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.



Thursday, July 5, 2012

Support for Debra Canada

I'm not one to ask for much other then a hand to keep Ren and I from disaster during dressing changes.  ie marital stress.

But.....

Debra Canada, which I am a board member is holding The 2012 Debra International Conference.
It is a meeting of minds and science that is very much needed in this family altering, life threatening rare disease. Please see  September Program

Last years meeting in the Netherlands was very important to me to see what exactly I'm up against as a father and caregiver for a child with EB.  I admit there were many moments where science and gigantic words based in a language hardly anyone speaks anymore, well Doctors, Scientists and Priests.

Anyway, this years line up looks to be very interesting and seemingly more interesting for the family care giver.

If anyone has thought to help out Raquel by any means, this is your opportunity.
Please help us board members find a way to grant all our requests from stricken families to being able to get the best minds together and share their knowledge amongst each other and families like mine.

Tell Erin and the Board that it's from Raquel, your EB hero.

Donate to debracanada.org

Now insert heart wrenching video of Super Kid getting a large blister lanced and drained.

I have to admit having watched it now from a video that is just over a year old, what I've learned over the two years there are many things different in how the wrap and draining procedure takes place now.  At least I have larger needles and great scissors (donated to Raquel from her plastic surgeon). Notice the sound of Cordelia unfazed bouncing a yoga ball in background.

But make no mistake, it's just as grewsome and just as painful for everyone doing the work and poor poor Raquel.  Hope this pulled at some heart strings.

It would be so nice for this years Presentation and Gathering be the best it can.

The Moja Coffee barista's have certainly been doing their part as they've raised another $1000 +.


Tuesday, July 3, 2012

MID DAY CHECK IN

On my way around town for work today I went by the home to check in on Raquel and her nurse.
As I walked in the door, Raquel laying on the couch appearing asleep swaddled in blankets facing the far wall she instantly cries out.
No Daddy
No Daddy
No Daddy.
No Bath.
No Bath.
No Daddy

hmmm
Instead of grabbing lunch I took some chips and salsa and left her from the fear of more pain on top of what she's feeling now.

FK EB!!!

It'll be interesting later this week as we will have a new nurse to break in to the darkness of writhing pain, torture of dressing changes and the deep anxiety of having to trust someone new to your very fragile child.

FK EB!!!

Weekend away

We did a little last minute getaway to the family cabin before the monthly change over.  I'd been trying since Easter to get away, but Ren & Raqu kept kiaboshing the extravagant plans for one EB reason or another.
This time Raquel has been in great spirits depite many nagging little issues so we bolted.
The Wentzel's our usual party crew were otherwise occupied so we had the rare small family cabin experience.
I think I've forgotten what it's like to be there with just Ren.  She held the law and only let me out twice for my fishing pleasure.  In fact I was so good that on my excursion to a mountain lake high on the opposite plateau I came home early.  It helps that I caught a monster very quickly and the loon discovered my fishing wizardry and kept a close eye on me once I pulled this guy in making further fishing difficult to say the least.



Raquel absolutely ate up the cabin experience, she walked around beaming and charging around during her waking hours.  We arrived well into the night (3am) and in the move from car to her bed she came alive.
The lake water was lapping loudly at the shores below she pressured Ren to walk down for a peek.
From that moment on her spirits were amazing.
It was like she was free.  The floor area of the cabin with the large deck made for great exploration and plenty of time free of our watchful eye and nervous guidance.

There was the moment she walked around the corner on her loop from side entry to slider lake side and didn't show up on the other side.
Ren worried at not seeing her for the 60 seconds went to look for her.  Thankfully at this moment I wasn't solely responsible for her safety, as I likely would have finally noticed her down by the lake.  Since it was me who barricaded the stairway to freedom I didn't think it possible that she somehow could move a 4'x8' sheet of plywood (1/2" thick).  When Ren found her she was down three stairs & just one step to freedom and a straight line down the bank to the lake.  Ren panicked some at the sight of her tender child so close to innumerable objects of disaster, so of course she cried out for her to stop.
In that moment Raquel looked up with a proud smile of her accomplishment and gleaming deviousness only a child of mine could have coupled with the confusion of her mothers strong disapproval she wavered, pivoted and started to fall backward off the last stair to the rocks and dirt.

The rest of the weekend was just that.  A continuation of lax parenting and near misses and massive blisters, but what remained and strong was the smile on her face.  As it was my paradise as a child, it's clear it's going to be hers too.

No.
She didn't fall, somehow she regained control and stayed put till Ren grabbed her and carried her kicking and defiant back to the closed circuit of the deck and cabin's main floor.

I was able to do part one in my dream for travels with my children into the wilderness at a later date.  We had a short canoe ride - except she now has a huge hole at the bottom of her chin where the life jacket started something that has clearly gotten out of control.

The drive home was also something we rarely try.
Daylight confinement.  It has so much potential for disastrous effects.  It just takes 20 minutes in a car seat with an irate kid with Collagen issues to lose a lot of skin, but with a 5 hr drive plus stops we only had a minor outburst which was resolved by dinner and some hideous music at a restaurant in Chilliwack.  The kid has got a passion for music and made every table she passed smile in amusment as she shook her booty all the way down the aisle and then continued outside till she finally saw Renata disappear behind a corner.


Today's dressing change was a complete blood bath.  I had an idea it wasn't going to be fun with how many new blisters she'd gained over the weekend.  So I made a call out to Dammit Janet for a little help.  I figured why ruin a great romantic weekend with my wife when I can yell at one of my oldest buddies if things go wrong.

In the middle of buying a house she took time out to come over.  In true reciprocal friendship I was late returning from winning a bet with a friend down a trail called Pipeline.  Happily I indulged in destroying his sense decent mountain bike ridership.  I'll try not rub it in any more, but after living with EB for two years giving him a break for having strep throat, bronchitis, dengue fever, flu ???  I think that was all of his excuses, anyway my level of compassion is a little thin, if any even with Dr. notes.  hahahahahaha...  Sorry Mr. Man but even with a 60 second head start on a trail that takes slightly over 6 minutes for me to travel he didn't even try.  Well he did I guess if wanting to change the match counts.

Oh yeah. Dammit Janet.  She rocked.  It was a horrific change.  Raquel was shaking with pain at various points till the pain meds really started to help out.  There were huge bloody blisters and pockets of fluid at nearly every appendage.  The floor was a mess, splattered with the various liquids.

Ren was a mess and shaken by the experience, her turn for that melancholy and infinite sadness.
Can't imagine not having help on that one.  It did turn out to be a three person job anyway.
I was lucky as my ride numbed my senses enough that it didn't mentally effect me as changes of this magnitude usually do.

Seeing that the week ahead is likely going to be a measured level of chaos and sleepless nights with screaming child I sent a quick text to the second/first or other super mom that's not mine or my wife who takes great and unmeasured care of Raquel - 'Hey Blood bath here, you up for 2 hours of respite this evening'.

Two minutes later Simona calls and tells us she'll first feed six people then come over (40 minute drive) in an hour to an hour and a quarter.

So we snuck out watched a movie about a bad vulgar teddy bear, bought food then came home to sleeping baby.
But not before getting her a bottle BC wine.
The name sold it for us.  Hopefully her and her husband will.

What did Dammit Janet get?
I stood in the kitchen avoiding thought texting taunts to my vanquished friend and didn't even say thank-you as she hurried away to possibly buy a house.

hmmm.

Raquel and her parents are very lucky to have somehow been able to keep such extrodinary people so close for so long.