Tuesday, April 26, 2011

Bad Daddy!!

I come home after watching the game seven of the hockey series that is going on right now between the Canucks and Blackhawks to find a drawing on the kitchen table (the Canucks won by the way).  The drawing clearly states by Cordelia's hand.

To Mom
No Dad.
leav.  (my interpritation is 'leave it alone!!')
hmmm
What's underneath?
The best Boochta (not exactly the Czech spelling) Renata knows how to make.
In fact it is sooo good I tell her not to make it that often, because I simply can not control myself.
I'm already on my second piece.
You know when people talk about setting someone up to fail.
Cordelia did that to me.  She got Renata to make a cake while I was out.  I come home after a couple pop and while in the kitchen looking for something interesting to eat I notice a neat little drawing from Cordelia and under it is my favorite cake.
Walking on by, was never ever going to happen.
Ryan gets Zero.
but mmmmmmm
It was slightly worth the crying I'll hear in the morning.
Raquel watched the game with me till about the second period when she finally started to settle (it was a party for her too).  It wasn't till the Canucks missed a few chances that I had to hand her off to someone a little less vocal.  She was so dead to the world that when the winning goal happened in overtime, she didn't even budge.
She has been great, outstanding, Wonderful since our last episode.
In fact our daily doses have dropped down considerably as she has been falling asleep shortly before the time to be given and wakes up happy without.
YES!!!
I was telling Raquel who Spock is


Focus like her sister.

Friday, April 22, 2011

Shaken Daddy Syndrome

The Kid Is Alright.
But it took her daddy a two days to recover.
It seems only a few weeks ago I was saying that we get a couple days a month to sulk and be sad about our lot in life and that of our kid (s).  I just took my two.
Wow, glad that's gone.
What it took was a great dressing change.
She was very well behaved and didn't mutter a cross word at us.

That doesn't include when it's time to do her hands and the mad flipping she does to try to rid my grip and the first few layers of visco paste.
Her dexterity is raising quite quickly of late. 

I've lessened the layering and stopped the complete wrap of her thumb.  We were given a soft splinting material to keep her with the rest of her species displaying oppose-able thumbs.
In our recent talk with the Plastic Surgeon he mentioned that even one good trauma to the finger could inhibit her range of motion down the road.  People have very different skin types and beside the fact Raquel has EB, will her skin heal tight or keep it's elasticity?  Of course after many broken and re-healed wounds, she will lose her elasticity.  Her feet have long been a glob of oozing, thin red sores with a few patches of healed close to what her normal colour might be.

(Just had a SCREAM FIT, from where I don't know, what I do know is - I'm no longer traumatized by her, as I didn't immediately hand off a screaming baby and hide somewhere till she stopped and was fast asleep for the night.  Which was exactly how the last two nights shook down.  'Shaken Daddy Syndrome'))

I talked today with our nurse that her feet don't seem to be healing deformed and tight due to what has taken place there.
Who knows?
I've slowly decided that we'd experiment with her hands.  It was bound to happen sooner or later.  Just that we jumped the four or five years originally suggested.  The strange stricture in the middle of her palm has forced this maneuver.  The word of encouragement from Casey's mom helped a lot.  Seemed I kept digging up this or that all the while I was talking to Raquel's Surgeon.  It seems her hands were doomed whether they were bound so we'll see what it's like when not so much.
She's in great spirits as I can hear the three of my girls reading a book from Cordelia's room.  Each of their voices can be heard in even intervals. NICE>
I guess that was the end of that valley.
Sleep.

Thursday, April 21, 2011

Day after - Good. Although I'm spent mentally.

My mom came back as was holding Raquel while I passed through at lunch.
Raqu looks as exhausted as Ren and I.   The wet hair look isn't that, it's the glaze of aquafor we used at her nose and eye area so that in her fits she doesn't push off all the skin.  The milia build up on her nose is quite evident here.  I've heard she had a good day.  I'm attempting work anywhere else but home.



Wednesday, April 20, 2011

Like Clock Work

As soon as I say, things couldn't be better it all spirals into chaos.
I've had a few dressing changes when I've not coped well with the stress, but today's brought me right to the edge. 
Renata had a feeling this wasn't going to be good and did her best to track down some more help from our nursing pool, hopefully to fill in some extra hours we're owed.
Renata was off to a Board Meeting for Cordelia's school and my mom was called in for reinforcement.
The hard facts with my mom helping is that when things go sideways I don't have the patience I should for her, despite the the fact she's there to help.  Why she keeps coming back after me acting like an ass I'll never know.
To start Renata had two hours of pure sleep last night that took place before the clock had actually passed over to the next day.  My night didn't involve much more pleasant sleep.  We took turns in the living room settling Raquel down in half hour blocks.  Raquel's morning wasn't much better, but at least for my sake I was off working staring into space as some major issues came up over a job I spent considerable time on in the previous few weeks, luckily the blame was spread fairly evenly around the office. 
Renata eventually took her off for a walk to get Raqu calmed down. 

During the dressing change and Raquel's violent thrashing we dealt with three separate mouth blisters not including the popped deep skin flap at her uvula that flapped like a flag in a hurricane during her protests and yelling.
Coincidence that our Plastic Surgeon the day previous gave me some very useful tools to get in there to pull and snip those flaps.  I was too chicken to do it as we don't really have a med on hand to calm her down enough that we won't make more of a mess keeping her mouth and head straight for such an invasive operation.  Plus it was clear her breathing wasn't impacted.  She never relented for almost the full hour plus and her half cries of F#@& off!!!! LEAVE MY HANDS FEET AND MY SORES ALONE!!! (Would she have my language skills).
I know the issue is her bottle.  We have yet to come up with anything that works, other then feeding her solid food full time with a spoon.  I get very pissed at myself that we haven't made it more of a urgent issue when times like these erupt.
I get crap for the hands often that the techniques aren't working.  In our recent trip to see our plastic surgeon he couldn't really give us any more info or techniques that we weren't doing already.  Seems one of Raquel's major Commenter on this blog is about the best tried and true informant we got.  Problem is a continent lay between.
I'm not sure if it's right that I fire back about the mouth issues considering much of her feeding and nutrition is Renata's gig.
Anyway, it was so tough I didn't actually finish getting her hand wraps on as I usually require.  It was more of an emergency layer that I'd expect from a fumbling ER staff.  (I'm not kicking what they do, just seen some bad wraps in the past (Cordelia's broken arm, my broken thumb, arm etc.).
I'm only vaguely worried they'll be off if she freaks out for any extended time this evening or next morning.  Only because my circuit board is fried.
She finally just woke from 2 hours of peace.  Started where she left off.  I still can't cope from the damage done during change. Renata has to.
Her throat is coarse and sore.
What a day.

What I can say is my friends are great.
I sent off a couple texts while I was in shut down mode after the change and Raqu asleep, mostly out of self preservation that I didn't walk into my garage and destroy what ever I could get my hands on out of pent up rage!!  Mike shows up with two beer and some salad.  Nice!  He did so despite being dead tired from overwork and his own family life, but not too tired from helping out. 

At the moment Raquel has blood all over the arms of her brand new silks.
Crying.
She can't actually scream any more, even tho it sure looks like she wants to.
My shift will start soon.
Joy.
I really hope her break through dose and Tylenol will work.
Tonight I hope we have

Strength.
Joy. (sarcasm)

But I'm sure we'll have
Blisters.
Pain.
Suffering.

Joy.

It would be so nice, if it were just the final push for her teeth that should be showing about now.
If it weren't for her kicking her feet and rubbing her eyes I'd let her have at it like we did Cordelia.  Still can't leave this girl alone for 30 seconds of a crying fit.  Damage can be too great and the cycle continues. 

Tests of a marriage.
Our other family member was exceptional, Cordelia the whole time was drawing contently and the dining room table and only bugged me for a glass of water.  When I passed it on with Aquafor grease she ran away disappointed and still thirsty and wasn't seen for another 45 minutes.

Elbow at change time


Elbow this morning

After an hour from when she woke she's still at it....
I still can't cope.
Renata is a star.

Another hour later.


Sleeping...

Sunday, April 17, 2011

Giggles

Mommy gone and we're doing great.

Grandma has been a great help.
I don't feel too guilty about it as Ren gets that help too.
Coco and Raqu have been a dream today.  Funny, I always have an excuse to disappear.
Work.
Play.
Something that won't happen again for a year, usually to do with snow or salmon runs.
Renata always lets me go.
She's super woman for certain.
This weekend she's out for her first real crack at a full nights sleep.
How is this possible?
If I don't have a decent sleep at least once a week I'm a wreck.
Ren is going on 9 months...
I should have gone to sleep hours ago, but now I mind as well wait for Raquel's 1am med and feeding time, there are odd rustles from her direction so it's approaching.
She's like clock work usually with her dosing.  Except, today.  She doesn't really seem to need it and has pushed over by an hour twice.  Hmm I'd like to get that back to 4 times a day, not the present 6.  She's been a really happy kid, less the usual thrash 10 minutes before she falls asleep.
Her face has healed up very nicely and the little pockets of milia are almost the only evidence of her disease while clothed.
Her lips are blistered and raw, but that's the look of any kid coming out of the desert.
Her body at the moment has only a couple dime sized spots on her elbows from her dressing change bath then the usual knicks from our finger nails while changing her diaper.
She's a super star.  And this good run is welcomed.  Saves lots of energy for when we need it.

Funny that the most stressful part would likely put a normal family through a loop.

We've noticed a funny tightening of her skin on her palm.
It's strange because I thought the strictures (? not sure technical word for it) on Recessive Dystrophic only happened when the skin has been blistered and the scaring is to blame.  We have a call in to meet her Plastic Surgeon on Tuesday to do some problem solving.
The many splints I've made work in many ways at preventing this and that, but now this.  Her fingers aren't drifting like they were anymore, but it won't take long to create a claw if her skin at her palm tightens up and heals together like it looks like it's heading with this new discovery.
I'm glad when Renata phoned the Surgeons office we could have had a meeting within hours.
Seems Raquel gets the VIP treatment.
I'm finding some of the medical things very interesting, problem is, with basic Raqu care, Work, Family and Fun getting a deep grasp on some of these issues is mind numbing.

Ren is lucky I haven't let her get an phone, or else I'd be invading her sleep just now.
Peaceful sleep in Victoria.
I'm actually surprised how cool and quiet it seems sans Mommy.
I haven't heard really a peep in four hours.
haha, it's almost never like then when Ren is here.

hahaha

Sunday, April 10, 2011

Trying Raquel with some easy words




Not sure even in my most persuasive ways I can convince my ESL wife those are words worthy of a triple word score in Scrabble.


Friday, April 8, 2011

Vancouver in the Spring

Cordelia, Renata and I had Grandmother look after Raqu for the morning while we went for a couple hour ski up our local mountain. 
The sky was clear for as far as the curving earth and distant mountain ranges would allow.
The mountain we ski has a tram so only batches of 51 can get into the alpine at a time, so for the first 2 hours the runs are bare.  The sun softened the snow significantly by second run and we enjoyed smooth arcs all the way down each run.  Cordelia showed Renata what was up.
It's been so long that Renata and I have skied, I'd forgotten how well she moves. haha..
She did a few spritely turns through a mogul patch and their were sparkles in my eyes.  Once and a while it still happens.  Haha.
These pictures are from two days prior.  We were having too much fun to stop and photo op.


I have three years to rig a sled contraption similar to a snow limo that I can get our littlest ball of excitement out to enjoy true family outings.  Really, I can't wait!!!!
Except the roll bars and cage will have to be titanium for the speed I'll like to travel...

Wednesday, April 6, 2011

Did I mention I dropped my phone on her nose.


Shame after all the tough moments we get I drop a phone on her nose, where it could have easily been avoided..  Somewhere between the phone being too slippery and me doing two things at once, ie. feeding her a bottle and showing her videos of Tripp, which she loves and can't take her eyes off the screen. 
I thought I was going to have to jump off our deck head first as I wasn't sure it hit her eye or where.  Total sickness. 
Cordelia remarked to Renata from the back room.  'Daddy said a bad word.'
The next day, at lunch was her actual first eye injury, to help put the rush to hospital in perspective.


We had a little mouth blister this evening.  We shortsightedly just finished changing her clothes so that she would be clean of blood going to bed. 
Not so much. 
Again she looks like a vampire with a bib of blood, but a beautiful Vampire at that.  The yellow of her shirt has gotten rather orange.
Once popped, without my recently typical missed shot, she was a pleasure and melted to sleep in my arms, sans iphone. 
I'm quite put off by this as I'll be forced to look at the many little Milia after the open wound has healed and gone away.  The Milia are those damn little white spots that form on her healing skin, her nose area along the side of her eye is full of them.  Thanks to daddy, add another 20. 
A side note - normally we wouldn't buy Renata a leopard print house coat, but Simona thought her old one (not leopard) was too old and no longer comfortable, so she tossed it and inserted this one.
It's sexy,
I think,
or so I'm told?? 
At least I get a giggle when Raquel breaks out into her fairly common playful ROAR and Renata is wearing it close by.

Monday, April 4, 2011

Eyes open and fine.

Turns out when we arrived at the Dr. office, Raquel first opened her eyes.
I have panics about the potential of eye injury with her.  I have this fear her eyes will puss up and the outer layer will fall off like the interior of her mouth did, way back when.  I'm not sure this is even possible, but I didn't think the way her mouth fell apart was possible either so what other nightmares are out there for us.
If she's going to be limited in so many ways of experiencing the world I'm very anxious she not lose one of the primary senses.  I fear with how I like to see the world and interpret it when so inclined, her potential loss of sight would be really tough on me.
Of course life changes with every little hurdle.  At first we were worried about her hands being wrapped so much, but now we both wish we could just get rid of her finger nails.  They cause more issues then they are worth.  Eyes?  Hopefully never.
So the panic level was high when Renata mentioned she'd scratched her eye.  We phoned our Pediatrician and Dermatologist then phoned another parent caseymommy for advice if such an occurrence had taken place with her son.  Renata reached her husband and his first words were, "It's not a national emergency", as well gave some more useful advice, if this quote wasn't the most. 
It was actually in the parking lot of Childrens that she first opened her eye, she looked as though it was painful, but she was willing to keep them open.  She grew more comfortable by the moment.  We waited for an hour to see our newly appointed Ophthalmologist, we had to tell him that during our visit.  Raquel was gold the whole time, by looking and getting the attention of all the kids around us.  She was great at her Tiger growl which received a few giggles at those she attempted to scare and waving her arm and turning her head at those who didn't.  Clearly her eye issue was a thing of the past.
The rest of the afternoon was a party by Raquel's standards.

We had no nurse today with a troubling trend when our few regulars get sick or can't make it.  We are forced to call in on our friends for H E L P.  Simona came over on an unscheduled visit, she was great although I still managed to get her irritated with me due to a few of my personality quirks. 
I don't always say goodbye when I leave the house or when someone else leaves my house, usually a family member.  So when it's not a blood relative it's actually a great compliment.  Well, not to Simona as she stood at the door exasperated staring me down.  Then when it hit me (after a nudge by Renata) she's waiting for a goodbye, I of course obliged.  In my defense I was looking down and feeding Raquel at the time, engrossed by her beautiful little face looking up at me and of course all the world around me ceased to exist.

Dinners from Cap Church have started again, so really, everything fell back in place quite nicely for the rest of the evening.
Grumpy turned 68, so we had a glass of scotch and a slice or two of Pavlova.

I have been looking at Tripp's videos Courtney has made.  Wow. 
She talks about the breakdown she's allowed once a month.  I agree.  She can have one.  But our tasks as providers of well being can afford only one a month despite how we feel as parents of special needs children.
In my weekend off with Karl fishing, that was my big thought.  Other then what went wrong by only being able to catch three fish.  Karl had none, so at least I'm on the right track with one of my endeavours.  The thought was the best thing I can provide for Raquel, other then being her advocate in all concerns medical and getting better at tasks I have with bandaging, is to be sure she's happy.  This disease certainly beats on the soul it has in it's grasp and those so close by, but if all the other times are good and based in happiness then her quality of life should not be a problem.  It's very clear to me that young souls need that deep nourishment of love, when that's clear a child missed it things so often trend to dark places.
A few minutes ago Raquel woke up in a fit.  She bashed her nose and went off for 10 minutes or so.
Renata was up with her, I had to also wobble into the living room to help give her medication.  I went back to bed shortly thereafter while the two cuddled sleeping on the couch, but I was overtaken by a cold sweat.  In minutes my hair was soaked.  Now at 2:30am I'm writing and adding to a post from earlier in the evening.
So many thoughts. 
So many issues. 
So little time. 
Have your break down.
Just don't let it last. 
It helps no one especially the ones who need you most.

Scratched Eye, The Next Mild 'Non National Emergency'.

Home for lunch to see kid screaming with eyes shut. Corneal abrasion, joy.
Off to ophthalmologist. So much for work. One to hold her, the other to drive.

Seems Daddy was missed.

After a weekend off the grid, I find out Raquel didn't sleep much and Renata's and my battle of "who's more tired" was a no contest.  Sleeping near a babbling brooke bound in a mummy bag on an air mattress after paddling and fishing for 8 hrs a day in slightly cold in near sub zero temps versus up every hour on the hour for two nights then wide awake to tender one semi sick kid and the other whom you can't let your eye off for many more then 60 seconds.
hmmm.
At least I wasn't greeted with my clothes in a box at the front door.
Now that I am home, Raquel only woke up twice during the night.
Seems I'm a little less shy about administering comfort doses to keep the edge off and her in fits of what ever it is she's going through.
Yummy Cuttroat and my TV dinner tray

After this how could I possibly complain when Renata gets her spa weekend?
Anyone taking bets she chickens out?  Problem is, she needs to find someone also willing to ditch out on family obligations.  I have no shortage of those kind of people.
Tent front door view