Thursday, December 27, 2012

Never leave glasses unattended.

We were cleaning table after a dinner and we hear clunking and a solo dialogue only to find this.




Enjoying what we get

Christmas Eve was a little exciting. So no pics as promised.
Moments after I posted that Raquel wasn't starting the day right, we had another little mishap.
Ren was doing her best to get our dinner going. For her the Christmas Eve is the all important family dinner.
Considering I was an hour late from returning from fun she was a little less humourous with Raquel laying on the floor in the kitchen, eyes closed and rather grumpy.
Things really went sideways when Ren stepped on her hand while crossing the kitchen. We each took our turn with elevated expressions of emotion.
First of course Raqu let it out, then I attacked Ren & once she'd had enough pressure clumped on by me and her own thoughts of guilt, having possibly smeared off all the skin on her daughters hand, she let me have it back. As mad and frustrated as I was seeing Ren up me by a few octaves and the deep red hue of her eyes and face I quietly let her run the course uninhibited.
I sent out a couple calls once she disappeared down the hall from the kitchen. One to Dammit Janet and the other to Andrew for an emergency change. It was clear Ren and I weren't going to be able to work this one as a team within the short term and help would be needed.
Once help did come it wasn't all that bad. Just two fingers had blistered and filled with blood. It took a little effort to get her to comply and the change went without a glitch. By the end Ren and I were on speaking terms although still not in agreement who's fault the stepping on the hand actually was.
I still think closest person passing by the prone child would be it, regardless of extrenuous circumstances. Even when the child who has few full sentences said, 'Mommy did it' moments after.
Hmmmm?

I'm only making light now as after a second change the fingers look remarkably well. I guess it helped she was layered in many blankets and we got to it quickly.
Funny this happened as earlier this evening Coco ran across the couch while Raqu lay covered and sleeping.
I stood amazed at the door watching Coco seemingly standing on top of her sister yet still listening to the sleeping child's deep breath in perfect rhythm. Then I lost it again.

It's been fun.

So for the entire Eve, Raqu opened her eyes a couple times. I'm pretty sure this was my fault as I medicated her into oblivion fearing the worst. Christmas morning she woke and all was normal. Happy little voice and she charged around all day as if nothing happened the day before.

Again, I don't get it.

At least her knees have healed and most of her current wounds. She's got more now in various spots than I think she ever has. I'm thinking the itch is going to be a problem once they all start the crusty scabby transformation to her normal collagen free milia dotted red blotchy skin.

The poor girl.
It's hard at times, but when we have great days like Christmas and today and watch a goofy fun loving kid do her thing it makes the hellish moments livable. We know pleasant times are close and there has been no way of telling when things will turn.





Monday, December 24, 2012

Alpen glow

4:30 cries.
wide awake at 6 and everyone in the household fast asleep so what to do?

Go for a hike up a mountain and enjoy the peace that only the an alpine buried in 3 meters of snow can.

I unfortunately didn't make sure my camera had a full charge.
Which means I alone watched the colour of orange light up the peaks above Vancouver.
The clouds were low and socked in the entire mountain but for the last 200' to the first pump.

The sun made it's break from the horizon just behind the tip of Baker across the boarder to the south.
That would have been the shot as a 100kms of a flat pillowy blanket tipped in vibrant orange separated the peaks.

Funny that I was sitting taking a break eating a couple oranges.

But before my camera died I got off this, 30mins before the sun rise.

Got home just in time for Raquel to wake.  Wasn't an easy wake either.
Lots of 'no like daddy' (s) and the yang MOMMY!!! as she walked out the door in perfect timing.

At the moment I have a full lap of child quietly hunkered down getting a tube feed, eyes closed, fresh blister under nose from wiping snot from her face and some blood trickles at edge of mouth.  We are quietly waiting for the pain meds to pull her into this world and other than whats going to be a dreadful dressing change we'll have a wonderful day.

It's the bianual Euro Christmas, so today we open presents.
I'm a little unclear how we'll explain Santas evening visit, then again how he'll deposit a few stockings over night.

Anyway,
Merry Christmas all.

I'll get a happy shot of my princesses sometime today in front of our CHARLIE BROWN tree.

Sunday, December 23, 2012

Pre Christmas Cheer

The relentless giving in which EB is capable, is quite amazing.
A few days ago, I was playing with the visco paste dressings and put a big patch on Raquel's knee.
She had some dry skin and I figured it was a nice easy way to put a little zinc on her knee and keep it there for a while in a way it wouldn't wipe off with clothing.  Ren was helping with a dressing change at that time and I guess the idea stuck.
STUCK.
A few hours later Raquel took a decent tumble and skinned the entire area of her knee.  The skin was mostly intact with a few rips at the top edge letting in the painful air and opened up the tender skin to the constant abrasion of her silks.  Ren figured on putting a little patch of visco paste bandaging to protect it.  Problem with that material is if it's left without a protective coating for too long it drys quickly then becomes stuck to the wound.  By the next morning when it was time for a diaper change the blood had dried and so to it the little bit of bandage.
I tried to soak it off, but in my attempt to put a damp cloth next to her knee she fought me off, tearing off the remainder of the semi protective dead layer.
Since then if her pain hasn't been managed she cries and limps around.
It didn't help that I tried to feed her a bit of banana.  She's now completely forgotten how to swallow anything but water and yogurt.  So she's had blood trails coming from her mouth to match her knee.

It's so refreshing to be reminded of how things have been as we see those sure signs of suffering by blood soaked sheets and pillows.  Thankfully she's not been tumbling out of control with issues.
Just one here, one there and easily administered.

Every other night is one that is slept through, so it's really not that bad.

If your name isn't Raquel Bella.


Tuesday, December 18, 2012

SNOW

We just got slammed with snow.
A whopping 4cms and traffic is at a stand still, as it quickly compacted and turned to ice.
All the coffee shops are empty, as everyone sits grumpily un-caffeinated in their cars, stewing at the stupidity of others and the mild guilt it could have easily been themselves that wiped out 6 static cars on the freeway.  Wondering why, this isn't the year they finally bought snow tires.

But since no one else doing ballet twirls with their cars down slick sloped streets bouncing off quarter panel after quarter panel has, why bother.

It is somehow strangely accepted, that we Vancouverites are all delayed three times a year because of the magical snow.  That we also kind of accept we're almost all reta%$#d when it comes to our safety and that of others.

Maybe it's that we've all done bumper cars as children and we feel invincible.

Kind of like me, before I found out the hard way I carried a screwed up gene that's made a significant part of my daughters life a living hell.

Won't go out in the snow without snow tires again will I.....?


My little buddy has taken a few big falls of late.
Skin missing from - nose, both elbows, both knees, forehead and finger with a side issue with her eyes.  Joy it's Christmas, even EB is in a giving mood.


Sunday, December 16, 2012

Payback is a ...

I just realized Raquel is purposely stepping on my heart. Not unexpected when I can be perceived as a bearer of pain and suffering.
Not like it is enough for her to nearly always cry for mommy when we are left alone, but she's taken it another step in the last few days, not exactly subtle either.
Simona came over to mind the little ones while Ren & I watched the Life of Pi.
Giggle Giggle.
So wonderful watching a movie with intensity and not have Ren walk out go to the kitchen, decide to fold towels in the laundry room, etc. This was awesome as the tiger lunged in 3D a few more times than most movies bother to. Ren couldn't hide, she couldn't run and every moment she figured it was over, fangs and claws jumped from the screen into our laps.
I guess the only reason I wasn't jumping and squealing was I was watching Ren and giggling beyond control.
Anyway,back at home I'm watching Raquel give Simona unrequested hug after hug. Big, warm and wrapped little girl necklace. Do I get one when we left or came home? It's like she knows giving everyone else the affection is starting to get at me.
Then for a coupe de gras, she (Raquel) was returning from depositing my banana peel in the compost. I asked her wasn't actually expecting she would walk to the kitchen and know exactly what to do. On her return I was so proud I put up my hand and said 'high five Quel' she smiled put up her palm and walked towards me hand extended and not two feet before we slap hand to silk mitten she sidesteps me and goes right to Ren giving her the five. She glances a coy sparkle in her eye and grin.
Like a knife to the heart.
Again, when diplomacy is taking too long who pins her down and yanks crusty scabs from her elbow, or lances a moving target and sinks the needle deep into the tender flesh or dresses her hands so quickly from freedom and touch.

Why would she?
I do all that and it's not like I do it with the soft painful eyes of sorrow. The more difficult the situation the more likely I am to snicker, or yell angrily when she's about to do some major damage to herself, because she refuses to sit still.

I figure this is the edge.
Glad, at least Coco his shaping up nicely as a daddies girl.

Monday, December 10, 2012

AOK

The kid has been fine.

Although that doesn't change the fact she likes to have her sessions at various times.
Screaming through the night for no apparent reason.

But who's complaining.
she has long eaten food other than yogurt, but doesn't stop her from feeding everyone else

Winter has come.
Hooray!! 
At least the dreary Vancouver existence can be altered once and a while by this.

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.

ASAP!

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

Crunchy

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,
Until....
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

BACK TO THE DARK NIGHTS OF EB

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

Johnathan

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

Haircut

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 
Eyes
Nose
Ears
Hands
Etc.
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?
haha


STILL - 5 FINGERS NO WEBBING









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
gasp....
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.
Relief.
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.




Wednesday, October 31, 2012

Man that chick can belt it out

Just finished a night of hell.
At first Raquel's cries were sounds of a needy baby, of which I'm much less then the soft soothing type.  After some time of advising Ren not to attend to her, I finally go in to attempt to stop the wild noises, so that the other child whom doesn't have high walls of containment isn't soon to be nestled between my wife and I in our bed.

A moment gone wrong.

Unaware 2 layers of her shirt had rode up her back and I had grabbed her bare skin with some force as I attempted to lay her back down, from the angry jumping and screaming at her rail. The thoughts of massive blister damage bashed my sleepy conscience and I was forced to give into her cries and follow her instruction.

"Daddeeee, kneeeeee, couch!!!!"

At least after an hour of asking for mommy she knew I was her only hope.
Within moments she's out cold and comfortable, but I was far from it and ended up watching 2 hrs of the seven wonders of the industrial revolution, with her curled up at my feet on the couch.  When I finally found my mind at rest I took her back to her crib.
The exact moment my head hits pillow she cries again....
After quietly waiting it out for a few minutes she's off to sleep, but for an 1 1/2hr.

When she wakes it's full on screams of pain and suffering.  None of the baby attention grab pleas from earlier, unless she's learned the exact levels and pitch of her screams to get her parents into fast action.
Ren was forced to take the early morning shift as I attempted unsuccessfully to bury my ears under all 4 pillows on our bed.
It wasn't till I was leaving my 7:30 am meeting, which I was late to, that I remembered that there is likely a XL hand print on her back.  (SHT!!!)  How does one forget that????  

Oh I know.  It's the family forget it's EB Awareness Week/Lifetime.


Our most regular nurse is with Raquel, so I can be afforded the opportunity to again do my best 'Avoidance Act' within my own home till I'm finished the few other work related responsibilities.  Mable's ability to sooth Raquel is unparalleled, as I know Raquel's grumpy dad can't do much better.  In fact I'm most certainly going to be greeted by her to 'No daddy, GO!'.

Unfortunately if needles and scissors are necessary for XL blisters gone wrong, then I'm the man for more suffering and implemented pain.

Why would I be blogging than checking in on my screaming baby?
Don't know.

I'm a bad dad.





Tuesday, October 30, 2012

EB AWARENESS WEEK.

AND ALL I'M INTERESTED IN IS NOT BEING AWARE EB IS IN MY GENES.
But for all of you.  I'm reminded that it's important for Raquel that you are aware, understanding and compassionate when required.

The little princess has been a version of Schwarzenegger's Terminator of late.

With every chance she's attempting to off her sister or tell her father to go away.
Basically she's fine, but if a little piece of muffin makes it past the pie hole, she's puking tiny bits of blood within moments.  Which tells me perfect or fine isn't exactly the case.

But if avoided like many other things we can live in the moment and forget EB, except then there's the bi-daily dressing changes I'm wishing could be spread off that extra day or lifetime.
Or the fact her toes are growing together at an alarming rate.  Blister or not from blister?
I'm content though that her hands are immaculate.

On the weekend Ren and I had a few to imbue and stayed up very late at a Halloween bash.  The following day as we struggled with staying alert and awake I took Raqu off to the Quay.
She cracks me up with her level of independence when given the opportunity to roam.  She was at every gift store or place where she could touch turn or pull at anything shiny or noisy.  She even broke down in dance a few times with her animated goofiness hopping and doing a Travolta type sideways point, except hers was to the floor not to the sky. 

It's interesting with the comments.
'Did she fall?'
'Is that really bad eczema?'
'Oh the poor child had a boo boo'.

ahh Nope...  She's got a skin disease.
Very fragile skin.
So much so that any friction pushes it off.

Gasps.  poor thing....
Or the one guy who didn't look like he was too impressed she was in his store, but still wanted me to buy a hat.   The conundrum...
Yeah, and in Vancouver there are bald eagles flying off in the distance in every other shot.

Tuesday, October 23, 2012

Little to add

Little to add, other than Raquel's big sister was able to change out another number to the years since she was cut and pulled lifelessly from a great gaping hole in her mothers belly covered in blood and marcomium - today.  Couldn't we have had a 'normal' birth?  Is that too much to wish for?

Raquel, a nurse and I went to another team meet today.  It was fairly uneventful as we have little to hammer out.  Well, there are lots of issues, but nothing seems urgent.

Eyes - Raquel didn't open them till after noon, very much putting in jeopardy her being able to make the hour car ride in the muted gray Vancouver weather.  Just as I was leaving she basically opened her eyes and was full of enthusiasm.  So she had to come.

Hypergranulation tissue - a large gooey skin fold that is trying to push out or wall off the g-tube,  often secreting a bloody drainage.

Teeth - even though now Raquel lets us brush her teeth every second day.  The odd days are full of screams and torture as we are forced to hold her down and wait for the pursed lips to release enough to get a brush at the thick layers of plaque.  We certainly need a serious clean for her.  It's just that now we aren't in there popping blisters everyday (although I did pop one on her lip today which is an anomaly of late) getting in her mouth is just one of those fights we've let go and now it's at the edge of being a big issue.

Pain - She really hasn't had much in the scale of what was going on from Spring to her birth.  So I think if we really dip into an extended zone of suffering I'm very worried we'll take a long time to get on top of it or ahead of it.  It could be that this is the first time I've not tried to ween her of it her pain meds and when 'it' hit the fan we were way behind.  Don't really know although I'm sure, if she looses the skin on two feet two knees and some eye issues at once with a nice little esophageal blister we're screwed.

Nutrition - We'd really like to put her on some blended people food, not her caned diet of sugar vitamins and daily required dose of everything else the body requires.  One can't really argue that it's not working well, as she is thriving for the better part of 6 months.  Since EB runs in virtually every level of her body and if one area falls it's like domino's.  So breaking up a good thing is something we're dragging our feet about.

Maybe it's the time required to process all that food and bag it, considering we usually only plan for dinner that day for the rest of us?
Maybe it's the cost of such a machine that makes the food fine enough to get past the G-tube?
Maybe I'm worried she'll get bunged up from carbs or whatever and ruin what has been the only place she's never had an issue?

Like everywhere else that she can scar.  If her intestines get all mangled and blistered the nutrients then have a hard time being absorbed through the hardened and deformed lining.  Like with scars everywhere else it only adds and gets worse.

To liquid diet of easily digestible food or not to liquid diet of semi easily digestible foods?

On top of this.  Her daddy and less faithful blogger has become the bad guy in the house hold.
It used to be that she'd say no daddy when the bandages were prepared on the table for when I came home from work to do a dressing change.  Now it's about every other time I'm around that she'd rather have anyone else then me.  The only time she really comes for daddy is when she hears Cordelia screaming with laughter and wants in on the action.  Just as soon as she arrives as the action stops, it's over..  Most of the kisses I steal from her are through silk padded hands flailing to injure.  Or she'll just cover her mouth like the speak no evil monkey when she knows I'm about take a peck.

Ren will get one and as Raquel hangs in her arms I'll look to her and she's already sealed off the soft wet wonderful family goodbyes or hellos with her palms as she peers wide eyed as if to suggest you ain't get'n noth'n daddy!

Some times I think it's a game she plays with me, but sometimes it's clear by the frown that mimics my forehead under duress that I know there's little affection for the guy who pops all the blisters and squeezes the pain into her body as the fluids are pushed out.

hmmm maybe there was more to add than I thought.

The great beautiful wonderful Simona showed up this evening for Coco's party baring some small plastic things, at least now when I throw them out I know GVRD doesn't have to open another landfill in a small arid corner of BC..
haha  she'll kill me for that..

But it was great as she stayed late and had Raqu on the cusp of sleep so I have the mind and energy to write.  So thank you.  I guess everyone else can tell, I missed her being around by the hard upper cut.

my two accidental sailors
I'm not too worried as I just pulled a clothes needle out of my foot from the dresses she bought my girls as I walked through the living room to get the camera to add photos. I guess we're even.

Tuesday, October 9, 2012

EB kid does crazy stunt

Cordelia clearly must suffer through all the activities I work her through as she gets kicks to the solar-plexus while remaining unguarded doing the wheelbarrel.  Raquel doesn't fair any better despite having a blistering disease where any friction produces blisters, as little thought was given to have her walk face down across the floor.  Any slip or lack of strength and it's smeared skin for certain.
Maybe the thought more importantly not contemplated was the possible hand pressures not previously tested???

I did layer the blankets but, there were no guarantees.....

Thanksgiving weekend come and gone

Nothing to report.
Just the same as before.
A wonderful holding pattern.  The odd bump still pushes off large areas of skin.  A brush here and there and it's another wound.
It just seems she's better suited now to push through pain as she understands it's part of life.  Gladly she doesn't comprehend that her pain isn't everyone else's pain.  She doesn't know why her big sister can run around nearly naked fall and keep trucking - among things.

Spent another great weekend at the cabin and Raquel has absolutely adapted to the point Ren was able to ask if we'd be able to try a Christmas up there?

Ummm no running water, about 8 hrs of light, possible -8 tempuratures and a pile of snow around the cabin???  Not likely just yet....

Coco, Ren and the Wentzel's did their wonderful speeches on giving thanks, I unfortunately was feeling the brunt of cabin fever as my fishing habit was underquenched and grumpy traits picked up from Grumpa (dad) was in full force, so instead of listing off nearly everything under the sun and hidden by the earth on the dark side I kept mine short - Thankful for places people are not.

When forced to expand to Cordelia I included our day we'd just had as an example.

Motoring down a quiet lake with two families (no wind had to pull our the 2 horse) where very few cabins were inhabited to a quiet beach to have a blissful picnic by a fire, in the warm fall air basically watching the leaves turn yellow along the mountainsides hemming in the tranquil lake water was the premise behind places were people were not.
None of the fire and brimstone as massive boats with a kajillion horse power barfing noise and pollution where ever they desired or odd people chucking spent cigarettes and beer cans all around the site with little regard for other future or present campers and day-trippers, let alone fish, ducks and other wild life.

Just the sun and the laughter of children playing on a beach, picking up snail shells and falling off logs into the cool autumn water.  Of course once one kid sees the other slip and fall and giggle it's his turn too.

No pics as I didn't bring my camera.
All I got is this.

On the way home we thought we'd hit the Adams river (one of the largest sockeye salmon runs on the planet) yet they hadn't arrived yet.  I guess it takes time to travel several hundred kms up river.
A little walk along the famous Adams.  Didn't see one fish.  Just fishermen, which really pulled at my sense of family

This is Raquel plotting against her sister.  She's a bit of a trouble maker once she's a little bored.
But then how could she not be bored after being in a car for nearly 10 hrs as we went from fishless river to home with a few stretch breaks?

Oh what I'm really thankful for on top of all that..
Is watching Raquel go for bite after bite of pumpkin pie.  Haha.  Ren won't touch the stuff.  Problem is, I've got another person in the family to fend off for the final piece.

Rellic & Bruno

Raquel eating on the run.

Wednesday, September 26, 2012

She pushed off the whole palm

In a moment when Raqu asserts her independence, Ren watches from a far as she walks across a concrete play park.  Of course she falls and comes within a mm of pushing off her face too.  The cries intensify and it's clear her hand is in a great deal of pain.
Once home we do an emergency dressing change to screams and shuddering pain.  I had to slice many areas of the blister to waving hands and squirms.  One cut looked as though my fears of scooping a chunk of flesh had finally come true... Luckily it was just that this blister was very bloody to begin with.
Once the aquafor and zinc bandaging material and pain meds kicked she was her self again.
Although slightly more goofy than usual.

It's clear pain meds are for all.  Even if the rest of the family isn't on them.  The anxiety dropped very quickly afterward as our coping method of out of sight out of mind was able to kick in.

Today's change was rather PG 13.  Minimal blood, small cuts at the edges and only 60 seconds of suffering as Raquel looked deep into my eyes wondering -
'WHY is it always YOU, that make me feel this way?'

The funny part at the beginning of the change was her saying a word that rhymed with 'it' when she dropped a toy.  The splayed arms in questioning and look on her face well copied another member of this house hold.  We won't say who, but it wasn't me or Coco.

Wednesday, September 19, 2012

Hand contractions and bigger issues (not Raqu)

I can tell you it was very stressful.
The thought passed through my mind to find a scalpel quick and give the scar pulling at her palm a short slice.  Then when the image of a German girl who's mother exclaimed she cuts at her hand webbing when ever she thinks it's moving out killed the idea.
I do remember for a long while wishing I could cut at Raquel's two toes webbed and the skin under pulling them down and under.  Much like the area between her teeth and gums pulling her smile flat at her upper lip.

Fk.

The signs are there.

I of course have spent the last 5 months ignoring these issues and enjoying all what has been largely unaffected by EB about Raquel.  Let me tell you when you are living high with your child there could always be this -

There was a little excerpt taken from a book Kidowed a mother of two babies with Junctional Herlitz now passed on gave at the Gala dinner.  I had the jolting pleasure to meet Jessica Kenley as she walked, shaken and stressed up through the lobby of the Hotel to the Congress.  This exactly 5 minutes after she was to speak to a room full of EB survivors and medical professionals.  Buses and the arduous travel from the US did not cooperate for her to make her entrance in a timely fashion.
Later in the evening and a reshuffle resulted in some hurt feelings and irrate behaviour from whom one would least expect.
Anyway....
I was talking to my mentor John and Leslie Rader (whom had once commented on this blog at it's very beginning).  There was a small exchange of hugs then John looks Jessica square in the face and says I've read your book, pause, then something to the effect - you were angry, I get it but you were angry.

Hmmm.

At the Gala I found out exactly how angry and at whom and what.  During her reading I was taken back by a few cusses and hard pointed comments to the void of EB knowledge at the hospital her son's last were spent.

I thought I was upset when Raquel's hand was so badly bandaged from the NICU nurses, when her skin fell off like a wet latex glove when or plastic surgeon and burn specialist was finally able to present proper materials?

I found myself at one point listening to Jessica's dark sarcastic demeanor exclaiming the ineptitude of a person advising her on her sons care and did a snort of equally dark sarcastic laughter.  It was the moment of who's helping who?  Immediately I realized it was loud and looked quickly if anyone leered my way in disapproval. 
Ha! 
Like I didn't get the gravity of the situation. 
I got it, I just should have contained my internal dialogue, but I think the general horror for nearly everyone there overshadowed my little moment.  Although, I'm not sure her comment wasn't meant to be funny...  What else is there, when things become so desperate? 

She carried on and brought up a paper written by the group in Groningen.  I was in fact sitting beside the head EB nurse from Groningen and noticed her talk to her colleague.  As the rest of the evening unfolded I wasn't able to get a comment.  I remembered during last years Congress a presentation by W.Y. Yuen on Terminal phase in Herlitz JEB: experiences and data, (PAGE 10) which talked in similar themes.  It was shocking to hear them talk about once finding with certainty the child has the Herlitz version that care should move to palliative than interventional care.

At that time back in Groningen I couldn't deal with what she said or her mentor defending her presentation when an audience member cried foul.  I thought it was cold, callous science and showed little respect for those present suffering from EB, parent, patient whomever.

It took the year watching poor Tripp be overcome, to get it. Then listening to Jessica go on and on about the details of her son's condition as he got worse and worse and suffered incredible pain.  I was basically left shell shocked as a good portion of the crowd listening to her recount some of the final hours of her son's life and the guide with which she struggled through her second child's even shorter brutish life.

Of course, so far there is a great exception to the Herlitz rule.
Daylon - see at side.  I do notice nothing new for months in the families blog entries.  In this house hold that's a good thing.
I'm not so sure about others.

If anyone is interested in how hard and brutish Herlitz Junctional is, with the odd, no sorry constant cuss and sideways comment check out this amazon link.
Kidowed
Stand back and be punted.
But please don't find a free copy!!!!!
Pay for it dammit!
Jessica only gets a $1 from each copy sold.


Monday, September 17, 2012

Publication for Debra International Congress 2011

Publication of 2011 Congress

Now that I've seen the 2011 publication I realize the 2012 will take a while.


This years International Congress

Just back from the Debra Congress in Toronto.
I was able to split and shuffle dressing duty to Andrew and nurses while away.

Of course I was very nervous and was told everything went well without hitch.
Upon seeing Raquel this morning for the first time since last Wednesday she peered at me in disbelief and basically turned away and hid her eyes.  I did get a little smile while I tried through waving arms to get a kiss, taking the defensive karate chops off the forehead and chin.  I did eventually get to place one, but with was difficult with her dancing like a butterfly and bobbing to and forth.

Yesterday she took a few falls, so her face and elbows aren't looking that hot.  But what's under the daily preventative bandaging is what concerns me most and there I'm told remains perfect.  We'll see today.

Sitting through the Congress was very interesting.
It was geared slightly more toward the patient and front line care giver than I thought last year in Groningen was.  I'm not sure if that's a good thing?  I'm still struggling with what these congress' purpose is.  I look at the mission statement and can't figure out if it's science or care?
 Mostly I quietly observed and listened then got excited when an interesting conversation broke out on the direction of where things should go and what was being done correctly. 

The one time I did speak out in private conversation to the COO John Dart about the current writing of the Best Practice Guides and the inherent overlap of information due to the systemic variables of EB and all it's forms, I was left to feeling I'd best keep my ideas inside for the near future.  It's not that my idea was rejected, it's just that maybe it's uselessly ahead of it's time as much of the work has yet to be done and keying it all together into a singular form being a rather monumental task.

Having listened to the Doctors and nurses in the midst of writing the Complex pain, Nutrition and Occupational Therapy Guidelines just getting to where they are seemed difficult and time consuming enough let alone have to relay to one another where each of them were at that time and how to overlap and key into one another is clearly impossible at this stage.

I just hope once all these guidelines have been completed within the next few years that the second or third rewrite, Debra International can have them bound in some logical way.
The disease is just so unbelievably complicated as one starts from nutrition and branches out toward everything else and if one fails then it will invariably have consequences further down the line.

Confusing, yes - I digress.

What was amazing is the near cohesiveness of the Doctor and national groups.  I kept talking about divergent philosophies as it's clear many ways to work and make people comfortable with EB rely on the immediate care group.

Eg.  While sitting in on a nutrition workshop on the final day I spoke up about Raquel.
A Russian man was gathering information on G-tube care and best manner in it's placement.
The topic bantered around a bit till the question of what can go through the peg (her belly port).
Currently Raquel only gets Pediasure (a total nutrient concoction to meet her growing needs), I would very much like to get ourselves a blender and start mashing up our foods and feed her that way.
I've had some resistance from our nutritionist as to how complex Raquel's mineral and vitamin requirements are and the importance of maintaining this balance.  Plus I'm lazy to add another regime and time consuming part to her care.  Isn't it enough that we are constantly balancing her pain meds, with bandage requirements, wounds and total blister prevention attention to keep her safe..............

In any case, the UK people suggested that putting natural foods through the G-tube was possibly unhygienic and not advised, but the US people suggested that hundreds of people do do exactly that and it hasn't been an issue in most cases.  In fact as long as a good plan was created that it was advisable.
So..??
Of course I want to try it, but I'm somewhat nervous.  The UK nutritionist did say later once I showed photos of Raquel that it was clear we were on a good path of hygiene and that she did think we could try. 
But at it's core this type of divergent philosophy isn't just there at nutrition, it's at wound care, complex pain (ie. gasps of telling people Raquel has had a steady diet of Morphine although the Dr. Goldschneider did go on at length this year about it's advantages), dietary (ie gasps of people when I suggest Raquel only takes possibly 3-10% of her food through her mouth) etc.....
But then when the opportunity allows I'd show this video of her hands while sitting during a dressing change..

Or this full body shot sent via text from Andrew the other day of her standing in a tub with body sturdy and virtually unaffected with blisters and wounds.

Zero collagen I'd inform them.  As these shots highlight her pain, nutrition, wound care, clothing (Derma Silks) are clearly being met due to the obvious overall health of this little princess.

I did see in some eyes that this can't possibly be the case (zero collagen), as in some aspects this flies in the face of years of teachings and the various formats taken in the care of EB.

Anyway, despite all this, one can not question everyone that presented and witnessed this years Congress their unwavering devotion to the cause and that these forums are absolutely neccessary to be able to share ideas, therapies and techniques.

As a parent and a Board Member of Debra Canada and as the Adviser to the Medical Assistance Fund I'm doing my best to pick, question, and move through the evolving information and procedures to best care for my daughter and open up the lines for whom ever else is interested and in need.

Again.
Support your Debra within your country.
If you work or own a large company, please suggest to guide your dollars open for charities toward the Debra groups or become a personal member of the Debra Organizations within your country and our umbrella Debra International.

I understand in many cases companies send money through the United Way.  If so talk to your accountant and please advise United Way to send a portion to DEBRA.

There are many things need funding such as the Best Practice Guides as well as the researchers who are working for various treatments and here in Canada fulfill the many shortcomings of our healthcare system and give the EB sufferers the equipment and procedures they require from this extremely debilitating disease.

Yeah I know.  Raquel looks perfect, but trust me the imagery and case studies I was exposed to during this congress would shake you to your core.

In the coming days I'll link some of the speeches and workshops I found interesting as they are or will shortly be linked on the Debra International web site.

Monday, September 10, 2012

I didnt utter those words

It's a simple sentence spoken between Ren and I, often in a quiet moment over dinner or a ride in the car that basically reflects on the erosive suffering EB can have on all our souls.

"I can't believe how well she is?"

Virtually with in moments of us saying it, Murphy wakes up and lunges at our collective well being.  I'm far from Stupidstitious and on any occasion I do my best to poke fun at it's impossibility (Superstition).
Although for arguments sake, lets just say I'm doing my best to not remark and just enjoy 'it' while the good time lasts.

The cycle returns.

It started with a dressing change.
I'm not laying a heavy here, but I was in the middle of letting go my dressing change responsibilities, by getting Andrew and one of our longest nurses back into the process.

I could tell the hand was going to be an issue by the pressure of the bandage going on, but maybe going for a sail was more on my mind than trying to pull all that bandage off again and stress out my students.....
Unfortunately it wasn't too far into the evening before she pointed to her wrist, I ignored it and waited till the next change day as her irritation toward her wrist was short lived.

On that following change when the hands came out from the clothing and tube sock, a huge bump was pushing the bandages up.
I did what I should never do but it was a reaction to my instant guilt of not checking the area sooner.
I quickly reached out with my scissors and cut the top right through the bandage.
The moment I cut it, I knew it was a stupid move and had my repeating visions of the day I finally cut at a blister and take a chunk of flesh with it, but the bubble released and poured a few table spoons of fluid to the floor soaking the bandages.  Once I was able to start pulling the rest of the bandages off the sensitivity of the wound had her crying out in pain.
From then on it's been a wiggly painful experience with her.
Thankfully after a brutal night of listening to her cry every 20 minutes till 3:30am she slept and woke up a dream child.
We then went to a birthday party for our friends child all set up in their rear yard.
Most of the people there have a pretty good idea of what's up with Raquel, but I'm sure it's not in too much depth.
Ren was off at a bridal shower and within the first five minutes of Raquel using the small plastic slides set up on the grass, she does one of her funny little happy hops, folds her ankle on the grass and does a face plant with a whiplash effect of her face bouncing back up after the impact.

OH JOY,,,,

At first inspection I didn't see any of the giant patches of missing skin from across her face I expected so I figured I'd dodged a huge bullet and then picked her up to my chest to calm her.
Clearly one can imagine the looks of concern I was gathering from the people close enough to hear Raquel belt out her pain and frustration.  I smiled as if it was just a grumpy kid with normal collagen levels whom had a little fall.  Of course this wasn't totally the case.  After a second peek she had a bloody nose and a bitten tongue.  The best part is I was wearing a white shirt so for the rest of the party I walked around with a blood/slobber patch on my shoulder.
Ren did eventually make it and it was nice to finally get a beer and two slices of carrot cake to quietly smooth out the bumps in my brain.

I'm headed off to Toronto for the Debra International Congress.  It looks to be a great line up of speakers and best of all one of our EB mentors will be there from NY giving a presentation.

I need sleep, but can't as thoughts of total chaos with Raquel are plaguing my mind, while I drink beer late into the night on Young Street.
Oh the horror.

I'm mostly fearful that having taken some time for Ren to take her own turn of testy/grumpy I'll be right back to sleeping in a tent.  Except this won't be the warm summery pleasure of sleeping down by the lake.  It'll be the under the cold torrent that washes us Westerners from Fall to Fall amongst mud piles and sawdust.

Tuesday, September 4, 2012

Just a little EB and a whole lot of support

We were off to a friends this last weekend for a special birthday party.
Special in the way that when a party is undertaken with a band and lots of friends the center of attention puts her desires forward to support EB and Raquel by having a $25+ entrance fee (of sorts).
At the end of a georgeous late summer evening there was a big bowl full of cash and cheques equaling $1685.00.
So, when anyone asks, what can I do?
Well,
take our great friends lead when you hold a little social of various magnitudes.
Any and every thing helps.

Believe me, that money is going to help someone with EB who is left out from various funding shortfalls in our countries governments and municipalities.

It was an amazing party.  I feel so grateful that Raquel and our family has been able to have so many incredible friends to lend their various levels of support.  It really makes getting from one point to the next that much easier.

As far as Raquel is going, she's been great.  She did skin both knees yesterday and woke up at 5 with some discomfort or itch at her feet from wounds getting better, but really totally minor.

The vast areas of her body are perfect soft baby skin we tickle and kiss as much as possible and we certainly don't take it for granted.

Her mood and mobility are virtually unhindered.  She watches older children play and wants nothing but to jump off stairs and rock walls towering well beyond her head.  She basically only knows two speeds when trudging around.  Walk or run. 
I still can't figure out how she does it as I know how raw her feet are on most days. 
She's the true model of grin and bear it.

She Rocks

Merissa rocks!!!

Thursday, August 30, 2012

Bad feet bad temper, but whatever

She's been a super healer of late.
Nearly every sore she's had, has gotten under control very quickly.  All it took was a little extra zinc in her diet.  Not sure if that's it exactly, but shortly afterward it seemed we'd only have to watch a sore for a few days before the wetness had disappeared.

We'd taken another little trip back to our cabin and over the four days the only blister or issue Raquel had other then her attitude was elbow blisters from the drive north.  She didn't fall once and was able to walk most of our property without incident.  I can't tell you the relief this brings.  As this family heirloom is the one travel beacon where I've envisioned her being in the wilderness of sorts and thriving.  Thankfully this is the early reality.

Changes have been OK.
Other then pushing out blood from blisters on her heel we've had no issues.

Of course every second change we find something.  It's clear my early concern of not having any blisters has drastically subsided.

Her eyes are as bright as ever and when she's not demanding with fits what she wants, she's been gradually turning into the goofball her big sister is. 

I think the only weak link in the family is Ren.  The grumpiness that proceeds her return to work from holidays can be tedious to say the least.
haha 
I'm going to suffer for that one...  If there is a sleeping bear, I just can't help myself but to poke at it.

Been a wonderful summer.

Just the EB Congress in Toronto to go to with my buddy Moe.

Not exactly ready to let the sunny days go, but salmon season has swung into full flow, so it's a little easier to make the winter transition with the loss of light and general warmth ----
Till those beautiful stormy December evenings before the mountain opens and Cordelia and I trudge our way up the trails for a bumpy treacherous ski in marginal conditions on the yet to be plowed runs.

Tuesday, August 21, 2012

Fairy Wings from Santa

Cordelia and Jakob have a request that they want to ask of Santa for this Christmas.
They want fairy wings for the family.
Coco says it's so Raquel won't have to walk and hurt her feet.  She can just flutter inches off the ground and be free and quick to roam unhindered.

I had to ask her if Santa's magic and the elves ingenuity come through, will we then be able to all eat rainbows and poop butterflies?

Of course this comment then brought about some very graphic discussion on the actual mechanical problems of attaching wings, eating rainbows and my favorite part pooping butterflies.  It was pretty much agreed that they'd come out in their cocoons.  I think I've tainted Cordelia's ever lasting images of how cocoons come to be as they fall from the sky and get caught on branches.

Of course when the time is right or her schooling catches up to her fathers fables the truth shall be known.

Saturday, August 18, 2012

A glimpse of a couple weeks of fun

The cabin.
I forgot to picture Maria Wentzel's BBQ'd chicken.
Some where along her dinner making I was asked to ask if she needed help.
So I did.
Her response was that she had it under control.

UMMM??

I was then put into quick action to create a substitute chicken dinner in moments.


Once served it was clear no one was interested in the cancer ball but me with a camera and blog intentions.






Pressure to POST....

When nothing happens on this site it's because we're having too much fun enjoying life and each other.
We've just come back from the family cabin my dad and uncle built in 76.
At the beginning we had a bunch of friends up with their families and it was soo nice.
Memories of my childhood flashed with scenes of 6 or more children playing at the lake side in varying degrees of deep water know how.  All from Raquel (the youngest) to Mattias the oldest either leading the way with modified paddle boards or chucking pebbles from the safety of shore.  The weather was hot and the water was warm, but somehow children kept arriving with sandy feet up at the cabin with blue lips and shivering demanding their parent get them warm.  Only to shortly disappear to reappear for another warming.

Again I remember my cousins brother and I all playing at the water as long as we could keep a parent down at the beach..
Wait.
Actually, I think I most remember the reason the cabin looks as it does now.
'Forced child labour'.
HA!  Cordelia is just shy of her work term.  I was able to talk her into doing the dishes a couple times.
Of course, much like her father before her learning the way through soap, cloth and rinsing most of the food left on the plates from the meals found it's way into the cupboards.

Mattias being the oldest bore most of my personality, poor kid.....

I feel bad about some of it other then the result of finding the cup of worms I bought for his fishing pleasure dried and shriveled baking in the sun after I twice reminded him on two consecutive days to be sure to put away into the fridge - yes of course just beside the ketchup and mustard. 
Then there was the time I talked him (forced) into sailing with me.
We were originally headed to borrow seadoos for some high adrenaline gas powered fun, but the wind started up as we motored down the lake to our neighbours world of speed and expensive machinery.  When I ask if he wanted to sail I get the NO almost instantly.
I asked if he's in a position to say NO when I was about to take him on the ride of his life?
I didn't realize then it was to be with the boat with small old sails and a 2 horse putter off the back to do it
As the sails go up, so do the gusts and very unpredictably I might add.
We manage the first few very well and the boat lumbers on at a steep lean till a very quiet patch runs through.
The sails basically go flat and the boat sits lifeless for a few moments.  I can see another big blast making it's way down the lake for us, so I attempt to angle the boat to take the wind and accelerate into it once it arrives.  I had already started the count down for Mattias to hold on and start hiking, when the wind hits us from the other direction pushing the sails across and the boat sideways.  I start to panic and do my best to fight it as with a quick look over my shoulder I realize we must be in that gust I had been following toward us.  I had just ducked the boom to the wrong side as the perceived direction of the wind finally hits. I wasn't quite ready and take the boom off the back of my head in a glancing bongggggg.  The boat is hammered, at a glance Mattias is basically climbing up the seats set perpendicular to the lake to the high side of the boat.  I didn't know it then but my feet were deep in the water pouring in the lee side of the boat.  I veered up into the wind and let go the main as best I could.  Just as quick as the initial wind hit us we are up and dead to wind with the sails flapping violently.  The boat felt like it was being chewed at by the lake as it pressured into the water as the wind rushed at us and over the haul.  I ask if Mattias was OK.  His eyes large he admitted that was far more scary then ripping down the lake at 60+km/hr on a seadoo.  We had to wait out another big pressure gust pointed safely into the wind before the sails went down quickly .



To think I used to bully that guy walking out of the lake in elementary school, by destroying his paper airplanes and using his pop bottle glasses to burn bugs.



One of the typical mornings Raquel would head down to the beach before the noon sun would come out past our few remaining large trees root rot, progress or a concerned neighbour hasn't felled.
She'd grab a bucket and fill it with some rocks buried under the sand walk to the edge and throw.

Over and over.

She started the week with the drool and swallowing issues directly related to Ren letting her walk around with a dried piece of flat bread the day before we left.  Seems a small piece made it past and messed her up.  There was a discussion on that subject I can tell you.

Then mid week Karl was chasing her on the deck.  I could hear them and the moment I thought to myself, wonder when she gets her first big body blister on the trip? Her feet fumbled and down she went.

The next day I devised a very simple knee pad that kept her great the rest of the time.

Now home she's as bright and as happy as I think she's ever been. 

Really.






Wednesday, August 1, 2012

Gore, EB and Brownpaperbagging it

Just to show life is still a rosy bag of blood and pain.
This was a non typical blister as the blood had turned to jelly.
What was most interesting was when I used my scissors to open up an area for drainage I cut out a clean little scoop like one would with a spoon from jello.  To my surprise the bulb of blood didn't even move.  I wasn't even able to squish it out and away from her foot. Of course the harder I pushed the more squished and painful Raquel's face became as her beautiful little eyes peered at my as if to ask why are you doing this to me again?

The joy of being the primary torcheror of my child.

Anyway, I went to a quiet place afterward and thankfully used the respite provided by our nurses to return a fully functioning member of the family unit.
Yes, avoidance works for me.

Monday, July 30, 2012

Terrible two

Raquel has certainly entered into her terrible twos.
At every opportunity she is trying to off her sister.

As soon as she gets a weapon of any type she's after her trying to whack her on the head.
Then her out burst when she doesn't get her way are way off the charts from what we experienced with Cordelia. 
Yesterday I was so fed up of her ups and downs I ended up using her crib as a deterrent.  She can't stand sleeping in there to begin with so I didn't think I could possibly do any more harm by the bad association of a jail.
It never really did work itself out yesterday, but I think her brain is close to figuring out cause and effect.  I hate that she gets so wild when she's pissed and the thrashing that ensues, but I'll take a bunch of blisters here and there for her to learn to behave and not smack her sister, me and listen when we say don't or no.\

I hope it's not because of the variance of care she gets as it's three to four nurses a week, grandma, Ren and me.

Raquel's got personality.
No question.

Friday, July 27, 2012

Coco Bean has landed.

Coco comes home and all we hear is 'Coco, come'.
After some unrelenting pursuit, a door closes and the little person who clearly idolizes her big sister is left standing in the hall banging on the door with her mitten hand.
Coco?
Coco?
Coco?
Coco, come.
Coco?
Coco?
Coco?

Raquel's ability for repetition and single minded ambition for what she wants is inspiring if not annoying the other half of the time.

It's both lovely and painful to watch and listen.

Apparently there are scenarios of too much love as she wants every piece of her sister she's missed for the last month and a bit.

Cordelia is really not going to be happy about having over a month of quiet sleeps to be crashed by what's going on here now.  Every 20 minutes or more is a cry or a whine, some escalating to some mild thrashing other petering out in moments.  Then we'll hear a wide awake voice call out for soup or something like that only to be followed by a long pause of silence.

It's very late and I just haven't seen the point to go to sleep.

Yet I've only had to get her once every hour for the last four.
ha.

Apparently there are scenarios of too much sleep, when you actually aren't getting any.

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.
Yeah,
never mind that......






Wednesday, July 18, 2012

Sailing the seas of cheese

Nothing to report.
Just enjoying normalcy.  The general feeling of a kid with good health is about.  In fact I have the irritation of doing dressing changes.  Her wounds aren't that bad, so I don't have the overpowering requirement to open it up and get her pain and suffering under control.
Although I think the disease has worn me down.  Where no sores were the goal maybe still only a few months ago, I'm OK with a big one on a foot and somewhere else.
Oh and there are the big ones on her hands.
I can't nail it down to anything in particular, but we've for the first time since birth had some very large ones at her hands.  I am fairly sure it's due to new help not understanding how to truly care for Raquel. It's like someone held firm to her hand and smudged her skin.  The scaring of course is my greatest concern and it's easy to see for the first time where the battle will eventually will be lost in hand mobility and contraction.
Like I said.  It's worn me down some.  I don't feel that deep harsh fighting blast against EB as I did.
Maybe it's the summer.  Maybe it's a moment where I've sunken into self preservation.
Maybe it's the summer as it seems to have finally come to the Wet Coast.

Was able to find a smidgen of time to tick off one of my personal 'bucket list' items (the list one has in mind for life accomplishments or avenues to that end) as it relates to my family.
Still evolving, but for an extremely affordable price with Andrew we have a little trailerable sail boat for fun and future family camping pleasure. 

How Raquel fits into this picture is the evolving part.

I figure if I took her down a North Shore trail in her trailer behind my mountain bike than I can figure out how to keep her reasonably safe while getting bumped by some waves.
Although letting go of sails and her shackles as we get over powered by a gust and a knock over imminent, I think I might have to carefully consider the entirety of my crafty nature and every possible consequence.


Andrew and I making it work.  Although before families and children get back on board there are some rigging issues that need to be resolved. 
Should I mention the first time we took it out with Andrew's kids on board, we forgot to put the transom plug in?
Should I mention that without the existing foam displacement the boat would have likely been at the bottom of the Indian Arm? 

Anyway, it works, floats and soon we'll have images of Raquel enjoying Sailing the Seas of Cheese at the Barriere.