Remembering you

Two days ago, on Sunday afternoon, we held your memorial party.

So many people came - some who met you, and some who never did - to help us remember you and say goodbye.

It was a beautiful, sun filled day; and the backyard was full of happy kids tearing about while we caught up with people we haven't seen all this long and harrowing year.

I hope that our family can find more balance now and make more time to catch up with friends; particularly those with playmates for Elsie.

Over this past month since you left us I haven't stopped thinking about you, Poppy.

Every place I go I've thought about the last time I took you there. Completely mundane places like the supermarket and the gym have become makeshift shrines to the life I had before I lost you.

I miss you so much, my little Popsicle. My little Pop-n-fresh. My little Poppy noodle.

I miss the you from before the Rett syndrome snuck up on us and stole you away.

I will never not wonder, each and every day, what that day would have been like with you by my side.

Poppy's bath time, 10 June 2017

Cremation day

Dear Little Poppy,

Today is your cremation day.

It is a beautiful, clear, sunny day.

Your sister and I have spent the morning at gymnastics and swimming, and I've tried at once not to forget you and not to remember you too often.

I cry a lot.

We have chosen not to attend your cremation because your father and I both believe that once you're gone, you're gone. Because you were just a little baby, only eight months old, you did not have any friends to mourn you; so there is no funeral.

We will pick up your ashes and treasure them always as a reminder of you. I have chosen a beautiful urn to put them in. Soon we will have a party to celebrate your short life.

Poppy at 6 days old, 1 January 2017

You were my perfect miracle, Poppy. My sixth pregnancy, second child, and first natural conception to make it into this world.

We were all so excited to have you in our family.

Poppy and Elsie at home, 26 January 2017

Your father and I felt so smug to have managed to give Elsie a little sister barely two years her junior. We felt sure you would be great friends to each other.

Poppy and Elsie having a cuddle, 19 May 2017

I'm sure you would have been.

But you were born with a cruel time bomb ticking in your DNA; and now we are all in a weird, unenviable place where our perfect family of four is only three.

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.

Moar hospital!

Oh, Poppy. You are in hospital again.

Eight months and one day, Poppy at Monash Children's, 27 August 2017

You came home with us for about four days, caught a chest infection off your sister, got steadily worse, and had to come back to Monash Children's for oxygen and suction.

You've been in for the best part of a week, and you finally seem to be on the mend.

I'm torn between my love for you and wanting you to be around and wanting to see what 'new Poppy' is or will be like; and the fact that we've already decided and agreed with all the doctors that you should have minimal intervention.

Where does quality of life care cross the line into life-prolonging intervention? It seems to be the 'vibe' of the thing. I feel like we've laid out some pretty clear guidelines, though: no form of ventilation, and no resuscitation.

Hopefully you will be able to come home in the next few days.

I can see that we're going to need to make a concerted effort to get Elsie to cover her mouth and nose when she coughs and sneezes if we are to avoid yoyoing in and out of hospital for the rest of your life.

Not looking too good, Monash Children's, 24 August 2017

OMG, going home? WTF!

Poppy! Poppy! Poppy!

We are going home today.

Nine. Weeks. Later.

The longest, darkest, hardest nine weeks of my life.

That's saying a lot, because I've had some pretty awful times in the past.

Off-hand I can think of at least three solid contenders for Worst Thing That Ever Happened To Me before this; but that's not what this blog's about so I'll leave them in the past where they can slowly fade.

And so, 10 days after I packed our suitcases and chose your cremation outfit, I'm taking you back.

My little baby is coming home.

So shiny

So, it took a few days but we are back in hospital.

Damien is a very action-oriented person, so he found waiting around over the weekend and half way through a new week supremely frustrating. But you can;t rush the public hospital system.

The enforced separation was really beginning to wear on Elsie, too. She just wants her family all back together - and who can blame her?

As Damien pointed out, this has been the worst holiday ever.

Little cutie,16 August 2017

Anyway, now we're in the swanky new Monash Children's hospital. It's bright and light and clean, and every ward has playroom and a parents' lounge. There's even a daybed in the window of your room for me to sleep on if I need to stay in with you.

You've had an EEG today and there is now seizure activity visible that was not observed in any of your previous three EEGs. Developing seizures is something that happens to Rett girls. You're going to start on some anti-seizure medications.

Nice hat, Poppy. 17 August 2017

You've also had a visit from the occupational therapist. She's made you some cute little splints to wear, to stop your hands from becoming useless. They look like tiny sparring gloves!

Still with us!

OMG Poppy, you're still here!

We'll be heading back to hospital this week to inform a longer-term treatment plan, observe your lung function and check for seizure activity.

You have a new version of 'crazy eyes' where you just stare up into the corner of your eye and vacate your body for minutes on end. It goes anywhere from 15 to 45 minutes. Other than that, you seem pretty good - insofar as one can tell.

You've definitely gotten weirder over the past eight weeks. The Rett syndrome has done a real number on all of us.

Still with us! 13 August 2017

New normal?

We are still at VSK.

You're defying all advice, and simply refuse to quit.

Now that you're off sedation and don't have a ventilator tube in your face, I can really see the progression of the Rett syndrome.

You're not the same person at all.

Elsie and my parents are staying at our house while we are here. They just come for visits. We're in a kind of limbo.

You could be progressively shutting down, or you might muddle along like this until a lung infection compromises you and knocks you off your perch. Certainly your breathing rates are not normal.

In the next few days we will have to seriously consider bringing you home. VSK say we can stay as long as necessary, but we really need to go home if this is just the new normal.

Extra time

You're defying the predictions and best guesses of all your doctors and muddling along.

Your father and I are hunkered down at VSK with you, waiting to see what happens.

Last night we set our alarms to get up every two hours and check that you were still breathing. We felt sure you'd succumb to low respiration overnight.

Poppy and Daddy in the morning, 8 August 2017

For a little while this morning I imagined what it would be like to take you home with us and keep you; but this evening you're a very funny colour, and I've no doubt your lungs will carry you off sooner or later. If an overnight apneoa doesn't get you then you'll likely develop pneumonia. It was a chest infection that landed you in hospital all those weeks ago.

I wish that you were gone already. I want a peaceful end for you, not a slow decline into illness.

For the moment, we are all enjoying our extra Poppy cuddles and trying not to get ahead of ourselves with the what-ifs.

Poppy in the evening, 8 August 2017

The very worst day

Somehow I slept last night.

This morning we had to be at the hospital by 9:30 to be sure we wouldn't miss your move to VSK. When we arrived, you had a cute little bow in your hair. Your nurse had dressed you up for your excursion.

You looked so beautiful.

Poppy's last day in PICU, 7 August 2017

It was a tense wait for the patient transport to arrive. Your dad and me snapped at each other a bit, it was not our finest hour - but why should it be?

I feel like I have some understanding of what death row inmates must feel like. Everything I did this morning was for the last time. The last shower, the last express of breast milk, the last cup of coffee, my last trip up the hospital stair well ... you get the idea.

I can't tell you how many cots I've seen wheeled out to the waiting wards of the swanky new children's hospital across the way, and how every single time I wished it was us. Now we're taking what feels more like a 'walk of shame'.

After 7 weeks in PICU, we are to be released under the shittiest of circumstances.

You're on a trolley, hooked up to a mobile ventilator, with an intensive care doctor and nurse in tow and two patient transport people. You've been given some chloral hydrate for the ride but, even so, when the cold air hits you as we exit the hospital for the last time you scrunch up your nose and shake your head in objection. It is a bitter day.

I'm not sure how long the ride from Monash to VSK takes, because my mind is racing against everything that's happening.

When we arrive, we are greeted at the door and ushered through the hospice to the family accommodation. The heaters are on, so it's lovely and warm, and there's a hospital-style cot for you to rest in. The medical staff transfer you and all your life-giving equipment from the transport trolley and the drivers depart.

We settle down with the ICU doctor and nurse to wait for your sister and grandparents to arrive. I won't let them remove the tube until everyone is here.

Donating life

We've decided to donate your heart valves and pericardium, Little Poppins.

We would donate more, but there are no babies in need of donor organs right now (luckily).

Poppy, 3 August 2017

Your dad and me are both registered organ donors, and it just seems to us like some good should come of this terrible situation.

Poppy cuddles, 3 August 2017

Popsicles and fart bags

For someone who was with us for such a short time you had an impressive array of names:

Poppy, Poppy-pants, Popsicle, Pop-tart, Poppin-fresh, Popticle, Poppins, Pops, Pickle, Chuckles, Chicken, Noodles, Fat-fat, Fatty, Fat face, Fart bag, Farty-pants, Baby Poppy the baby.

Little Fat-fat, 18 May 2017

Very Special Kids

Today we went to see the place you'll die.

Very Special Kids will help with your final palliative care.

There is family accommodation there where we can all stay together, and they can provide family counselling and help with the logistics afterwards. It's an amazing service.

Once we remove the breathing tube, we expect to lose you within a matter of hours.

Diagnosis

Today the world caved in.

Your genetics results are back, and they reveal a mutation on the MECP2 gene.

You have a severe form of Rett syndrome.

I can't tell you how much we cried, little Poppy.

Rett syndrome is just horrible.

Infants and children with the disorder usually develop normally until approximately age 6 to 18 months. They then cease to acquire new skills and gradually or suddenly lose previously acquired abilities, such as conscious control of the hands and the ability to vocalise most sounds or words.

This diagnosis doesn't explain all your symptoms, but that really doesn't matter because Rett syndrome is degenerative and fatal and you definitely have it.

The prognosis for children with Rett is grim. Whatever else is wrong with you, Rett is the thing that breaks a mother's heart.

Those eyelashes, tho

Those eyelashes, 28 July 2017

Quality of life?

Poppy's playtime, 27 July 2017

I'm trying really hard to enrich your day with the time we spend together. You spend so much time just staring at the ceiling and it can't be good for you.

You've always been a bit passive, Popsicle; and in a way I'm glad you're not a crier or much of one to complain because I reckon you'd be breaking my heart after a month on your back in PICU.

One thing you really seem to like is music. I sing to you a lot, and we've got a Wiggles CD on high rotation that sends you off to sleep.

Today I got you up into a tumble chair to see if you'd enjoy a change of perspective sitting up for once. I don't think you really liked it much. You shook your head a lot and worried at the intubation tube. It's soul destroying to watch.

7 Months Old

Dear Little Poppy,

Today you are 7 months old.

I have to say that this is not what I expected or was hoping for. This is a shit show of negative experiences, right here: you, me and us.

I am sad and sorry for you, little Poppy, because we don't know what's wrong with you and we can't make it better. You hate the intubation tube that's helping you breathe and you hate being swaddled so that you can't pull it out.

You've always preferred to have your arms free and wave them all about.

You get really frustrated and shake your head from side to side a lot, which is obviously not great for the tube. It reminds me of a caged animal pacing. I just want you to be free.

Over the last couple of days I've taken to giving you arms-out-time. I have to stand over you and make sure you don't pull at the tube. You're pretty good, really. After a little while you settle down and just enjoy the freedom.

Poppy at 7 months, 26 July 2017

It's hard to separate all the bad things I'm feeling into discrete silos. The avalanche of worry I feel for you affects me viscerally. That sounds like bad teenage poetry, but it's the simple truth.

I worry most about your daily experience of the world. 24 hours a day in bed, mostly swaddled, staring at a hospital ward, with tubes and IV lines poking out of you and a constant hum of ward activity and monitor alarms is just an objectively awful way to pass your time.

Hovering over your bed for several hours each day to sing and talk to you is murder on my back, but it's not like I have a choice. It's the very least I can do for you, Poppy.

I love you so much, little Poppy.

If you could just start breathing on your own, that would be an enormous step forward. We could extubate you and you could get out of bed, and off the sedatives; and I could have my sweet, weird little baby back.

The one who never cries and can't really sit up like a 7 month old should; but who laughs and gurgles all the same.

Poppy at nearly 4 months, 21 April 2017

Nobody knows, it's a mystery!

Poppy, Poppy, Poppy ... what the fuck is wrong with you? Nobody has the slightest idea.

So far you have had an MRI on your brain, a heart ultrasound, two EEGs, several ECGs, and two lumbar punctures. They've taken so much blood that you got anaemic and needed a transfusion. Everything the doctors can think of has been tested, and nothing has yielded a result other than 'normal'.

While we wait for the big guns – an exome sequence, rushed through specially because you are so critically ill – your doctors are proceeding to treat you for Opsoclonus Myoclonus Syndrome (OMS).

Today is the third day you've been on extremely high dose steroids, and the first day in two weeks or so that I feel like you're here with me.

I haven't seen the crazy eyes at all today and you're quite serene.

I definitely think you're responding to this treatment. You're connecting with me.

It's beautiful and heartening, but also frightening because OMS is a life sentence. I can see myself giving up any thought of going back to work and instead devoting my time to home schooling you to protect you from any exposure to infections which might set you off.

Crazy Eyes

One of the things that's worried me about you since (almost) day one is the 'crazy eyes'.

When you were born, you were only 2.002kg and you spent the first four weeks of your life in the Special Care Nursery at Knox Private Hospital.

So tiny! Poppy and me, 27 December 2016

During that time I saw what I would come to think of as the 'crazy eyes'; a wild, chaotic rolling and flickering movement that went for 30 to 60 seconds several times a day.

At first I thought that you were just having trouble focussing your newborn eyes on the world around you. I figured you'd grow out of it. You didn't.

So that's one of the things you do, Poppy, that just ain't right.

I don't know how many people I've mentioned it or pointed it out to, but it wasn't until your health centre nurse saw it that anybody agreed with me that it might be super weird and maybe just a little bit worrisome.

She suggested I video the episodes to show to your paediatrician. Why didn't I think of that?

These videos have been instrumental in getting you the help you need. Every doctor I show them to is fascinated, but they've never seen anything like them before. They all agree that they have to be key to some diagnosis.

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.

Dear Baby

You've been in hospital for 28 days already; and I don't know when, or if, you'll ever come home.

We miss you.

Here is what we know so far:

You're crap at breathing.

On Friday night, 23 June 2017, I gave you your usual liquid iron supplement on the change table. Instead of choking on it or swallowing it, you just let it sit in the back of your throat while you turned blue from lack of oxygen. It's difficult to assess these things in the moment, but I think you stopped breathing for around 5 minutes.

Or maybe it was an eternity. It felt like an eternity.

When you finally spat out the liquid and started to breathe, you were not really with us for a good 3 minutes while you caught up with the whole concept of just being in this world.

By the time the ambo's arrived, you were a bit shocked but otherwise OK; or so we thought.

By the time I got to Maroondah hospital with you and waited in the hallway for an ED bed, you were actually smiling and laughing with me.

Poppy on the trolley at Maroondah Hospital ED, 23 June 2017.

I thought they'd monitor you for a bit and send you home. HA!

When we finally got an ED bed, they hooked you up to an oxygen monitor. You de-saturated frequently and continually. It became clear that you would not be coming home.

Maroondah wanted to transfer you to Box Hill for overnight observation but the people from PIPER said, "Hell to the no!" because Box Hill has no PICU. They took you to Monash Medical Centre around 2am on 24 June.

In hindsight, that was a VERY wise decision.