Monday, February 22, 2010

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

Sunday, February 21, 2010

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

Thursday, January 14, 2010

7 Weeks And Counting

It's been more than 7 weeks...
7 weeks and 4 days to be exact...
7 weeks and 4 days since I sat here...
And last journaled our journeys - mine and Ella's...

It was November 23, and I recounted...
The day we left BCCH after our 2nd stay...
The day in 2008 when our hope was big and grand...
As we prepared to take our baby home again...

That was 7 weeks and 4 days ago...
And so much has happened since...
7 weeks and 4 days of being back at work...
Even if more than half of that was only part-time...
A first Christmas... without Ella...
A first New Year... without Ella...
A brand new decade... without Ella...
A visit to Montreal...
My first since we took Ella...
To meet her families in October '08....

An emotional flight in, it was...
An entertaining flight back I had...
And quite a fun surprise for my familly I offered in between...

And such a blessing Ella gave me...
By showing me I needed to be with my family...
At this part of our journeys...

And then there is...
A larger bereavement group...
ELLA's bereavement group...
A fact I find so sad because I know...
I know the pain...
The pain that each parent of angels feels...
As they start down this dark journey...
They did not choose to be on...

And yet, it make me grateful...
That through Ella's journey...
Parents come together...
To support each other...

Then there is...
Another grouping of parents...
The X-linked PVNH group...
That Ella also brought together...

A group of parents so strong...
For their families are affected...
By that same rare disease...
That took Ella from us...
They chose to gather...
In one spot together...
To lend support...
And seek help...

And through it all...
I am learning...
Learning to take care of myself...
That I come first...

Back to yoga...
Added acunpunture...
And did I mention my Christmas Tree...
And Ella's Tree...
Are both still up...
Shining their brights lights...
Every night when I get home...

As I reflect on my last 7 weeks and 4 days...
And as I am told by my friends who've been by my side all along...
I know I am making progress...

At my pace...
On my terms...
As little...
Or as much...
As I can handle...

Honouring my daughter...
Thinking of new ways...
To pay it forward...
And living my life on purpose...
For as long as I live...

ELLA - Everyone Loves Little Angels




Thursday, December 24, 2009

Holiday Wishes & Snow Angels


Holiday Wishes...
And snow Angels...
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That was the message I shared...
With my family...
My friends...
With Ella's nurses...
And her doctors...

With my Facebook groups...
And all of my Facebook friends...
With people I met...
On this journey we've been on...

With all of you of have been...
There for me...
Day in... and out...
To cheer me onto a new day...

This was my Holiday Wish...
This is my new Year' goal...
And it's comprised...
Of all the lessons that Ella taught me...
Maman t'aime si fort mon coeur!

ELLA - Everyone Loves Little Angels


Monday, November 23, 2009

Grateful... Just Plain Grateful...


A year ago tomorrow will be the day that we took Ella home after a month-long stay at BCCH. The second of our three stays there with Ella...

A year ago tomorrow, I was fortunate to live again with my daughter at home for nearly two weeks... Though she was on oxygen therapy, she at least was home with me...

Throughout it all, through the last year, through the good days and the bad days, through the panic and the grief, I survived my ordeal because I felt loved and supported by my family: my mom, my dad, my sister and my brother, their spouses and my nephew and Goddaughter...

But beyond the love and support I received from my family is the other kind love and support I felt...

The love and support that comes from friends who without any expections in return sent my and Ella's way...

There were visits at BCCH, presents for Ella, an onion tart to feed her maman and other kinds of tasty goodies, calls to check in on us, Guinness smuggled in to our room on one of those days, messages and encouragements being sent on Facebook by mail and by email, donations made in Ella's memory, hundreds of cards and even ladybugs mementos... And through it all, love and faith carried Ella and I forward...

As I started my first day back at work in 15 months today, just like it was the case throughout Ella's illness and after her passing, I felt the love and support of my friends and family uplift me...

Since I decided I would attempt a return to work, there were more words of encouragements, emails, cheers and calls...
And as I headed to work today, more messages, cheers, calls, even flowers - pink of course and embraces came my way...

Since I came back from my first day's work tonight, there's been so much more love and support...

What else can I say but "thank you! / merci " to the hundreds of family members and friends, here in Vancouver, throughtout Canada, in the US and even overseas who have carried me, my grief, my broken heart and my angel forward on this journey back to life...

I am grateful... Just plain grateful... for your love and your support!

I could not be so strong without you having my back and complete confidence in me. And on behalf of Ella, my little angel who I know wants nothing more than for her maman to be happy again, I'll say it once more: "Merci! / Thank you!"

ELLA - Everyone Loves Little Angels

Friday, November 20, 2009

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

Wednesday, November 18, 2009

Memorial, Lanterns and Mischief

On November 8, 2009, a day after Ella's 15-month birthday was a very special event. The kind of event one would pray to never be invited to, if it even crossed their mind as they became a parent... As only a select few do get invited... or so it seemed to me in March…

But the reality of it is there are a lot more select “us" out there than one might think. Those "us" are bereaved parents... And the event was the Remembering Our Children event...

Organized by Canuck Place volunteers, the event consisted of a brief, joyful yet intense memorial service followed by a lantern-lit walk at Canuck Place. And here is how it played for me that night...

I showed up to the memorial service by myself.... my support friend not being able to join me after all that evening because life was simply taking over that day… (no criticism here, I understand how life goes, believe me!).

Fortunately, I thought, I had lined up another support friend. And there was still a chance that Ella's dad might show up; as he said he would come...

Well, as luck would have it, my second support friend was late and Ella's dad did not show up.... And because I sat in the 2nd row, toward the corner of the room - in front of the musicians - I could not easily be reached by my friend who showed up late... Silly, I know. And I won't make that mistake again...

So, as it happened, my friend Linda, chaplain at BCCH and my family for this special event sat in the back - and though I looked for her, I just could not see her... I was actually looking back right over her head, not realizing she was there... It was kind of funny actually, in retrospect, as I must have turned around 50 times, out of fear, desperation and reflex - all thrown into one....

Why fear and desperation? Because I was alone... Well, not really... but yeah, so I thought... in the moment…

While I was surrounded by fellow angel parents and their families, most of whom I know from the Canuck Place bereavement group, I was the only one unaccompanied… (remember I thought Linda was a no show)... And while a few of those parents and I spoke, exchanged stories, even laughed and one offered for me to sit with her and her husband before the ceremony started, I stayed where I was with an empty seat next to me, praying that Linda would show up...

But back to the fear and desperation...

It began a few minutes before the actual service started… Those who know me know how much music is an intrinsic part of my life… And it became clear that I was going to lose it as two guitarists started playing and singing a Beatles song …

But I tried my best not to lose it…

By the time the service started, tears were rolling off my cheeks like a cascade…
And as Eric, chaplain of Canuck Place, spoke I was already a complete mush…

I don’t know if it hit me so hard because Ella’s passing is still very raw… Or because I was by myself… Or because it was an opportunity to take in the enormity of it all… something I did not really do at Ella’s funeral because I was in “I must keep it together” mode that day…

All I know is that the service, as beautiful as it was and as serene as it was meant to be, hit me real hard… It must have done something similar to a few moms around me as we glanced at each others’ in understanding with tearful eyes...

There are 3 moments in the service that stand in my mind…

The first? A poem that Eric read, which upon hearing it, sent me even further into a spin… It’s called “We remember you” and though Eric adapted it to speak of many children whose families were present; it spoke to me and me alone at that moment as it had when it was read at Ella’s funeral on April 4, 2009 by, if I recall correctly, her Godfather Will…

The second is how a member of each family was offered the opportunity to light a candle during the ceremony and place it into a sandbox (which for me was the perfect metaphor – playful and exactly as children’s memories and souls should be) and we then mentioned their name out to the group… I got through it and that very candle is on my mantle now, surrounded by pictures of Ella’s and moulds of her little hands and feet…

The third came at the very end when Kathryn, a clinical counsellor, invited everyone to shine to the sky a little gadget that beans a ray of light as the group sang “Twinkle Twinkle Little Star”… And right there, I lost it again…

You see, Ella fell asleep every night at BCCH on that very music… And as if that was not enough, that same melody is that of a song I sang to her from the day I knew I was pregnant “Ah-vous-dirais-je Maman”, to soother her - and me… To the very day she died… Every single day… And even now…

By the time we’d sang the song and the service ended, I was physically and emotionally drained, having cried all the tears in my body…

And that’s when Ella first interfered!

Linda, my friend and my family was “revealed” to me… She gave me a huge hug and all the fears and despair were lifted… But that was only the beginning…

Linda and I now headed, as did all the families, back to Canuck Place. It had rained all day. The ground was wet, it was a dark night and the air was crisp… But that did not matter, as Canuck Place was festive…

Not festive as in decked out for Christmas… But festive in a very solemn, yet very bright and beautiful way… Oh, and the rained had stopped!

There seemed to be 100s of lanterns on the garden grounds…. I actually don’t know how many there were and did not dare to ask as the answer would have daunting – each lantern representing the soul of a child taken too soon to become an angel…

The staff of Canuck Place welcomed us, offered us some hot cider (a cheerful treat on such a cool Fall evening) and then sent us to walk around the gardens to find the lantern which belonged to our child…

As Linda and I started exploring, looking at every single lantern’s name to find Ella’s, we quickly commented how amazingly beautiful it all was, and yet how tragic it was… to see sooooo many lanterns – each for a child who’d died…

As we diligently went about checking each lantern, I commented on the fact that in true Ella style, she’ll make sure that we find her last… And as we came across one lantern which had run out of candle and we got that fixed, Linda thought to herself, something wonky will happen… And that feeling intensified, I later found out, as we came closer to where we’d started…

After a very thorough tour around the Canuck Place gardens, my heart started to sink… I had not found Ella’s lantern…

So, we made our way to the volunteer who knew where each lantern was placed and asked where Ella’s was…

Can you believe that my darling, sweet girl’s lantern was actually at the starting/ending point of our tour – right where Linda and I actually started looking at names and where I told Linda that we would find Ella on the last lantern??? But again, in true Ella style, there was a twist…

A twist I attribute to my mischievous angel… J

Ella’s lantern was not lit… And as we both came to the realization of it - Linda and I just cracked up giggling because we just knew that Ella had been by our side all along, waiting anxiously for us to get to the end / the start and laughing as she got passed us to go “Look Maman - Pffft, pffft, pffft” to blow out the candle with her now healthy lungs!!!

We continued to giggle after lighting the candle as we walked to the new waterfall feature in the gardens to place a memorial rock in the water on which is inscribed “Ella Dupont Bedassie, Love You, Maman”. Not every inspired, I know… I guess my brain was fried from all the tears and giggles!

And then Linda and I both headed home, all the while still laughing and amazed by the turn of events that night…

As I returned to Canuck Place last evening to attend my bereavement group, I went back to the waterfall… And right there, just as I’d left it over a week ago, under the light that beams on it so intently is Ella’s rock! And it is surrounded by all the rocks angel parents placed in memory of their darling child…. just like I know those little angels surround each other up there in Heaven and cheer us on as we attempt to honour them as best as we can while they continue to play tricks on us to keep us on our toes…

ELLA – Everyone Loves Little Angels