Sunday, February 24, 2013

Blood and Suffering

Often my mind is a running mess trying to anticipate or rationalize Raquel's needs or what we think they are.
Last evening I was told she is tippy toeing around, as I'd been off working for the day and not seen Raquel for most of her waking hours.  The question was how bad does her weight bearing look?.
Ren asks should we do a change?
I responded tired and ready for bed, go see what Raquel thinks.  I hear from a way away that she doesn't want one.
I seriously take a moment to think about it.
Let her suffer through the night and be sure to get up every other hour or do it now, but now is time to go hang with Coco and read french books and tell her spider stories and use the excuse of a little spider to tickle her relentlessly and giggle ourselves to sleep.

After staring at the ceiling for a few minutes it was clear what we had to do.

We hop Raqu up some to side step some of the pain on it's way, then start a quick procedure on the bothersome foot.  Once her sock is off its clear from the bumpy bandage being pushed apart from below with crimson red that it's big and ugly and the right decision was made, but by the looks of it 24 hrs too late.
Ho hum.
After some screams and cries and a deluge of fluid to the floor shes nearly asleep in the chair as the final wraps and tying off of the conforming wrap are placed.
Fifteen minutes and its over and we've handled the biggest blister three years ago I couldn't ever have imagined.
By the time she's out of her chair and getting her night clothes on she has perked up and is acting like a party rocker.  Chit chat with mommy and happy camper all around.
The edge of pain is gone, but I still find myself contemplating the whole pro and con of our pain scheme.
Yet as I go for a night kiss Raquel holds up her foot and says 'I can walk now, I'm good'.  Her eyes are clear (not swirling in her head) and voice natural.

To suffer or not to suffer.
What is pain for a child that cant really describe the level or the source in most cases?

I understand many or most kids with EB aren't on a base dose of Morphine among other drugs, so I keep having to justify it to my self, second guess, ignore.....

It's tiring managing day after day after day.
.4 for morning, .8 in afternoon, every four hours .6 or every six hours .8, add tylenol, or advil as required, maybe an extra dose of anti itch for daylight hours? Constantly watching and adjusting depending on if she's less inclined to walk, open her eyes or more inclined to attempt to sleep off suffering and often enough cry for hours on end.

Bla bla bla, feeling sorry for our lot and EB sucks.

She didn't wake up before I left this morning, so at least the nurse makes the call this morning.

Saturday, February 23, 2013

Debra Canada T-shirts and Broomball event

We are trying at Debra Canada to raise a few $'s and if in the near future we can attain a similar bracket as our European and American counterparts.
In the last year I've been volunteering as a Director on the Board for Debra Canada as well hold a position as the Medical Assistance Fund Liaison.
Which covers cases where our medical funding runs short or doesn't cover items that can help people suffering from the severe effects of EB get closer to normal function.  I'll take the case and present to the rest of the board, where we then decide if we should or can fund the applicant in part, full or unfortunately sometimes not at all. The solution is based invariably on our resources and or the resourcefulness of our fellow board members.

As a little awareness and small money maker a few of us board members created these shirts for sale.
They are $20 cdn plus tax and shipping, go check it out.

Also in March Grouse Mountain will host a fundraising event for a semi holds barred broomball tournament.
We're hoping to attract all age brackets and companies to donate and take part in a fun picturesque mountain top experience on the skating rink.  If you want a small glimpse into the precarious life with EB try running around on an ice rink without traction control while chasing a ball with a broom, defending and attacking a small net.  One slip of many and you'll have a nice little bruise to remember that someone like Raquel will be missing skin from any such mishap and in her case it's cumulative and dystrophic (disfiguring).

Check out the Grouse Mountain website come Tuesday for details and registration.  All proceeds go to Debra Canada and for those who know Grouse the Broomball fee for play is very similar to the skyride ticket, which shall give you a good idea of how great Grouse Mountain has been in helping develop and run this event.  There is a bar, restaurant, cafe, coffee bar, movie theater, ski and boarding, snowshoeing, zip lining and of course the best view in the Lowermainland - BAR NONE.


If you have a company and would like to donate some door prizes to most bruised athlete, most scored on team, youngest player to score, oldest player to score, most likely single adult to score and of course the most likely married adult not to score. Think about it and I'll provide all the necessary links in the coming days.

Wednesday, February 20, 2013

Raquel Helmet

Dammit Janet had us for dinner.
I thought I'd have Raquel tell her what the name is.

Tuesday, February 19, 2013

Not blogging because I hate the upanddown

One to two days we don't see an eye ball.
Then out of the blue there they are and it's smiles giggles and a peaceful night sleep.
So quiet that when you wake you feel the worst possible scenario has happened while your body was sunk sleep deprived into an unstirable deepness.
Then just like it ended, it's whiny cry baby with voice and suffering that can only be likened to needles and glass shards scraping and poking at the back of your neck.

Just a humdrum existence using nurses to help keep the love and sanity for what this stupid disease has done to my little beauty.

Simona sent this from a week or so ago.
At the moment Raquel looks like she's run into a prize fighter.
The last bad spell her face took the brunt of her suffering.

Tuesday, February 12, 2013

Sore but happy

We never did go in for Raquel's plaque removal.
It was due to no more rooms in the Inn.  Considering Raquel is just having plaque removed, the more serious issues to do with life and death took precedence.
Understood, but fairly annoying having rushed and stressed and sent Ren off to the Hospital with one of our nurses with me just on route to find out that we'd been pushed back in time indefinitely.

Raquel was pleasently given a wooden block set for her troubles.
Ren was given the opportunity for stress counseling for her troubles.

Raquel now has some serious blisters holes on both feet and tippy toes around.
I can say we've managed her pain exceptionally well in last week or so, as she complains seldom, but it's easy to see the suffering and underlying issue.  There are only a few other sores present that look painful.

It's just simply shocking to me that she can be so wonderful in temperament for most of her waking hours.  I only had to jump in at 2 am for a top up of the anti itch meds and pry her feet apart while it took hold and she melted back to sleep on my chest.

It's been a while coming, but she's actually started to ask for me on occasion and I'm not feeling the third fiddle to my mother and Ren.

Images of peace and a few hours respite Sunday.


Wednesday, February 6, 2013

The Van Strangler

Ren wondered when I'd get to this, in fact feared when I'd get to this.....

I'm not sure if the pause was because the event needed to percolate some or that I had to wait and be sure not to have too much residual irritation toward her that would sour the post - ie our marriage......

Our two princesses have issues going to bed and that's just a fact.
We take full responsibility in that we don't really want them off so quickly, meaning 8pm or earlier as we don't get to play and have fun as coming home from work, dressing changes etc would only allow a solid two hours.  So things get drawn out, sometimes a lot.

Never mind that for the last 2.5 yrs Raquel almost always wants one of us to drift out upon.
For some reason out of the norm Ren had Coco going to bed at 8.  So the full pre-bed rituals took place much earlier.  But has habit trains the mind and body, Coco couldn't relax and every few moments she'd rise and walk out, thus disturbing the work being done on settling Raquel.  At one point they were both almost asleep so when Coco comes out at 9 asking for something to eat and Raquel jumping up in anticipation of getting to feed someone again, the second wind came in at full force.  Within minutes they are as active as at 8am and are milling about causing a ruckus.

I think I was taking a back seat to this operation as they were both clammering for Mommy to snuggle with them.  So off to Coco's room they go.
Seconds later I hear angry/scared elevated words from Ren repeated by Sh&$ in a quick staccato rhythm going on and on.
Welling in the background are the cries and screams of Raquel.
Within a second or two I'm standing at the threshold of the bedroom to see Raquel standing in the middle of the floor crying and Ren sitting on Coco's bed still repeating her singular SH*#.

It's a little gray the conversation at that point as Ren is nearly incoherent as is Raqu.  I'm pretty sure it was Coco that explained what happened before Ren was able to fill in the details.

Short story long - Raquel was despirately trying to get onto Coco's bed to join in on the cuddles and used her only way up.  So one foot on the baseboard heater, one knee on the blister maker bed rail then a semi jump climb to the pillow with Coco.  Problem was that Cordelia has a long twine rope hanging from her window that has puffy cloth letters for her name spaced evenly over the 5' they hang to the bedside.  Raqu, while climbing up had this twine rope tangled at her neck.  As Ren panicked that her knees were about to take a beating she picked her up (in the dark room) and walked toward the door to hand her off to me.  Except, after two steps the rope tightened....

I think it's OK to blog about wanting to tighten that same rope around Ren's neck moments later, but I'm sure common sense will always prevail as life as a solo EB dad in jail could be rather daunting.

I picked up Raqu and went to the change table and did a little wound work at an impossible part of her body full of folds and bends to have things hold and not cause more damage.
Once she was in better spirits we went back down the hall and Raqu walked in and told her crying mommy

"look mommy, I'm OK, daddy fixed me".

Except a good portion of her skin from the front right side of her neck up around the long way to the back right side of her neck had a distinctive slasher look helped by the drops and streaks of blood covering her collar and shoulder.

I still think it was harder for me not to get mad at Ren than is was for Ren to get her self to stop crying from her darkened mindless blunder.
I have to admit I've seen Raquel actually wrap that rope around her neck before and when I freak out to tell her to stop doing it she only holds it tighter and tries to get away from me with it still attached.
So as much as I bug Andrew about Darwin's theories and potential awards for his boy, Raquel has some basic evolutionary issues of her own less the missing gene Ren and I were not able to pass on.

The next morning our nurse had taken Raquel to a toy store for a walk and a little play time.
I'm sure it's easy to imagine 'the horror' of the mothers present with their children in the area looking at a child with distinctive strangulation marks around her neck and large patches of red raw oozing dermis.
Meanwhile Raquel was about business as usual.  Joyous, playful and determined not to let a mere flesh wound get in her way.
Still - Monty Python And The Holy Grail (mere Flesh Wound)

Monday, February 4, 2013

How much would this hurt?

Ren has asked that I give her parents and family East of the Atlantic some more images of our crew.
Except as I was finally roused this morning from a flying knee to the kidney from my first born, without malice I'm sure, I hear a stream of Czech that includes a few words I can decipher mixed with the helpful handful of international words that she's complaining I'm getting up late.
A late riser is not my M.O., that's for sure.  So the one Sunday I do every 6 months or so I'd be nice not to get pulled  under the bus by my wife to her parents.

Not sure if I've written the story where her father gave me a quick judo move to get my attention when seemingly unsuccessfully telling me to treat his daughter with love & respect in broken Czechglish a couple days before we got married?  Of course I always intended to be wonderful to Ren, as long before we were married she was one of my best friends and it was my relentless pursuit that finally broke her down so that she'd marry me.  At that time I just didn't feel a speech like the other boring father-son 'birds and bees' talks was necessary. 

Our talk ended as a man at 60 had me weightless and pivoted on his hip in a gentle mile-second.  A struggle by me either way and my forehead or the back of my skull would have met granite cobblestone.  Of course I quickly agreed that I'd be the best husband 'EVER' before he let me down gently to my feet.

So, I'm slightly sensitive when it comes to him and my perceived good or bad deeds.  This runs deep as I don't even know how to thank him for all the great things he's done for us in the last two years to keep us still standing on our feet.

All I have is some gore.  This way I can at least get a mercy moment based on my daughters suffering....

Although, Raquel had been completely unfazed by this and has 'slept like a baby'.
I see the itch coming...

This week we have some serious tooth plaque issues that will be dealt with finally.
Since we've stopped Raquel from eating the plaque builds up and in places has completely covered over her teeth.  Irritatingly the holistic issues spin around and as one issue gets solved of sorts another surfaces.  Save her mouth from scars and getting quickly to the point she can't open her mouth by eating normal food and having 2 dialations a year or work on the plaque.
Clearly plaque is the lesser evil.  Except she'll be intubated this week, which if done poorly will bump up the next dialatation and a week or more of suffering and sleepless nights.
Oh joy... 

This is Raquel with furrowed brow guarding her drawing from her dad.  Her right hand is very quick to block and jab.  She really is quick to react and goes right for the eyes should you get too close.
This is a very similar look and battle that takes place during dinner when she's guarding me from Ren and Coco's dinner and desert plates.  Raqu gets furious if she sees me attempt an extra piece of chicken or chocolate.
Since she doesn't eat per say she is very diligent to feed us and make sure we eat our allotted amount.

Friday, February 1, 2013

To inhibit or not to inhibit.

Raquel has been good, bad, moody and excellent, roll the dice on the day.
All the indicators I thought I had no longer hold true for any length of time.

Yesterday we cut a giant blister on her foot that dropped a big puddle of fluid on our floor.  I was shocked at it's size as I haven't seen one that size since last spring.
It might have had to do with her jumping in the snow.

I'm proud to say she's hit a new milestone in agility - sort of..  I watched her in glee as we had her in the snow six days in a row playing to her hearts content.  She's finally figured out that she can jump simultaneously with both feet to get air then the hobbled one two step she used to do. She did it over and over till she was sure we all understood her new ability.  I cringed each and every bounce, but I couldn't bring myself to stop her.  I just held to an internal theory that the soft snow underfoot would lessen any friction at any point of her foot.  Clearly the theory was wrong.  Didn't help that we waited two days to do the change there after.

Once snipping the side and watching the three second pour to the floor, I pushed out the remnant fluid and traces of blood, Raquel flinched, shuddered and cried.  Blood stains quickly pushed through once the bandaging had been reapplied as she changed from pain to her typical pain in the neck personality.  Hardly showing that moments before I'd been hurting her.  Then she continued in good humour to finish her waking hours then sleeping through the night without hardly a blip.

I don't get it..  Again EB is a mystery.

On top of that she's had the most body blisters I've seen on her yet.  I'm sensing a point that I might have to wrap larger parts of her torso should this continue.

The hard part, is that while at home we're quite rambunctious with play and she carries that on without much fear in the less safer zones of her daily life.

As much as taking Raquel skiing in a backpack may seem a little edgy, going tubing down a run in Coco's lap was NOT!! going to happen.

To inhibit or not to inhibit?