Sunday, February 27, 2011

It's always the same.

Just when I think we're normal and seemingly better off then lots of new parents with babies, Raquel pulls what she just pulled.
Had the Wentzels over for dinner and just as they were leaving Raquel started into a little crying fit.
She became more and more distressed, escalating by the minute.
Cordelia was off to bed with Renata and me doing my best to keep her quiet, which is often not enough in Renata's eyes.  Normally she just gets over tired and squirms and fights sleep.  Except this went on and on.  We have a magic number somewhere around 10 minutes, where we have to start considering there might be something up somewhere if not evident.  The tough part was she cried her throat hoarse.
Then there is thinking, the whole esophagus is about to collapse like the wall of her mouth did recently and really stop any breathing.  I'm not sure that can actually happen, but it seems plausible.
The moments were getting tense.
Some verbiage was used etc. etc. In times like these I wonder how Renata can still say she loves me.  Who's grumpy now?
I decided we had to pull off a bandage from the foot with a blister on Friday.  Hoping we could find a simple blister needing to be relieved.
Once off all was normal and healing well.  Hmmm next foot.  There it is.
A little blister pushing off one of her last remaining toe nails, in fact the only one that once looked truly normal...  Looking at the scale of the blister to my finger, you'd think, how can that hurt?  But then I've never had a blister push my nail off any finger or toe.
The pressure was quite hard as blood squirted out and she started to ease, but not that instant gratifying relief.
I had to rethink my regular toe wrap as I usually pull the conforming bandage quite tight across her toes to hold the foam shoe I create every time and thought up a new layer to protect her toe from any bumps and abrasion, but also not have any pressure on it ??? (we'll see if it worked tomorrow).
By this time I'm fried. 
9:30 pm is time to set up tomorrows schedule, think about the week ahead and cuddle with Renata for some type of mind numbing entertainment (TV, MOVIES (D.J.! that's twice I've had to edit my post the next morning due to a text I received in the morning for inappropriate content).  Instead we're stressed and arguing about nothing freaking out about our little girl in fits.
It's actually not over.  She's not down for real, I'm just recharging and staying away till I'm needed for real ((grandma holding her doing her magic) also for what might lay ahead for the rest of the night.
Once the feet were neatly bound Renata noticed the left hand bandage has slipped off and was held loosely in place by her silks.  Usually hands go pretty quick for a re wrap.
Totally out of sorts it took me three tries (this is when Grandma showed up) and it wasn't until she was quietly taking the bottle from grandma and no longer kicking and fussing that I got a good wrap on.  It's a three person job when it's like this minimum.  Plus we're completely dry on the EBM, Renata produces it goes to the bottle and it's gone, which is also a first and totally disturbing for Renata.  JOY!!!
Anyway, it's quiet back there, kind of.
Changing pace, last night was cool.
Dammit Janet talked an acquaintance of hers into helping out a family in need.
WE'RE NOT THAT BAD OFF! 
Some times I feel like a feeble father and human, needing and getting so much help, outside my typical time for play, but it's a thought I do my best to keep deep inside.  Most of the time as when stuff is done for us and helped out (dinners) our quality of life goes way way up.. Mostly Renata gets a breath.
So, gladly we welcomed tickets to the Canucks VS Boston, row one, just inside the blue line, well within the club section.  What a night it was.  Both kids taken care of in separate houses, Renata and I out on the town.  Free parking from Simona's son Simon, three blocks way from the rink.
A little romance,
NICE!!
Thank You.
We lost, but what a view.  Now that I've been that close to the action, maybe once and a while it is worth the ticket price.  We also gladly put in a good amount to the 50/50 draw, as those proceeds go to Canuck Place.  Hospice to Kids and families.  I don't anticipate that scale of respite for us, but it has been suggested that it may exists for families like ours.
I text a friend this image and he told me to get off the ice, I didn't get it till I was home and looked at the image again
 The City was blanketed in snow and in the middle of a nice storm.  Our typical warm coastal weather turned the beautiful white stuff into instant ice once a car or foot squished it to the concrete.  Police, Stretch Limos and many high-end sports cars struggled in zero forward movement at intersections with boisterous crowds of One Year Post Olympic Celebration Yahoos in fits of laughter and cheer.  I wasn't in the mood for that type of anarchy or fender benders and diligently put on our chains and seemed to be the only car with them driving out of the city. 
What was more odd was the next morning watching people with summer tires or bald all seasons attempt to drive up Seymour Mountain.  An 18km road from sea level to the alpine.  Still snowing and in a long convoy of eager pow lovers, every thing was fine till one car could go no further and started sliding sideways off the road.  The line of cars would stop and then only 50% were able to sneak out and around and beyond. Cutting out the chaff.  Scary was, those trying to come up after these guys were attempting the decent defeated with the same tires that couldn't go up, I can't imagine braking was something they were going to be able to do.  Don't these people understand gravity??
Je ne comprends pas.
Honestly, Vancouver has the worst winter drivers in Canada.  No question.  Problem is and maybe a semi valid excuse for not winter or all season tires is that we're the only little pocket in Canada that may not actually get it in any given year.  Chains though, they should have chains!
Distracted.
Distracting moments before the rest of our night...
I should be sleeping knowing that it's quiet out there still.
Charge the batteries.

Wednesday, February 23, 2011

Raquel did her first day of Montessori

Renata was very happy with the little things she could do and her focus.
Again, normal kid with some issues with her skin.
Dressing change day.
She only has a couple dots here and there.
Should be good.
Well, not including the horror flick that is her mouth.

Friday, February 18, 2011

Raquel has been doing very well

Not much has changed since the ambulance visit.
She's been great.  Normal kid, doing normal things.
The big meeting of the minds with her medical team didn't bring to light much.  I just informed them of the ER visit and a few on going concerns with her hands.  Everyone was fine with the status quo, as Renata and I seem to have morphed into a real comfort zone with Raquel in almost every way.
She's crying and making a fuss right now in the back ground, but I'm sure it's just normal grumpy baby.
Yup grumpy kid with an appetite.
We're going in on Tuesday for a test build of the mitt contraption I'm thinking to use a few hours before dressing changes.  A way I think might help get her hands to breathe a little and give her the opportunity to grasp a little better outside her bandages.  Hopefully my vision of what to do and what comes to pass will be similar and not a waste of everyone's time.  It's worth a try, I'm glad they think so too.  Although I am having second thoughts about how her quirky nails will slide easily in them and the sewn end at her web space.... Let's hope the DermaSilk cut sent from Italy (thank-you Dino) will work in this application. 
Yes about that.
The guys from Alpretec have been incredibly generous with us.  Raquel is finely dressed for a good half year of growth in those invaluable silks.  As well now the new company carrying the silks here in Canada, Western Allergy has been helpful too, in keeping up the flow of clothing.
The last huge blister she had on her back I meant to take some two day interval images of it in the healing process.  But after about the 5th day the blister became of little significance compared to other things going on that I forgot to continue my photo montage.  Next one. haha.  Not really haha.  The nervous haha of the inevitable.  Self defense mechanism I have.  Laugh in the face of danger.. After the fact..  Laugh at the back of dangers head.  hmm getting late.
I still don't get it.  We don't know exactly how the last rash of back blisters happened, but now we've had none for a couple weeks.  ZERO>
The only blemishes are at her face from all the abuse it takes from the split wrapped in her hand bandages.
Keep the splints in and her hands look straighter, but her face a mess.  I stop for a week or so and the hands look bad, her face beautiful.  It's a really annoying cycle.  I see past the blisters now anyway, more for the people in the grocery store that wonder if she's contagious and my visions of her later life when she's cured and doesn't have to scold me by saying,
DAD, COULDN'T YOU HAVE DONE BETTER IN HELPING ME NOT HAVE SCARS ON MY FACE!!
As she smears on lipstick and runs out the door to which ever establishment dad's across the world cringe wondering how their baby grew up so fast....
hmm getting late..

Monday, February 14, 2011

Nurses faired well.

Last Friday we did the first in 'Operation Gimme a Break'.
Dammit Janet and one of our other nurses did a dressing change with me watching out.  Each taking a foot and hand.  I can't tell how hard it was for me to sit back and let them do it, even if it meant, I get a break from it once and a while.  In the end they prevailed, but as they walked out the door they asked what I thought, what's the grade so to speak.  I wasn't too liberal with my marks, as I was forced to do a little re-wrap and explaining 'angle the bandage at 45 degrees across the instep then angle it again at 45 toward the heal' was like talking Greek, but in then end it's how Raquel fared through the weekend.  Was a wrap to fall off, or was there to be many new blisters by Monday? 
It's Monday and the change was great.  No new blisters and the ones that were there were well on their way to healing. 
I even complained to them about the time it took, but when we actually looked at the time, it wasn't any different then one of my early changes.  So really, even if I tried (which I did) I have no complaints and Raquel was as happy and active as she has ever been.  I have to learn not to be so overbearing like my father was to me while trying to use a table saw, or throwing a baseball etc. etc. etc. or else I'll get no break ever, nor will they want to come on by, even if they are paid.
Raquel really is in very high spirits these days as every little milestone with her is amplified in my eyes.  Best of all are all the giggles and the great impression of a lion.

Tomorrow is the gaggle of Doctors and OT's and whom ever else. 
I'm going to push the idea Dammit Janet helped out with for the burn unit hand wraps.  Hard to explain.  Hopefully tomorrow evening we can have a prototype I can put on Raquel's hands an hour or so before dressing changes.  It's purpose, to let her hands breath and maybe have a chance at her saving some of her nails.  Renata laments that often.  I always seem to think we have always bigger fish to fry, but maybe...  I hope it works. 
Now to spend some of the remaining Valentines day with Renata...
Cordelia's Queen created from one of her drawings

The girls with Jakob

Friday, February 11, 2011

After a week to normalize, we're back to normal.

That was a tough week thinking about stuff.  The kind of stuff I'd done a good job talking about, but likely better explained by an out of body experience while my mouth was moving.  Maybe I didn't see how difficult it really was and can be till last Tuesday's ER visit.  Things got stressful here and there, but really, we've had it pretty good.  I'm not saying I haven't gone through the frequent lament here and there, but that was not a day turn around for me at all.
The first day I held Raquel during her first real dressing change and the skin was dripping from her hand add the mind numbing reality of the situation, somehow I was able to go away have a good  bike ride, come back the next day and say I'm ready to take over and head the bandage procedure.
Watching your daughter stop breathing for reasons not exactly clear in a moment of chaos is something else entirely.
I wasn't naive to the idea that such and event wasn't inevitable or that she may eventually require a trach or how ever you spell it.  Kind of like, who wants to know how to spell Epidermolysis Bullosa?
Anyway, a couple good emails from friends and new ones with a common path helped a great deal.
Although like anything time and distance heals all.  Just make sure that learning has taken place in that time.  It has, but like everything, there is going to be so many situations ahead that we just can't plan for.
What I can say is, this week has been GREAT.
Raquel is right in the middle of a very good stretch.  She had Queen Bee here Tuesday for the entire day. Renata was able to get some great respite and our house was cleaned, spotless.  I don't really help my case when I walk in the door with a cloud of concrete dust following me to the mud room.  Why don't I enter that door instead of tromp through the corner of the living room?  I'm quite sure if I keep that up she'll consider us a lost cause and maybe stop that wonderful help...  Simona has indicated our laundry room was next and that she might call for help to move things around. ???
Yeah that room drives me insane, but I can't imagine what she has in mind.  I'm finding my intrigue to what she will do slightly masochistic in that I'm almost 95% sure I'll be put out in some way doing by best to bite my tongue, 'for the greater good I suppose'.

Back to Raquel, best parts these days are her new sounds and upright sitting and in some cases her new ability to hold objects.

She's such a bright light when she's checking stuff out and doing her ahh sounds as if to say hello to whomever walks in the door.  I'm ashamed to say it, but I missed this with Cordelia.  I couldn't wait for her to be BIG.  Funny, I was the same with myself, I couldn't wait to be BIG I couldn't wait to take on the world myself.  I couldn't wait to see an independence in Cordelia.  I don't have this issue with Raquel.  I'm the polar opposite.  I don't want her to have teeth.  I don't want her to crawl. I don't want her to get older.  I don't want a lot of things with her.  Blisters being the BIGgest of my I don't wants.
Those selfish dark thoughts again...
BREATH Ryan!!!



I remember going to my buddies family restaurant (miss you Mike) we were very young and barely had a clue about life.  He came up to our table to mutter under his breath 'check out the table by the window, but DO NOT stare or talk too loud when we look'.  He probably should have started with 'Do not look now, but'.  Turned out it didn't matter.  I was so stunned by what I saw that I couldn't say much, although I messed up on part one, I couldn't stop staring.  I was astonished by the man at the window without any arms.  He had probably been there before we even entered and our peripheral vision noticed nothing out of the ordinary.  He was seated comfortably with a full meal of hearty Greek foot (food hahaha) and a glass of wine.  His shoes placed neatly under his chair and his feet as arms holding the wine glass or holding knife and fork eating, carrying on a conversation with laughter and not even a flicker he was doing something different than anyone in the place.  I was young, I did a little learning that day too.  It's obvious to me there is no limit to the things people can adapt.  We just do, as parents and as siblings. 
Pictures of the week.

I'm seeing a theme here, shots from my studio door across to Vancouver over the water.





Sunday, February 6, 2011

Took a day off, all of us. Except Babi

An old river boat turned fly-boat
No fish for dinner, although I had an incredible kayak. Cleared a few cob webs and checked out some lakes up the valley I'd not been to in 8-12 years.  Never mind that it was 5 degrees and raining heavily with a steady wind coming down from the mountains.  I've forgotten how quiet being out on the water in the winter is.  Not a soul anywhere for miles on each of the lakes I visited.  I saw every colour from dark green to dark gray.  The photo above was in colour, really.  It's too bad it was raining so heavily, as I didn't dare take my camera out on the boat.  The lakes are man made and have many dead trees poking out of the water.  It's incredible in the mist, especially in the moments when the fog cut visibility down to a km or so.  Just a tall treed shoreline with giant hulking stumps and barren poles edging out into the water.  Despite how dreary it was in relative terms (Vancouver to the rest of the world) I still don't get auslanders complaining about our weather here in Vancouver.  It's beautiful. 

Raquel was exceptional all day I was told and kindly saved a tongue blister for the moment I walked in the door.  Simple, less the 30 minutes of painful crying post lancing, followed by a few intermittent squirms and thrusts of discomfort.  I'm always in amazement of how strong her core is.  When she wants to arch her back and straighten out, she is very hard to hold onto and once she starts she keeps on doing it over and over.  It's always a scramble to rearrange her sheep skin and under-layering.  Trying frantically to lay it flat before the next surge backward.  There is always the fear the rumpled bedding will cause undue friction at her back and all that entails.  Then also make sure the extra blanket is firmly wrapped around and between her legs and feet.  She is so strong and the ferocity at which she rubs and kicks her feet puts me into deep dread. Any prolonged foot bashing are disaster to her feet.  The evolving foot wrap has been working very well in my estimation, but it only takes moments of this type of thrashing to go right back to square one.  Plus we just drained a massive blister on her foot Friday. But, she's happy now, as likely nothing transpired (insert image of fingers crossed).  Renata and Raquel are in their usual nightly embrace on the couch, sleeping.  Actually, Renata doesn't look very comfortable and will probably complain about a sore neck tomorrow.  I'll rescue her when I'm done re-editing this blog.

Cordelia went snorkeling up the mountain with Sasha (another Czech transplant -Renata's maid of honor). Tough at the top of a mountain with her skis on, but after two or three runs in the pouring rain they did their best to soak every millimeter of their bodies. The prize - hot chocolate.  When was life that simple???  Cordelko clearly went to bed happy as she passed out in seconds.

Renata was a Czech tour guide today and also had a great time.  She too came home relaxed, happy and achem, much later then me.  The text I received while driving into town was 'I'm at a restaurant (Granville Island Brewery) go get Cordelia from Sasha and then save your mother from Raquel. 

Just in the nick of time, apparently.

Friday, February 4, 2011

Tired and Flat

I wasn't quite ready for how much the other night took out of me.
Lucky Renata missed it all and didn't get what feels like Post Traumatic Stress.
Raquel's mouth is extra tender from about last night till now.  The only way we get any food in is if she's had some Tylenol within 20 minutes.  It's easy to imagine the pain she's in.
Again I was whimpering a few days ago when I sliced a little chunk of skin off the tip of my finger while sharpening my skis and the many times I'd touch something wrong or something would get in it.
I think of a sore from biting my cheek.  How annoying are they?
Now add the entire area of your mouth.
At least it finally looks clean and pink.
Usually there are big white scabs everywhere in her mouth.
Now it's that pinky red every one's mouth looks like.  It's just that she has no skin in there on top.

We took her to an art opening last night.  So she's still a metropolitan urban hipster, despite the suffering.
Her sister cracks me up though.  She likes to dress herself and does not take kindly to anyone suggesting what she wears. (good grief!!!! she's only 5!!  I'm scared already)  She had white and red striped tights.  A pink frilled skirt with black polka-dots on white, layered like a too-too. Then a grey zip up hoody with fur (polypropylene) trim with an abstract red, blue green squares and lines through it.
She was clearly the artist of the crowd.
Even the young hipster women showing had nothing on Cordelia.
I think a friend took a picture of us as were leaving to avert our max allowable stay.
I'll post it, as I'm still getting giggles thinking of her outfit.

Here's some hipstomatic shots I've taken for the visual imagery installment of the last few days.
Probably the feeding the dropped all that skin, Gerber owes Raquel big





Sun set out to Vancouver from my studio

Wednesday, February 2, 2011

9:30 and all is well





9:45 am and all is quiet on the Western Front.

Having great difficulty going out the door to make my concrete castings.

Chaos and our first ambulance trip

Came home from work, started my dinner as Cordelia was taken out to go get her Beau Jakob (from across the street) with his mom.  Renata was about to go to the airport to welcome another Czech beauty to Vancouver.  As I started to eat, Raquel in my arm she started to fuss and Renata closed the door and gone for the evening.
oh, back track a few moments.
About half way through cooking my dinner (heating up Renata's cooking - mmm) I walked into the living room and Renata was on all fours head down in some strange breathing pattern.
'Having a panic attack?' I asked, not in the warm soft caring mode I'm sorry to admit.
Yes she was.
So I asked her about her day and all was well.
So then I gave her a nudge and a brief pep talk. 'buck up my dear'.
Foreshadow maybe.  Mothers intuition?

In goes the bottle, out comes a mouthful of blood then directly into a choking frenzy.  A couple seconds go by and no gasp.  She was able to get in a few then back into it with deep cries between gasps and the death gurgle you hear in war and horror movies.
Luckily my mom was near by to help out.  I'll not throw her under the bus, but it's really difficult thinking of how to care for a child with breathing difficulties when you are wondering if you'll have to call another ambulance for the two of them.

After four of the long breaks in breathing I called 911.

Basically I was thinking I could get some advise on the line, not exactly a fire truck and an ambulance and five guys in my house in 10 minutes.
As they walked in I started my speech about the rareness of her disease and the latex gloves they had on un-lubricated would create blisters the size of their fingers, nearly instantly if she was handled in the wrong way.
They each were extremely respectful of her situation and listened.  By that time we had (mom) calmed Raquel, Mom was doing fine she was back in my arms,looking about wide eyed.
In a few occasions she'd cough and this strange bubble would come out her mouth.  I realized early on it was a blister that had broken and blocked her airway, but I wasn't sure how big by that point.  The weird bubble coming out her mouth freaked me out to say the least.  I can also say with firm belief even the hardened ambulance medics and firemen were in uncharted territory and had an uneasy look on their faces.
We decided we'd give it a go and try to pull the blister out with some excellent tweezers they had (I definitely have to get me some of those), the medic suggested I be the one who do it.

Gladly.

Mom and and the medic held her steady while another medic shone a light into her mouth while I pulled at the massive skin flap that was her ENTIRE MOUTH!!!
The part that really got to me was the neat little hole that appeared to be where her uvula was.
It was shocking to see the entire layer of skin in her mouth including her tongue come off.
While I pulled it was attached very firmly at her lips and I felt a weakness deep inside me, as I tugged and watched it tear at the firm skin at her lips from the inside outward.  I did my best to twist the tweezers to rip the bubble to create a bigger hole and maybe stop it from covering her blow hole, since it wasn't coming out in pieces easily. Problem is that skin is way thicker then the skin elsewhere on her body.

We agreed it needed to be cut off.

I suggested scalpel.

geesh no wonder I'd not get into medical school with half baked thoughts like that.  A firm C+ average and a passion for art not anatomy wasn't going to make me much of a doctor.  Clarify, inner body parts and their workings didn't used to be all that interesting and chemistry diagrams very very boring,  I like anatomies just fine.  Brains are interesting too, but still way too much schooling.

The medic asked or suggested that we best take that trip to the hospital.
I agreed.

I tried to quickly assemble my 'go box' or 'butterfly box' (red box) but was dazed and basically forgot most of the important contents as I'd not yet replenished it from our ski trip.
I did have time to have a vision of doing this procedure in the gas station along the #1 highway at Lyton, with a few boxes of -40 window fluid as a table with the many insightful slogans written everywhere to keep us company.

Once in the hospital I text Dammit Janet, to tell her I was at her place of work and sent a pic of Raquel with her mouth open, clearly in the ER intake hallway. 
the circle is the hole of the uvula fallen forward, The white part isn't her tongue but all the skin from her pallet and tongue
It took some time for her to reply, but she was in the hospital before long to help out.  Great considering they are her colleagues. I didn't ask, but knew I didn't likely have to, the image was probably enough to maybe motivate one of Raquel's personal nurses to some unpaid overtime.  I was very grateful she came. I had to also lean on mom to bring over some of the things I forgot in my moment of haze.
After going through the spiel again about her skin and the dos and don'ts the doctor figured out we give her a shot of a morphine and midazolam to calm her before we tug around on that flap.  M&M's

hehe Renata just showed me the tweezers the medics forgot.  Wonderful, the silver lining.

Back to it.
We held her down while the doctor pulled out the skin flap and cut it off in two tries.  There remains a big flap at the back and it still flutters with her breathing.  I can hear the flutter now while she sleeps beside me on the couch.  She's on her side so that the bigger flap lays flat to the cheek.

The choice was, make more damage trying to get at it or let it go.  We let it go.
The other part of the silver lining was I was able to bring up the fungus and smelly foot from our last dressing change.
We set up a tray and did a foot change there in the ER, while a nurse took a swab and handed me the bactroban type ointment and we were on our way.
Dammit Janet you rocked!
I'm very grateful I didn't have to train a new nurse how to hold her or take up the ER nurses time either.

Although she did text me later to poke at my resolve saying she saw me waver when the Doctor had the scissors and was cutting the skin flap very close to my crying babies lips.  He was young and agile so it passed quickly.  But it was still interesting to see him slump his shoulders in relief when he realized we were done and he didn't have to go back in and it was a seeming success.

By tomorrow morning at 10 am I'll judge if it was a success and that I didn't have to see him again.

I have those wonderful tweezers now, so just as long as Renata doesn't go into another panic attack we should be able to nip it.
We just don't have the M&Ms in our repertoire for sedation.
I have to admit. I haven't seen Raquel that stoned since one of the first morphine doses she got while a couple days old in the NICU.

It's wearing off now though.
I foresee very little sleep tonight.

hmmm where's Cordelia??