Thursday, March 31, 2011

Getting anxious.

I'm going away for a little trip sans family.
All is well.  Had to wait to confirm till quite late this afternoon to see if the improvement was on going.
Cordelia has stopped puking and has been up and at it somewhat all day.
I'm shocked at how much weight she lost in four days.
Before, I thought she was all knees and elbows, but now it's all ribs and spine.
Seems she's even lost some of her muscle tone.
Now I get why a little extra on the bones is a good thing.
Raquel has been all beans today and for a while.  We're definitely relishing this run for the good.  All she wants to do is play and explore textures and shapes.  She's finally shaping into a human.
Her skin shows no real trauma anywhere.
Her feet have healed to where there are no open sores under the dressings and her fingers are getting straighter due to the new wrapping techniques.
Of course she has the the odd little nick from the finger nails of her care givers (me) and her mouth is a patch work of white gushy scabs, but nothing that makes me think anything will change in a moment.  Although in past times this is exactly when things go to HELL.

Renata is out for a movie with her buddy.  Enjoying the last little bit of peace and quiet she'll get for the weekend.  I expect she'll tell me when she gets home she slept through much of the movie.

With a little time to ponder my anxious mood I think I'm worried about the trade I made.  Weekend for me weekend for her.  YIKES!!  Two kids?  All weekend?  Nearly alone??? 
Not entirely wired that way, but we'll be fine and likely figure it out.  Cordelia when healthy is a superstar.  I guess other then carrying around her limp body from bed to couch over the last few days was pretty easy too.
Late evening trips for ice-cream with a few moments of panic whipped in.
It'll be fine....

Wednesday, March 30, 2011

One goes down the other a dream

Raquel has been a babbling fun kid the last two days.
Cordelia, not so much.  Since splashing the Sunday floor with chewed crepe and whip cream, she's just gotten worse.  She's had a few sips of soup, but up till this afternoon she's kept nothing down.  I've heard a few friends talk about having sick kids recently.  They've all had it come and go, but it didn't stop me from bragging how rarely mine get sick. Leaving Raquel out of this conversation, although she hasn't really been sick, past the red eye and plugged nose.
haha jokes on me.
Dammit Janet suggested gravol suppositories to help the vomiting.
Ahh nope.  Not me.  I'm not giving that to my other child after all the pain and discomfort Raquel will soon associate with me!!
I figure sticking Raquel with needles while pinning her down during screams and cries due to a plethora of issues should surely get me a 'get out of jail card' on this one.  Plain and simple.  'Mean Daddy' will likely be my handle anyway, lets not let this get out of hand.
Plus Janet is the nurse, she's the pro and suggested it, maybe she should have done it for us as we let her help out with Raquel. 
But no.  Thankfully
I was able to convince Renata this was her call to step up to the plate and take one for the team.
The first pill taking routine was toughish for our little gaffer.  Too bad for Cordelia, there wasn't too much soft nurturing.  Seems even Raquel gets much much less now too. 
Renata and I are slowly getting like the doctor you see in ER.  You'll be bleeding out of your eyeballs and they'll look at you like a mechanic looks at an engine.  Although when I brought Raquel into the ER no-one seemed to look at us that way. 
The second pill popping was down right hysterical.  Not the hysteria you'd expect from a child refusing and crying from an uncomfortable situation, but Renata and I giggling at her excuses not to and the defiant 'I will squeeze forever!!'
Just then, her eyes widened, like I've never seen her become aware of the world.
Both my little girls, fighting to the end.
I was just wondering how old Raquel will be before she calms down and figures out I'm actually helping her when I approach with needles.  Obviously Raquel is much more like her sister and me then Renata.  Not to much give, scrapping till the dust settles. 
Hopefully for Cordelia's sake she doesn't have to see one of those little white bullets again.  Although I doubt she saw the first two?

Monday, March 28, 2011

Couple images from weekend.

Saturday 8am - Taking a break with Karl on the Squamish R.
Sunday noon - The start of a few hours of pain and suffering.
Notice furrowed brow and blood blister at lip.
Sunday evening - All sorted.

Friday 9 pm - John's just sealed concrete bar, hours before drinks.
Would have been nice to see it cure for a day or so before Red wine was spilled.
Seems I'm used to things not going as planned these days for various objects and people I've had a big part in creating.

A thank you.

Today we went to say thank you to many people who helped us out with dinners through two months around Christmas.  With so many difficult decisions and actions to deal with on a weekly basis concerning Raquel my fear of public speaking was nothing by comparison, despite standing in front of a crowded room of the Capilano Church Congregation.  When in College I actually went blind with fear during a Model UN Conference in a PolySci course.  I couldn't actually see past the edge of the desk during my part of the assignment/argument, then promptly sat down and passed the notes to my partner hoping she'd be able to decipher my chicken scratch and eek out a decent mark.
I didn't have that problem today.  It's much easier talking from a point of personal gratitude then pretending to be Malaysia defending the destruction of the Borneo Forests and indigenous cultures within.
Those dinners got us past the point of feeling like a headless chicken, to near the end where we felt slightly sheepish due the extravagance of it.  It's still amazing to me that so many people had gone out of their way to bring dinners at around 5pm so that we (basic strangers) could take those few extra moments off in an evening and basically deal with our crazy emotions and the procedures at hand.
During the part where I was speaking, I keep thinking to myself - 'stay on track', 'don't freak everyone out with details', 'don't forget to say thank you'  'don't studder',  'don't talk forever', which is easy for me with a captive audience and most of all 'don't um and ahh'.
Funny, my buddy Mike who introduced us couldn't think of any other reason for me to be his friend other then a fishing partner (?) how about - mountain biking, skiing, architecture, and the valuable philosophizing about family, life, film, beer. etc. etc. maybe he was nervous, I'll give him a pass.
I didn't plan it but it was nice holding Raquel in my arms while I spoke, there is something about holding that warm little body that gives strength.  Pastor Kim was great too as we had a few phone conversations beforehand and it was very comfortable talking to her, also having Renata, Cordelia, Mike and his wife Jenny standing up front.  Jenny also said a nice prayer for us to wrap it up.
I'm very impressed with their community and outreach.  It's something that our society as a whole can do more of.  I wish it were the case.
Hopefully I got it across how touched we are as somewhat outsiders to be helped along in what I can say was our greatest time of need.  What I did forget to add was it's not all gloom and doom in our daily adventure.  Raquel is, a happy kid, full of life and our little butterfly.

The day for us disintegrated past the wonderful service.
Actually that wasn't so.  I missed the end of Kim's service with the poke on my back and a young man telling me my wife needs me in the hall with Raquel (which turned out to be nothing serious).
Cordelia got her stomach virus back and hasn't kept anything down since the sing along part of the service.  At least Raquel waited to get home to show a big blood blister on her lip.  Then later in the afternoon to show a nasty one on her tongue.
Raquel screamed for about 2 hrs straight while we attempted to pop blisters deep on her tongue.  Of course I lanced her many times in the lip, gum and arm (?) during the three separate attempts to get it.  She's getting too smart and evasive without the much need practical sense of self preservation.  She has recently developed some great karate blocking techniques. I hope it doesn't take her too long to figure out when her daddy comes with a sharp object it's best she stay calm and cooperate.  It took three of us twice unsuccessfully, then Grumpy and I were able to finally pop it. 
Raquel ate quickly afterward and was a dream the rest of the afternoon to evening.
I can comfortably say there is peace in our household now.
Renata and Raquel sleep behind me on the couch.
Cordelia in her bed and they are all seemingly relaxed and sleeping well.
Time to charge the batteries of the mind.
Now sleep.

Nope, Cordelia had to gag out some more bile.
I told her in her drowsy sleep her breath smelled like my old dog Burny, she smiled and fell back asleep.
I think neither tonight.

Sunday, March 27, 2011

A Sad Story.

Since Raquel was born I've had my google account send me any interweb news with the call word Recessive Dystrophic Epidermolysis Bullosa.  This past week I was sent this article about Alice Irvin.
I spent the week telling my friends about this amazing young woman with spirit and seemingly indomitable will to let the stack dealt to her get in the way of how she saw the world and how she was to act within it.  She seemed brave and a great role model for our little family.  Each of us.  Unfortunately with every EB story I hear there are always a few new details very difficult to internalize.  I'm told EB effects every child differently, but we are likely to go down this path or that.  Almost none are remotely preferable over the next.

I had every intention of somehow tracking Alice down and was mentally preparing how I was to approach her and what I might say.  It's a seeming huge wall when we reach out to another family with EB.  It's scary in terms that many unknown issues become less so.
Ignorance is bliss.
But of course we can't be ignorant.  We can't afford to be ignorant, for Raquels sake, for our sanity. 

On Friday while I was off working, Simona was over helping out again.  Renata and her started to talk about Alice, apparently Simona had heard of her too, except her story wasn't as uplifting as Renata's.
Renata informed me late last night this news and all I can say is,
A great sadness.

The community that is stricken with the extreme forms of EB is very small.  So when a new soul is born or another has passed away the effect on us is eye opening to say the least.

I have so much to say, but it's just a incoherent jumble so I'll not bother.

Wednesday, March 23, 2011

Pain Management a Success.

We've luckily gotten past the bump in the road.  
She had been getting a very regular dose of Tylenol, on top of that we've had to use her dressing dose of morphine far more often then in recent memory.  In the last two weeks it was a given we'd be forced to use it, for each of her changes.  This was something I was very proud we'd been able to work past because of either how well her bandages were working or how we'd been able to avoid many other blisters and issues due to her attentive care (Renata, Nurses and Grandparents).  
Of course we have had the odd blip where things lapsed into chaos, but a week or a few days would go by and we'd get out of it and she'd be a happy kid. Many moments in a day we'd be able to look at her and see a bright, beautiful happy child (yes she's always beautiful, but dark clouds of grumpiness SUCK! and can be very trying to one's outlook).  This past two weeks or so it was getting difficult to see that playful fun kid on a regular basis, through my eyes anyway.  
We were simply not able to get over the hump.  
The sores on her feet as evidenced by the recent photos shows the obvious pain she would be in or what I now suspect being a prime culprit - 'The Itch'.  I've started to believe this is one of the issues which drove her and us to our wits end through the evenings.
She'd wake in an uncomfortable squirm rubbing her legs and feet ferociously.  
I have my own little battle with psoriasis and it finally occurred to me, 
'When is it most itchy?'.  
When I'm at the edge of deep sleep or calming down for the evening.  Depending on what I've eaten (chocolate) or drank (beer, scotch) I'll go into fits of scratching mayhem that drive Renata as batty as myself. 
This pushed us to make an earlier then scheduled visit to our pain management Dr.
After some deliberation of what we are seeing in her and how we were able to get past other cycles of pain and obvious suffering, it was decided to up her daily dose for the time being by 50%.  Same dose every 4 hours not every 6 as was a couple days ago.
What we have since is a wonderful baby, back to normal.
Talking, engaging, happy to be held, interested in everything, content.  No grog, no dreary eyes, no constipation, nothing negative (just yet, but this is being monitored very closely).  
We had to leaned on Janet today to come in for help on her bad foot for a change outside the weekly Monday Wednesday Friday routine.  The foot was smelling too much like a brewery and the green layers in her bandage was reason enough to get it cleaned daily.
Today's change went really well.
Honestly I was shocked at how quickly this foot had healed in 24 hrs.
March 21st, unfortunately it's covered in Bactroban but it's easy to see how big the red raw area is

March 22nd and a solid 24hrs of pain management

What a difference.
She had been waring on me a bit lately.
As much as I love her, it is very difficult attempting to be the calming influence when you are spending half the time while holding your child in panic struggling to wedge a hand or various blankets between her ankles so that she doesn't continue to push any of the remaining and healing skin off her feet.  
Deep into the night over the past two weeks it would often require both of us to hold her down to take either her morphine, Tylenol, or get her started on the bottle or simply stop her thrashing.  Otherwise her feet and face get pounded which of course eventually occurred.

Anyway, things are good and I expect a good sleep tonight.

This is Raquel in her first experience with a cat.
I was very proud of her that her first inclination wasn't to pat to the head of a cat, but to give it a firm right jab to the chin.

That will teach this cat not to sit on the couch next to a kid with boxing gloves.

Friday, March 18, 2011


The night was measured chaos, morning filled with blisters and new wraps.
Renata showed up at my work for lunch and Raquel was happy and lively.
Another evening of pain, suffering and the typical dressing change of raw foot and hand due to blisters from last nights chucking of hand wrap.
Dammit Janet is in for reinforcements along with our regular nurse during dressing change, while Renata goes out for beer with her Czech friends and gets the evening of respite she needs.
While I get to go sailing in a regatta all weekend.

Thursday, March 17, 2011

Been a tough week.

Raquel has gone back quite a few steps.
Her feet have been a mess and now some new blisters have popped up on her hands.
She's been waking at 1am 3am 4am regularly and going from deep sleep to outright screams of pain and frustration.  Which in suite, do we.
A few days in a row we've also been battling the dreaded mouth blisters.
This morning was a blood bath.  She's completely wised to my needles and keeps her mouth closed while I'm trying to lance them which means I lance her lip, gum and tongue before I'm actually able to help her out.
Really, how much fun is it to see blood gushing from all the areas around the pocket of blood that you don't even want to lance in the first place.
It's like getting spanked with a wooden spoon as a kid (do people do that anymore?) and using your hand to block it.  Not at all clever, as I'd take a shot on the fatty bottom then the bony knuckles given the choice and time to reason it out, well I'm guessing I would have made that choice at 9 years old.  Too bad I can't do any of this type of reasoning with Raquel.
Then this morning half way to work I'm requested to do an about face because she's chucked a hand dressing.  hmm joy, I didn't even get my second espresso as the barista was in a bit of a tizzy and I couldn't be bothered to follow through with my near daily routine.
Apparently Raquel has been great during the days, but when I've been coming home from work in the last 7 days or so there have been nice moments, but ends in restless evenings, nights and mornings.
Her feet have reverted to what they were like before we discovered the foam layer wound into the conform wrap.
I don't see it a problem due to the wrap, just in the deep cycle of pain and kicking.  We've put a call out to our pain management Dr. to see what levels we can use to get her healed and out of this terrible cycle.
It doesn't help that when she plays it's often in a kicking motion.
Renata seems happy.
At least that's one of us just now.
Maybe it's the $600 she splurged on clothing for herself.  Renata usually takes 2 months to buy a pair of shoes and by the time she's ready to buy them they are gone.  So to see her bring home a bigbagoclothes was shocking.  Danger.  Two in a family with splurge buying habits is real bad.
What ever it takes at this point I guess.  Especially when I need a new pair of waders for Steelhead and Coho season this year.  My argument platform is much better positioned just now, not that getting hypothermia and or drowning isn't.

I just got a call from Simona about how to better adjust her hand wrap and add a correct splint.
I agree.  We had one, but it was so big she covered her face in cuts and blisters in hours.  She mentioned what cashiers use when they get carpal tunnel syndrome.  Some type of internal frame, maybe wrapped in to the conform like the foam on her feet is done?  Anyway, we'll figure that out with her OT skills and the bits of forming plastic given to us from the hospital.

Renata also had a great talk to another mom with a child with EB, Renata was despirate to call her and I'm glad she finally did it, then talk every other day about thinking about making the call.  She was able to get some more useful info on care of hands and feet, as well as talk to a mom just a few years ahead of where we sit.  The call was over and hour long and across continent, she huddled herself in the back room for most of it but now its over I'm getting it from Renata too. 
I can't anymore tonight, I can't think one more moment about Raquel's care today.  It's pained me all day while sanding and grinding a concrete bar top for a buddies restaurant (gratis).  In the loud droning moments of polish and sand all my minds eye could see was a bloody foot and a wet bib of blood on her clothing from our early morning mouth lancing ordeal.
I just want a sleeping baby and a cuddle on the couch with my wife.  Nothing else.

hmm Raquel and Renata are now out, sleeping on the couch.
I'll cherish this moment till it's broken to bits at midnight or 1am.

Thursday, March 10, 2011

A couple more pictures with Renata's Grandfather and our wedding in the Czech Republic

We were married by him in the Church he still sometimes gives sermons.  In fact he had just given one there on Sunday.
Here are the few photos I have of him on our great day.
He spoke in English and Czech, so I wasn't too lost.

This was a very emotional moment for all three of us.
Still don't know how to translate

This was Renata's highlight of the whole reception.

Had to add this because Renata is so damn beautiful.  
The moment for my first sighting of Renata wasn't like the typical wedding here.  Bride walks down isle and groom is standing, waiting, stunned and awed.  In my typical panic (typical now, wasn't then) of 'where is Renata, why isn't she ready'?
There was me and a few of my buddies waiting outside looking down the streets we knew nothing about, hoping to see her fast approaching.  I'm not a fan of being late, especially when there is a huge crowd of people waiting for us.
Then she appears walking down the street and I got my first look.  Holy Moly.  I hadn't seen her in either makeup or a dress that well crafted.  Neither had my friends.  As she got up close probably only seconds before this photo, I could hear my buddy Mike X (who is unfortunately now past), blurt out with me in ear shot "who is that?".  I'm guessing he'd found a brides maid he'd be able to chit chat with later, he was so quick to call out his special interest, that there not be any conflict with the boys later he didn't actually look at who he was talking about.  With Andrew, John and I holding back our laughter in bits and pieces out of respect for those that probably shouldn't have heard that and the magnitude of this somewhat inappropriate moment to be hitting on the bride, minutes before she was to be married to a friend of his since the 8th grade.  They told him somewhat quietly to take another look at who he was all excited about, bringing him thankfully back to reality.  Still one of my great fond memories of my long lost buddy.  He had many, and plenty far more awkward and funnier then that!

Mike telling me my wife is hot and how lucky I am.
Got side tracked there.  When I see those shots I can't help thinking of that moment and also Mike in general.  Miss you BUD!

Wednesday, March 9, 2011

Great Grandfather Josef Vesely turns 97 today.

Happy Birthday.  Stastne narozeniny Dedecku!!

I've been told Martina (Renata's sister) brings the pages I write about our family once a week to her Grandfather.  Pressure... But today I'm not into the written language.  Just pictures and tidbits.  Especially now that I've spent 1 hr trying unsuccesfully to get my printer to be in tuned with this laptop....  Good thing it takes USB and I can transfer that way ???

Renata's Blister Day 6 (No bandage just her Dermasilks)
The foot wrap only looks like that after a wash. Notice foam pieces I make into a boot.

Tuesday, March 8, 2011

The Hultman Twilight

She wasn't out all night feasting on helpless humans, but sure looked like it when I left for work.
Raquel is wising to our ways and fighting with every bit of energy and strength she has.  This is the result of Renata and I trying to pop a blister at her front edge of her gum before I trudged off to work.  She keeps her mouth closed now till she has to make a yell of GET OFF ME!!! STOP POKING ME WITH THOSE DAMN NEEDLES!!  But I wish it lasted as long to say all that.  It now only lasts for a WAA!  I'd be happy with a WAAAAAA with six A's.  This mornings two a's meant I lanced her lip twice, tongue once and the gum just past the blister once before I was able to get it twice to my satisfaction it wouldn't grow anymore.  She knew where it was and protected it well.  If she didn't have EB I bet with a similar athletic ability of her sister combined with her ability to squirm in protection of what ever it be, her strength, determination and this growing innate savvy I'm starting to notice, would make her a shoe in for the Canadian Woman's National Hockey Team.  It took all Renata and I had to keep her from fidget and I still missed repeatedly.

Yesterday's dressing change was also a pain.  She has come to the age of awareness.  When her hands come out of bandages, she gets very excited and loves examining them.  Then when it's time to hide them from distruction of her face and hands themselves she get's pissed!  Lucky Simona was there yesterday to read to her.  Renata is rather flu ridden and only good for short spirts.  Did the reading ever make a difference.  She looked through a couple books multiple times quietly and intently, while the nurse and I were able to finish up.
I started to let the nurse do the change, but as Raquel got fussy I took over again to make sure we were quick.  Hmm, not the way to teach.  Learn by fire is usually how I like it.  Just maybe not teach. I think the quiet days of smooth dressing changes are a realm of the past.  It was absolutely a three person job yesterday and even that became a problem with one hand airing while two of us we were at her feet.  Then when one person had to do something else outside of the immediate job of occupying a limb, it became chaos for the other two.  As in changing books.  Apparently they don't make children's picture books at novel length for 9 month olds to get us through the hour plus dressing changes.

Saturday, March 5, 2011

Another day in paradise.

The day started in good spirits.
But by the end Raquel was covered by blisters.
For those with EB or have lived with it for a while, it must seem strange to think back to the early days and the panic over every little blister.  Some are of little significance, true, but some look so painful and uncomfortable that it's hard to not feel her pain or often envision it even if she doesn't seem to notice.
I had the splints on her hands and they always seem to make a mess. 
Good hands for when there is a cure, or bad hands and having them somewhat useful? Vaguely paraphrased from a new friend of ours whom also has a son with EB. 
We have the big chair Simona brought.  Raquel loves it!  But she bangs and beats her hands against the tray in excitement for nearly everything that happens around her.  The first couple days, nothing happened to her arms from this action, but now she's started to pile on the red open sores on her arms and elbow.  A place previously almost unblemished.
I feel like taking my jig saw and chopping out a good deal of the plastic from the leading edge of the tray and glue some foam on it for protection, but I fear I'll probably destroy it in the process.
Maybe I should just pay Simona for it now and chop it tomorrow. 
Raquel also has a giant loose waterskinbag that goes from her collar bone down and around to half way up her shoulder on her back.  The worst place I figure to have blisters less the mouth area for her right now.  Renata finally admitted to it by thinking back to where it likely happened. 
Raquel on her side in an awkward position, freaking out because she was pinned by what ever.  Renata reaches down to pull her up and there it is a 6" blister.
As disturbing as it sounds, she's been a dream since.  The dressing change wasn't so bad either.  Although I think she's starting to figure out it's a time she probably doesn't want to be on the table, as the fuss and fights have been escalating over the last week or so.  Joy.  What's another month of awareness going to do?  She'll start the fuss as soon as she sees the nurses or me open the big white door with all her supplies.
I know this fuss isn't pain because of the early days, plus there is a certain pitch to her cries that can bite deep into my being when it's pain.  Problem now is she would rather hang out in her various chairs and watch her goofy sister do her goofy best, who wouldn't?
The High today for me unfortunately didn't come by route of Raquel.  It was her big sister.  At bed time I was told to go into Cordelia's room for a surprise.  Was it ever.  Cordelia read me a book.  For the first time. 
I often pretend not to know the words to some Czech songs she likes to sing before bed, so that I can have her sing them to me instead of the other way around when Renata puts her to bed.  Maybe I'll have to pretend not to read for a few weeks and sneak a few more of the cutest bed time stories ever. 
Discovering her hands, yes I know the drift looks bad on the center finger!
Raquel now does a few signs.  She's very intent to what we are doing and is getting good at mimicking our movements.  Yesterday Renata was trying different things from waving her hand goodbye and hello, then clapping etc., then the hand quickly oscillating to her mouth making the ohh ohh ohh ohh ohh war cry sound. When Raquel's over sized hand with hard plastic splint and three layers of foam went up to her face repeatedly bonking her nose in somewhat of a spastic reflection of Renata, Renata quickly realized her brain cramp and that was probably a sign we'll not attempt again.

Wednesday, March 2, 2011

Back to normal

Raquel has stabalized into her regular routine of mouth blisters and what not.  A few spots here and there on her body, but I still marvel at how spotless her chest and back are right now.
It's so beautiful to see such a huge area of her body pink and fleshy perfect and soft.
Simona bought us a child (giant plastic) seat for Raquel.  Normally I'd not have let it in the door.  Firstly I wasn't there when it arrived, but I could see it's purpose so I humoured Simona.  The fact it has wheels was proably the main if not only reason it stayed.  At least when I'm tired of looking at plastic in our living room or dining area (same place really) I can wheel it to kitchen or office area.

The best use I've found are during the times where Raquel and I chase Cordelia around our place.
And when Cordelia falls,
We run her over.
So much fun.

A couple nights ago with Renata and I preparing a dinner for friends in the kitchen Cordelia wheeled Raquel into the bathroom with her while she did #2.  Renata saw this happening and mentioned the door should not be closed.  But from where I was standing that little parameter set out by her mother didn't quite get past the ear drum, I watched the door close.  We could hear them talking back and forth and giggles here and there by each.  It was amazing to see - achem, hear them off on their own, with minor supervision.  Being a team.  A very very happy moment in parenthood for Renata and I to say the least.
We don't have a lock on our bathroom door, just in case anyone thinks we're crazy for letting a 5 year old wheel away a 8 month old with semi-predictable skin issues into a potentially locked room.
Babi doing her magic
Butterfly and Cocoon (I'm in awe at Cordelia's growing ability with her hand eye coordination)