Thursday, March 29, 2012

Been taking the blog tour

There is a little boy from Russia that was born a twin with RDEB and his brother with normal collagen levels.  Anton was soon after abandoned in the hospital by his birth parents.  There was a world call for someone to take over for this boy and eventually someone did.
He is now in the US and I think just had a G-Tube put in.
It's pretty amazing the devotion a family will have to make for a child with EB.  I can't imagine choosing this as a path.  I just can't.
I don't even care how selfish that may sound.  Of course I hear from many that they couldn't do what we do.  I rarely believe them.  Yes it's tough.  It's also incredible to see the simple gains your child makes in their development and not take it for granted one moment like I never have.

But to be sitting waiting for an esophagus probe and have a little bag inserted in her throat to open up what  are obvious strictures, there isn't any fun in that.
This is just one thing for this new family to deal with. 
There is also this constant attention to detail that you have to have.  If you're already OCD, YIKES!!! I didn't know I had much of this in me.  Again, why I rarely believe them when they think they couldn't do it.....

One of our health care providers called me pappa bear today.  Her way of making light that I was being slightly hasty in my attempted push through the red tape.
Red tape.
I can't imagine the Red tape of bringing a boy with EB from a broken communist regime to a land of unbalanced health care.  There must have been walls of it on both sides. 

Point being.  Anton looks to be in the best possible scenario.  His parents now have a battle that I really hope they understood before hand.  So far so good, I hope.


Here's my team having a healthy dinner.
Cordelia has a bowl of plain pasta (?), really....  I was poking at the broken communist regime above.  Well, that damn ideology had it's effect on the populous.  Poor Ren, growing up they didn't seem to have many options like we've been accustomed to here for dinner.  When we met back in the early 90's I was shocked that she'd eat plain rice with a plain piece of chicken and maybe a little wedge of butter and be content with it. 
Now nearly 20 years later I'm sitting across from my daughter after Ren cooked dinner and she too seems content with pasta without sauce, cheese or flavor enhancement.  Dammit she's infected my daughters mind too!!!  I guess since she's genetically half her I shouldn't be surprised but mercy!!!
Forget the fact she's eating from a bowl so full it'd take me three full meals to finish.



Sunday, March 25, 2012

Spring is here.

Saturday was more of enjoying the weather.
Renata Cordelia, Jakob and I took off with the canoe.  Raquel stayed with Grandma as she's about two years from such an excursion.  Maybe we can get a little one this summer at the cabin.



It was as nice of a day as I've seen in months and months.  Jake and Cordelia were stars in the boat.
I didn't have to tell them to be careful once.  Renata did, but that was after I blamed them for rocking the boat every time Ren attempted to paddle.  Also we were only able to go up the river as far as Ren's paddling skills would allow.  I've had that boat twenty years now and I think Ren's been in it only a handful of times.  I learned from previous 'friends' that maybe I shouldn't bring my wife along or especially a person I'd hope to be my wife.


This morning Raquel and Cordelia talked for about twenty minutes in Raquel's crib.  So I get it.  These are the times that having two really rules.  We can slowly wake up from our room listening to the sweet music of them giggle and chat.  Or what ever it is that Raquel does.
I showed her the moon and sky today.  It was so brief in me mentioning it to her but she got it.
I really can't wait till she can join in the conversations.  She has every indication that she'll be just like her sister. 
This mornings Crazy 8's game over a muffin and an espresso.
I actually had a momentarily lapse of judgement and gave Raquel a few pieces of my muffin till she eventually started to choke.  Helplessly I watched and waited for the last piece to make it back out.



I have to say that in a half hour we in Vancouver can do this is amazing.
Except I don't want to share my spots, so shhh.


Trust me.  Raquel doesn't get left out of all the fun.  This is her derby crash'em up car.
I sling shot Raquel down the hall in her plastic go cart even when it tips and she rolls out bumping her head she always calls out for more....  Although it's usually more padded then what is seen here.

Friday, March 23, 2012

The Gouge

Yesterday was a joy.
Our new nurse was given the full scope of Hultman temperamentalism.
We tried an early morning dressing change.  Raquel had a rough night and didn't wake up till somewhere around 9.  She was a little grouchy to start and once her early feed was done and some time for morning adjustment we went through the procedure.
Every thing was fine till we were getting her into her chair.  We have this wooden bar that goes across her waist to hold her in and it needs to be pushed into place as if trying to string a bow.  Renata nor many of the nurses like doing it as it takes considerable force to install.
When Cordelia used the chair we'd just leave the bar in place, but with Raquel it's impossible to get all the protective blanket layers within.
Everything was going somewhat awkward as the nurse was so eager to help, as we kept getting in each others way till.....
I was trying to pigeon hole the first side of the wooden bar and was having some trouble as it was getting caught up in the many blanket layers we use.  It didn't help that Raquel was somewhat fussy and squirming back and forth. As I was pushing from one end the nurse helped in another and the bar flicked up and hit Raquel in the face.  I couldn't tell but it looked in the eye as I was standing behind her and from the resistance I felt it felt serious.  There was a small scratch down her forehead toward the eye, but didn't look very serious.  From then on Raquel was fighting and fussing and had her eyes closed, generally a good indication I'd scratched her eye. 
My temper shot through the roof and for the remainder of the morning I had the feeling I'd made a permanent gouge in her eye.  If little flicks cause issues for weeks what would a big rough pointy end of a wooden bow do? 
Anyway, poor nurse.
Raquel cried and cried and cried and I had the darkest cloud pouring acid rain as I tramped around for the next hour before I had to leave to work.
I was able to settled Raquel before I left with the sweet sounds of Ween's 'Spinal Meningitis (Got Me Down)', 'Baby Bitch' and the ever joyful 'Don't Shit Where You Eat'.
I've never been much of a lyric listener when enjoying rhythms and beats, nor titles for that matter.  I wasn't sure if our nurse was wondering what she'd signed on for as she looked rather nervous for that period. Was it me? Was it Raquel? Was it the choice of musical lullaby?
It didn't matter at that point to me as Raquel was quiet and bobbing back and forth to the sounds.  I do realize I'll have to put that record down soon or again as her English comprehension gains.  Cordelia catches this word or another then asks questions that are sometimes difficult to go about in a PC manner.
I guess if my children are so lucky to born and live in what will likely be a war free zone for another couple generations at least I can do my part to bring a little flavor to sunny afternoons and dew drops.
Just hope on our nurses second full day with Raquel and first change our flavor won't be judged.

Anyway, my evening was spent walking along a uninhabited river bank far up the Squamish valley.
In the end I didn't find fishing very interesting, plus I didn't have that predator mind set to stalk the many little pools and runs for hiding trout.  Though I already knew I didn't end up gouging her eye, I just couldn't kick the dysfunctional mood of my brain.  I did end up finding refuge by walking far up the river along a small winding path amongst the moss and dead fall. 


All the Hultmans in this little unit seem in good spirits, today.....

Monday, March 19, 2012

Oopeesee

New word.
Oopsy.
Except it's not pronounced that way.
I had my brother over and we ate fudge brownies on the couch watching Better Off Ted while mildly attending to my little princesses.  (obviously Ren was out)
Raquel was doing her adult drone impersonation sitting between us while we giggled at bizarre scientists and quirky executives.  I think something was either dropped in the show or broken, I can't remember which but seconds later Raquel is standing at my brothers shoulder staring into his face saying Ooo-pee-see.  She must have said it five or six times over till she went on to playing with Cordelia.  Moments later Cordelia comes out proudly from the kitchen showing us the new word she taught Raquel.
Oopsy.
Raquel repeats
Oo  - pee  - see

A small time later as I'm doing the straight jacket routine to get Raquel to stop wandering around and avoid the indiscriminate screaming and beating her self when she's supposed to be sleeping or getting ready for her crib and sleep, I asked Ren for a glass of water, please.
Raquel pipes up.
NOoo! MEeeee! Peees.
It was funny to see her so intent that she get the water and most of all that she hijack my thirst.

Me.
No.
Please.
Eight.
Nine.  (Also no, we've had a few Germans through here in recent time.  She's seen the video where Andrew and I attempted to get Jo to drop into a double black diamond run. 'Nien'.  By the way Jo,  Cordelia skied that run today.)
Ten.
There are many words now.

It's nice to see all the projections people have given me about Raquel's personality and learning compared to other children without disability over time.  That she'll likely grasp on to certain things and learn at a different rate than some.
Cordelia didn't speak till she was nearly three.  It was mumble jumble.  I'm pretty sure it didn't help she was being spoken to in Czech, Slovak, English and my contribution of broken German and French.
With Raquel it's been fairly streamlined, a full dose of English speaking parents, grandparents and nurses as Ren doesn't seem to have the energy with Raquel to speak in Czech.  Yet Cordelia still does (?)
Anyway.

With her levels of future physical disability, communication will be key.

Sunday we invited over a fishing buddy and his kids for freshly ripped apart and boiled crab and clams.  Shortly before they left Raquel made a simple fall on the carpet.  It wasn't anything she doesn't do normally, but the knee landed first with sufficient forward momentum, as she sat there looking back with tears popping out from from her eyes I knew it was going to be a tragic mess.  Renata and I with our guests sat quite still at the table discussing the possibility of what just happened.  We let her have her moment then carry on with the kids.  Once they left we carried her screaming to the diaper area where we often tend to these type of injuries we deal with her quick fall's gruesome aftermath.
Just like that she'd lost the entire skin cap of her knee.
It wasn't debilitating like I'd suppose a wound like would do to me, but perspective rains down again on a family thinking they were edging on normal again.
Ooopeesee.

I'd be remiss not to mention Cordelia and I skipped Mondays responsibilities and had a day in Whistler.  The weather couldn't have been better, or the snow.
We lived the highs and lows of the EB roller coaster and family trying to carry on.
One sad moment, then followed by a few proud ones.
Coco waist deep in powder making turns and Raquel with a giant blister on her knee charging around the house not complaining for a second coming up with new words with her ever attentive big sister.
Ooopeesee.

Tuesday, March 13, 2012

Still, thankfully little to report.

I'm up at 3 am.  So there's that.
It's minor though as she has many little blisters at the hard crusty scab level that are probably causing some itching and mild irritation when she's sleeping.  I've completely forgotten what it's like to wake up to crying child on a regular basis.
Ha!
Two nights in a row for 15 minutes now constitutes for a regular basis.
Well maybe we listened to her low level cries for 20 minutes before we responded as a team to get her back down quickly.  I'm in the rare moment where my brain is humming along without much interest in rest.
Raquel's words just keep coming.  It seems like she gets a new couple every other day.
Yesterday was about getting ready.
'shoes', but for Cordelia 'boots' as that's all she wears these days.
Coat
'Jacket'
'Hat'
'Got it'.

As she directed Renata and Cordelia to also get dressed so that we could go outside.  Ren was headed off in another direction for the rare exercise she finds time for.  Cordelia, Raquel and I were off for something all together counter productive to indulgence control.
Gelato.
Seems I have two berry monsters.  I unfortunately picked some strange pistachio mistake that tasted more like a perfume sundae.  Cordelia had the blueberry and Raquel was very eager to get as many spoons as possible.  Of course I was panicked a bit about all the small seeds that might be still stuck in her esophagus.  It now occurs to me that the blood Ren thought she saw near her mouth while we were putting her down earlier in the evening was probably blueberry stain.  I tried to sneak bits of mine in, but she was wise to how terrible it tasted.  Considering she doesn't have any thing that looks like a taste bud on her tongue (all calloused over and shinny smooth from the continuous damage feeding and sucking on bottles prior to G-Tube), I'm continually surprised at how responsive she is to taste.

It was also funny as when we brought her into the Gelato store I carried her in in my arms as soon as I put her down she trudged off back toward the door pointing to the kids seat.  I didn't even see it when I entered.  Not only did she see it, she saw which one she wanted.
Blue?
Red?
Yup in following in her sisters colour preference Red it was.  (pink!!)

Like her words.
Her personality is coming and asserting its self every day.
She's a happy go lucky - ham.  Much like her big sister.
And for all the reasons for her not to be.  She hasn't a care.  

Skiing with Coco and the boys.

video
I have to admit I was a little scared for her when I turned off the camera.  The slope somehow seemed way steeper with her little body against the fall line.  Didn't help that she was in the middle of a no fall zone. 

Andrew moments before he went flying off a little bump then crashed heavily into another little bump.
Chicken legs, nearly did himself in.  Later that night we had the Wentzels over for dinner and the guy could barely walk.  MAN would it have sucked to be part of the evening news for all the wrong awareness reasons.
The snow was about as difficult to ski in as you'll find anywhere.  Once the skis cut through the freeze crust wind layer it was like being a train and all the while wanting to be a car.  There was very little opportunity to do any fun turns, we were basically locked in till the flow of the turn would naturally take you into the next.  Which is a big reason I got to split a gut watching Andrew pound his face into a snow bank.
It was amazing and we were wishing we could stay and climb all day, but then there are wives and children and instead of a train wanting to be a car........?
Ummm, I think if I write the other analogy I'm thinking of I might get boxed by either Andrew's wife or mine. 

Thursday, March 8, 2012

Bath tub

Over all it's going as well as possible right now.
It's easy to over look the fact her pillow has had a few blood stains or this afternoons trickle of blood coming from her mouth and the gurgles that usually follow such events during diaper changes.
She's just so happy the rest of the time and her new words have been coming in fast fire that all that seems like nothing.

We've been watching UP during dressing changes and other moments we need her still with minimal parental involvement (ie when she's plugged into a feed).  There's a few times in the movie where all the dogs go bizzerk when they think they see a squirrel.  Raquel now points and says squirrel to every squirrel she sees.
Similar to the craw craw when she sees a crow.
Vancouver is full of both as nearly every small stand of trees has one or the other.

The baths have become remarkably less about screaming.  Unfortunately for me, it was shortly after Ren started to go back to work as she has seldom been part of the changes anymore.  It could be that Raquel is maturing, or that I'm super-duper dad.
I think it's obvious to those who've read many of these blogs to know which I'm leaning to.

Today, though.

We had to call Dammit Janet in for help as there wasn't a day nurse today to help with the change.  Her second spawn Lyric was also here helping.  I'm not sure why after doing changes every other day for over a year now that I still forget half my supplies in the other room.  It often happens when we're half way though a foot and there is no conform bandage.
Lyric was very helpful today as I'd forgotten more then usual.  Probably because of what went down before hand.

Back a bit to the bathroom and Raquel is standing in her blowup insert tub full of water and bleach grabbing her various water toys and chucking them into the tub.  Something she does when Coco's having her bath.  In fact it's a real pain as she inevitably gets her dressing soaked.

Most of the time during these baths I have a blanket and am steadying her under her arms while the nurse or whom ever sponges and washes her.  Generally the whole time she's nervously telling me 'done', done' 'done' but nothing overly serious.
But when her hair is washed all restraint is gone.  At that point it's crazy in there as she's hopping, screaming and doing her best to ward off other hands from her head.
Today we'd done the hair and every thing had calmed back down and she was pushing her toys under the water from a crouched position.  Yeah, forget getting her to sit in the tub.
I stopped holding her for likely no reason whatsoever and look away.  In that second she teeters and falls backward into the wall and bump out of the tub, rubbing her back and elbows as she slips right back into the insert blowup tub.  My heart and nerves were shot instantly.  I wasn't holding the fleece blanket and didn't dare grab her with my bare hands and basically had to wait and watch her slump into a static position.
There were a few curse words followed by some minor panic before I was able to get her back up and safe, wondering how badly damaged her back would be.

When Ren came home I tried to explain the bathtub incident as well as another one which had Raquel jumping  on her chair with Cordelia as safety adviser, she ended up doing an endo off the side and landing on her head.
Funny how all this happened within 45 minutes of Ren coming home.
Lucky for me I exaggerate often and she didn't believe me off the bat, so the initial shock of the possibility of two giant blisters was eased into reality.

Thankfully the reality of two giant blisters wasn't that.  She only had one about two inches round.

Ren puts padding at the front and rear of the insert tub.
Had I,
nothing would have happened.

Dammit Janet!!!!!


Monday, March 5, 2012

Painting. Now it's daddies turn.


Cordelia has been having fun for the recent while, as we send her off to one of my old studio mates atelier to learn.  I'm a big fan of what she's been doing and love how much she's getting out of it.  There is that funny thing when parents live vicariously through their children that's starting to emerge here.  I'd been wondering for years if I'd ever get any time or energy to quell the passions for painting and sculpture of years gone by.  I haven't painted since Cordelia was born, now with Raquel and all that follows her around, the concept seemed even further from a dream. 

Except, after the EB blues the other day, attacking a languishing canvas was extremely rewarding.
The under-painting was part of a war series I worked on. Fun composition to start from, glad it was killed (so to speak) and now with a new narrative.

Sunday, March 4, 2012

EB Blues

Couldn't actually pin what it was or why it was.
Maybe it was just a little low in the battle front.
Maybe it was spawn out of a moment where while poking a blister Raquel flinched and kicked plunging a needle right through her skin to be stopped by bone.

Joy.

She only stopped fussing for the second to look me in the eye and give a small instantaneous look of shock and wonder.  I was then reminded by the nurse that hitting bone doesn't actually hurt. 

Hurt who, is the question?  I had a nauseous moment but, damn kid for not staying still!!!!!

Maybe the blue was getting a link from the company Polymem.
One of their products used in wound care similar to a Mepitel.  Foam stick bandage - the tacky stick EB friendly, unlike the tape used on Raquel's belly day 1 that left the square pattern.

This is the imagery I looked over.
PolyMem

We fight with little wounds I understand.  At least from what they could be.
Saying we've lost the skin on the entire upper of her foot is one thing.  The poor girl who had those incredible sores on her back.  I still feel somewhat isolated from the horrors that will come.
I often talk frankly about the eventuality of her hands becoming blocks, even the guarded expectations of life our Pediatrician so elegantly put it in the TV interview but living beyond this wonderful time with Raquel is such a black out.

Maybe I used up my fighting spirit for the next couple weeks during the interview while so eagerly talking about the horror that was her birth.

Anyway
This evening while she was settling down for sleep on my lap she grabbed my phone.
Turned it on with the bottom button.
Pushed her thumb across the unlock screen - unlocking the phone.
Then pushed the message button and proceeded to make a nearly legible word then pushed send.

All the while doing her best to hold me off with her elbows and shoulder position.
So far so good.
What's to be blue about?

Thursday, March 1, 2012

Missing Simona's frank reality

So is Raquel.
After such a busy last week our most cherished extended family fixture has flown the coup for a trip that I've dreamed about since 1992.
A bicycle trip around Cuba.  Tropics, ancient cars and lost in time.
The ancient car part is very important when thinking of a bicycle journey.  Having ridden from Lisbon to Frankfurt in not an exact straight time line or route, being passed from the under powered cars of Portugal to the insane drivers of Italy, I can attest to the great difference and possibly false sense of security of long listening to a car approach from behind vs the shock and instant wind cyclone sucking you in and out of traffic..

Not that I've ever smoked, but the smell of clean fresh tobacco is actually quite nice and I imagine the little bars or what ever there is along the roads would be interesting, if not the fields growing it.
I don't know if this is fact or the fiction of my mind, I just know I've always wanted to go there.  Taking in what ever experience comes our way.

These are the kind of trips I've planned to take my kids on half a life ago.  On several occasions traveling Europe by bicycle I'd seen families packed and bundled onto tandem-cycles slowly meandering through the country side.  The kids loved it and the freedom is something else.  Unlike much of the worlds perception of freedom = speed.  The pace and working for what you see is something I'd always loved, not to mention the gratification that is associated with it.  Especially when you've finally found yourself in the Swiss Alps and thinking back to the thousands of KM you've covered.  Ho hum, the glory of another lifetime.

Back to drawing board.  Currently I'm thinking sailing will be the key to this type of travel post EB.  Just haven't figured out how we'll get Raquel past rough seas or monster motor boat without pushing off every bit of skin she has.  Especially her butt.  It can be very monotonous hitting wave after wave after wave for 4, 6, 10 hrs at a time.
Maybe we pack her into a mini sumo suit?  I guess that way Raquel will also float when she gets bounced off deck.

Anyone have a Vangaard Nomad for sail?  I mean sale?