Day 1 - The Night Our World Changed Forever

This is my first FIRES diary entry from 22 May 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 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.

Sam had an assessment, and nothing of significance was found, apart from the fact he had a red throat so he was prescribed penicillin as a precaution.

Just minutes after returning home, whilst Sam was sit on our kitchen table, he started to blow bubbles and didn't respond to me when I asked him if he was alright. 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 was so scared watching what was happening to him, I was beside myself with fear. I genuinely 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 know 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. But, I cannot believe it's happening to us.

I plead for antibiotics and explain that we had been to the Walk-In Centre that evening, and they said he had a red throat so gave him penicillin as a precaution. They seem reluctant to give him any. I know there is something seriously wrong with Sam, 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, which contributed to delays in escalating his treatment. It also helped me understand why I felt I was being dismissed by the doctor at the time.)

20:51 - Texts to my friend, Amanda

I received a text from my friend asking how Sam was - she had seen an ambulance coming down our street, and she knew Sam had been poorly.

Me: We are at hospital with Sam. He fitted it's been horrendous

Friend: I was so worried when I saw the ambulance, and worst fear. Do they think rigour due to temp? Can I do anything?

Me: Yes potentially. It lasted 30 minutes. Amanda I cannot begin to say how frightening it was. He is asleep we are waiting for a doctor. He hasn't talked yet.

Friend: They are the most horrendous thing ever to witness. He is in the best place, he will be fully investigated. Kids are often very out of it afterwards. Hopefully it is just related to the temp. Wish I could come down and support you.

Me: Yes, he was fitting foaming at the mouth for around 30 minutes. The ambulance had to stabilise him first.

Friend: I am crying for you. Nothing more frightening ever in the world than see your child go through that. If you need anything at all day or night just message me. Hugs and lots of love.

Me: I thought I was going to lose him Amanda. I'm still worried - he hasn't talked yet.

21:00 - Text from my sister

I received a text from my sister in Blackpool:

Sister: Me and Mum are going to leave in five minutes. I don’t think we will be allowed into the hospital, because of Covid, but we can wait outside. It’s not a problem driving down tonight, we are packed.

22:30 - We are in Resus

Sam has has now had a further two seizures and he still hasn’t properly come round. He hasn't spoken at all since his first seizure. Seeing him have further seizures is incredibly distressing. His oxygen levels kept dropping during the seizures and he still hasn't woken up properly. They have done blood tests and a CT scan of his head, but they can’t see what is wrong.

On his third seizure the medical team inserted an airway (an oropharyngeal airway) into his mouth to help keep his airway open because he isn’t maintaining his airway properly. I know there is something seriously wrong. I feel sick with worry.

23:24 - Texts to my friend, Amanda

Me: Sam is going to HDU he has had three seizures, he hasn't come round properly.

Friend: I don't know what to say. You must be worried sick. Do they have any idea why? Can I do anything? I am so sorry you are all having to go through this. Poor Sam and you all. Sending the most positive get well prayers I have my lovely. Keep strong, I know it's so very hard. Lots of love.

Friend: They know Sam had covid in December don't they? Sorry my brain is in overdrive. Just making sure it is not this MIS-C that I Have read about that can happen weeks after Covid in children. Sorry hun if I am not being helpful.

Me: Absolutely spot on with that connection. The hospital has asked about that and are testing him for it. Thank you so much. He has had a CT scan and a shed load of blood tests. They have now given him antibiotics and anti-virals (although, I later learned that there was a delay giving him anti-virals).

02:00 – Sam is admitted to HDU at Derby Hospital

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

02:30 – Sam has more seizures

As the nurse again tries to reassure me that Sam is fine and that I should try to sleep, his oxygen saturations suddenly drop 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 have 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 felt 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 then started to lose his airway, they were having to bag him.

The ward eventually started to fill with more and more medics. It was clear something was catastrophically wrong. I genuinely thought Sam was going to die. The fear was indescribable. I was shaking and I could barely breathe - all I could do was stand there watching it all unfold. No one was really telling me what was happening. I felt completely helpless.

Sam ended up with a team of doctors and nurses around him, and I remember this overwhelming urge to remind them that this is my little boy, my whole world, lying there. I needed 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 would have come in. Sam should have been assessed 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 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 text 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 in last night to be with us and have been to the hospital.

05:30 - Texts to my friend, Amanda

Me: Sam has been intubated, so placed into a coma. He is being transferred to Nottingham Paediatric Critical Care. Please can you let school know for me.

Friend: I have no words. I am sorry, I have no words. Love you both lots and lots. And yes of course I will, I had already thought of that. Do you need anything brining over?

Me: Thank you Amanda. My mum and sister are at my house. They have been to see us at hospital, the team let them in. The transfer team are here and preparing Sam for transfer.

Friend: The transfer ambulance team are amazing Rachel and will spend ages making sure he is stable. I assume he is going to QMC? Are they any closer to a diagnosis? I can't believe.

Me: They think is definitely an infection from his tonsillitis. His temperature has been changing very quickly, which can be a cause apparently and also pathogens. I just wish I had insisted he had seen someone at the weekend.

Friend: Never ever think that lovely. You did all the right things and spoke to ask advice. Chances are he could have been seen and sent away. Never doubt yourself. Hindsight is a wonderful thing. You are the most caring mummy. So they are thinking sepsis rather than MIs-C? Besides Sunday morning after his bad night, he was eating toast which wouldn't make you think rip roaring tonsillitis either. Stay strong both of you. If there is anything I can do please shout.

06:00 - Text to my friend, Becky

Friend: When are they moving Sam?

Me: Hopefully in the next 30 minutes.

Friend: Let me know if I can do anything or if you need anything fetching from your house.

Me: Thankyou. Mum and sister are going to my house.

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.

07:55 - Texts with my friend, Becky

Friend: Are you at Nottingham now? How is he? Let your mum know I can have Daisy if that helps.

Me: Not yet, they are still getting him stable to travel. His veins are closing and they are struggling with putting cannulas in. Mum and my sister are here at the moment, but will call if I need anything.

Friend: I am here if you need anything, or want to talk. Lots of love.

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.

11:03 - Texts with my friend, Becky

Friend: Is there any change? Have they managed to transfer Sam to Nottingham?

Me: He is on route now and we are following. He is stable.

- A Mother’s Love

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

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