Monday, December 27, 2010

Typical Foot Blister of The Large Variety

Oh Joy.
Not sure how this happened.
There was the start of something last dressing change.
I was very surprised it grew like this.  We've been chalking her displeasure up to teething of late.
I thought I'd show a little video of how the operation looks like.
The back ground bouncing sound is Cordelia playing basketball with our yoga ball; in the living room, of course.  The last comment before I was cut off was "we can blame that one on Andrew".  He was to busy looking good for the camera. haha..
Over all.  I'd say this went very well.  Raquel was calm and fed right through, less the moment I pushed the fluid out of the blister.

Raquel blowing bubbles (top side)



I've recently discovered a super star kid with EB.  Talk about your courage.  I like the part where his dad told him he was a good kid because he didn't wreck anything when he was young, yet his glasses made it under the tire of a car.  Back to my future visions of Raquel.  Just a little before the mysterious woman in a veil is a happy kid full of life like Zack.

Oh yeah.
We've had a bout of a mild version of pink eye in the family.  Tough wiping guck out of a kids eye you don't dare wipe guck out of.



Hopefully this little photo montage didn't start it all over again.

Sunday, December 26, 2010

Christmas Day

A much much better day.
Just the normal baby stuff with older sister beyond tired for the entire day.
I didn't notice how tired Cordelia looked till I took a picture with our new camera.

I'm a little sheepish to how that was a surprise!

The old camera was a good one but likely had too many sunset shots (5yrs-don't make'em like they used to), a voice calls from my recent memory while taking a ferry from a small island to Malta, my friend asked, how many sunset photos can a man take?  In my case lots and lots, especially in the middle of the Mediterranean Sea.....
Anyway...
Christmas Eve didn't have the scheduled change due to many factors, (mainly me coming home two hours late from a fishing trip heavily dampened by the pouring rain) so we decided to do it Christmas day.  We were to go to the Wentzel's for dinner.  Luckily Andrew called to ask if he could be of help, just in time too, as he helped out for a nurse-less dressing change.  He probably phoned because he realized we'd be that much longer if we didn't get the help and dinner might have been cold waiting for us.
All went really well, she coo'd at us and I only had to smack his hand two or three times for him to focus or move his fingers from the areas to  be placed under a few layers of bandage.  It's a little different having a man's forearm blocking your vision then the nursing help or the few other friends signed in for emergency changes (all of whom are women - except Grumpy (dad)).

Once Raquel was fresh and one outstanding blister of little consequence lanced we were off to the Wentzel's for another fabulous meal.
Penny (Andrew's mom) has trained him well, as they teamed up and did an outstanding job, while the ladies and I played with kids and mildly imbued.
It was a Merry Christmas no question, but often there is something little or big that defines certain times or Christmas giving.  What blew me away was the gift the Moja Coffee crew handed us for Raquel.  I often walk in there for an espresso or two at various times of the day.  Usually giving the guys behind the bar grief if I can.  Someone has to look out for Andrew when the mice come out to play and the cat isn't around.  Knowing how they do their thing ($ for Haiti and a few other causes) I was stunned to see they pulled through for Raquel in such a manner.
Over all I should have said thanks to others who have put in various amounts of money to her account.
I have found it strange being on this side of the fence, having people reach out to help us.  It's honestly slightly unnerving at times but amazing all the same.  So really thank you all so very much!!!!
And for everyone who has lent a hand.

Really, you all ROCK!  To each and everyone of you a Merry Christmas.

I keep putting shots of these two sleeping.
It's my favorite view these days, Renata and Raquel fast asleep.  Apparently, Cordelia has to stay awake to keep me company.  Tough job for a five year old.

Friday, December 24, 2010

Christmas Eve

We are in the dark side of a few great days.
From sleeping straight through a night to not eating for a 6+ hrs because of those pesky mouth blisters.  Then there's the new blister taking up the entire area of her arm pit.  It was hard to hold it together when that on became evident.
The combination of the two made for a very enjoyable Christmas crab dinner.
She cried, right on through.
Jumping from Mom, to Renata to me.  Grumpy (my dad) looked as out of sorts as she was.  Eventually all the commotion seemed too much for her (or maybe me -who's grumpy now?) as everyone was sent off.  Raquel is in a five minute quiet time of light sleep.
Hopefully it'll last an hour or so, so that we can get in some food when she wakes, it's usually the time it takes from popping blisters to her finally being able to eat.
It's was actually funny now that before and during dinner Renata and I were pissed off at one thing or another, Raquel was pissed off at a few things in particular and Cordelia is still pissed no one can focus and open some presents with her. 
I'm not so sure about the Euro Christmas giving and opening Christmas eve with the combo of what happens in our typical Christmas morning. Too much opportunity for being spoiled I think.  That being said, I'm pretty happy just now Raquel is giving me 15 minutes of peace and quiet.  There are still wet tear streams down the side of her face, sweaty forehead and furrowed brow.
Raquel's basic health and comfort this week was so great.  I figured we'd be able to make some rounds and say hello to some great friends of ours for a drink here and there.

No such opportunity this evening.
Well.
Maybe.

I've been off doing a few things just now and Raquel is still deep asleep.
Wunderbar!!
Renata is walking across the street to see our neighbours with Cordelia, I guess I'll watch 'Duck, You Sucker', again.

Hopefully Raquel won't hear all the pyrotechnics.

Merry Christmas.

Wednesday, December 22, 2010

Super child

Today was exceptional yet again.  We're soaking it up, believe me.
The only thing to say is she's teething for sure.
She's drooling like crazy and trying hard to stick her bound hands in her mouth.
She's had a regular dose of Tylenol to keep that at bay and it's worked.  We're too worried from past experiences of how a solid hour of fuss and discomfort makes for many blisters in all places.  Most of her facial scabs have fallen off and if this keeps up she'll be a glowing baby in a few more days.

We left her with my mom for a few hours to check out the German Christmas market in town.
Some nice Pork sandwiches, a Bratwurst and sour kraut (I actually ate it Frau Blank, for you and only you. I'm sure you smiled and wondered why it has taken so long with a common wag of your finger at me in disapproval of all those dinners I past up due to that skinky stuff) The Sauer Kraut was chased by a few Pauliner Lager mmmmmmm.  Renata went to the Gluwien.  Cordelia made a chocolate on a stick and was proudly walking down the street with it on our way home, till an older slightly drunken man grabed it and ran away.  He eventually gave it back after she chased him down, but Renata and I weren't too interested in her eating it with his melted finger prints across the middle.
Oh well.  When we picked up Raquel all was well.  Dad didn't even break a sweat (he gets slightly panicked in his old age at any difference of pace). 

Time to join Raquel in a peaceful sleep.

Tuesday, December 21, 2010

Sleeping Beauty

Dressing change was great.
Renata talked about how wonderful she was today.
No new blisters.
Everything is healing well.
We are effectively at the top of the roller-coaster.

Saturday, December 18, 2010

Pictures From a Change

It was a great change.
Raquel was in such good spirits that we decided to not give her her typical dose.




She wasn't at all upset.

Wednesday, December 15, 2010

Mouth Blisters.

Today was something special.
The blisters hidden under the bandages foot and hand were looking fairly well.


Yet Raquel was in painful fits.
She cried through most of the change, it wasn't till near the end when we noticed the giant blood blister inside of her mouth.  It basically took up almost the entire upper palate to her right.
When she lays back the blister flows to the back, there it's hard to access or even notice.
I tried to get it with the needle as she was in a sitting position.
That didn't work as my fingers couldn't get past her lips.  So we extended the needle with an oral syringe.  My dexterity was a bit off as I didn't feel too much of a connection to where the end was.

I missed judged the tip a few times puncturing her lip and a couple spots at the back of her mouth.  Fine if you're an acupuncturist and you're thinking of relieving pain that way...  My terrible aim only added to our pain and suffering.
I finally laid down on the floor and Ren held her over top of me so that the blister would flow to the front of her mouth and onto her gums.  With my head lamp I was able to see in and lance it a few times.  Although it was basically hanging out of her mouth in that position.

Tonight was Coco's Christmas concert, that was to start a half hour after the change.  Raqu went from the dressing table to the car seat, within moments of the lancing and buckling of the seat Raqu was out.  Talk about your instant relief.
She watched a little of the concert and went slightly spastic during the clapping, but generally loved the action and music.  We've hardly heard a unsatisfactorily peep from her since.

I felt slightly guilty about my last blog mentioning the wine we received at one point with our dinners.
For those who are helping out with some dinners for dressing change evenings to come, I wasn't making a call out for alcohol to medicate us of sorts and smooth out some stress.  We can deal well enough.  The dinners are amazing enough.  Please don't feel it necessary or the norm.  Although, today, the wine will taste soo gooood...  My wits and patience were shot, low blood sugars or what ever.
I didn't quite get to say 'Dammit Janet'.  Could have, but I'll wait till the moment is less stressful for us and our new nurse.

Raquel just pipped up.
Feeding well.
ahhhh
time for a glass of wine.

This little bit of info didn't let my day go smooth either.

Patient 13 coming-home?


I unfortunately found it hard to be happy for this poor boy.  Probably more to do with our household and a few of those sentences written. patient 12 and 14. 21 years etc.

I gotta stop...
glass of wine.

Back to see the crew of Doctors.

We decided not to do our typical dressing change at the hospital today.  Last time we came home with one of her largest blisters ever.  Hard to focus with questions and ideas on her care while concentrating on a moving target.
Pain issues and over all comfort was a main area of discussion.
Where is the line between fussy moments with a baby without EB and one with EB? 
In a annoyed second of hunger she can produce a dime sized blister as she raises her arms quickly to her cheek or eyes. She has a Dr. Jekyll and Miss Hyde thing going on now.  Her obviously dominant right hand has turned the right side of her face into the surface of the moon, her left side is still quite angelic and not much different looking then Cordelia at 5 months.

In the last week most of her sessions of pain or discomfort have been a result of either mouth blisters or her continued and spreading sores on her feet.

The ones in her mouth are quite scary.  She was choking a bit last week.  I could tell the water in the blisters was rolling back under the top layer of her tongue and past her uvula.  For a few hours one day she'd cough her EBM up and cry in pain.  I knew she had mouth blisters at the time, but usually when we spray the milk from outside her mouth she'll still swallow.  This time I could get it in past the blisters on her tongue and gums but what ever was going on in her throat was having none of it.

It's painful to read stories for operations on DEB kids to take away the built up scar tissue there...  Let's keep that from Renata still...

Can you tell, we eagerly await the addition of solid food?
One of my buddies used to make light of a Simply Red song directed to others of older generations stuck in the 70's 60's etc.  The glory years.  It's just a quiet under the breath "Jee, Holding Back the Years".  Usually I get a little giggle.  It's not so funny right now as it's not even years I'm looking to hold back.  It's the months, weeks, days for us.  When I think of him now, most of us boys from 20yrs of friendships are well into family lives kids etc., hahaha he's still a swinger playing vinyl in clubs to all hours.  I'm not so sure he'll play a Simply Red song anytime soon, but his snide humourous comment seems reflected now.

In any case, our conversations brought us to stay ahead of pain with medication.
We usually see when dressing changes are going to be difficult before hand.  As she tends to have fussy outbreaks that progressively get worse.  She's been a wonderful kid in the disgruntled child department so when she's not happy it's not because she just plane hates the world.  There was only a month or so of the evening colic I've heard about from others.
Now she just arches he back and cries a bit then you'll know she's moments off from sleep.  Anything else at different times will usually give a signal that something is up.  We usually find out eventually, but sometimes because of a Monday, Wednesday, Friday dressing change regime what's hidden usually stays hidden and she and we live through it.

The great part, (if there can possibly be a positive to EB) is if a blister does make itself seen, is how quickly she settles after lancing it.

I wonder if it's like removing a big ugly sliver from the tip of your finger?

The next part of our visit was to design new boots with the OT department.
hehe

They look like moon boots or those surfer boots that went urban hipster a few years back.
I'm hoping they work with some minor modifications.
So far I've only put one on.
I'll deal with the unwrap of the other foot tomorrow.

Renata already forgot to put it on after a diaper change.  I caught Raquel at the beginning of a kicking motion.  I'm not very confident she didn't do some more damage before I isolated her foot.  She's been sleeping well for a few hours now, so maybe we dodged a bullet and tomorrows change will only be about monitoring her healing feet.





Cordelia, Raquel and I were playing shoot the rabbit in our living room last night.  Cordelia was the rabbit, with Raquel on my lap I'd hold her arms and pretend to shoot Cordelia as she ran back and forth in front of us.  Cordelia would pause and we couldn't shoot or we'd be out.  So she'd be standing in some very funny positions with contorted facial expressions, Raquel was giggling something fierce the whole time.  She absolutely loves her big sister and being in the middle of the action, that is clear.

Sunday, December 12, 2010

Simona - Summerized by either 'Queen Bee' 'Worker Bee' or 'Bee in the Bonnet'

I've mentioned Simona before in this blog. 

Simona was the one who offered her basement to us in the first hours of going to the Children's Hospital.
The proximity was a short walk with free parking.  Renata and I in fact started our live-in relationship there as tenants.  As well, a long drawn out story I tell only when I have a captured audience.

Simona was the one who offered to help Renata out of a tear filled call to her parents. Where more blubbery sobs then actual coherent sentences would have been the case.  It was necessary and helpful that Simona too has Czech as a first language, as it wasn't going to be a call I was able to make on Renata's behalf.  I'm OK with the many colourful and practicle words heard in a Czech Tavern, that's about it.

Simona was the one who while Renata and I were headless chickens and loosing our marbles in the first days, brought in full bag of toys and devices for Raquel's mental development.  A few were promptly attached to the incubator for her viewing and listening pleasure.  The fact was, Renata nor I were even sure Raquel would be there for the rest of the year let alone need those early building blocks for her development well on into life.
Once we were able to digest what Simona was doing and see past the new sores and obvious pain, it made a world of difference in us understanding Raquel as a normal baby, but with skin issues.  It also helped getting past dark thoughts, to imagining Raquel living a long and enriched life.  A reality it took a little time to expect.
 
After Renata and I had spent long days in the hospital and needed to go home to be with Cordelia and recharge, Simona would take the evening.  We also had Louisa in that department too.  The two of them would go in on alternating shifts and read or talk to Raquel, hold her and be a loving family presence for her, for us, as another needy girl was at home.  I can't think of too many nights she was without someone deep into the night.

I have to watch how much I praise Simona due to the connection of the 'bee in the bonnet' part.

Simona was asked to accompany us for a few of the early Doctor appointments, and ask the hard questions we were either to stunned to think of or scared to ask.  Sometimes her questions would go a little off topic.  But giving a valuable pause in the action to think of how to frame questions and contemplate vague answers.  The best part was watching the doctors or other professionals squirm by her rapid fire questions and knowledge of the system.

Ask for a pitbull and receive a pitbull.

Simona set up a dinner run of neighbours so we'd be nourished during this time, the many bottles of wine that accompanied the food was very much appreciated and kept the evenings soft and nights deep in sleep.

Once the time had come to go home from the Hospital, Simona was here too.

She did a few night shifts as Renata and I slept.  In the time Raquel dozed she'd pick a cleaning project, our house was cleaned from ceiling to baseboard.  Our oven, our fridge, our bathroom, our hood vent, Cordelia's room and everyone of her toys.  I made sure to suggest a few places of off limits.  She hears what she wants.

I look over my shoulder now and realize Simona hasn't been here in a while.  hmmm.

We had many disagreements over placement of this or that.  Certain lighting situations were to be fixed.  Beds to be moved, furniture to be bought, all was in question and much had to be argued for or firmly stated with a NO. Still if she really wanted to do it, it was done, regardless.

We had bleach in our house for the first time due to Raquel's baths.  Simona was still able to up the anti and brought in a whole whack of new products we only see in commercials.  We actually don't bother walking down those isles in the store.

After a while I or we couldn't help but put a foot down.  We suggested that if she must, please get some of the more natural products.  And yes we know they clean like SH^*
The toys, beds, rockers or 'learning tools' became a problem as plastic toys and other CR#P have always made my skin crawl.
In one case I came home from work to find new giant machine in the middle of our living room.  I still can't figure out how Renata didn't nix it from the start.

I was assured the battery filled rocker would go when we'd have no more use for it.  I thought we'd have none, but in a moment late, when morning was a more appropriate term, Raquel squirming, crying and in total discomfort the big blue swing was taken into service.  At first I'd push the bottom with my foot or hand thinking I'd get away with not using the batteries.  After inertia settled and the chair moved no longer, Raquel would act up again.  I did that for a few nights, till sleep deprivation reared it's lazy head.

It was easy, push button, twist dial to add sound and movement to the spinning mobile and daze off with Raquel to the click, knock rhythm of this now wonderful chair of neglect.  In the following weeks I'd get fits every time I turned it on imagining the inevitable; sending those ten or so sized D batteries to a landfill.  Later leaching into the rivers I like to go pester fish in. 

But really, there are still many things here that are useful that we use every day, 80% brought by Simona.  In fact sometimes I feel for her husband when a new object arrives, as he now works for Raquel. 

Cordelia also was not forgotten in this either.
For every object Raquel received, Cordelia would have an equal.

So for every uncomfortable moment when I feel like a nail is being scratched down a chalk board placed in the middle of my spine there are three other moments of complete gratitude.

Thank you, Simona.

and a very happy names day!!!

Sunday, December 5, 2010

Still don't know.

It's very bizarre that in daily contact with Raqu, Ren and I still have no idea how exactly the blisters appear.  A big scratch or a poorly constructed diaper/bandage change will do the trick for certain.
There are those other moments when we (me) slip into a comfort zone and forget what this child is all about and simply grab her around the chest and pick her up as I would have Coco.
A momentary lapse of brain function gets shocked to the present while looking face to face with her in a grasp seldom tried and mostly out of bounds.
Panic strikes and the question rings loud like the giant cathedral bells of Europe.
Have I just given her two giant hand prints all around her torso?
I'll lay her down on to her sheep skin bedding, take a moment, then shift into denial once it's clear she's not in discomfort till I talk to Ren next.  Then quietly say, not any different then the times she'll look over to me and quietly repeat the sentence.
"I think I just gave Raqu a big BLISTER."

Feeling each others pain we wait.
Wait till we do a diaper change or something else happens.
But depending on the firmness of grasp or if she'll be wearing two layers, nothing happens. Mostly she's carted around with her sheep skin bedding and a bamboo towel at her chest.  Always at least two layers between our hands and her skin.

Wednesday during her bath I almost figured out that wrapping the foam into her bandaging had it's first real flaw when left on during a bath. 
As the bandage soaked it gained weight, lost it's form then slipped off during the transfer from bath to table.  I was still cutting the pieces of mepilex and visopaste for the rewrap when I noticed her near bare foot being kicked by her other foot in one of her fussy "I don't want out of the bath" moments. 
We secured the foot (held her down) and then proceeded to bandage it quickly.  I didn't notice any immediate blisters or signs of trauma.  I figured we'd averted a minor disaster.  Although a few cross words left my mouth, mostly out of fear of what was to come.  Hopefully Raqu's nurses can see past those moments of panic and frustration.  I've seen them take it from Doctors on other occasions, but obviously this is a different situation.
It took till Friday when I fully realized the foam in bath was not going to happen again.  Haha, we'll see if I remember tomorrow (Monday) won't we?
The bandage again started to slip off, luckily we were ready for it and proceeded to unwrap the layers, only to find a giant blister had formed that covered her most of the area between her ankle and her toes down well into her instep.  It was a bubble of water that pushed out her skin like a balloon.  Where under all that dressing material such an amount of liquid can form I'll never know. The skin was thin and transparent, she fussed a little while I lanced it 10-15 times and slowly pushed the fluid out.  She had a minor meltdown when I added the saline solution, but all in all the situation was quite manageable.  The other foot has come so far where the skin is actually thickening, never mind those three toes that don't seem to want to grow.

Side note, I wonder now because of her toes that her ears are a little small, have they stopped growing too?  Good thing I know ears and nose are the few parts that never stop growing.

Today I felt inclined to wash the none blistered foot off in a rubbing manner.  Lighter, but similar to how I clean and hold her hands.  This was a first and rather gratifying.  The only rubbing motion to her feet so far have been to apply a large goop of Aquaphor before the zinc oxide paste.

Our friend asked today how we hold her or if we can?  We do, as stated above but layering of clothing is essential outside the controlled moments in the bath.

Ren finally stopped being sad about the naked belly time she'll never have. Although Ren did dig up a few other "will not ever get to dos" with Raqu this evening.
I can't help getting short with her in those moments.  It's just different with Raqu.  We'll just need more of an imagination with her. It pains me I can't be rough doing the gymnastics routine on the couch Coco and I often do (which 30% of the time have tears involved at one point - bonked head, crumpled shoulder etc.) go off jumps, mountain bike, ski etc..

OR the neck tickles that will send her into those spastic convulsions and giggles of her big sister..
It's all about the famous saying from the Dead Poets Society.
So far so good.  She smiles more then she cries and has good changes far more then the bad ones.

She has started to drool, hmmm next step teeth I guess.