Friday, April 27, 2012

Dodging Bullets? Taking Fire....

Two situations in one day.
First was a excited stressed call from a nurse mid day while I was at work.
"Can you come home now please Ryan?!!"
In the back ground I hear Raquel freaking out in distress.
I was down the stairs before I realize I didn't even say yes.  I tore through a place of business as it happened to be my quickest line to car nearly chucking customers to the side in my wake.

Forget about testing Subaru's AWD system.  Brakes aren't as good as they should be for that type of driving...  Maybe I need to hit up my sailing skippers at Park Shore BMW for an X1 so at least I can be safer on these mad treks home.  Well one I'd have to be able to work.  Two I'd have to be able to work to afford a car.
Nice car.
Day dreaming...

Then I get held by the one light between my house and my work.
No cars.
No traffic camera's
DO I??
Deep breath.
I really wish I had a car that could peel out, again.
So satisfying in these situations.
Even if you are still siting in one place after you have the go ahead while your tires smoke around you.

Images of a giant smear of skin across the entire side of her face are running through my head as a scenario of her falling off her change table.  Or some where else on her body.
Then there was the choking scenario and arriving to near dead kid.
I can say the adrenaline was rather thick in my veins and my mind quite clear for the circumstances.
I'm happy in fact I didn't ask what was wrong before I charged off.  Thinking of near dead kid all the way home would have certainly had me blow that red light.

In the driveway and the fluttering ABS nearly had me take out the few concrete planters I have loitering at the deep end of our driveway.
Once in the door Raquel is sitting quietly on the nurses lap taking her feed.
All the rage and fear I had dissipated and was strangely contained in the tiny little tear still evident on Raquel's cheek, just under her eye.  We'd been linked, but just not in real time.

Back to work.
Except how do you concentrate on work after that?
How do you conceptualize (in my work) the 3rd dimension?
Well 2D but thinking in the 3rd while using the 4th. Is a computer the fouth?

All went well for rest of day.  Except between a builder and I.
Although I guess that went down before the choking incident with Raquel.

Oh right Raquel, she aspirated reflux.

At 1:30 am we had a second aspiration.  From her bed we could hear her struggling to breathe.
Within seconds we had her out of her crib and into the living room holding her not having a fking clue what to do.
After many seconds she stops choking and starts to breath normally.
Within minutes she's passed out on my chest.
Yet another night on the couch, with her rolled up at my feet, just in case I need to be with her at a seconds notice.
Not like I still have a fking clue what to do, but to hold her and live her panic.


RDEB, gotta love it.

Thursday, April 26, 2012

Stricture apparent

We had Raquel do the barium swallow.
Not exactly a procedure I was fond of.
It ended with her choking and gagging up chocolate barium screaming under an xray machine while three of us tried to keep her still.  Of course three blisters resulted at her elbow, armpit etc.
It worked though.  I was able to rest easy that my thoughts of a severe stricture in her upper esophagus was correct.
Rest easy I say.....
Rest easy that there is a decision to make of whether or not she actually needs one....
An esophagus that is.
I went over some of the options with one of our nurses "Mana" as Raquel puts it.
Another intubation, is it really worth it?
Just so she can eat/drink ice cream, water and yogurt?
Certainly regular food is never going to happen as the issues they cause.  We didn't even get to 15 months and she was in this situation.
In my opinion we've dodged many bullets to get her to this point with virtually no esophagus.

I'm going in no apparent line here.
I can't think about it anymore today.  Back to ignorance till her next GI meeting.

Monday, April 23, 2012

Now that Raquel has turned the corner.

Renata and I are tired and I'm a lot crusty around the edges.
I have a headache and maybe Renata is just letting me be the moody one for a day or so till she takes a turn.
Raquel was great today, although past dressing change I did my best to avoid her.  Saving my energy for the scream fest tonight as it'll be my turn.  Ren slept with her on the couch after I had enough at about 2am. 

Not walking much, but that's to be expected.

 Raquel hamming it up between feet.

 Here it's obvious how her toes have been deformed since birth.  I don't understand why her big toe was stunted with the next two.  They were like that from birth.  I asked the Dr's about it, but got little answer as if they were thinking "you got much more to worry about than toes buddy".  Also after this round of foot and hand blisters it's very easy for me to see how quickly her webbing can and will likely happen.  Those three toes above are a mess and the pad of her foot is looking quite odd.  I've just noticed the splinting material I've been using is setting less and less into her webbing ie it's starting to not exist.  The other foot is fine (kind of).  shh don't tell Ren I think that.
I sent her away when she came in the door for today's dressing change.  Ignorance is bliss.

It's easy to see why she's been going out of her mind with the itch of late.
These shots are after a good soak prior to re-bandage.
I'm starting to wonder if my technique has reached some limits with her.
I don't think so, but it's hard not to think about the whole procedure.

Tomorrow is Children's Hospital and a pre-check on her esophagus.
I'm very nervous.
Not about going or the non invasive procedure (I'm told) but more about what I expect they'll find.

Back to the itch

Hell hath no fury like the mind and body of a child consumed by 'the itch'.
Where her parents hold her in near straight-jacket form.
Finally some clarity to Raquel's current ailment. As I hold her (blogging while waiting for her to drift off) her body is twitching in rapid fire. She gets off the odd deep dig with a toe or heel in an attempt of an itch but as long as I'm in charge I'm doing my best to hold her off.
Its taken a few songs but now she only requires one hand to keep her from damaging herself.  The stress level is somewhat palpable although the smell is terrible radiating from her foot.

I was a bad dad today, as I forgot my plan to do a change today. Too busy dragging a tree around and digging a hole to replant it. With many helpers and advice from nearly every passersby it may live.
Bigger hole, bone meal, not deeper than the crown, lots of water etc etc. What is it about sunny days and work in the yard where virtually ever person walking/driving by has to impart advice?
I was totally content battling it out with Grumpa.

Anyway I'm hoping there's a lot more healing going on under those bandages.
Earlier at friends house she unspooled her hand dressing and (gasp?) went without protection for a couple hours.
It's probably exactly what's going to lessen my laws of total control.  More free time without issue.

She's finally calm.

Saturday, April 21, 2012

The foot is starting to look good.

Maybe not, at least one of her toes looks good.

When she wakes like this and it takes two hours to open her eyes you know you're gonna have a day like the night before.

Thursday, April 19, 2012

To prove she's in good spirits

This is the two sisters going at it moments after the above post and 24hrs past the photo of blister at foot.

Finding the line

We've got Raquel back to her old self, but it took catching up to her pain and discomfort with her various meds.  We have a fairly open guide for her care in that regard and she's had so few larger blister issues that we'd backed it all off.  When the blisters all come so quickly we just were not able to catch up.  All those terrible two moments, was basically a child irritated  beyond her control (I hope).

She's been great of late and yet she's still getting huge new blisters on blisters.

Wrapping under the foot has been a problem for today.
Yet, she's behind me being bossy and giggling yelling at her sister.

Tuesday, April 17, 2012



I'd forgotten that she gets big blisters all over her body and they are irritable.  So short and selective is my memory.

We've finally caught up and have her pain/irritation in control and she's back to that happy go lucky kid with humour and lots of personality.
It's amazing to watch her talk more and interact more.  It really shows in a little window, two days in fact, how chronic pain can delay all her development from speech to what ever.

She has two feet and a hand looking like this at the moment.
The hand was troubling, but at least it's on the back of her hand and not on the fingers or web space.

I can only wonder it was from the flailing of her arms in recent days.

I couldn't get back from work in time to do a change with the nurse so my mom helped.
Out side of a few gruff comments by me she was excellent.  I was only a jerk due to the internal strife looking and dealing with bloody mucky painful areas on my daughter often exudes the grouch.

Sunday, April 15, 2012

The battler

Raquel doesn't budge an inch.
She's been a monster of late.  Flailing fits of rage and total irritation.
She has woken grumpy of late and pushed it on through the entire day.

I'm in shock.  This isn't that happy go lucky kid of just a few months ago. 

It's like she's 1.5 turning 16.5.

The evening sleeps, again part of the same.
Her feet  are likely very sore and itchy, but it's nearly everything that sets her off.
I'm not so sure it's not partly about how much we hold her back.  She just wants to go.
Walk around the grass.
Climb up the concrete stairs.
Sit in the dirt and sniff the flowers.
Rough house with Cordelia and I.

It's not that we don't let her do all that, but it's with so many conditions and heavy guidance.  It's clear she just wants her freedom.

Wow.  Is it ever going to get complicated with our lifestyle and Raquel's expectations.
What a F'n terrible disease. The mental parts are now rearing their heads.  I'm seeing how babies are actually quite simple.  I had thought once reason came into the equation we'd be alright.

Problem is we have no guarantee she's be a reasonable kid.

We're entering into the stages now where many of the things we do are definitely going to start to effect her personality, her goals and limits she places for herself.  Her spirit is undeniably strong.  She just does not break.  So much she basically falls asleep before it appears she's given in.  Then when she wakes back up in the morning or worse late in the night and goes right back at it - full bore.

This mornings example.
She started screaming as soon as she woke up.  I tired to feed her but she was thrashing so much I could no longer hold her and because I couldn't control her thrashes I put her back into her padded crib.  She basically screamed her self to choking with a little blood in her saliva.  By that time she'd let it out, but it was a fine line from then on all day.  Didn't matter what Ren and I were doing.  Eventually the overflow of grumpiness took hold of another family member.  For once it wasn't me.

But now to figure out how to keep her strong and without borders in the sense of spirit, yet get that mean streak of hers in control.  Oh BoY do I hope this is just a passing phase.  I think in the last year and a half I've proved to myself I can handle the disease, I'm now starting to worry I'm not going to be able to handle the person affected by it...

Wednesday, April 11, 2012

Been a while since the feet have been bad

Raquel has been having some harder times of late.
Her personality has still been goofy and her temperamental outbursts have been considerably less.  I only say this as I feel less irritated by when she does have one at the moment.
Although she still does break down in full fledged fits if something isn't going her way.

Problem is she watches Cordelia or other children do things, then tries her self and gets grabbed, held and the big NO.  I've read this topic in many other EB blog sites.  Poor kids watching from the side lines.

The biggest concern continues to be Raquel's esophagus.  She nearly choked when she nibbled a banana the other day.  Then there was the chocolate egg she found and chewed on with foil still attached.  Damn Easter Bunny!!
That creature is getting eaten next year no doubt.  No more Mr. Niceguy.
I'm going to lay some leg hold traps all around the house and stuff him with help of a taxidermist I know.
Anyway, it's the little end of a piece of anything and that's likely all it's going to take having watched her spend considerable time trying to push what ever it was back out the top.

We've been waiting some time now and all we'll get is a preview to see a Dr.
I threatened part of our team whom really didn't deserve the comment that I'd bring her into ER or the (did I already mention this in the blog??)
- memory fading - or the office of our GI guy and feed her something and watch how quickly they admit us.

It's strange.
The G-tube was so fast.
This, which is actually life threatening this day or the next has been pushed off.  It's frankly our first experience with the medical system where we haven't had preferential treatment.
hmmm maybe that's it.  I'm just pissed because we're like everyone else with a chronic issues.


I don't get it.
I guess I have to be a squeakier wheel.

She has two rather large holes on each of her feet now as well.  Lots of blood from one today and it was very difficult getting her to hold still while I used my special scissors to cut little whisks of flesh from the bottom edges of the blister.  One of these days like way back when I'd sink the needle into her tongue or gum when lancing the long ago mouth blister, now I'll actually clip off some real flesh instead of that ultra thin sagging bag of fluid.
It's been so long that she's had feet problems considering how much she walks.  Her first foot to blister is healing very well.  So hopefully this won't last long.
Her recent rapid growth I think has moved her feet onto the next size.  Too bad we don't have a shoe like that soft leather pair, I just have to remember to remind everyone that maybe the extra sock we use for her walking around the house doesn't need to go into the shoe too.  It once helped to fill the shoe, but obviously now is a hindrance.

photo Jenny Nelson

Photo Jenny Nelson / Dirty White Planter Ryan Hultman

photo Jenny Nelson

Monday, April 9, 2012

Easter Morning started even worse than Christmas

Usually we're up at our cabin tip toeing around the small patches of dirt between the landscape of snow.
The morning was a couple kids wandering around the property picking out the chocolates I wasn't able to get to, either because I was being distracted by Ren  or otherwise made to feel guilty because it's for the kids.
Kids at those ages weight about as much as one of my legs, so by proportion they are due 1/5 the chocolate I am.
3:30am a little body appears in the bed and is talking as if it's 7am.  Ren and I take turns consulting on the benefits of a return to her room among the odd mild threat to the amount of fun the long coming daylight hours may or may not be to her.
Little is accomplished as reality flashes in and out every half hour as either a quizative voice is heard or cold shoulders shocking the system to find the covers that are usually comforting me from the elements blowing the remaining days of winter through the window.
All is lost when #2 cries us up and into our day.  Once in the living room the Easter bunny had left various chocolates around for the kid already bouncing off the walls in excitement.
Honestly I don't get it.
How is this possible.  I thought Easter two years ago we had that bunny for dinner?  In fact I'm pretty sure we had enough cooked rabbit that we should have wiped out a generation of Easter bunnies.  Maybe with his magic and incredible ability to poop pre-wrapped chocolate eggs he/she was able to evade capture and the delicious Czech cuisine BC mountain style to drop eggs another day.

Oh well. 

Raquel in all of this was in heaven like the oldest.  Her little rant lasted only moments this morning.
By 6:30 am the four of us were off for what is Cordelia and I's Sunday routine a muffin and a game of UNO.  I finally beat Cordelia too.  Maybe having her pester me from such an early time made me slightly more competitive than usual.  She did make a late and nearly stunning comeback, but I was able to thwart the 6 year old in the end.  Raquel sitting beside Cordelia and I at first was loaded into a wooden child seat.  She's sat in it before, but maybe I'd had a second blanket or maybe she didn't struggle to get out as much as when Renata isn't there.

Anyway, once home after a second Bunny chocolate run at a friends house that started at 8am we noticed a giant blister that can be squarely blamed on me.  Literally when I lanced it a pin prick of fluid arched from the backside of her knee to the counter beyond her change mat and when I finally got under the water sack to slice the bottom the remaining fluid poured out in a flush pooling on her blankets.

Raquel let out only a mild whimper.  She shivered a bit when I attempted a little mepitel cutting to maybe hold the skin back down to reattach.  It's been working when we get to this type of unpunctured wound quickly.  How I'm going to get this one to reattach is beyond me.  It covers the entire area behind her knee and it's ugly...

Really how painful is that going to be?

Then off to the Wentzel's for the third Easter Egg hunt.  Raquel was well looked after.  She was given little plastic eggs with jelly beans and things Ren & I quickly discarded (ate).
By the end Ren and I couldn't get out fast enough to get in a little nap.  Thankfully we were able to lose a child.  Raqu fell asleep before we even got home so it was a wonderful afternoon nap.
I then returned the favor of looking after Kai for a few hours.
Everything was going so well.
Until Ren wanted another little break to go read her book, Raquel noticed the conversation leading up to Renata leaving and started to cry and fuss wanting mommy.  It was then she started at the door (open as spring has arrived).  Half laying down I reached for her arm, she pulled,I pulled leading to a quick pivot and face plant onto the carpet.
The crying brings Ren and me feeling rather small and very stupid.

It gets better.

Kai is bouncing on our yoga ball shortly after Andrew arrived to take him back home.  We are chitchatting and I get Kai to bounce on the ball with me helping getting a little air.  Cordelia and I have done similar things where I pull her hips down and let go, she gets slightly vaulted into the air and lands on her feet or has to make a safety roll of sorts.  Lots of these games used to end up with some tears.  Since Raquel has been born, Cordelia has rarely been damaged because one of my plans goes slightly askew.  I'm either getting OLD or maybe more conscientious.

So why wasn't I careful with Kai?  Why did I bounce him off the ball straight up in the air and then straight back down onto the carpet with his face breaking the fall?  


Adults too get that over chocification that often makes the children wild and irrational.
 Kai's is a little harder to see, but I can assure the readers it wasn't a simple faceplant by loosing one's footing.  Raquel's on the other hand was only a small graze and by comparison for my days work a visual nightmare.
 Those questions we get in public?  This and the two huge skinless areas under her chin will surely get a raised eyebrow.  Hopefully they are the same people that saw us on TV and or in the paper.

Take me to a quiet place.

Tuesday, April 3, 2012

Poking Blisters

Once I lanced the blister, Raquel was actually irritated that I destroyed her painful play thing.
It took some time, but I finally warmed to her today after this morning.  She was a joy most of the day although our nurse did suggest Raquel had another outburst.

Oh well.

She sure is a fan of that CD.  I still rather play something slightly more subversive.
Oh well.

The war of wills

1 1/2 year old baby vs. a 40 year old.
This morning was the first time I've actually been mad at Raquel.
I guess it was coming as I've tired of her wicked spirited acts of attitude.
Renata and I listened to her a few times last night fussing and it was clear she was only up for attention, but around 5am her new wrist wound was itchy as we could hear a thumping of an appendage hitting her padded crib walls.  In we run and off to the living room to calm her.  We administered what we thought appropriate for the circumstance then I tried to hold her till she went back to sleep, but she wasn't interested in Dad.  So Ren was sucked into duty while I slinked back into bed.
Once the morning finally arrived Raquel was bitter and angry.
Screaming and flailing for Renata as she prepared Cordelia's lunch and herself for work.  She actually hit new levels of fits and pure rage I'd not ever seen before. 
So who's going to break???
I did my best to talk to her and every now and again I'd get her attention and she'd instantly stop screaming and do the yes or no or this or that.  Which made it all the more unnerving when she'd jump right back into screaming and fighting when she realized she wasn't getting Renata.
By then end of it Ren and I were mad at each other for meaningless crap so much that Ren was about to leave many of my work papers in the middle of our driveway in the pouring rain.  haha.. (yes I deserved it, kind of....)

As soon as the nurse arrived I was gone too.  I couldn't get out of there fast enough.  Cordelia was witness to many colourful words and had large eyes quietly observing the break down of the rest of her family.

Anyway, Raquel is in for it.
She's in for a black out.  A father with vengeance can't be good.  She'll get the point.
Cordelia had a couple similar situations, I just hope Raquel has some bend in her cause when mad I certainly don't....

Hopefully that how much she looks like me, isn't how much her personality is like mine.

Monday, April 2, 2012

Little Miss Go!

No reports, because I don't have to vent, complain or pick at others through my diversionary projectionist idiosyncrasies.

Raquel is building that little bullish personality I've been fearing by the day.  It's getting scary and I think soon I'm going to have to lay down the brutal law like only a father can.
To date there are only two people that haven't had the GO! shouted at them when they walk in the front door, both nurses, which will give everyone a good idea at how great they are to Raquel and within our home.

I've been working a lot lately and playing too so by my absence I've been the recipient of many.  I'll even be the first into her room in the morning as she's calling out for someone to pick her up.  She'll see me or hear me (depending if she's not yet interested in opening her eyes) and just roll over onto her head with butt in air and flick out her arm saying GO.  (I want) MOMMY.  I'll then have to have a small conversation with her asking her many questions that I know she'll say no to, then start putting in a few I know she'll likely say yes to.  Finally I'll break her down and she'll say 'yes' and if I'm good in my psychological warfare with a 1 year old 'Pees, UP'.
She's similar to Cordelia (and me) by not staying grumpy too long.  It's easy to prod her into a smile or at least away from the hostility she's starting to show for people she's not ready for.

Sunday I was away for the better part of the day (not hard to guess where, I suppose)
photo - Mike Nelson

and Raquel was complaining about her wrist. 

Two weeks ago I brought up a few scenarios that Renata would have to do bandage changes.  I urged her to change her mentality from 'no chance, not ever' to being able to step in once a month if not eventually once a week.  I've had some fun emails with one of our guidance families about their roles in the bandaging and I'm very glad to share, that they back me up on this one.

But to my glee and happiness Ren has moved on did a re-wrap on Raquel's hand with the help of my mom.  She apparently pulled out some of the old card board cut outs one of our nurses made (now moved on) and did her best.  Of course Raquel still had a large blister still there today, but it was a good start and not worth pestering her at what was done wrong.  Actually, the wrapping was dare I say perfect, they just didn't cut enough of the edges so that it didn't swell back up again.  I can understand why it wasn't something they were into.  You really have to get in close with a big needle and swipe at the edges to slice/rip them.  It's tough and stressful especially when Raquel isn't interested in sitting still.

This morning was also a first for Cordelia.  Apparently Cordelia took the initiative to remove the green grimy piece of mepitel from around Raquel's G-tube, then cut out a new one which helps to prevent Raquel's G-Tube from pushing off all the skin around it.  Once cut and ready Cordelia went over to Raquel to put on, at this moment my mom walked in the door.  Uneasy to see the little blonde surgeon attend to her patient she tried to stop Cordelia, but the patient, not patient at all raised the arm and face of her palm and uttered the familiar refrain -

Once Raquel stopped her grandmother in her tracks she then pulled up her shirt to help Cordelia apply the fresh patch.

Team work.

Soon my work will here be redundant and I can ski all day everyday.  Oh right, I'm too old and ungraceful to be a pro-skier to be able to afford such illusions of grandeur.