Gone, just like that

This morning started like any other day.

I was up at 6:30am to give you your pre-feed medications (ordine and paracetemol) then feed you via your NGT while expressing more milk.

You were a bit congested, so I suctioned your nose and mouth which annoyed you quite a bit. You settled down soon enough, though; and by the time your feed was finished around 7:40am you were settled and sleeping.

At 8am I topped you up with your morning round of daily medications (topiramate, vit D, iron and omeprazole) and left you to snooze while I ate my own breakfast and entertained your sister.

Elsie making a crown, 13 September 2017

Your sister is a handful, Poppy. Two and a half and full of beans.

This morning, we made a crown out of a cereal box and stuck sequin 'jewels' to it while you snoozed to your music in the nursery.

At 10:45am I checked your stomach contents, gave you your pre-feed meds again (ordine and ibuprofen this time), and went to heat up your bottle and ready the expressing kit.

At 11am when I went back in to you, you were pale and frothing a little around the mouth so I suctioned your mouth and nose to free up your airway. I observed that you were breathing very infrequently, but deeply. I could tell straight away that something was unusual with you.

I picked you up and changed your nappy. You were very floppy. there was literally no resistance in your muscles. I put you back in your cot and watched you breathe for a few minutes.

I took this video at 11:07am.

 
You were pausing for a minute or more at a time before taking a few, deep breaths. I had to decide, standing alone in your small nursery on a Wednesday morning while your sister watched ABC kids in the lounge:  was this IT?

At 11:09am I called your father at the office. I said I couldn't tell, but I thought you might be dying. I described my observations. He dropped everything to come home and see you. He got here in 20 minutes flat.

At 11:13am I called Eastern Palliative Care. They did not discount the possibility that you were actively dying. In fact, they said they would come over straight away.

I sat down beside you and watched. You continued to breather deeply but extremely infrequently.

At around 11:25am you took four deep, haggard sounding breaths. I started the stopwatch on my phone to better quantify your pauses. It just felt like I should be doing something.

Five minutes later, when your dad came in, I told him, "I'm counting her breaths. I'm waiting for the next ones. She's breathing very slowly."

I showed him my phone. It continued to count up from 5:25.

"I think she's gone," he said.

I couldn't believe it. I'd sat there watching you the whole time, and I never saw you leave. And yet, I know objectively that you had not taken a breath in over five minutes.

We moved you to the change table and took your temperature. You were already beginning to cool. The thermometer read 35.8C.

You were gone.

Further regression?

Over the past few days there have been less and less times when you're 'with' me. You're mostly asleep or having one of your agitated face rubbing sessions.

Nothing can compel you to stop, and the more you go at it the more agitated you become. In the end I find it is better to swaddle you. You usually calm down pretty quickly after that and drift off to sleep because you're exhausted.

There have also been more times when you're quite out of it and very grey because you're breathing too slowly or shallowly.

Poppy looking grey, 7 September 2017

Our palliative care doctor says not to feel like we have put you on oxygen during these episodes; only if it makes you more comfortable. It doesn't, so we won't.

You've also slowed down in the eating department. The first few feeds of the day seem to process through OK, but the later ones are just sitting in your tummy and we're bringing back a lot of milk or curds when we aspirate ahead of the next feed.

We've started a new regimen of five feeds only per day, each at four hours with an eight hour break overnight. This way, we are under less pressure and you have more time to digest. You won't be getting your prescribed daily volume, but at least you won't be vomiting.

Poppy, this is really hard.

I love you so much, but watching you this way makes me wonder whether yours is any kind of life at all for you. We've got you sedated pretty much 100% of the time. Ordine is my new best friend.

Rett syndrome has four stages. I think you're in stage 2 (rapid regression) at the moment, and I think it's getting worse.

From ED to PICU

When we arrived at Monash emergency, around 3am on 24 June, I still thought you'd just spontaneously get better in a few days.

But over the course of the next 5 hours you seemed to withdraw more and more and get more lethargic. Your eyes were red, and your oxygen saturation was poor.

Poppy on high flow oxygen and feeding tube, 24 June 2017

The doctors put you on high flow oxygen. Your stats improved immediately, indicating that there was no underlying heart issue behind all of this. You stayed on the oxygen while we waited for a bed to open up on a ward for you.

Around 2pm you had a nasogastric feeding tube inserted because you were feeding very poorly. This was a relief to your dad and me because we'd been struggling to feed you properly for about a month. Your weight had almost plateaued. Anything that would get food into you without a fight could only be a good thing at this point, as far as we were concerned.

At 8pm the medical staff were just preparing to transfer you to a ward when you took a massive dive and had to be intubated. It was lucky you were still in the ED and not in a hallway en route.

That's how we ended up in PICU. That's how we ended up here, where you've been now for 30 days.

You've had so many tests, but no one knows what is wrong with you yet. Everything comes up negative or normal and yet you continue to suck at breathing on your own.

Initially, the doctors identified a chest infection caused by a couple of different bugs including a nasty one called VRE. It explained your immediate breathing difficulties, but not some of your other odd symptoms.

Before coming into hospital, I'd already been trying to have you checked out for come worrying behaviours.