Sunday, February 26, 2012

News spot on Global News

We had a TV crew in on Friday doing a small piece on Raquel.
She was a super star and was amazing for the camera.
Cordelia, I feared would steal the show.  As soon as the tripod was secure and camera on top, she was in front of Raqu blocking her with her big blue eyes staring squarely into the lens.
The camera man then had to give her a little speech about what is supposed to happen with the camera,
but ummmmm now to think of it, I think I stared at it a few times.  Cordelia, got it anyhow, after the not so idle threat of sending her off somewhere outside in the rain by herself.  Clearly the camera man had similar concepts on Crime and Punishment as I.  Raquel was excellent if not a made for TV personality.  I guess I should hold up gushing about my dearest till the piece comes out.   She did have a nice sized smudge from a fall on my mothers carpet that I still haven't apologized to my mom for losing it over.

I'll do it in person if she ever comes back from her work holiday from Grumpa.

For the camera
We did a few of our natural days activities - playing, drawing, then had a small hands free time which are becoming more of our routine.  Then we had a quick hand dressing moment to get those hands secure, again Raquel was perfect.  She protested for a moment or two, but Cordelia was there to side track Raquel's attention so that a movie wasn't required for background distraction.
Our nurse, whom may also star in this little short helped out from the outlying areas till she was required to hold Raquel's arm at this point.  If she does get filmed then I'll have to pull in her first name to these posts. 

Afterward I was interviewed and hopefully didn't stutter too much.  I know I pulled a few big blanks forgetting what I was talking about and probably droned on in some other abstract departure talking in circles.  This only occurred to me after I was asked a question and I spoke for a few minutes on the subject, then the question was posed again in the same manner.

I flushed and had no idea I was speaking in the wrong direction what so ever.
Then I tried to speak of DEBRA Canada and did very poorly.  I think by that time I'd lost my confidence.

I'm hoping the editing will be good.  For those of you (Simona, Dammit Janet & Mike N) that I pick on in many occasions, feel free to go nuts on this one.

I've been told it should air on Monday and even saw a preview on the website for the 6 o'clock news titled 'Meet a little girl with a rare genetic disorder'.

I'm starting to realize disorder is a better word than disease.
The later sounds contagious and far more scary at first blush.

I do need to thank Erin again.
Her article started this ball rolling.
Now there is a great push for EB awareness in the span of just over a week.

This is part of Raquel's crash the day before the camera crew came.

Never mind what her knee looks like.

It's been a freaking mess.
Thank science for Mepitel.
I did a poor job dressing it the first day and just moments before the camera came by when doing a diaper change we tore off a huge piece of skin and the dripping scab that healed right into the fabric holes of the silks.  That's never happened, we've had subtle sticking, but nothing a little warm saline water didn't fix quickly.  Which is a testament to how freaking tough this kid is.

Above is the knee when it was fresh.  Four days later it's certainly not looking any less painful.  Let alone what it looked like when we pulled off some of that skin.
One can see I've got a very firm grip on her leg below the knee and Ren is pushing down the other knee with considerable force.
Raquel was pissed!!!!
I finally figured out how to bandage it.
Three strips of Mepitel that go right across her knee layered like some of my mountain bike armour.
It was a funny little inner dialogue I had figuring that out and what I have for my knees when falling 10' off a rocky or wooden feature.

Which reminds me
Even after all this commotion I still have this to recharge for Vampire Soup.
It's been snowing like crazy in our mountains of late.
This is what it looks like for the non professional skier who misjudges slope, speed, trajectory, obstacles, snow depth, and a camera man. 

I'm still not sure how my whole body is so neatly squished behind that exploding shower of debris I so ungracefully sent onto Tom and his camera.
But to think, we climb and ski mountains in treacherous conditions only during the day....
I was trying to explain to Elena that if the hole by the cute little tree sticking up a foot out of the snow behind her goes down the near 4+ meters of depth we are standing on, Tom and my shovels aren't likely as quickly assembled as she thinks or would hope.

ahh a momentary exit from EB.
Even looking back at the photos of the week.

Can't wait to see how Raquel will adapt to all this.
Of course by then I'll probably be able to explain to Renata better why we "NEED" a family snowmobile...


  1. I just saw your story on the news. I am not sure it is the same as my cousin's condition, but I suspect that she too has EB. I was always told that the name of her condition was "Blister Baby". I do know that I have never touched her skin; if she is not used to your touch then she will instantly blister. Her childhood was similar; the blanket she was wrapped in as a baby would take the skin right off; and she was usually bandaged. She is not a blood relative; my grandmother and grandfather were widowers that married when I was 2 and it is my step-grandfather's niece, but obviously I have known her most of my life.

    My reason for stopping by; to congratulate you on your determination and to give you some glimpse of the future. My cousin is a few years older than me, which would put her in her late forties to early fifties. When she was born her parents were told to prepare for her to pass; at the time no blister baby had ever lived past I believe 2 years. But she is now an adult with a career at the local hospital and has been married for almost 20 years. Yes, people she does not know still cannot touch her. She wears jeans every day because her body has formed a permanent scar ring around the waist where the jean waist touches and now it no longer blisters her (other pant materials would). But she leads a fairly normal life; one she was never supposed to have, and I am confident that what has been learned from her and others after her will lead to a better life than hers for your daughter. So while it may seem there is little information with which your doctors to work; it is more than in the past. :)

    1. Yup, I get that most certainly, bandages and care have vastly improved, but we're only barely out of the dark ages in EB.
      My best to your cousin.
      Thank you for your comment.

  2. I saw your story on the news tonight. Raquel is such a sweetheart. What a strong and brave little girl. Hopes and prayers to your family that they can discover a cure for this nasty, painful disorder.