, families, animals and nature at their core. In addition, we would love to help children who may not have access to some of the amazing childhood experiences that we were blessed to be able to give Sam; some of which may have life changing impact on them, or to help families who have difficulties in accessing help or support their children need.

Helping to fund and promote projects that support the amazing benefits that come from the therapeutic healing and nurturing power of nature, wildlife and animals will be central. This includes training Sam’s dog Daisy as a community therapy dog, and sponsoring projects which support outdoor play and sensory gardens (starting with supporting a sensory garden at Sam’s school), nature and wildlife projects, or education days. All of which will also raise awareness of the importance of being kind and caring, together with looking after nature and the environment.

Even if one child or family is helped it becomes a force for change and allows for greater good to be spread in the community around us. We are just starting out on this legacy journey, but your support is greatly appreciated, and I know we will make a difference for families and children in Sam’s name.

Our key contact details are below. Please follow our journey on Facebook or twitter. We will shortly be on Instagram where Daisy will have her own Blog, and we will be giving regular updates on our social media channels.

#SamsSuperheroes #Love2Sam

Website: www.samssuperoes.co.uk

Facebook Page: @teamsamssuperheroes

Twitter: @superheroessam

Email: rachel@samssuperheroes.co.uk

We are also looking for support from people in many ways such as: your skills, support and connections to help us hold an annual black tie and family fun day event, fundraising and sponsorship, joining our little team of super hero volunteers making crafts, knitting, crocheting, sewing bunting, baking cakes, growing plants from seed, so we can sell these things at summer and Christmas fairs and online, and those who can donate craft supplies or wool etc.

With many thanks and much love

Rachel, Sam's mummy