Day 22 of Sam's FIRES Diary
Tuesday 13 April 2021
Creating Awareness of Febrile Infection Related Epilepsy Syndrome


Location: Nottingham Queens Medical Centre, Paediatric Critical Care Unit

 

Extracts taken from diary and text updates sent to family and friends.

 

7am I hate waking up, the moment between sleeping and waking up, when your eyes flicker open. It takes a split second for my brain to adjust to reality; that I am waking up in a hospital room facing the nightmare of all nightmares, my son critically ill in an induced coma fighting for his life, with brain damage and continuous catastrophic seizures that no one knows how to stop. It is not surprising that I am struggling and I struggle to even breath sometimes.

 

Sam had a steady night, his ketamine remains unchanged, but they have managed to reduce his midazolam, and thiopental. He started his IVIG last night, which is invitro immunoglobulin, an immune suppression drug and used to manage various conditions including automimmune, infectious and inflammatory states. The ultimate goal of it is to normalize a compromised immune system. The thinking is inflammation is presumed to play a causal role in FIRES, so treatments that modulate the immune system may help the underlying cause of his seizures.

 

For reader background, the treatment of FIRES represents a significant challenge for medical teams as it is associated with such low success rates, especially during the acute stage (which is what Sam is in), patients are at significant risk of dying, and cognitive and functional impairment is common, alongside lifelong epilepsy. There is no efficacy for any treatment, apart from the ketogenic diet, IVIG and burst-suppression coma with barbiturates. Whilst there has been some reported success using other treatments in small cases studies there is no one treatment that stands out as effective.

 

So, as you can see what Sam is facing is utterly horrific. To put it bluntly, we are getting to the stage where it’s literally “let’s try it and see”, we have no choice, Sam’s seizures need to be stopped. They are killing him.

 

12pm Sam has still not had a poo. They put him on his rocuronium holiday yesterday (an anaesthetic which prevents him from moving) to try and get his intestines going as it was felt this may be stopping his bowel and digestive system from working. The team are going to try stronger laxatives. His feeding tube was coiled, so they needed to remove that and start reinserting it in him. This is a slow job they literally insert it bit by bit to prevent it coiling up. They think because his gut is so static it is not moving the tube with his internal flow. The night nurse was doing physio on Sam to try and help his gut get moving and move his poo.

 

The dieticians have been today and they are making up an IV TPN ketogenic diet so hopefully he will be on that by the end of today or tomorrow.   

 

9pm The loading dose of Sam’s phenobarbitone has gone really well. It has shown improvements pretty much straight away on his CFAM brain monitoring. In fact too well, as it has achieved full burst suppression, i.e. no brain waves showing, a flat line! They have therefore already reduced his horrible thiopental. We should be off his midazolam by tomorrow morning if all goes well. His feeding tube is now in place as they used an x-ray, or something like that to guide them in real time.  

 

I have spent most of the day once again reading books to Sam, I have helped with his cares including moisturizing his body which helps with circulation and keeping his skin in good condition, moving his limbs as shown by the physio, helping with the bed bath, and making sure his lips aren’t too dry.

 

The fabulous nurses put bunting up with Sam’s name on around a week ago so I attached his photos on it. Now when anyone approaches his bed they are greeted with photos of him. I think it’s really important for people to see Sam before he became ill, it will help them get to know him more, connect with him, and realise just what a special little boy he is.

 

11.30pm All in all today has been a stable day for Sam. His reduction in meds has gone ok and his new medication phenobarbitone is doing well. I need to go to bed as we will have an early start and a busy day with his MRI at 11.30am tomorrow. I am feeling so full of nerves about his MRI and I am praying and hoping that Sam has no further brain damage.

 

Once I feel Sam is stable, I say his good night prayers with him, sing him his good night song, tell him how loved he is, and how proud we are of him. I also tell him that we will get through this, and not to worry he just needs to keep fighting.

 

As like every night, I will myself to sleep by praying and hoping for a brighter day tomorrow.

 

Day 23 diary update will be out tomorrow. Until then I will leave you with these beautiful words.

 

~~~

Respond to your children with love in their worst moments,

Their broken moments,

Their angry moments,

Their selfish moments...

Because it is in their most unlovable human moments,

That they most need to feel loved.

~~~

Credit: L R Knost

 

With love and thanks,

Sam's mummy