Countdown Has Begun

It's official, the coundown has begun...
And I can't do anything about it...
Not, one single thing...

Time won't stop...
No matter how clever I think...
I can be at willing it...

The countdown was always there...
Ticking away...

But with the shift from Feb. 28...
To March 1...
It has stuck me...
As the most cruel...

With all of us now in March...
I can't escape it...

Every day is a stronger reminder...
Than the next...
On what's to come on March 27...

Every day is full of memories...
Some that were hopeful...
And then the reality of the events...
Hits me right in the face...

And as if that was not enough...
It is a constant reminder...
Of what I wanted to do...
But could not do...
To honour Ella yet...

A long list await...
But life gets in the way...

Life for now equates with work...
The place where although I know it helps...
I can't focus on my daughter as much as I'd like...

The place where I started having panick attacks again...
The place where I find myself in tears, more and more daily...
Not because someone is mean...
Not because of the work load...
But simply because the countdown is on...

The place, like the rest of the world, where everyone has a life...
A real life..
Although maybe not always happy...
A life without the black cloud...

A place that I must be at...
Because otherwise I won't be able...
To keep my home, Ella's home...

Rare Disease Day went un-noticed...
The Olympics celebrations nearly had the best of me...
Not because I was out partying and enjoying it...
But rather because I cannot celebrate life...
The way that it would have been celebrated...
If Ella was with me physically still...

The countdown has begun...
And I can't do anything about it...

ELLA - Everyone Loves Little Angels

The Faces of PVNH: Ella

So, now we know, It's called PVNH...
But really, what does that mean?

First, a name...
Periventricular Nodular Heterotopia...

Then, a lesson in medicine...
A rare disease characterised by some neurons taking a wrong turn and not ending in the proper location in the brain, causing some havoc along the way...

Passed on from mother to child, it is thought to be lethal early for males (future posts will show you this theory does not hold true anymore)....

This condition, like so many of the nearly 8,000 rare diseases identified, can also happen spontaneously - that is with no other family link found...

In medical litterature, PVNH is described as a disease associated with a mutation in the gene which produces a protein called Filamin A (FLNA), a binding protein....

To this day, our genetic team tells me that Ella's case was actually not a mutation but near complete or complete deletion of the gene...

This, as far as they know, has never been documented to date...

But I told you Ella was a zebra... And zebras are no horses...

So in true Ella fashion... Ella's symptoms did not really reflect the typical symptoms expected with this disease...

No delay... No seizures... No hyperflexible joints...

But there was a cardiac defect, something which helps put weigth on the PVNH diagnosis... And then, the neuronal migration defect was there too...

But the biggest symptom Ella had...
Cannot be explained clearly and definitely by PVNH...

To this day, it is impossible to confirm...
That Ella's PVNH caused her end-stage emphysema on both lungs...

And that, is the reality of rare diseases...

While I am blessed to have a diagnosis...
A luxury many families affected by rare diseases are not granted...
I continue to search for answers....

Answers on what happened to Ella...
Answers on what it means for my family...
The one Ella and I have...
And the one I am wishing to grow in her memory...

ELLA - Everyone Loves Little Angels

Ella The Zebra

Since October 2008, we've known...
Known that Ella was a zebra...
A little girl like no other...
Full of life and bright as the sun...

And though we tried our best...
We could not...
While she was alive...
Find out what was causing her illness...

And so, Ella lived...
On oxygen...
In the hospital...
For nearly 6 out of her 8 months of life...

And what a life!

Almost always a perfect smile...
Playful and loving...
Full of character...

Looking so happy...
And healthy on the outside...
You'd never think...
Her little lungs were destroyed...

But every once in a while...
There was a flash of fear...
Usually around the time...
She would start to truggle to breathe...

And so we learned to read the signs...
And manage the symptoms...
Of Ella the Zebra...
So she could live her life...
For as long as she could...

And though Ella lost the battle...
Her fight did not end...
On the contrary...
It is now stronger...
Stronger than ever...
Carried by her so large spririt...

It took a year...
To get to the elusive diagnosis...
But the one question remains...
Is this the only one...
The diagnosis that caused it all..

An with that...
the work is laid out...
For us, Ella's army...
To change the face...
Of rare diseases...
Starting with...
The one answer we have...
Called PVNH...

Ella - Everyone Loves Littles Angels

One step at a time...

On November 16, 2009 I should have been celebrating the beginning of my 5th year in Vancouver...

On November 16, 2009 I should have been able to tuck my daughter in, sing her gently to sleep and tell her how much I love her...

On November 16, 2009 I should have been able to hear my daughter say "Je t'aime" as she trailed into dreamland...

Instead, on November 16, 2009 I was faced with the gross realization that it had been 7 months and 20 days since my daughter passed away in my arms...

And that the next day, she'll have been an angel longer than she's been alive by my side...

What a way to start a week, ugh?

But as I reflect on that day and those thoughts more specifically, I know that as this 5th year in Vancouver starts there is a wind of change that lifts me up. A wind that is powered by two very small lungs... Two very healthy lungs that belong to one hell of an angel... Angel Ella, my baby girl!

She is the force that makes me get up and breathe...

She is the strength that allows me to feel, feel with every ounce of my body... something I was probably unable to do very well before she came into my life....

She heightens my sense of smell... She is the spring in my step... and the song in my heart... The pupil of my eyes...

She brings me to tears and always follows through with a smile or a good giggle...

Without Ella, I might have gone on to live my life as uneventfully as I did before...

Without Ella, I might have not paid it forward as much...

Without Ella, I might not have really known what it was like to live and to really love....

And as I go about my day, there is not one moment that she is not on my mind..

I can still sense Ella's touch on my cheeks as she reaches for my hair...

I can still smell her baby smell...

I can still see every roll of her body as she dances playfully...

I can still feel the slight tug of my hair as she twists it over and over around her little finger...

And who could forget about "le doigt d'honneur" - that middle finger that would pop up on one hand as the other would give you a piece sign... F you and peace all at once - what a diplomat she was :)

I so wish I could have it all back, just for one more minute... to hold her... to sing to her... to kiss her... to tell her I love her...

That won't be possible until I meet her in afterlife, I know... And so I've decided that I would continue to do what I've been doing in the last nearly eight months and which has been working quite well so far: live knowing that Ella is always with me...

I know... you probably think I've lost my marbles... But why not?

Ella got me back on a horse after 4 years away... Afterall, that was supposed to be "our" thing.

Ella got me to start yoga... Okay, that one is not as much her as what happened to her. I just needed to something to help ground me. But I know she gets me through it!

Ella showed me pink glittery gum boots I had to buy last week, a couple of days after I went looking for puddles to play in (and did play in) because if Ella had been alive, that's what we would do together...

As Ella would have been 16 months in 2 weeks, we would be at the stage of doing crafts together and now thanks to her, I have a desire to be more creative - something from deep inside me that I am rediscovering... All in her memory but also because if Ella was with me, that's what we would do...

But in her passing, Ella gave me a beautiful gift... The gift of our lives together and our story... and though it is indeed a tragic story, it is a story to which I would make no changes except to bring her back...

Today, I was able to share that story with a room full of colleagues as I prepare to return to work on Monday... Some I know very well and some I'd never met... And just like it has been the case since Ella's passing, she continues to touch people... Or is it that we, together as mother and daughter, touch people...

Someone told me yesterday that Ella and I have a very special gift to share....

Someone told me last month that at 35 his life is not nearly as full and fulfilling as Ella's life has been and that he is inspired by her and how she continues to motivate people to be better individuals...

As I embark on new adventures, Ella will be with me just as she was today when I faced my fears head on. It will not be an easy road... It will not be a steady road... But with a rock-solid little girl like Ella with me, there is absolutely no way I can get lost...

ELLA - Everyone Loves Little Angels

7 months and 20 days...

7 months and 20 days....
Not what you’d normally call…
A life time...
But it is…
It is…
Ella's life time...

7 months and 20 days...
2 months of which were "healthy"...
And well, the rest you now figure out...

And yet, Ella was "healthy"!
As healthy as you can be…
When your lungs are poisoning you...Definitely so ill inside...
But remarkably so healthy-looking outside...
And in her mind…

Although Ella had end-stage emphysema…
Before she was even 3 months old...
You could definitely not tell...

Ella has a sparkle in her eye…
Ella has a grin on her face…
Ella has an intent way of looking right through you…
And captures your heart…

Feet up…
Hands waiving…
Ella dances to the music that plays around her…
And to the songs that drum in her head…

With King Louie…
With Pinky…
With Mula Visa…
Grand-Maman…
And Grandma…
With Maman..,
And Daddy…
Always with Ralph looking out for her…

Mischievously playful…
Peacefully restful…
From frail to plump…
Holding on to Daddy’s finger…
Smiling for Maman…
Ella grew…

And as Ella grew…
Her lungs got worse…
But you could hardly tell…

From the day she was born…
To the day she died…
Ella lived a full life…
The way Ella intended to…

Announcing herself as a surprise…
Keeping me on my toes and off my feet…
Peeking her head ahead of time…
Taking Maman for a ride…
Ella lived…

Ella went to a pub 18 years before it was even legal for her to do so...
Ella was on Facebook when kids 10 years her senior are not allowed...
Ella travelled across the country to meet her families…
Ella went to work… (Maman’s workplace that it…)

Ella was a die-hard football fan (go 49ers, go Dolphins!)…
Ella was a Canucks lover…
And Ella roared fiercely for the Lions…

Ella attended an LGBT travel show…
Where she was the star…
With the Dallas Cowboys (the shirtless kind!)…

Ella was a supermodel in Montreal…
Posing for an indoor photo shoot complete with lighting, talent and 3 photographers…

Ella was a supermodel in Vancouver…
Subjected to her mom’s kid Kodak reflexes…
And immortalized by friends, nurses and Jane from Now I Lay Me Down to Sleep…

Ella went to the beach…
And Ella ate out at restaurants…
Ella was touched by the rain…
And thanks to Michele, one of her nurses, also by snow…

Ella went to birthday parties…
And collected stuffies…
Ella loved watching Stewie…

Ella breastfed…
And she held her own bottle…
Ella grinned as she lay on her sleeping Daddy’s stomach…
And Ella played rhymes with her Grand-Maman…

Ella had a first love, beside her dad: nurse Ryan…
And Ella had a beau (sweet Gavin)…
A best girl, Emma, who visited her so often…
And Ella learned to count…

Ella celebrated Halloween…
And Thanksgiving…
Christmas… and New Year…
Hawaiian Day…
And Daddy’s birthday…
But was sadly two days short of wishing Maman Happy Birthday…

Although she’d learned to say "Maman" – a precious gift that came as she was about to leave.

Ella was baptized…
And Ella was proclaimed a Saint…
By the father who presided at her funerals…

Ella felt the wind…
And the love of a thousand friends…
From far and wide…
Who 7 months and 20 days ago…
Had their hearts ripped out…
When Ella became an Angel…

And as I started the day…

The 7 month and 21st day....
The day when my daughter has been an angel longer than she’d been by my side…
I remembered the words of a very wise nurse…
Who believes like I do…
That we are all put here for a reason…
And that we all follow our own path…

Ella was and is beautiful…

As she continues to inspire me…
My family…
My friends…
No matter how long our journeys are…

7 months and 20 days is a life time…
7 months and 20 days is Ella’s life time…
As long as I live and honour her…
Ella’s life time will go on…

ELLA - Everyone Loves Little Angels

Ella's First Crush

I originally wrote this text a couple of weeks ago to add in to the series of moments in Ella's life I docu- mented for BCCH Supercommunity ELLA - Everyone Loves Littles Angels. It should have been added yesterday, in place of Ella's First Halloween which I should have written today. Oh well, can't dictate a mother's heart and where the inspiration comes from right? Oh, and I've made slight additions to it too :)

So here it is.

It was October 30, 2008 and Ella had just been re-admitted to BCCH for the second time the day before. By this time, we knew Ella's condition was serious enough that she required oxygen therapy. We were on 3M, in quiet a corner room. And on that day, as I rocked my daughter, we met nurse Ryan.

Ryan was our third male nurse. Chris had been our ongoing nurse extraordinaire when Ella was born at BC Women's. And then on Ella's first hospital stay, we were fortunate to be cared for by Mark on 3R. But as we started this stay at BCCH, I noticed something in my baby girl's eyes when Ryan entered the room.... It was as though Ella had a glimmer in her eyes :)

It did not take long for both Ella's dad and I to understand that the glimmer was actually Ella's way of telling us she had a crush on Ryan. She would become so focused on him as he cared for her, and would be so playful, even more so than with other nurses. And she would just have that happy look every time he was around. We were blessed to have Ryan look after us (I say us because I was staying with Ella 24 hours/7) and Ryan was of such great help to me throughout this difficult time.

Fast-forward a month and a half and this time, we have been readmitted in to BCCH, and have already been transfered down to the ICU because Ella's state has greatly deteriorated. I am walking back to 3F where Ella had spent two nights to update the staff on Ella's state and I ran into Ryan. Upon hearing of Ella's return, he promised to stop by to see her. And sure enough, at the end of his shift, Ella received her first visitor in the ICU: her nurse Ryan. Although Ella was pretty sedated, I know in my heart she was aware Ryan was there to say hi.

And as we started what would be a very long stay in ICU, I thought my baby girl was so blessed to have a first love, besides Maman and Daddy, that loves her right back!

I am thinking of you today Ryan. And of all the wonderful caregivers that spend their life looking after other people's children in hope of making them better. And though sometimes, they can't do that as much as they'd like because life takes over, you always manage to make it easier on us, parents, and our little ones too.

Thank you - from Ella, from her dad, from me and from every single parent out there who's had a child spend a scary day/night in a hospital! With all my love and gratitude.

ELLA - Everyone Loves Little Angels

I thought....

I thought I was doing so well...
Going about each day, honouring you as best as I could...

I thought I was about to start a new chapter...
Meeting new friends and focusing on fresh interests....
That is until reality struck me right in the face...
And I found myself unable to face it...

I tought I could take it...
But instead I took the back door and left like a thief....
Ashamed of my feelings of even caring for what was once...
And the fact that it was all a scam...

I thought someone had my back...
And realized that no one but me can actually look out for me...

I thought I was wiser...
But now I know that a lesson in friendship.....
A lesson in love....
A lesson in life...
Can show itself at any time of day...
Whether you are ready for it or not...

What a difference a year makes....

As I start to type, it occurs to me that a year ago, at this very moment (12:07am), I was fast asleep on the futon, with a plastic nap under the sheet, in case my water broke. I'd just been induced that morning with the assurance from my OB that my baby girl would take at least 48 hours to show up. I'd been having what I thought were Braxton Hicks that evening after I had spent the day at the beach with my girlfriend Mireille. I was crampy but I was feeling fine.

NOT!!!

My water broke at 12:20 am (thanks to all the good advice I got from friends for saving my futon!), and soon, the whirlwind started: call daddy to be who was still in Whistler celebrating a friend's wedding (thank goodness for voicemail and text messaging!), call the doula to say "what do I do?", call the hospital to see if I should come in, grab my bag and drive myself to BC Women's Hospital (the Oak/Broadway street contraction is the only one I remember...). Park the car, make my way in to assessment room, meet my doula there (1am), double over while daddy to be finally calls to say he's on his way and then all I remember is my doula showing me how to push away the pain; how I am in pain; how much I want drugs, and how much it hurts so I end up actually puking a couple of times from the pain! (at 1:38am with contractions every 3 minutes) What a mom won't do for her girl :)

By 3:10am: 100% effaced and baby's head is ready to come out. Nurse Rose has been great. And soon Nurse Kelsey takes over. But not without the supervision of our doula Jacquie. By 3:35am, daddy to be finally shows up... I am too much in pain to actually yell at him for not being here sooner. It's time for the epidural, thank you very much! (4am).

It won't be long before I actually realize that everything I've planned is going out the window. The computer with my push, relax and "ye, she is coming and is here"music mix is at home and not in the car where it was before my doc told me I had at least 48 hours... The camcorder, the battery candle that looks so real, the camera... all in the car... But in the midst of it all, I managed to exclaim "I'm a supestar!!!" (note that the "r" is missing for a reason - great drugs!)

By 5am, I've convinced Chris to get the camera, and as Dr. Kenyon comes it's finally time to push. It seemed to be many pushes but according to Doula Jacquie's notes, 3-4 pushes was all it took. I'm told it was all very smooth.

At 5:47am, Ella Jacqueline Dupont Bedassie came into this world. What a precious little girl. Our miracle!

She's put on my tummy and quickly, Chris cuts the cord, the doc takes her away to do their stuff for a few minutes, then she's back with me and Chris when she just grabs on to his fingers. It was the first of so many moments where our darling little Ella grabs on to her dad. A moment we managed to immortalize in so many pictures and with a LifeCast statue.

I apparently said "I can't believe it was that easy!". And I remember saying to the staff and our doula who wanted to know her name that Ella was "named for one of the most famous jazz singer: Ella Fitzgerald".

Ella Dupont Jacqueline Bedassie weighs 5 lbs 7 oz, measures 18.9 inches and sports a full head of dark hair, just as I did when I was born. What a wonderful start for yor precious life!

After all the excitement, dad set up his cot and quickly passed out, snoring as if he'd done all the work! And mom concentrates on breastfeeding little Ella. By mid-morning, we were transferred to Balsam where our first nurse Chris would take such special care of the 3 of us. He turned out to be our nurse for 3 out of the 5 days we spent at Women's Hospital.

There will be many more tales to come as I journey the first days, weeks and months of our life with Ella. But in the meantime, allow me to say:

"Happy Birthday my darling Ella. Maman and Daddy love you to infinity and beyond, forever and always!"


Maman

Children know best

As I sit tonight and reflect on the last four months, I can't believe how quickly time flies and how badly I want to find a time machine to go back in time...

I very well know I can't do that and so I ever so gently move forward in my journey, counting my blessings for every beautiful thought and memory of Ella, so happy that she got to meet her paternal and maternal grandparents, her cousins and her aunt. I now strive to see the world for her. 1 step forward, 3 steps back is how it feel but it's probably more like 1 step forward, 2 steps back.

On the evening of Ella's passing, after spending the entire day with my baby girl in my arms saying my goodbye, giving her one last bath, making moulds of her hands and feet, touching her cheeks, stroking her hair and dancing with her so that I never ever forget the feeling of her in my arms, I was fortunate to hold a very special boy who beat all the odds. We'll call him baby A, a twin who before he was born showed a very serious heart condition to his parents and medical team which required open heart surgery days after he stepped out into this world.

Baby A was back for a visit in Vancouver in March and our paths crossed as it had happened when his mom and I spent time together during our pregnancy. This time though, I knew he was not out of the woods and held on to him as I held on to Ella in those last few months. With the hope that all would be okay, with the fear that life could always take a turn we would not expect.

I always felt that baby A knew exactly how I felt that night, and what had happened to my baby girl who hours earlier had earned her wings and went to sit at the BIG table. Baby A not only got me through that evening without me feeling any panic and distress, baby A who I still see and hold every once and a while, gets me through the current pain of outliving my daughter. It's like he knows that I am a mommy with a broken heart and he protects me.

I like to think that Ella makes sure both of us are protected by her brave heart and soul. Baby A is doing well, and though he is smaller than his brother, he definitely has a force, a wiseness already present beyond his almost one year.

Thank you baby A for showing me it's ok to hold a baby and not feel guilty. Thank you Ella for allowing me those few precious moments of peacefulness and for making me want to make every day count.