Monday, November 11, 2013

Tough enough

Yesterday, I was planning a quick excursion up Seymour to see the little skiff of snow and show Coco, Raqu and Jake.  It's a yearly event for me since I could get myself up the mountain.
As I was asking Jake's dad for permission to take him with us Raqu happy and overly excited about such an excursion forgets to look down and basically in her run, trips on my foot and does a face plant straight onto a paver driveway.  The sound of her skull making the impact with the concrete was very distinct and very much concussion sounding.

I look to him and say "Nope, not going." I quickly picked her up and off to home and see what damage has resulted.
I was rather sick to stomach wondering how much of her face was missing skin as I didn't dare check till I had all the materials and tools necessary to deal with such a crash.
In the moment I picked her up she finally let out a cry and it wasn't the usual screaming that accompanies this type of accident but something surprisingly low key.

We make it to our place and sit on the couch as I do my best to console her and check her face and hair line.  After a couple moments she finally puts a few words together explaining why she's so upset and it's not what I expected to hear.

'I want to see the snow' in a quivering pout.
How tough is she?

As Jake's dad shows up at our door to check in on Raqu, I have to explain that the mountain hike is on.

We don't get far, but were able to have a snow ball fight, play with ice shards and then there is the part where Jake found some frog eggs in a giant puddle.  I had to giggle as he figured someone had dropped a jelly bean in the water and was looking like he was going to give it a try.  It's clear to me how very lucky Jake is that I am not his father or else I quite sure I would have kept quiet to see what happened during his inquisitive moment of what a jelly bean from a pond might taste like.

That being said.  I am a dad of a Coco-nut.
The death thing that she was trying through a while back has morphed into something much larger and gaining a wicked amount of speed.   I haven't a clue....  I think the void of heavy stress from the EB front has finally started to crack the family.
I can handle EB and all it's shit.  I can't seem to handle a burgeoning OCD, clean freak, and unrealistic expectations on top of it all, add sensitive mother (not mine). Shit yeah, her too.

Just when EB was somewhat licked - meaning it's a good long stretch where we have been able to handle it with authority and grace, all the hidden psychological effects of everyone but the center of this family are bursting at the seams.

So who's the same around here?
Raqu, that's who.  Even while up at mountain this painful skin puddle was growing within.

The video is from her after we've gone through the pain of the popped blister.
I guess I'm still a bad daddy and Simona is best.



Tuesday, September 10, 2013

No drugs. No problem.

It's been a big battle keeping Raquel comfortable through her all the ordeals she's up against yet we seem to be pushing through a new milestone of sorts.

No Drugs Required.
No not really, she still gets something for that damned itch.

A little child of age 3 with skin so fragile a nick with a finger nail peels off skin filling your nail as if it were butter saying 'No' you aren't doing it right, 'use your nails'.
She say this during a struggle to keep her heel from hammering the top of her opposite foot in every attempt to kill the itch.

This killing would more accurately be described as breaking down the last months healing.  Soon to follow the limp, then the ugly scream fest of a dressing change, then the anger at me for cutting blisters and manipulating her feet and then return to the itch.

I'm not sure who, of us two has more issues with loss of control on her bandaging?

As I so desperately want to dig and remove every last scrap of sloughed skin and her so dead against me even looking.  For her I'm able to apply a big goopy layer of aquafor, but not take a moment to look, inspect, ponder and touch in any way that is not directly related to me putting the bandages right back on.

Some days of late have ended in a stand off, I've been able to do the bare minimum and she's stopped me from digging and the discomfort it creates.
Unfortunately it's the battle for her digits that I'm not in any way interested in losing.
Yet her toes are going going.....

No Drugs Required, right I was on track from something.  Got side tracked.

It's been a small grade down to today where she's had none.  We've done this before and I've regretted it, but she wasn't like she is now.  She has an awareness that I don't think I saw in her before and the start of her ability to push through pain and go for it what ever it is.

We walked out to a beach near here.
There are two dykes built up.
One to contain and push Vancouver's sewage way out into the ocean and the other to contain the great Frazer River's north arm.  The one that's the poop shoot is paved and has a parking lot full of people wandering it's high thin banks to it's terminus and back with the constant deep thuderous roar of jets making their way across the planet.  The other has a gate not stroller friendly, nor a path stroller friendly or a few hundred meters of river and ocean debris of piled boats and logs very view friendly, but once one breaks through, it's sand and stunning beauty.

After a KM or so pulling a stroller backward through soft sand with a lump of sleeping child we came to an area open and interesting enough that I could fish and the kids could play and Ren could, well not be concerned or irritated I was fishing.
Once the turkey woke it was everything 1 year ago we'd be losing our minds over.  Raquel playing in the sand making castles and so effective was she that her older sister had to join in.  In any event she was determined to climb on logs and balance 3' up from certain EB nightmares and she had a blast.  She didn't care her knees were crusty and raw or her feet either.  Only that she was part of the gang and making all of it.
Her typical investigative squat.  We're always on her not to kneel.

The castle before Coco discovered it.

haha you mac people won't be able to see this video.  Too bad, so sad.

My usual ten finger salute.  'get lost dad'

Monday, June 17, 2013

The Good, Bad and Ugly.

Asking, does the irritating person.
'So, do you want the good news first or the bad?'

That moment in a perfect world, has the person holding the information for effect also holding a handful of chicklets. 

The Bad.
Ren has gone away away for family reasons that are tough and difficult to say the least.  We're holding down day 3 of 21.
The Ugly.
Day 3 of 21 saw a 5am wake-up, a difficultly consoled then eyes shut till shortly afternoon by the reigning short person in the family.
In the mean time I had my first Heritage Commission meeting discussing a year old project that's seen many turns, breakthroughs and fall backs to amble in to a meeting of make or break it.

Ummm, can you spell stress?

It didn't break, so on we go!!!

Then a nurse and my mother with Raquel met me at Children's Hospital within the hour for a consultation that was to give us caregivers the upper hand at dressing changes when her feet in particular need special attention.

It was a bloodbath...

It kind of went like this.

Deep breath.

Wait for Big M to take hold.
Remove dressings on hands so she can play and be distracted while we go to feet where I could finally get at some really ugly areas under her toes trimmed and tidy.  So that, in my estimation giving her a chance at good balance and walking pleasure should an eventual CURE or not come our way - bad IDEA!!!
As distracted, she was.  But to at any cost not let us finish up her dressings anywhere on her body.
Start foot.
Nope, not cooperating.
Try hand instead of foot.
Nope not cooperating.
Wait a little for the drug to get in there some more....
Shit, she's got a blister on her palm from pushing around on bed while she battles us.
OK try bad foot as the bandage seemed squishy and likely had big blister.
Massive blister located.
Snip and the deluge, bloodying the bed...
Raqu majorly stoned and restless shuddering in pain while flopping all over.  Very difficult to contain.
Shit now she's nearly naked and out of control and how to minimize more blisters?
Do coverup on this foot and go to somewhere else on body to come back later when more drugs are on board.
Shit she's got another blister on her elbow from wrestling us over her hands or feet.
Fuck, who cares just get this kid back to her safe mode wrapped and bandaged....
Finally we finish a foot and one hand.
Dr. tries Fentenol.
Not really working in getting her to comply.

Takes time but she's still not giving in to let us finish her remaining hand.
Go back to bad foot snip more of fluid filled bloody balloon that gravity had previously pushed out of my sight. The foot still wasn't an easy bandage and there was still no way she was going to let me poke and prod at the giant scab, scar, pile of dead/living skin pulling her toes under in the EB nightmare that is Recessive Dystrophic.

A second Fentinol.
We finally do last hand with a minor wrestle and a little distraction, but still she's tough as nails and like her father, no sense of backing down when mind made up.


Back at home and it's 8 long after our day there and she's KO'd.
No wonder.
I was able to take a nap with her for an hour, but it was only one of us to rouse when her big sisters foot steps and giggles came stomping in the house from her day of school and play.

The Good.
Fathers day was bliss.
Took my mom and two girls sailing on Das Boot for five hours and the only (I realize now where it came from) issue was Cordelia kneeling on Raquel's foot while climbing around in the spacious cabin.  Raquel spent all the good windy bits where mom's eyes were soccer balls as a nice little breeze shuddered the little boat and rocketed us up wind to home sleeping upfront and in the shade.  Probably the only time I was content our saggy sails were closer to sacs than the carbon sails I'm used to seeing on Mpower (Farr30) while racing with the A-team.


Waiting for wind.  Raquel sleeping in the cab.

shhhhhh! don't tell Andrew how messy a sailor I am when he's not there to observe.

Raquel not taking no for an answer about helming the boat.

My knees look a lot like Raquel's when my mountain bike works

Indian Arm.

Thursday, May 23, 2013

Raquel's body 05/23/13

Webbed middle toes.  Her skin quite translucent and in bright light it almost looks like I can see through the onetime space.

This one in particular is very itchy for Raquel.  And she rubbed it with other foot and I did emergency change to realize the blood had transferred from other foot. It's clear here how the webbing takes place as under the toes becomes bridged to pad as toes are pulled and contorted by the pressure of tightening skin and scar tissue.
When we see her limping about it's either a bad one here or bottom.  A few posts ago it was both and she refused me to help. Imagine this, after a weekend hike in new boots.

What happens when wounds aren't managed promptly? The fluid sags and releases the epidermis, slumping further till we get scissors to cut away at edges to stop gravities evil work on her skin.  Red areas are scissor cuts.

Where I dropped her.  Amazing wound recovery.

Saw this for first time today, after what I heard was a scooter accident. Wasn't impressed that a scooter accident happened in the first place but it made me feel sick and terribly sad. 

I'm still extremely proud of her hands. Even though this happens once and a while.

Battered knees at 3 years old.  Milia are the pimple typed deposits.

The perfect foot.

 Perfect, except for the fact the big toe is being pulled under by scarring and the index getting pushed up and over.  Raquel and I battle relentlessly till I usually give up in fear of ripping skin off her foot or leg trying to hold her still while I cut at the thick scar tissue building up and pulling the toe under.  She's been getting her hands in the way when I'm about to cut fending me off with vigor.  An accident is going to happen!!  I can't wait for some M&M power!!!! (yes pain clinic - hear me)

Sunday, April 14, 2013

Debra Book

Coco made this.
It's her Debra - EB awareness booklet.
She's clearly headed for a publishing career.


Monday, April 1, 2013

Broomball images

I'll write later when I have time to think about it. It was basically far beyond what I expected for raising money for Debra Canada. I had made a few suggestions of what we could make, but I didn't think we'd blow the higher number out of the water.
Here are some images from a friend.
The ice had over heated from the sun and plus 15 degrees and turned to slush. Probably not a bad thing for how hard people were working to play the game.
Good Nights Sweep vs Braydenators
Montessori North Stars vs Boom Boom POW
Trudge Club Broomsteres Vs Moe Tappins
Mr Moe Tapp giving his team a pep talk
Raqu and Nurse Weezie
Just what I was looking for - blood and suffering from a non EB sufferer
Surveying the game with our NINJA friend rarely caught on camera
Brayden's dad chopping down the competition. I actually watched a Good Night's Sweep player hip check Brayden.  So I'm not surprised his dad was out for retribution.

Monday, July 23, 2012

The importance of collagen 7

In moments of my tragic decision making skills I'm reminding how Effing bizarre and fragile my daughter is.  It's not that I need to be reminded of this while riding my bike home semi drunk from a party at 2am and have my nearly flat tire give way to metal rim on pavement while traveling fast down and up around a corner.

It was a fairly exciting moment when I felt the tire not exactly go where I wanted it to go.  I caught a glimpse of a few sparks as all balance was lost and gravity and inertia did their number on me.  Dressed in a nice short sleeved dress shirt and jeans I hit the pavement nearly instantly and skidded to a stop after some distance.  Usually I'm wearing armour, gloves etc when ripping through our forests, but a pleasant ride home at night down the hills of NV didn't seem like the occasion for that level of protection.
Anyway, after dusting myself off and making sure nothing was broken, the burning pain of road rash on my forearm flashed.  Some how the fabric of my shirt was hardly affected as was the skin directly below.

There's the thing.
Raquel falls over the threshold of a door and she looses all the skin on her knee and elbow.  At about 36" in height it's not far to fall, yet she's headed for two weeks of discomfort.  From Dad poking her with needles to relieve surface tension from ballooning skin pockets to resultant - raw, bare and open sores to the dry itchy healing skin flakes.
Or where she didn't pick up her foot high enough and falls to the carpet skinning her cheek, on that aggressive and evil texture to the collagen deficient.
Jakob and Raqu watching Coraline yesterday

Me, I'm nearly invincible by comparison as this is all I get after hitting the asphalt with bare skin at about 40 km/hr.  Well today there are some deeper bruises and pains showing up at many areas of my body not initially connected to where I though most of the damage might have been.  Must have happened in the tumble and effort it took to keep my melon from cracking on the pavement too.
never mind that......