Monday, January 30, 2012

Definitely on the mend

Raqu has been in wonderful spirits in the last few days.
What a relief.
She has been playing talking walking and starting her terrible twos just a little ahead of schedule.

Try pulling something out of her hand she shouldn't be holding.
Scissors, felt pens, knives

She just seems to arrive at the right place at the right time for maximum possible destruction.
I'll be filling the dishwasher and suddenly she's shown up from the other side and is already looking closely at the blade of a chopping knife.  Etc etc.

I have 'My German' here for a visit.  When we were kids he used to introduce me as that.
'My Canadian', the accessory every German must have.  Anyway, it got some millage for me this time.
Andrew and I brought him up for a ski to Whistler.  It was my goal to get him to a 50+ degree slope with a bottom a km away.  Watch his legs shake and have a good giggle as Andrew and I nurse him down to safety.  There were a few things that got in the way.
A- he's known me long enough to know something is up when I'm telling him, 'don't worry this slope will be fun'.
B- he's known me long enough to know something is up when I'm telling him, 'don't worry this slope will be fun'.
C- he got some type of vertigo from the white out conditions above tree line which made skiing for him that much more difficult.
D- we tired him out before I got to say, 'don't worry this slope will be fun'.
By the time we were down and in for an √Ępre ski beer, like a good beer drinking German he started to feel normal again.
Dammit!  Why didn't I think of it earlier.   Fill him full of beer first, then take him to 'slope of doom'.
I guess there was an
E- large injury.
I took his sister there some 12 years ago with a few of her friends, one of which was a beautiful German woman, whom while on the slope of death busted her knee.  It was a (achem) good four weeks as she wasn't able to fly home and had to be nursed back to heath.  I don't think I'd be all that interested going the extra bit for Jo.  Carrying him to bed kind of things.  Plus, I'm quite sure his wife would find a way to shoot me from a third of a planet away.

Andrew turned 40 yesterday and now joins an ever growing group of buddies from way back to be officially
O
L
D
It's no fun if you can't poke at your buddies in a moment of weakness.


Yeah, so Raquel is good.  Nothing to report as nothing is stressful, no difficult late night hours, just the odd outburst of a totally normal slightly spoiled 1 and a half year old well on her way to being a full fledged terrible two.

Sunday, January 22, 2012

Update 01/22/12

Raquel had been fighting an ear infection, so much of the turmoil of last week had a direct correlation.
It was only shortly before we met with the doctor when we figured that be the root of her suffering and ours.
Looking for EB and we find baby.
Now that she's on her first course of antibiotics everything to do with her has leveled.

Of course once her feet finally skinned over on both feet we get this today.
She has been telling us lately when things hurt, so now we'll investigate quickly.
I'm ashamed to admit we weren't quick enough on this one.
OR this one.
I circulated the next image to my friends titled-
Vampire Soup

I was shocked that no-one found it funny or thought it engaging enough to respond.
Oh well.

I think when Raquel looks like this while I'm pressing her punctured blisters like a grape to squeeze the remaining blood and fluid out, I can start to find humour in whatever ails this family.
It was a disastrous week for me on nearly every front.
So much so, that my car sits outside motionless, as I can add two sets of keys to my lost radio and wallet for the year.
No wonder I thought Vampire Soup was funny. 


I'm losing my mind....

At least Cordelia and I had a wonderful night skiing on Saturday.  After our hot chocolate we took the tram down with all the teenaged hooligans as the mountain closed.  The ride was full of colourful language and disrespectful youth.  Hopefully I'll be the better role model for her than what she hears and sees at all the places I take her at hours beyond most other 6 yr olds. 
haha
Probably not.
'No soup for you.'

Tuesday, January 17, 2012

Almost breaking the cycle

If it weren't for Raquel's hints of a fever or flu.
She's thrown up her food a few times in recent days and has been a super heater.
Actually most of her full feeds come back up.  The best part is when we see the streaks of blood mixed in.
No, the best part is the nights.  Which have been horrendous,  Raquel has been clinical with almost an hourly precision of waking, crying, flopping and twitching.
How nice it would be to have only one or two symptoms.
Blood in mucus + fever.
Or large open area on one foot + sore throat.
Maybe even pain in belly + with a high temperature.
Drugs are our friends at this point.
Falling so far in such a short time.

It was fun when Ren was gone.  I slept through the night, had lots of space to stretch out.  Now it's sharing couch with baby till Ren finishes her first work week in a year and a half.

Feels like I haven't really worked in a year and a half either actually.

I think last night I only spent 3 hrs in my bed.  Glad our couch is extreamly comfortable for nearly every slouching position.

Raquel's blisters are still coming fast and furious.  New ones of various sizes pop up regularly.
Three more on the exterior not sure what's making her vomit streaked in rouge though.

I only wish that when I remove the outer layers from her dressings -
A  it doesn't smell like a brewery / fermenting flesh
&
B  I don't feel like I've found an ancient Egyptian mummy and am removing odd and discoloured wraps that look centuries old, if it weren't for A.


Her knees have looked like this for months and months.
I was hoping they'd look better once she started walking.
Maybe not just yet.



Despite it all she still looks rather stoic.



I had a message from Casey's mom Beth today with like images of painful looking feet and other stories of EB horror.  She mentioned we should sing a little EB chorus of disdain.

I'll do a take on the Canadian chorus so often heard in hockey rinks.

EB you SUCK!!
EB you SUCK!!

Thursday, January 12, 2012

It's not getting easier

Raquel has been in tough for a while now and it's not getting better at all.
I had hoped by last blog that we'd turned the corner, but this is not the case.
She keeps getting blisters and the healing ones look nasty at this point.  She's been shaking in her sleep by painful tremors that shoot up her leg and the issues carried on into the morning.  Yesterday I finally phoned our pain Dr. for some advice about my taking her off her morphine a few weeks ago.  Roughly a week before it started to fall apart for Raquel.
In fact I think we're now in new territory with the amount and size of blisters on her body.  It's really hard to imagine this type of scenario while Renata would have been away in Czech.  Or worse me being away and having Andrew or Dammit Janet doing changes.
I'd have lost my marbles looking at the images from afar.
But still, once Raquel gets her chance and she has enough medication to break through she's back to her playful self.  It's been quite amazing to watch hour to hour, day to day, balance vs mental and social breakdown. 
I hate the fact I have feelings sadness when I look at my battered child's skin. 
I hate that I have to make her so uncomfortable while lancing and cleaning her sores.
Thankfully today she was out of it, due to her low dose requirement from being off it for so long and didn't suffer too much while I was poking and prodding at her feet while draining two new large blisters.

Anyway, she'll get over it.
Soon, I can only hope.

Sunday, January 8, 2012

Moody Lady

Three weeks ago I had strange thoughts my daughter may have magically grown a gene.
Things were actually on the border of being that good.
But then Renata starts to feel the pressure of going back to work.

And all starts to spiral and all the ladies in the house hold have become rather moody.

Raquel gets a pass, kind of....
Shes the only one without Invisible Monsters.
All hers are there for us all to see.  She is absolutely covered in raw sores right now.  So much that I'm having a hard time remembering her having so many large open areas ever.
The previous blog post of her blisters being a non event has turned out not to be the case.  It's been a problem, it's been an issue, although the odd thing is - recently Ren nor I any longer feel the stress we used to when she's been in such a state.
I turned the corner while in Netherlands, Ren turned the corner while in Czech and possibly from her meeting with Dr Diem at the Salzburg EB clinic.

I'm not saying it doesn't break our hearts to see Raquel suddenly stop what she's doing and point to her wrist

and say
Owww.
Owww.

Ren today noticed her point to her knee and explain Owwww.  Then Raquel on her own accord bent over and kissed her own knee better.  Soon she'll do her own bandages.

Every dressing change over the last couple weeks has shown two or three more large blisters.  Yet today, finally we've had no more.
Has our down slide ended?  Even better Ren and I didn't have a stupid argument about nothing.

Hopefully last night was the end as we were woken to her mumbles of pain and discovered a large circle of blood on her pillow.
The source a the trail of blood at the side of her mouth.
Standing there trying to figure out how I was going to get my arms safely under my crying thrashing baby I moved on to thinking, shouldn't I be freaking out right now? 
It was simple.  Pick her up, hopefully safely then wait till she calms.  (Kind of)

The other two girls in my house have also been a little out of sorts. 
One grumpy school has started and the other nearly off her rocker as work looms ever closer.

It's about loss of freedom. 
Freedom? 
8 hours to think about something else then at an instant your child could fall and wipe the skin off half her face.


Anyway, it's not been a pleasant little while as we've been climbing to this seeming apex of chaos.

Back in the day, I'd have disappeared for days on end.  Out of a hat I'd find some fishing trip or a ski tour to camp in a quiet alpine meadow and bask in the void of responsibility.  Umm other than not freezing to death as the sun dipped out of sight for the next 14 hrs.

But not this time.  I must have finally become a parent dedicated to the cause.
Oh right and the current mountain conditions are terrible and dangerous.
Someone please tell the Pacific we don't want any more air coming from Hawaii. 
Build a stable front and let it come down from the Arctic.  PLEASE!!


Thursday, January 5, 2012

Blisters shown to be a non event

Virtually nothing has happened as a result of all her blisters.  Her mood or evenings have hardly changed.  Even today's dressing change happened without much of a fuss.
Last night she had a few moments where she flinched, the pain or discomfort likely originating from her foot.  Over all, she's just cruising along.
I can't figure her pain.
Every time I think I know, I find more reasons to not know how she can deal with it or we can deal with it.
She's tough. 

I lost a bit of my epidermis yesterday moving a planter with an abrasive edge.  It simply wore off a nice patch of skin at my knuckle.  Of course every time I forget it's there and I pull on a shoe the abrasiveness of my sock creates a decent bit of discomfort.  OR when Cordelia grabs my hand.
Either way I whine about it and I've done more whining about my knuckle then Raquel has about having areas 80x the size in a similar state all over her body.
She's tough.
She's our Super Trooper


Monday, January 2, 2012

End of Days.

What a turn of events.
Renata and I were in the easy chair relaxing in the common glow of a family without strife or suffering.
Raquel had no major blisters, other than this moose without antlers or legs -
Started Boxing Day Dec. 26 - above Jan 2
In a couple years, Raquel will probably wish she could just have the moose on a T-shirt.
Maybe it's a female moose standing in a marsh.

Then today strikes.

I guess it started at 4am last night with the strange problem of not being able to comfort her.
We've completely forgotten what that was all about.  Since the morphine had been dropped from a regular occurrence we'd been smooth sailing for almost a month now. 
I tired to wait it out last night, but after two hours of thrashing and waking the moment I moved to take her from my chest we broke it out.
Once her first diaper and clothes change happened in the morning, Renata found a big blister on each elbow and one knee, as well as one that wrapped up and under her armpit.  Later during the dressing change we discovered even more.  The one at her wrist nearly wrapped right around, then this monster below.
Ones like this below make me want to do changes everyday, like I know others do.
This one had enough fluid to fill an espresso cup.  A real espresso cup.

Today was the first time that I got a sense while lancing it that Raquel was pissed at me for hurting her.
Maybe that wasn't it.  But while she cried and shuddered in pain there was a different look in her eye.
Once we slathered it in Aquafor and wrapped it in zinc she settled considerably, she even continued to walk around after, but that new cry of hers might become an issue for me later.

She's grown at what I think is her biggest jump so far in the last month.  It's been amazing to witness.  Which is probably why I haven't written much lately.
A-there was no time
B-everything was blissful.
Who wants to hear about good times??

Now that we are edging back into a very uncomfortable run while these areas all heal, I'm sure I'll be writing to get it out so I don't carry it to sleep and through my days.


This is how Raquel tries to avoid her hands being re-wrapped.
I think she thinks if she can't see her hands I must not either.
It's very cute.  I try to only start wrapping once she drops them.  It's a bit of a game, but ..
By the way, the new technique of using tubes of the Derma Silk under her conforming wrap works great.
The only problem was that during the last dressing change I left it a bit bunched near her wrist and was the obvious factor in what happened today.  Again, a lazy slip in bandaging.
Won't happen again.  Although I just had the idea to cut up her silks that are now too small for her to wear and slip them underneath her conform bandage.

Then there was Cordelia trying to be amused while we worked on Raquel.  In the hour it took to do the change Coco depleted her hair band stock.  I asked why she didn't also do the front of my head?  She shrugged her shoulders and explained that she ran out.  It wasn't till the painful removal of each of the elastics, that I believed it to be true.


Renata will be happy that Raquel will no longer be using this chair.  I have to say it was very useful while Renata was in Europe.  I could count on her staying in it while being g-tube fed if I had to do some cleaning in the kitchen.
 But this one is far better.  (Thanks Santa! it even matches Renata and my couch) Not only can you feed her then split to the kitchen, we can have Cordelia sit watch at a comfortable proximity and keep her company.

Our typical night.
Dad watches a drama that bores Raquel to sleep.
Except Rango is now offered on Netflix, Cordelia battled me to turn it off while she tried to sleep since she was only able to watch the first half.
Some how I was able to convince her to close her door versus me turning it off....
Gasp....
The dragons or monsters didn't even get her with her door closed????????