Day 8 of Sam in Paediatric Critical Care

Location: Nottingham QMC, Paediatric Critical Care Unit

Date: Monday 29 March 2021

A Mother's FIRES* Diary

*Febrile Infection Related Epilepsy Syndrome

9am:  I feel sick with nerves. Today is a big day — Sam is due to have his MRI scan at 2pm. We have everything crossed that it can go ahead. The medical team will decide whether he is stable enough.

I meant to mention this yesterday - it was a late last night as we had someone came to try and fit a bite guard for Sam, as he is biting his breathing tube during seizures. They weren’t very nice. They didn’t speak to Sam at all and barely spoke to me or the nurse. The nurse picked up on it immediately and said, “we are not having them back, they didn’t even talk to Sam.” They were really annoyed. It made me realise just how much they truly care for him. I can’t begin to say how much this meant to me. I love this nurse.

I am struggling being in the main ward. A patient nearby has a big TV on at the bottom of their bed - I can hear music, laughter, life carrying on. I feel selfish even saying it, but I don’t want to hear it. I don’t want to feel like life is just going on when my little boy is fighting for his life. I am trying to block it out, to get used to it… but it is so hard. I want to scream and walk over and turn it off.

The nurses are letting me be involved in Sam’s care. We help wash him, brush his teeth, put Vaseline on his lips because they get so dry from the breathing tube, put cream in his eyes as they get dry too, and change his nappy. I haven’t brushed his teeth - I’m too frightened of disturbing his breathing tube. They’ve asked me to moisturise his skin too, to keep it healthy, prevent sores, and support circulation. That sounds like a challenge to me - my boy will have the most moisturised skin in PCCU. I’ve asked my Mum to bring in his Childs Farm moisturising and bathing products from home. We’ve used them since he was a baby.

Last night made my heart sing. The nurses made Spiderman bunting with his name on and put it up above his bed. They are just incredible. It lifted me so much - Sam would absolutely love it. They’ve also put a Toni box by his bed with Julia Donaldson stories. Sam loves those books. We have the audiobooks in the car, so hearing those familiar stories will mean so much. We are so grateful.

They confirmed last night that Sam is tolerating his feeds, which is brilliant news. He had a stable night. Sam has had a blood transfusion to top up his red blood cells, which were low. His count was 68 - just below the threshold of 70 - whereas normal is around 100 to 120. It went well and he hasn’t had any reaction. It has helped support his blood pressure, which was up and down yesterday and made me so anxious. There’s a device on his arterial line that shows his blood pressure constantly. I couldn’t stop watching it - panicking when it went too low, and panicking when it went too high. They had to use adrenaline a lot yesterday to keep it up.

12pm: The MRI has been moved to 3:30pm. It’s frustrating, as we will probably not get the results until tomorrow now. But they have given Sam the biggest time slot they had, so it’s the right thing for him.

I ordered some new books for Sam this morning. I love reading to him and I am running out of books. My brother has ordered some too, which is lovely. I’ve also ordered a portable CD player so I can play films and music he knows and loves. I appreciate he is in a coma and sedated to general anaesthetic levels, but I am hoping it will help – somehow.

Sam is struggling to maintain a consistent temperature, it continues to be very up and down.

His anti-seizure medication has been massively increased. He is on incredibly high levels. Just to give some context, a normal dose of midazolam would be around 1ml per hour - Sam is on 4.5ml per hour.

I’m not sure if his seizures are reducing, maybe slightly, but that’s likely because of all the medication. I feel so disappointed that the steroids haven’t been the magic answer I had hoped for. I really thought they would make more of a difference.

They are trying to reduce some of his medications so he can safely go for the MRI. His oxygen has had to be increased as he is a bit rattly, but the physio team are looking after him so well.

One thing I know for sure – my boy is a fighter.

1pm: The nurse preparing Sam for transfer is absolutely brilliant. He needs a specialist transport team which includes a nurse and two doctors. The nurses prepares him for transfer and they are simply fantastic. They are so calm, so efficient, and so caring.

They are also going to try therapeutic hypothermia – which means they will reduce his core body temperature to 34 degrees. One of the consultants has seen some success with this before. It may only be temporary, but even buying time is everything right now.

Sam is also being moved onto a ketogenic diet, which may help with seizures. The dietician has been to see him and worked out exactly what he needs.

8pm: They managed to do the MRI. It was a mammoth, complex operation. I hated it. I felt on edge the entire time, terrified something would go wrong.

The nurse and doctor accompanying Sam were incredible. And so was the doctor who met us in the MRI area. hey kept talking me through everything, double-checking everything, staying so calm. It helped me more than I can explain. I felt like Sam was in the safest possible hands.

Sam had to be moved between four different ventilators - from critical care, to a mobile one, to the MRI waiting room, and then onto the MRI ventilator. I couldn’t watch when they switched them over. I felt physically sick.

We stayed by Sam’s side every step of the way, apart from the lift - there wasn’t enough room. He end up with a team of four with him, including a consultant. The team around him were first class. I feel so incredibly grateful.

It was very emotional - my mum was able to see Sam briefly as we passed through the corridor. We paused for 30 seconds so she could see him. Because of COVID restrictions, only parents are allowed to visit. It is so hard on everyone.

MRI Update: We hadn’t been back on the ward long before the results came through. I was shocked - I thought we’d have to wait until tomorrow.

They haven’t found an underlying cause...

But what they have found has devasted us...I didn't think thing could get any worse but it just has...

The seizures are damaging Sam’s brain. We don’t know if it is permanent. Some of the damage is near his brain stem.

I am in utter shock.

I cannot believe how much our lives have changed in just seven days.

Every night I pray so so hard that we will find a cure for Sam. Tonight, will be no different.

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 🩷