Wednesday, June 19, 2013

Maintain the level.

After the dressing change ordeal the following day was up and down.  I think our newish new nurse doesn't fully understand the brunt of Raquel's drug requirements when her body is sore and what is required to maintain a level where she's not stoned or in pain.  This likely my fault for not laying out exact doses for day as I disappear for work for large stretches of day.
So when the drugs wore off and SH*& it the fan it was big time catch up and the stress levels and tension in her voice were alarming.  Then a few of the background issues that don't seem to be taken care of were pressed upon.

Such as Raquel's g-tube site, which has had ooze and blood coming from it.
I exclaimed it's been like that for a while. 
Yeah maybe.
Should we do something about it?
Hmmmm.  What does that mean I think to myself?  Another hospital visit? ER? hahaha

I did mentioned it to a nurse at Children's the other day if they could find someone to come look at it.  She asked who was the Doctor responsible for it.  I gave the GI guy, but that wasn't who she wanted or thought should see it.  In the chaos that was a 4 hr dressing change of suffering by all, she stood in the doorway asking who??  I think I ignored her or was more focused on Raquel.  She disappared then as soon as we got Raquel's final hand wrapped so did we. 

We've been given silver nitrate or told to soak it to submission with saline.  Neither works as soaking requires her to lay still in middle of day when nursing help is on.
The silver nitrate is used to burn it off.  That being another one of the evil things I do to her which requires two people to hold her down while I dab basically a flame at her innards pushing the feeding port out...  I've been told it's not supposed to hurt unless we get it on her skin.  clearly that's not the case.  There always seems to be a tender part of it that makes her squirm and cry.  More of the bad daddy.  So the burning of her granulation tissue gets overlooked when possible.


Don't misunderstand any of this or from the other day.  I don't blame anyone for any misstep or moment of chaos we're just moving through the best we can and we are all learning.  I don't even think we're doing a bad job even as some things slip by that would make me shudder in fear should Coco have any of these issues, being of full of collagen 7.

I hate excuses.
Yes it's bloody.
Oh well... EB SUCKS.

But then last night, when Raquel's suffering was eliminated the three of us played our rambunctious nightly game of monsters under the covers at their usual 9:30 to 10pm bed time. 

Everything was perfect.

She was doing her somersaults and her backward free falls into the down-blankets.  The kind where team builders use as trust, but Raquel is more interested in stepping off and rolling the dice that Coco and I are by chance watching her and ready to be out of the way and ready to catch her should she have not lined up where the bed was behind her.

Last night she caught my knee on the bounce back of her blind daredevil maneuvers and sports a 25cent skin smudge in the middle of her forehead.
Oh well.  eb sucks.

Good blister or bad blister?

Good I guess, as we just carried on once I applied a patch of mepilex across the length of her forehead.

Monday, June 17, 2013

The Good, Bad and Ugly.

Asking, does the irritating person.
'So, do you want the good news first or the bad?'

That moment in a perfect world, has the person holding the information for effect also holding a handful of chicklets. 

The Bad.
Ren has gone away away for family reasons that are tough and difficult to say the least.  We're holding down day 3 of 21.
The Ugly.
Day 3 of 21 saw a 5am wake-up, a difficultly consoled then eyes shut till shortly afternoon by the reigning short person in the family.
In the mean time I had my first Heritage Commission meeting discussing a year old project that's seen many turns, breakthroughs and fall backs to amble in to a meeting of make or break it.

Ummm, can you spell stress?

It didn't break, so on we go!!!

Then a nurse and my mother with Raquel met me at Children's Hospital within the hour for a consultation that was to give us caregivers the upper hand at dressing changes when her feet in particular need special attention.

It was a bloodbath...

It kind of went like this.

Deep breath.

Wait for Big M to take hold.
Remove dressings on hands so she can play and be distracted while we go to feet where I could finally get at some really ugly areas under her toes trimmed and tidy.  So that, in my estimation giving her a chance at good balance and walking pleasure should an eventual CURE or not come our way - bad IDEA!!!
As distracted, she was.  But to at any cost not let us finish up her dressings anywhere on her body.
Start foot.
Nope, not cooperating.
Try hand instead of foot.
Nope not cooperating.
Wait a little for the drug to get in there some more....
Shit, she's got a blister on her palm from pushing around on bed while she battles us.
OK try bad foot as the bandage seemed squishy and likely had big blister.
Massive blister located.
Snip and the deluge, bloodying the bed...
Raqu majorly stoned and restless shuddering in pain while flopping all over.  Very difficult to contain.
Shit now she's nearly naked and out of control and how to minimize more blisters?
Do coverup on this foot and go to somewhere else on body to come back later when more drugs are on board.
Shit she's got another blister on her elbow from wrestling us over her hands or feet.
Fuck, who cares just get this kid back to her safe mode wrapped and bandaged....
Finally we finish a foot and one hand.
Dr. tries Fentenol.
Not really working in getting her to comply.

Takes time but she's still not giving in to let us finish her remaining hand.
Go back to bad foot snip more of fluid filled bloody balloon that gravity had previously pushed out of my sight. The foot still wasn't an easy bandage and there was still no way she was going to let me poke and prod at the giant scab, scar, pile of dead/living skin pulling her toes under in the EB nightmare that is Recessive Dystrophic.

A second Fentinol.
We finally do last hand with a minor wrestle and a little distraction, but still she's tough as nails and like her father, no sense of backing down when mind made up.


Back at home and it's 8 long after our day there and she's KO'd.
No wonder.
I was able to take a nap with her for an hour, but it was only one of us to rouse when her big sisters foot steps and giggles came stomping in the house from her day of school and play.

The Good.
Fathers day was bliss.
Took my mom and two girls sailing on Das Boot for five hours and the only (I realize now where it came from) issue was Cordelia kneeling on Raquel's foot while climbing around in the spacious cabin.  Raquel spent all the good windy bits where mom's eyes were soccer balls as a nice little breeze shuddered the little boat and rocketed us up wind to home sleeping upfront and in the shade.  Probably the only time I was content our saggy sails were closer to sacs than the carbon sails I'm used to seeing on Mpower (Farr30) while racing with the A-team.


Waiting for wind.  Raquel sleeping in the cab.

shhhhhh! don't tell Andrew how messy a sailor I am when he's not there to observe.

Raquel not taking no for an answer about helming the boat.

My knees look a lot like Raquel's when my mountain bike works

Indian Arm.

Sunday, June 2, 2013

The hockey proverb

Lots going on.
Raquel is clearly becoming herself, between the moments she's attempting to off her sister by which ever method readily available. We often come running into a room with a child under duress and it's mostly Coco pinned in a compromising position with Raqu pounding at her. The terrible part, if that's not enough is that if Coco moves Raqu will likely be the one with huge wounds. It's surprising despite being beaten, she understands the catch 22. Is it she fears me or for her sisters well being?

We went to a little bday party and our friends made sure Raqu had her own private bouncy gym. The other kids had a huge air jungle gym which was an EB kids parent worst nightmare. 10 kids laughing and screaming running and bouncing about. Every few minutes the odd kid comes out crying because of this or that. Or then there was the evil child (not Raquel at this juncture) chucking the small plastic balls directly at the face of whomever, eventually hitting the right mark setting off a mini war of maleness. At that point one can only shake their head and contemplate perpetual world suffering. Well I guess one could do more as I contemplated and watched the bigger kid take aim and release with great precision time after time, it was a different parent instantly prompted with the need to break in on the violence and eventual tears.
Apple falling far from the tree, you might ask?
But as Raqu bounced in her little space landing on her bum yelling daddy watch this, as she further tested her agility and tumbled with joyous abandon, it was easy to smile and let her be free.
Hmmmm, as usual after some time it was clear her feet were sore. A little later on she refused to walk and hung off either Ren or I till we departed. The experience fell under 'worth while' despite potential pain and suffering. But as usual, time will tell how 'worth while' it actually was.

It's 2am and Raqu is on my lap after she was pulled from her bed screaming. Every few minutes as I type on her iPad her leg twitches or is kicked with some sort of suffering.

Once home we did a quick peek and of course there was a blister of various types developing on each foot.

The interesting one started off as a thin faint outline of red at the top of her arch. It was hard to initially decide if it was a colouration of an old one or something brand new. I gave it a quick EB safe rub with my thumb.

and Gasp..

It was shocking as before my eyes it started to grow and fill with blood. In seconds it was thick and worm like then started to push her seemingly healthy epidermis from the dermis.
Then the next struggle erupted between Raqu and I, as when I have a needle or scissor pointed to the demise of a blister on her foot.

I can't exclaim how effffing annoying it is for me to have only morphine in my arsenal to keep her calm in these instances.
I'm always sure she has enough on board to take away the shivering edge, as air hits large raw patches of flesh.
There is nothing for the anxiety even though we've had a meet with Dr.s and have solutions. The anger she must have for me after a needle's deep poke which missed the mark due to her ever stronger fidgets and or writhing.

But then there are worse things going on here.

Like the long emblazoned refrain in many a Canadian household. 'Keep your head up and your stick on the ice'.

Funny, the meat heads of the world had a deep thought or two.

Don't pout keep your head in the game.

Beware at all times for tragedy or opportunity.

These options best apply to this team as we jump over the boards from our slumbers to attend a suffering kid or our day once the nights have come and gone.