Day 1 - The Night Our World Changed Forever

This is my first diary entry from 22 March 2021. It was the day our perfectly ordinary lives changed forever, when Sam, my five-year-old son, was suddenly struck down with FIRES (Febrile Infection Related Epilepsy Syndrome).

FIRES is part of a wider condition called NORSE (New Onset Refractory Status Epilepticus). Both are rare and catastrophic neurological emergencies that strike previously healthy people without warning, although children and young adults are most commonly affected.

I am sharing my diaries to raise awareness of this devastating condition, to show the reality of what families go through, and to explain why research and support for families facing the unimaginable are so desperately needed.

There is currently no cure for NORSE and FIRES, no consistently effective treatment, and the cause is still not fully understood. Around one in five people do not survive the acute stage. While recovery is possible, many survivors are left with significant lifelong neurological disability, chronic epilepsy, and complex care needs.

Sam was a healthy, happy and loving little boy, from Derby in the UK - he was rarely ill. Just after his fifth birthday, on 22 March 2021 at 8pm, the fight of our lives began when Sam had his first ever seizure after what appeared to be a routine viral illness. Read more below.

Monday 22 March 2021

7.55pm - Sam's first ever seizure

Sam had been ill over the weekend, with a high and difficult to control temperature and a strange red rash on his cheeks. When we reached out to the various out of hours doctors services we were assured it was just a virus. Sam seemed to be getting better on Monday, but by the evening his high temperature had returned so we took him to the Walk in Centre in Derby to be properly assessed.

Nothing of significance was found, apart from the fact he had a red throat so he was prescribed penicillin as a precaution.

Then just minutes after returning home from the doctors, whilst Sam was sat on our kitchen table, he started to blow bubbles and didn't respond to me. He then collapsed unconscious into my arms, having a seizure. This was Sam's first ever seizure.

Screaming I immediately rang 999 and with Sam in my arms I ran to my neighbour's house for help.

Sam remained unconscious for 35 minutes and during that time he was having clusters of seizures. I thought I was going to lose him.

Sam was rarely ill. He was such a healthy, happy little boy. To see this happening to him made no sense at all.

20:45 – A&E at the Royal Derby Hospital

When Sam started to regain consciousness he was immediately blue lighted to Derby Children's A&E. In A&E whilst he was sitting up on the bed, he was not interacting, not talking to me, he seems to be just staring straight ahead. He normally would have wanted cuddles from me, he should have been frightened, but there was nothing. Call it mothers intuition, but I knew something was seriously wrong. Sam hadn’t spoken since his first seizure at 8pm - I was so worried.

It felt like the staff were dismissing me as an over-anxious parent. You hear of this happening - it's every parent's worst nightmare, and I could not believe it was happening to us.

I pleaded for antibiotics and explained that we had been to the Walk-In Centre that evening, where they gave Sam penicillin as a precaution. They seem reluctant to give him any. I know there is something seriously wrong, but they are not taking me seriously.

(I later learned that, due to communication errors during handover between East Midlands Ambulance Service and the hospital, the A&E team believed Sam had experienced a one-minute febrile convulsion at home, rather than 30 minutes of back-to-back seizures. His Glasgow Coma Scale assessment had also been incorrectly calculated. Both of these errors masked how seriously unwell Sam was and the neurological emergency he was facing. These errors contributed to delays in escalating his treatment. It also helped explain why I felt my concerns were being dismissed by the doctor at the time.)

22:30 - Resus

Sam had a further two seizures and still hadn’t properly come round. He hadn't spoken at all since his first seizure. Seeing him have further seizures was incredibly distressing. His oxygen levels kept dropping during the seizures and he still wasn't talking or displaying any emotion. He had numerous blood tests and a CT scan of his head, but nothing was found.

On his third seizure the medical team inserted an airway (an oropharyngeal airway) into his mouth to help keep his airway open because he wasn't maintaining it properly. I knew there was something seriously wrong, and I felt sick with worry.

02:00 – Sam was admitted to HDU at Royal Derby Hospital

Once the A&E team felt Sam had stabilised he was admitted to HDU. I was given a fold-down bed opposite him and encouraged to go to sleep as Sam was “fine”. But I knew Sam wasn’t fine, and there was no way I could go to sleep whilst my little boy was so critically ill. I was wired with anxiety and worry - I knew something was massively wrong with him.

02:30 – Sam had more seizures

As the nurse again tried to reassure me Sam was fine and that I should try to sleep, his oxygen saturations suddenly dropped again. He started to have more seizures, they were coming in clusters, one after the other, and he started to become hypoxic. Machines were bleeping everywhere. I had never felt fear like this in my life.

The atmosphere in the ward changed immediately - I could tell that something was very, very wrong. I was terrified, and it felt his medical team were terrified too. They were panicked, there was shouting, if felt they were unsure what to do. Sam started to go into respiratory arrest, they were having to bag him.

The ward eventually started to fill with more and more medics. It was clear something was catastrophically wrong. The fear was indescribable and all I could do was stand there watching it all unfold. No one was really telling me what was happening.

Sam ended up with a team of doctors and nurses around him, and I remember this overwhelming urge to remind them that this was my little boy, my whole world, lying there. So I told themneeded them to focus on making him better. It all felt so chaotic.

(I later learned that the on-call consultant had not been informed that Sam had been admitted to HDU, as they should have come in. Sam should have been identified by the duty doctor as a complex case with unexplained seizures. This meant a senior doctor did not have oversight of his care during the early hours in HDU when he became critically ill. We also found out that the HDU doctor had also not been informed that Sam had now had four consecutive seizures - he thought he had only had a one seizure)

03:30 - Sam is intubated

The consultant was called in from home, the crash team arrived, and the anaesthetist . A decision was made to intubate Sam and place him into a coma. This would ensure his airway was protected and would hopefully help stop his seizures.

Arrangements were immediately made for him to be transferred to Nottingham QMC's Paediatric Critical Care Unit.

The Children's Medical Emergency Transport Team (CoMET) arrived around 7am and they prepared Sam for the road transfer by ambulance to Nottingham QMC. I felt so relieved when they arrived. It felt like Sam was finally in a safe pair of hands. They looked like real-life superheroes walking in.

It took nearly four hours to prepare him. They had to stabilise him for travel - his veins were closing.

I texted my friend to ask her to let school know what had happened.

My mum and sister had arrived in Derby - they drove all the way from Blackpool last night to be with us and have been to see me at the hospital.

07:30 - Infection is thought to be the root cause

The consultant at Derby thinks an infection is the root cause. Sam's temperature is changing very quickly, and is high, which can cause seizures. I just wish I had insisted he had physically seen a doctor at the weekend when we called 111. But it is the tail end of Covid - and I was trying to do the right thing.

11:00 - Sam is transferred to the critical care unit at QMC Nottingham

The CoMET team transferred Sam out of Royal Derby Hospital to go to Nottingham Paediatric Critical Care Unit at QMC. We walked with them as the transfer team took Sam out of HDU to the waiting ambulance. It felt like an out-of-body experience seeing my son connected to all these machines that were keeping him alive.

Me and Sam's dad couldn't go with him in the ambulance - there was not enough room for us. Can you imagine not being able to go in an ambulance with your critically ill child? But I did feel reassured that he was in very good care now.

Myself and Sam's dad rushed home and packed an overnight bag and immediately drove to Nottingham QMC. I packed some of Sam’s things like his toothbrush and PJs. I called one of my close friends on route to let her know what has happened. She keeps me calm and tells me everything is going to be OK.

Sam’s FIRES Diary – Day 2 will be shared tomorrow.

Sam, if love alone could have saved you, you would have lived forever.