Day 46 of Sam in Paediatric Critical Care

Location: Paediatric Critical Care Unit - QMC Nottingham

Date: 6 May 2021

Day: 46

Sam: 5 years old - previously healthy

A Mother's FIRES Diary

This is day 46 of my five year old son being in a coma, as doctors fight to stop his relentless seizures. They came out of nowhere after what appeared to be a mild virus. They have named this FIRES (Febrile Infection Related Epilepsy Syndrome)

⚠️Please note: this content may be distressing to read ⚠️

Sam's last remaining anaesthetic (isoflurane gas) that was keeping him in an induced coma was removed yesterday so he could have his MRI scan. We need to understand whether there has been any more damage to Sam's brain - if it has got any worse. The anaesthetic gas was holding off the worst of his seizures. Before it was removed I was told I would need to be brave by his neurologist - it was likely be incredibly distressing for me to see him being in a state of continuous seizures. They were right.

Today is a significant day. Sam will have a neurological exam by his neuro team, followed by a discussion about his MRI results from yesterday and what the next steps are for him. I feel desperate, scared and numb at the same time.

Sam continues to experience relentless waves of seizures. He gets brief moments of respite, but they don’t last long. I’m watching Sam like a hawk, trying to spot any sign of consciousness. I noticed him starting to yawn, and I got so, so excited. It looked just like my boy when he was tired - a big yawn. I told his neurologist, but they cautioned me that it was likely just a reflex. The only other responses we’ve seen are reflexes to pain, and occasionally, when being suctioned, he’s moved his arm toward his head. I am deeply worried that he can feel pain and discomfort - but he can't communicate it. I am also desperately worried he is frightened - and again can't communicate it. It feels absolute hell and I am simply terrified for my boy.

Sam still has a breathing tube in place and is triggering his own breaths. But as I said yesterday, something about his breathing seems strange to me. When I watch him inhale, it just doesn’t look or seem right.

MRI update

We’re taken into the parents' room. There are around three critical care consultants, two neurologists, Sam’s lead nurse, and our palliative care nurse. The news we receive is utterly devastating.

The outcome of his neurological exam was catastrophic. He showed no responses in his arms or legs and had no gag reflex - something necessary for independent breathing. Without a gag reflex, there’s a risk of choking. Sam’s pupils were dilated and unresponsive to light - they had been like this shortly after his admission, around the time of his brain swelling.

The MRI scan results from yesterday were simply earth shattering. His brain damage had progressed and was now catastrophic. It was widespread and irreversible. It was likened to a form of ultra-fast dementia. There were also holes in his brain, now filled with pockets of fluid.

Considering both the MRI and neurological exam, it became clear all hope of any meaningful recovery or any kind of meaningful quality of life for our son had slipped away. It felt like FIRES had eaten away at his brain. Whatever this was, it wasn’t going to stop until there was nothing left of his brain.

Sam’s dad and I asked so many questions. Was there truly no chance of recovery? How could we know for sure? Could the high doses of other medications he was on be keeping him in a coma and not waking up? But the fact remained - his brain damage was catastrophic. He couldn’t have a quality of life.

I was numb - completely in shock and disbelief. Words are simply not enough to describe how we felt. All I could see was the suffering my brave, darling, innocent boy was going through at only five years old.

A decision was made by the medical team that Sam’s breathing tube, which had been maintaining his airway, would be removed. Sam should be allowed to decide for himself whether he could survive.

As parents who love our son more than life itself, we knew we had to support that decision. We had no choice, we had a responsibility to our son. I didn’t want my gorgeous son left in a subconscious state, with no quality of life. We had to put our own emotions to one side and do what was right for Sam.

The breathing tube was scheduled to be removed in four days, on the Monday (it was Thursday). That would give us four more days with Sam, in case the unthinkable happened. We began making the hardest phone calls of our lives to family and our closest friends sharing the earth-shattering news.

When I returned to Sam’s room, one of the doctors was there, but was just leaving. I stood in the doorway to Sam's room and whilst crying asked if we could try a drug that I know we hadn't yet - called Tocilizumab. I had read about it on the NORSE Institute website - it’s sometimes used instead of Anakinra, which Sam had already received, but it didn't stop his seizures. Both tocilizumab and Anakinra target inflammatory cytokines - but different ones. I was utterly broken and sobbing in the doorway whilst I asked this question - the doctor gently guided me inside Sam's room and told me it just wasn’t possible.

I asked the team if we could seek second opinions on Sam’s brain scan from the Great Ormond Street team who had been supporting Sam’s treatment, and from a friend at another London hospital. Sam’s doctors readily agreed. I wasn’t questioning their judgment - I just needed to hear it from others.

I am incredibly thankful for the amazing team of nurses caring for Sam over the past few days. His lead nurse was on that day, looking after both him and us with such compassion.

We also talked about whether Sam should be moved to the local hospice, Rainbows. But we decided we didn’t want him moved. The PCCU team felt like extended family. They cared for Sam deeply, they knew him inside out, and he was still incredibly complex. I trusted them completely. I didn’t want him anywhere else.

Sam’s dad and I talked about the hardest questions: What if Sam is trying to tell us he wants to let go, but he can’t? What if he’s in pain and can’t communicate it? We kept coming back to one thing - this has to be about what us best for Sam.

I continued helping with Sam’s care, just like yesterday. I’m on autopilot. I talk to him, reassure him. Tell him everything will be okay. Tell him how much we love him, how brave he is, and how proud we are.

I gently whisper to Sam and tell him "mummy is going to sort everything out - and not to worry".

Once I feel Sam is stable, I say our good night prayers together, sing him his good night song, tell him how loved he is, and how proud we are of him and I gently reassure him mummy will never leave his side, we will get through this, and he just needs to keep fighting. I then head to bed.

I lay there in bed completely and utterly in shock. Seven weeks ago, I had a healthy boy. Now he has catastrophic, irreversible brain damage. And I’m terrified - terrified of what lies ahead for Sam, my beautiful, kind, loving, perfect five-year-old boy. As like every night, I will myself to sleep by praying and hoping for Sam to have a brighter day tomorrow. And I hope that Sam’s nurse has a boring and uneventful night.

Thank you for taking the time to read - I am incredibly grateful. I hope it gives you insights into FIRES and why our charity's work is so vital.

Our next diary update will be out tomorrow.

💥WHY WE ARE SHARING THESE DIARIES💥

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.

Five year old 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, and our world changed forever.

With love and thanks,

Sam's mama bear 🩷

Sam - my beautiful, healthy and happy little boy in Summer 2020

Sam in an induced coma in paediatric critical care at Nottingham QMC in May 2021.