Wednesday, November 28, 2012

Back to Earth

Raquel has finally come back to earth, but not before some constipation.
She's had her second and third solid poops in two years and the only place I've never seen blood emerge from her body has now been checked from the list.  I don't think it's serious, but just shocking.  It's like her hands, eyes and throat.  It's one of the four major places I do not want to see or know there are any blisters and scaring for the apparent RDEB debilitating side effects.  So much so that I've based her care philosophies at avoiding them at virtually all costs.

We went to see our Ophthalmologist yesterday at Children's.  Before hand I threw out a pile of emails to Doctors and fellow Debra Board members for the presentation slides from the International Congress.  They haven't been compiled into a web version as our President and other board members are all volunteers and the time it takes to undertake is rather consuming.
Next to the wound speech by a Colorado Dr. the eye presentation was by far the most interesting and informative.  So it was very relieving I was able to have much of it's contents in hand with my notes taken during the Congress to ask questions for the present and be ready for the future.  Of course there are many avenues to an end.  It's just very important from my view to be aware and not purely be pushed by the flow.  Direct it where necessary and feasible, but if pushed - swim.

I have to thank everyone who helped get this information so quickly.  Amazing!!!

On a side note.
Things have settled that I've been able to enjoy a little hike or two.
It just sucks that we live so far north and darkness comes so fast in winter and the only option before the snow comes in earnest is to hike in pitch black in the forest with all the spooky sounds and critters jumping from tree to tree.

We're good, but

We're good, but I have been reading and living heavy via Kourtney's blog.
I trust she's getting some serious rest now so that she can build some strength and get healthy.


Keep up the fight Kourtney!  We're behind you and sending our strength of will.
I hope somehow that can be captured by you and used for good health.
I also hope we can come for a visit in the near future.

Monday, November 19, 2012


Raqu has finally started to act more like herself.
The flailing arms and screams that she only wants "mommy" have subsided to every few hours.
I seem to only get a few moments of joy and play till the bubonic plague Ren had catches up to her system and she becomes crusty and cranky again.
In those few moments of clarity or mild drug induced decent behavior, Raquel is surprisingly quite charming.  So much so, she finally asked if she could go out with me. Only me....
Like a date?  With her Daddy?
Of course!!!
Since she hasn't quite gotten to the age where she likes the early morning Sunday excursions to play cards eat muffins as Coco and I do, I was more than happy to figure out a new excursion Raqu and I could do.
So why not head to the wine bar?
Minors accompanied by and adult can have a small glass of wine.  With everything else that goes through her body, wine is by far the least of my concerns.
Plus, it's very likely by the time she's 19 her mouth will be so full of scars and tongue virtually already void of taste buds she'll hardly have the chance to appreciate the complex flavors wine can provide.  So, why not start now, at 2.

We went down to the The Republic's Servant ordered a 1/2 litre of an Argentinian Malbec (she's already has a larger appreciation for the full bodied reds than any white), we started with a taco chips and fruit salsa, then some crispy calamari, the main course was Duck and roast potato with a beautiful assortment of seasonal vegetables - considering we live in the near tundra and it's nearly December I'm pretty sure it was California's endless growing season which was referred to.  For desert we had Creme brule.  By this time Raqu was in heaven.  Half drunk and nearly satiated she enjoyed cracking the glassed caramel with the ridge edge of her spoon.  I'd taken off her mittens and bandages before we left so she was able to pick up the shards and crispy calamari with ease and crunch them with every flavorful bite.
It was amazing living in the moment,
The peace and fun was broken from loud cries echoing in the distance as the subconscious traded out mindful fancy for the inevitable reality of life.  The shrieking voice was that of a child, grumpy and upset calling out, yet again for 'momma'!!

Saturday, November 17, 2012

Raqu & Coco morning sketches

Raquel and her family. Not sure why we all have three eyes?

Coco's Robot Playground
At an age where I was likely drawing tanks and airplanes of destruction, I think to myself how happy I am to have girls.

There is a hospital, hair dresser and squid eating popcorn. Not to mention that there is a kid puking blood, pretty easy to figure out who that is.

Friday, November 16, 2012

A typical day in Squamish

This has nothing to do with EB.
Other than I was on one of my many chronicled fishing mornings attempting to loose my bent mindful of EB.
At the time of the incident I was very peacefully standing in the river watching the dozens of eagles nestled motionless in the giant Cottonwood's at rivers edge.  Gazing beyond their black silhouettes and white heads there was the freshly dusted mountain peaks of the Tantulus and Garibaldi ranges, the sun was pushing bands of light though clouds at faces of glistening granite cliffs with countless veins of waterfalls plummeting thousands of feet to the river's edge.
There were large splashes and flops of the spawning salmon competing for dominance and spawning rights.  As well a few fierce fights at the end of my line from the tiger striped Chum with fanged white teeth..
When suddenly without warning at the back of my legs a very large object pushes up against me.
At this point I'm waist deep in water and the current is somewhat trouble some as I have to stand on my toes nestled onto larger rocks for balance and that little bit of elevation to get clear and long casts into to pockets of moving salmon.
So having something in the 100+lb range push at me broadside was freaky, brushing me quickly from my precarious foot hold.
At the first instant I was worried a log had crept up behind me and was about to sweep me to my early demise, so I really started to freak and attempted to bounce down river to get away from what ever it was, except it was going with the current and it was virtually stuck to my legs.  Anyway, it finally passed the apex of my leg and started to get around me, I could feel it sticking to me somewhat as the fur was pushing back at my waders.
I had the visual sensation of how my ski touring skins open up when in use for ascension.  SO it was pretty clear what was pushing at me once I saw the torpedo like shape with dark spotted colours.
I was waiting for it to bite me or swim quickly away, but it tumbled down the river on a few rocks then came to a stop closer to shore in some slack water with it's head pointed upstream.
Oh, did I say that somewhere in there I squealed like a little girl finding 30 spiders in her bed.
Or that I molted my skin?
If there was ever a feeling of being electric.  That was it.  I could have lit a small suburb of Vancouver in those few seconds.
I'm convinced my screams are still bouncing around the Squamish valley.
By the time it stopped I was fairly sure it was dead.
But it didn't stop me from getting well beyond the waters edge.
It's bad enough having a moldy Salmon bump into your legs at 14-20 lbs never mind a mammal.
Once my nerves had settled I went over to inspect the Seal and quickly saw a large wedge from it's forehead missing.
FKing IDIOTS with GUNS!!!!
I'm not saying I haven't chucked a rock at a seal's general vicinity that has pestered me on an ocean beach, but blowing it's head off in a river with a few million salmon flopping at nearly every back eddy is moronic and offenseable to the highest degree.

After my heart dropped to normal beats and I was tired of mangy fish, since it was clear none of the pretty bright Coho I'd hoped to tag into were hanging around in these waters I left and started the sea to sky highway home.  Half way up the Howe Sound I could see well below the highway and huge circle of frothing sea water.  Most of the Sound was calm and without ripple and this area looked like the effluent splurging from the depths at the side of a mill or storm discharge at oceans edge.  At closer inspection as I slowed on the highway I realize with every second there were the dark outlines of 30 or so Dolphins. They were densely packed together headed south and a quick guess would be that there were well more than a 100.  Amazing.  I've seen this once before but the pod was stretched out across the 1km width of the sound.  This tightly packed pod was amazing to see as they cut up the tranquil waters.

Good day.

I didn't exactly tell Ren I was fishing that morning as I decided to go late in the evening and wasn't sure a bargaining session of time & fun allocation was going to be fun/in my favor, so I just packed and left like a thief before she awoke.
Sooooo,,,  if anyone asks Ren about this?
I was at work and it's all fiction.

Then later in evening.  I was playing flip Raquel games, she ended up not getting her hands out of the way of her face when she flipped onto the couch and skinned her lip.  Fun and games gone wrong.
Yet again.

Friday, November 9, 2012


It's like the EB spinning top.
Image from Debra International Awareness Campaign
We are living in a bit of a spinning dervish at all times.
Any wrong move and hands are cut up, esophagus is shred, elbow skinned, eyes mangled or feet freed from the comfort of flesh.  On and on it goes as any one issue kicks the spinning axis out of control and with every lash and flailing limb more and more of her system becomes at danger.
Somehow we have to find the equilibrium and pump the top to regain balance while everything around is being shredded and falling apart.

For months we were able to see a slight wobble and keep the momentum from working against us and maintain our sanity with a relatively healthy child. 

It seems the axis is starting to wobble out of control right now.  We get moments like last night were she finally stopped holding mouthfuls of gob and talked and ripped around our place acting herself.

Then within hours of falling asleep, all was lost.

Ren held her nearly the whole night as she cried in pain and discomfort.  Neither of us had much of an idea if it was a badly skinned elbow, the continuing throat issues, a new blister well below her bandages, the beginnings of a cold, all of it, none of it or something new? 

It was a wide awake night with a child bent out of shape with her eyes closed screaming that even the street light outside shining virtually a half block away at two am through our curtains, was "TOO BRIGHT!!!!!!!"

Actually, there was a positive, she wasn't writhing, just screaming.

Wednesday, November 7, 2012


I met Johnathan and his mother while in Toronto at this years Debra International Congress.
What an exceptional kid.
He hears it all the time and was offered to take a few trips next year for EB awareness.

Anyway, here's the link to his TV appearance last week.
CTV live

Tuesday, November 6, 2012


Babi gave Raquel a little hair cut today.
She was very cute and strangely compliant.

mom won't like these shots, but oh well....

Things have improved some, but there is still a ways to go. She spoke for the first time adding more than a word or two without gobs welling from her mouth while describing a drawing she made.
Bright and perky she engaged me with her mitts pointing to 
It was music to my ears, but when she was finished it was back to mouthfuls of saliva pooling at the edge of her lips.  Yet she was undaunted in her awareness and ability to be a brat there soon after.

Ren has had a fairly wicked stomach flu and expelled most of her contents moments before she was supposed to go to work the other day. She clearly isn't put together like Coco, Raquel and I whom rarely get sick or stay sick long. It's actually starting to be a running joke around here. I'll feel 2hrs of nausea, Coco maybe 6, Raquel, well who knows when she's sick as her typical pain and suffering can be little differentiated from sick and suffering. Plus she gets the good drugs to get past it as unhindered as possible, but Ren, she'll be in bed for two days. Which is exactly the case this time around. I can't imagine how families who don't have nursing help function should anyone get sick. We'd have been hooped.

If Raquel gets what she had I can't imagine.

Without swallowing how would the vomit force it's way up?
The pain of burning acid locked at tender areas within her esophagus would be insane. For certain she'd run a serious risk of aspirating.
I'm quite sure with her hygiene, the fact she wears covers on her hands is a very good barrier from many contaminates making their way to her mouth. Also, if it wasn't for many of Ren's blunders of things she lets her chew and stick in her mouth we'd be free of this question and 99% of lip and mouth blisters.

The darkness of my fury is barely contained when week after week I'll see things I thought we'd agreed on was a no no.

Oh well. She's still lovable (Ren) even if common sense in EB care aren't her strong points. I guess if that was within the criteria early on in dating I'd likely have overlooked it, who knew?


Monday, November 5, 2012

Starting to worry

Raquel's breath now is getting bad due to her continued throat issues.  She hasn't swallowed much more than water just warmed from ice since her apple munching.
She's still charging around, but with a full mouth of saliva.  There are a couple bowls that follow her around which quickly fill.  After a good gob dump, she'll get in a word or two, then it's back to holding it.
The main issue is her forgetting and attempting to swallow then the choking that insues.
I'm listening to her cough now in her bed.  It sucks.

This evening Coco Raqu and I were reading some of Coco's French books while Raqu was playing at my side.  I reached to pat her head, but the issue was I wasn't actually watching and Raquel jumped up quickly.  All I felt was my nail dig deep into the edge of her eye socket and skim her nose.
In a flash I figured that was the end of that eye.  Except when I looked quickly at her face her eyes were both open and starring at me with a tidy little gouge just at the inside edge of her eye.
I felt sick to my stomach.  Not swallowing is one thing, a deep gouge to the eye would be another we'd not likely get over in a week or so.....

I'm wide awake and its late.
Can't sleep.  EB is pissing me off.

Friday, November 2, 2012

Apple and suffering

Raquel had a little piece of apple last night.  Our nurse figured it was mushed enough, but the result was choking and hacked up saliva mixed with blood.
The remainder of the night was rather stressful while I watched her slip into sleep and then awake suddenly to expel the mouth gob full of saliva into a cup, her blanket or the famous couch.

It was thought to be a very nasty night, but she settled and
Asked for Daddy when she woke at a reasonable hour in the morning.

NO APPLE Please.

I can't blame her as Raquel grabbed a piece of muffin last Sunday and did the similar thing.  Although luckily then it only lasted a few moments.  Interesting holding your kid while she choked no stressing and calmly waiting for her to get it out while people in the coffee shop watch in nervous horror.

After so many terrible nights and massive blood and fluid letting sessions a little lack of breath is hardly something to get anxious about.  Well.  I'm counting and know when the numbers get to X that it's time to freak!

EB sucks.

Thursday, November 1, 2012

The XL smudges not there.

No XL hand marks on my child.
I can't explain enough how odd it is to have one kid I can play very rough with and then the next a touch of her bare skin with hand that isn't in a fully controlled moment creates incredible levels of anxiety.
Stranger still.  Raquel has been very nice to me and hasn't pushed me away but been very loving since our all-nighter.