Thursday, June 30, 2011

Update.

Raquel is doing very well of late.
She's crawling around and acting like as normal of a kid as we can hope for.
She has a couple blisters healing on one of her knees that are 5 days old.  It's interesting watching her crawl around and changes to tripod mode once off her safe zone.  Keeping the sore knee up with her foot flat and bent knee on the good side. She protects it well and no new blisters have formed on the butterfly pattern on her knee.  The other knee just has little nicks and spots, nothing major.
Oh one pinky has a decent blister covering it's entire length and should be painful, but she's coping very well.

She's finally moved out of our room and seems to be sleeping better where she is.  This was because Renata's sister and our niece is her occupying Cordelia's room.  Cordelia took over Raquel's crib which is very funny as it's all kid.  No spare inches there anymore.  Full house and it's 5 girls 1 boy.  Raqu has been great or we don't hear her or her us and seems only to need a little top up of food to get through the night.

I've been working very hard lately as Renata's sister is here to help out, so I disappear early and come home late.  It's been great, finally getting lots of work done.  Except I only get to see Cordelia and Raquel an hour or so of  each day over the last week...
Well last night was because I went for a four hour bike ride that ended up a friends house and beer...  Not sure I can call it a four hour bike ride then, oh well, I was covered in mud from head to toe so I looked the part when I arrived at home.

We're headed to the cabin for 10 days.
Should be good, except Summer has not come to Canada yet!!!!  Cordelia is excited to continue her dominance in the fishing department.
If I catch one bigger than hers I'll be in trouble.  She's gone a month and a half talking over and over that she's caught the biggest fish to date....  17" dad!

We've decided that we're going to start to push Raquel for a G-tube and avoid what seems 'the inevitable'.  We've spent the last week reaffirming our idea that when she gets that real bad esophagus blister and resultant blockage and then add something else with her health, we won't be behind the eight ball.  She'll be able to get the nutrition, vitamins and medication and what ever it is will have a better chance at healing with the minimal suffering for all.....

Last night was funny.  I often let Raqu play with my Iphone and she's figured out how to check out different photos and push the arrow to watch videos.  Clever little girl.  Problem is when her hands are dry (her silks) she can't slide the images.  So I have to dampen them (Water + expensive electronics=...)
Anyway, she's a monster.  I take it away to help and she freaks out. 
I love the sound of 'pissed off' Raquel as opposed to all to common 'suffering' Raquel.  I actually enjoy the little giggle I get when she's misbehaving like a 'normal child'.

Yes times are good.  But like I've started to notice in our previous cycles we've about used up our good days in this stretch and am looking to our next big down turn any moment now. 
Week on week off.
Problem is where we're headed for the cabin it's an hour and a half from the next city.
Oh well, we're pro's now.  We can handle anything.

Lastly.  I'm not sure if I've mentioned this or not.  Vancouver has another little child with EB born just over a month ago.  Not sure details, just know little tid bits. 
It's the SH*)S.
So much suffering. 
I was hoping that at least with the odds of 1-1000000 D-EB we'd taken Vancouver out of the game for a decade or so.  Really sad.  I wish the family all the strength.  I'm glad we've been able to lay some ground work at the hospital and in the community though.  I imagine it's taken a good deal of stress away.  Although each situation is what it is.  People adjust to the stress no matter what it is.  It sounds like they have it managed and are doing well as can be.  Hopefully they contact us sooner or later and we can be of some assistance.  Mind you, I can't imagine the other father want's to hear me critiquing his dressings like I do to our nurses. haha

Friday, June 24, 2011

The slippery mountain slope.

Well, was that a smooth fall from bliss or what?
Currently we are in chaos mode, again.
It's lucky for me my memory is short and I'm able to live in the present of good times, save good feelings in some subconscious bank then dole it out when we're back to suffering sleepless nights of watching our little girl bash her skin off.
It's been three days of her not wanting to open her eyes.  Ren is off to children's again since this isn't going away.
She gets her eyes open for a couple hours, but it's usually when the back ground irritations are at a minimum and or the Morphine and Tylenol are in perfect working quantities.
Then, in the time it takes to look at your wife and say,
'I think we're past it'.

Raquel rolls over does a face plant into either of our knees, her padded safe zone or the dangerous wood floor and scrubs off a nickle sized piece of once beautiful living flesh and cycle repeats itself.
I'm reminded of the French labs back in school with the earphones.  Repeat after me.
Le nez de mon enfant est un désordre rouge et ensanglanté.










Rudolph our Red Nosed Reindeer, once it starts to heal anyway.
Today I'm shattered...

Ren?
She's so much stronger then me....

Sunday, June 19, 2011

Life is sooo good.

If things can get any better I don't know.
Raqu is crawling and wandering and we've backed off big time.
Her level of world interaction has gone through the roof.
She's a fan of cables, which is a little troubling, but after 11 months of a pooping crying sack of baby drool, her quickly becoming human can have it's initial loose boundaries. She's so happy and determined clamping down on her in this great age of enlightenment seems cruel. So until she pulls a laptop on to her head it's free rain!!!

Saturday, June 18, 2011

I'm getting greif from my mom

She's a little irritated with me that I don't often include how great Raquel is.
Mom goes on about how great she is when she comes for a visit and enters the door and Raquel points up baba!
The smiles and goofy antics (now that she's fastly becoming human).
We had a bunch of tests recently.
She's on the curve for expected growth in exactly where she should be and right on what Cordelia was.  The area where she's ahead of the curve is the giant HEAD she has.  I could do a few quotes of "So I Married an Axe Murderer", but much of the world won't understand the references to a little bit of Canadiana.
Oh Ren just informed me.
She's tenth percentile in growth, but 90th in head size.
So her X-girl super powers just might be within the size of that Cranium.

We've had a very very good patch this week.
Raquel has been happy and crawling up a storm.  She just puts her head down and trudges on.  She likes the power cables dangling from the lap top we watch netflicks from.
I just heard a story from Czech that my nefew found some scissors and cut the cables to my sister in-laws laptop.  hmm.
That would have been fun.  Lucky for him that the handles were plastic....

The european current is a little more intense then what we have here in North America.

I'll add some videos of Raquel charging around once Cordelia lets me on my computer.
She's got a little fever and has been watching Grossology.  It's all about Boogers and Colon monsters.  I've been told there is an educational element to the cartoon, but I haven't seen it.

Tuesday, June 14, 2011

Midnight Howling and My Little Xgirl

The little quiet moments we had today, where I'd thought we'd turned the corner, was - ahh premature.
Full fledged howling for an hour or so last night at about midnight, rocked our world once again.
In a moment of desperation I took her to the front door and outside for a cool breath in hopes of calming her. 
The shine from the nearly full moon lit up our faces.  She did settle some, as the change of atmosphere started to show it's effect.
My mind started to wander while I rocked her nervously.  The mind is a funny thing when it's stressed tired and not having a fricking clue what tomorrow holds.
With Cordelko life was simple. 
It was a dream in nearly all cases.  So what if she'd scream in her bed for an hour because she wanted someone to fall asleep with her?  Her parents just wanted the evening to cuddle and relax.
Just turn the volume up. 
Maybe change movies and watch an action movie. 

Fight fire with fire. 

In the end and what ever antic or malady came our way never amounted to anything.  She's simply a well loved, healthy, semi adjusted 5 year old who knows when the fix is in.  Even when I'm at my manipulative best, she never lets her daddy get one over on her of who's gonna eat the last chocolate, berry, pancake etc...  Coy and in fun, she plans ahead and almost always wins.  Then on occasion shows her humanity and shares with her vanquished foe.

With Raquel it's just not the same. 
What lengths will she go through in her battles of will and determination once an adult?
Not only does she have some strange gene coding that screws up the production of vital proteins in her skin.  That in a moment of miss care, clumsy movement by us or a miss guided exploration by her can turning her entire body into the surface of the moon in what I can only relate as a August meteor shower.  To what is a truly eventual episode.  Not exactly so regular as our wonderful northern summer nights watching stars fall on our atmosphere.  The world is fortunate to have the equivalent of Collagen 7 keeping out all those nasty objects from pocking the earths surface.  Destroying the beautiful forests, plains, wonderful cities, villages and scenery.
With Raquel it's the other anomaly in her genetic coding that troubles me.  A mutant coding.  A strange and so far one of a kind sequence not yet explained or shared in medical journals. 
It's random and confused. 
Out of sync.
What is it doing?
What will it do?
Is it something that will surface later in life?
Has it already started to take effect?
The full light of the moon was still shining on our faces, her forehead was scrunched in pain, irritation, hunger, over tiredness, stomach ache, morphine requirement or the clausterphobia of her wraps, any of it, all of it.
Who knows? 
Just then in the rock of my arms her forehead and cheeks slowly started to melt into a relaxed calm sleep.
A beautiful, quiet, fulfilling sleep...
I paniced.
I raced back inside pulling her out of the moonlight.
I can't have that mutant unexplained gene coding turn her into some type of Xgirl. 
One day she'll be off to save the world from doom and evil.
Ren and I will be the old couple sitting on our worn out armchairs watching her on the news waiting and wondering when she'll come home from her obligations that so clearly would be out of her control.
She's my little girl.  My beautiful little girl, I don't care that she causes great stress and suffering in Ren and I.
I just want a normal family.
The world can find another superhero somewhere else.




Monday, June 13, 2011

Turned the Corner.

The up's and downs of Recessive Dystrophic Epidermolysis Bullosa.  It's nearly always the same.
When I say to myself. 'Wow, we can't get better, Raquel is happy, minor sores, Renata and I are getting along well.'
Then it all falls apart....
Raquel is waking every couple hours in a screaming writhing fit, till her morphine kicks in, then once the first ebb comes back in she's an uncomfortable mess till she wakes full time in the morning.
That was three days previous our ER visit on Saturday.
It had just gotten too much and we phoned our pain guy, went over a few things on phone, but he advised us to go get a check.
I was certain with a little advise from Casey's Mommy that she had an esophagus blister and that time was needed.
But at the third day and it was the same thing all over again coupled with one of her longer pain cycles in recent memory.  The thought it could be something else, out side of our expert EB knowledge had to be nullified.  These days had pushed her to these limits of pain, irritation, as well I was getting well past my limit on how much nurturing I could provide.
Renata has a bottomless well of compassion.
I do not.
Can't help it, it's gotta be the way males and females are wired in it's simplistic form.  Me anyway.
In the last couple days after 30 minutes or slightly more of holding her in my arms and keeping her appendages from mauling the rest of her body while immersed in a solid scream, little bits of anger peak into my conscious.  Maybe it's futility.  I don't know, although I do know it's as far as I can take.  I'd had reached the point where I'd have to lay her down on the foam pad on the floor and just watch, distanced, but there, putting my hand in once and a while to keep her thrashes away from her face and the hard pushes and kicks to her feet and lower legs.
She's a mess, lots of superficial wounds around her face, but all major appendages have been spared this time around.
Ren, she can hold her walking around for hours.
And the dirty looks I'd get when she'd walk in with me laying at the edge of the couch watching Raquel writhe on the floor, is something else...

Superwoman...
Ren is Superwoman.

Anyway, once in hospital she finally ate more then 1/4 of her milk at a time, she cried for only moments and we knew while we were fast tracked (120 min wait for everyone else - 15 minutes for us) that we'd past the worst of it.  Her voice was hoarse but the pain was gone.  Although we were approved for 1/3 larger of a dose to get her through.  So?

In the end we had a reasonable weekend and by last night, Raquel actually started to crawl.
She was in control and made it off the reservation.
Jasonette Borne.
She was exploring areas she'd not been on her own in the small living room we have.
An arms reach for us, a world away for her two days previous.

So a beautiful upswing I suspect is in our near future...
video 
Cordelia's crying in the back ground is from going into the bath with a small 
abrasion on her knee from tackle soccer we had played earlier.  You can only imagine the compassion she must have had from her parents? haha

Which also means, we'll be living life and I'll not be blogging.
Oh yeah.
Renata turned 41 yesterday.  My wife officially has
O
L
D
haha
I'm of course still in my 30's.

And the Canucks can become Stanley Cup Champions today..
Go! Crush those B's who whine about a little finger knawing.
Funny, but other then some of our media, not many Canuck fans are complaining about Boston's antics.  Business as usual.
The Ascot for beer and cheer anyone?

Friday, June 10, 2011

It's amazing how much a little person can belt it out in the depths of a deep cry.
Amazing also is how strong that little body is.  Today I had two professionals helping on the dressing change and it was one giant struggle right on through.  She behaved during the bathing portion.  She always does.  I read all the stories of the other kids suffering through them, Raquel hasn't had one bad bath the entire time (?).  It's the peaceful fun portion of these regular affairs.  In fact the dressing changes have very few moments where I wish she had some M&M to calm her into a complacent state.  Of course today was an issue.  Glad Ren took off once the help had arrived and missed the whole shabang. 
To start at a two hour interval last night Raqu would start up and writhe in discomfort, the night previous it was about every 4 hours.  I apparently don't have heavy enough pillows to block out the sound... The most troubling is there are not any clear indicators to what is wrong.  She has no real surface for trauma that would bother her other then the thick ugly scabby skin at her wrist and thumb.  She does however have a mouth full of teeth all of the sudden and is a drooling mess much of the day.
Two top and bottom already through, but a second two at top are just about piercing on through to the other side. 
Joy.  Teeth brushing.  If it's not one thing I don't want to do in her care that another near impossible lifelong regular maintenance procedure should arise..
A water pick?
Air brush?
I can't imagine even the softest of tooth brushes go in her mess of a mouth.

What a life.  Not mine.
Hers!!
fu&8ing EVIL eb is.

We found out some genetic anomalies in the recent testing.
Apparently Raquel has some code issues past T.
I have no idea what that means.  Only that the T group of DNA doesn't exist in her.  What happens after that apparently is a mess. 
Like this blog wih ou he .
A jumbled mess of Shi  !
Pissed off.
Usually I can handle one or two problems with Raqu running concurrently, but when they add up like no sleep, stressful dressing change, a new anomaly gene mutation previously un-seen (don't know yet if that's a world anomaly or a clinic based statement), and no clear idea why Raquel is freaking out every couple hours to writhing levels, that's just too much for the day.

Once I get a geneticist to sit down with me directly and tell me what the results mean and not Renata's garbly gook 2nd hand info I'll pass on what it means.  I googled T-gene and nothing...
So really I have no idea what she's on about.

Off to see the Canucks give it to Boston. 
They'd better after what just happened in the last two games of the Stanley Cup Finals.  Maybe then I can get a vapid happiness by cheering for a bunch of millionaires playing games where they get to pound the living daylights out of each other. 
Yeah!!!!
That's what I want to see.
More carnage.

Tuesday, June 7, 2011

The joy of coming home to bandage changes.



Basically, I'm used to blood and bags of fluid pushing my daughters skin to creepy shapes and sizes.
But when confronted with it all after taking a even just a few day break, it's freaking shocking all over again, add jabbing her in the wrist with a large needle (accident), the pain I caused and blood soaked dressing bed after. I see how it'd be easy to check out full time.  Go hide in the arctic tundra eating berries and moose.

Monday, June 6, 2011

Great weekend except

While I was off fishing on the yearly Link Lake Special with a few of the boys Raquel developed a giant finger blister.  Since till this date I've been the only one to do her hands out side of the hospital Janet was up against it.
She even helped out that week and I was doing my best to show her what the newer techniques are.  As they change quite often do to growing hand and the subtle issues that arise.
Anyway, we'll be redoing the change now.
Hope Dammit Janet didn't make too much of a mess of the bandaging.
This is Ella's finger too.  It's had some issues of late.