Day 7 of Sam in Paediatric Critical Care

Location: Nottingham QMC, Paediatric Critical Care Unit

Date: Sunday 28 March 2021

A Mother's FIRES* Diary

*Febrile Infection Related Epilepsy Syndrome

10.30am: The main ward is much noisier than the isolation room, so Sam is now wearing earplugs. We think noise and touch may be triggering his seizures. Last night, the nurse also placed ear defenders on him as well.

The nursing team are just so caring. I cannot fault the level of attention they are giving him - it brings me a small sense of comfort to feel he is in such caring and safe hands.

Sam is positioned slightly upright in bed, with a teddy tucked under each arm. For those reading this, one of them is his doggie called “Spot”… although you would be forgiven for thinking he had spots. He doesn’t. He is actually a Corgi that Sam chose himself from the Sandringham gift shop when we were on holiday in Norfolk with our best friends Becky and her daughter Holly.

Spot’s home is now my bed.

2pm There is no real improvement today.

Sam’s oxygen levels have dropped and are dipping to 92% - another thing to worry about.

The physio team visit Sam twice a day. And during his morning visit they took secretion samples, and they may start him on a nebuliser to help loosen them. Yet again, they are another team I am so blown away with - efficient, kind, and incredibly gentle.

I love how they always say hello to Sam when they walk in, just like they would if he was awake. And they talk to him throughout, even as they carry out lung exercises.

I had no idea how vital physiotherapists are in critical care. I will admit, I always thought of them as working mainly with muscles and bones. But here, they are specialists in respiratory care. When someone is intubated, they can’t clear their own secretions, which can build up and affect breathing or lead to infection. The physios use different techniques such as vibration, simulated coughing to help manage this. They are the real experts in this space.

I have been talking to the duty Registrar and they explained that if the steroids don’t successfully dampen Sam’s immune response, there are other drugs they can use - but these are at the far end of the spectrum. We had previously talked to them about using Anakinra, as it was used successfully on a girl in America – it stopped their seizures after 28 days. But for now, they are waiting to see if the steroids work. Sam has only been on them for 12 hours.

Any next steps would be discussed between the PCCU consultants and the neurology team. It really is a huge multidisciplinary effort to care for Sam.

4pm I have been reading Sam a story. The medical team continue to do everything they can, and Sam is still fighting as hard as he possibly can.

I am beyond proud of Sam, but my heart is breaking.

6.30pm I went for a lie down and managed about an hour’s sleep while Sam’s dad stayed with him. We make sure one of us is always with him.

We have fallen into a routine. Lunch around 1pm, dinner around 7:30pm. But my appetite has completely gone. I just can’t eat.

9pm: Sam’s temperature remains high and keeps trying to climb. The cooling machine isn’t working properly, so they are replacing it.

They still don’t know whether Sam’s temperature is due to infection, or whether his brain is unable to regulate it. I can’t help but feel it is his brain. Before his very first seizure, when he was ill over the weekend, his temperature was so erratic, it just made no sense, it was so strange. It would spike incredibly high, come down briefly with medication, and then rise again.

They are changing his antibiotics and sending off his blood, urine and lung secretion samples to investigate further.

Sam’s red blood cell count is borderline low, so he will have a transfusion today or tonight. He has had so much blood taken – so it is not surprising he needs some back. I just hope it helps him feel a little stronger.

If I’m honest, today has been truly awful. I have barely messaged my close friends any updates. I feel incredibly low, full of anxiety, and a bag of nerves.

There is perhaps one small positive - Sam’s seizures now appear to be sub-clinical. These are seizures you can’t physically see, unlike the clinical seizures he has been having for the past five days.

Clinical seizures - the visible ones - can be very traumatic for the brain when they are prolonged and uncontrolled. That is why the team have been doing everything they can to stop them.

I had no idea before all of this how damaging ongoing seizures could be.

Sam’s dad and I have fallen into a routine where I call or text his dad as soon as I wake up. He tends to get to the ward very early, around 5am. It is so I can prepare myself before I walk in.

I just need to hear his dad’s voice first. To steady myself. To prepare for whatever I am about to walk into.

11pm: I usually get to bed around 11pm to midnight. I wake around 6am. But I don’t get up straight away. I lie there, completely paralysed with fear.

I cannot begin to explain the fear I feel when I wake up. The dread that this nightmare we are living in is still real for another day - or even worse, that Sam may have deteriorated.

But alongside that fear, there is always this tiny, fragile piece of hope. The thought that maybe today will be the day.

And yet I am so frightened to let myself believe it. We have had so many false hopes with each new medication, each one bringing a flicker of possibility, only for it to come crashing down again.

I don’t know how much more Sam - or me and his dad - can take. This is just so horrific.

I can’t believe my healthy, happy little boy is facing something so unimaginable. A nightmare I never even knew existed, let alone thought would become our reality.

Every night I pray so so hard that we will find a cure for Sam. Tonight, will be no different. But I will also pray that Sam’s MRI will be able to go ahead tomorrow.

A photo of Sam at Warwick Castle in 2019, after he had completed his 'Knight fighting' course!

Sam in an induced coma at Nottingham QMC fighting FIRES - March 2021

Thank you for reading. Every read of Sam’s FIRES diary helps 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.

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 mummy 🩷