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.