Monday, May 30, 2011

All is really well.

Raqu is a dream lately.
Lots of speaking and high energy on her little matt.
If you are laying on the floor with her she wants to crawl all over you.  It's really really fun.  The big  - squished nose, squinty eye, three toothed grin....  Is melty butter heart kind of stuff.

Her hands are in fabulous shape, feet as well.
I'm thinking her feet haven't had a blister in somewhere around two months.
Forget the mouth.  That's Raquel's Mulligan.
It seems even with the thought of getting more nursing hours has made the over all mood in this place amazing.
Life in constant stress has been alleviated somewhat.
I'm going to drag Raquel here one day in the trailer for my bike.

Thursday, May 26, 2011


Apparently we've been approved from more nursing hours.
Maybe Renata and I can get back to happy family, by having the energy to work through each others low moments.
It means more people in our house, but it's summer now (or so I've been told) and we'll be able to spread out a little.
Raqu had some great days of overall pleasure, but something is up this morning. None of her approved pain therapy even dented what she's going through.
I can't tell what it is, only that she's trashing and keeping her eyes shut.  I'm hoping it's because she didn't sleep well and is refusing to open them.  I doubt it.
I couldn't get out of the house fast enough again this morning.  Although I forgot half my work there and will have to go back shortly.  Damn....
At least I'll be able to give Ren the hug she needs this morning, one I was unable to give then under the pressure of getting No 1 to school and my shattered ears a break.  Maybe I can bring a tasty peace offering in the shape of a coffee from Moja - problem is she'll only get to enjoy if she gets to put the ball of fuss down for 5 minutes.
But joy "we have more hours!!!" to recuperate in the back ground for the all too frequent occasions such as this.

Wednesday, May 25, 2011

Her Tumb nail fell off not her bitten finger



I'll see if there is Finger-nail Fairy.
I feel slightly queasy.

Tuesday, May 24, 2011

Pics from the cabin

Thumb day 4

Nail moments after bite

The Fang

Biggest of the weekend

If she can't go out on the boat with me to fish she can play 'Go fish'.
Way home near Merrit, BC

OK OK I won't call her Fang anymore.
Maria and I thought we'd use this opportunity to teach our children sign

Good weekend.

All and all Raquel was great.
Most of my fears of Raquel bursting out in loud screams of pain or colic or what ever babies do didn't surface.  At all.
It was also the first time Renata was able to sleep in the same bed as Raquel.  I did a little turn one night, but was too freaked out to be that close to her in a bed while sleeping.  I sleep light, but one thud of an arm or a roll onto her arm and I'd probably never sleep tight again.  We rented our neighbour's secondary suite and it was very odd stepping out of the rustic chaos that is our cabin into floors that are spotless, central heating, a shower, and a dishwasher that didn't have two arms and name wasn't Lubo or the evening scurry of little furry animals.
There were a few highlights.
Cordelia catching the biggest fish of the weekend.
Raquel being a very normal baby and having great dressing changes.
Ella biting the finger nail off Raquel.


During one of our dinners, someone reacts with a nervous Ella! I look to see Ellas mouth and teeth firmly gripped around Raquels bandaged hand.  With a big pull of obvious pain Raquel was able to get her hand to freedom from being bitten clean off.  A good deal of crying happened as a result.  I think for Ella and my sake I was sandwiched between two people a wall and a table to defend Raqu in the seconds her teeth were gnawing at my daughters fingers.  The usual hopeful banter of not much happened took place between the nervous adults, yet I was quite sure I saw the moment her teeth pulled past Raqu's first knuckle and slipped off the end of her bandage.
Once Raquel calmed I did a few tests to see if damage was done by squeezing lightly her fingers.  Each time I got a good reaction.
After dinner we did a small change of the hand dressings to find indeed a decent blister pushing up the whole end of her finger.  As I poked at her nail it was clear there was likely to be a short end to it, as it moved independently of her finger. 
I often joke about pulling her nails out one by one, to get it over with, but at least we'd be using M&M or some other pain/amnesia-tic concoction and laid out comfortably on a hospital bed.
That's for me, then what ever Raquel needs!! 

The visual of her hand red and raw after the big blister on her thumb from before we left on Thursday and the loose finger nail a few millimeters up and floating above a bag of skin and water looked very much like that of an atomic bomb survivor.

I really wouldn't want those who weren't there to miss this one.
I'll add pictures later.

MW will have to deal with the nick name Ella will now have The Fang.
The next day when it was time to do a full change I was able to put our biggest needle for draining and puncturing wounds well under her nail.  Raqu didn't even budge and it was her usual dressing change dose of morphine that I don't really think is sufficient for real pain episodes.
Now that's a tough kid!  I see and deal with all of it daily.  I don't understand.

I currently have a little sliver in the side of my left thumb.  It's been there for a week, I can't see it or get it out and now it's starting to get hot and sensitive.
What a baby I act like when I bump it.

I think the nail falls off either Wednesday or Friday.
I should have been putting Raquel's toe and finger nails under her pillow to see if there is a Fingernail Fairy.
In hopes she'll drop a couple hundred each.
I'd invest it with the hopes Lee Press-on will have a product for her when she becomes 13.

Although, overall it was great to be at the cabin, hopefully the others thought so, despite the dark stormy cloud that followed me around.  Not sure sometimes why I have any friends.

Thursday, May 19, 2011

I've got Blisters on my Fingers!!!

Large Variety Blister

post popping and clipping with new scissors

Probably from last nights Thrash

Just hours before we stick her in a car seat for 5 hours.

Frey'd Knot

We're getting at each others throats.
We had plans to be packed and nearly ready for our trip to cabin, but Raquel again had a deep throat blister.  At first I jumped to blame the feeding techniques Renata carries on with.  We've had a few conversations about what Raquel should be eating from, but other then her solid food, we've done nothing that's lasted.  Then when things go south and she's in a lot of pain it's impossible to get in new methods, so it's back to the trouble.  We had an identical moment on Monday with the nurse and an emergency call into Dammit Janet to help with a dressing change.  That was 45 min of suffering and thrashing by Raqu and almost a run to ER for some M&M so we could be sure to get any skin flap that might cause problems later.  This evening it didn't appear to be that big and she was calming down in 20 minutes with her special meds.  She's laying in a ball on the floor beside me and fusses every 10 minutes.
I think once I go off at 1 am to bed, Ren will have a pleasurable couple hours.
I have no answer why we haven't moved on in this regard.  It's senseless.  We've adapted at all other concerns and her care has been very fluid with what ever comes up.
Right now, we're going to have another terrible night.
Eyes with scratches + big deep blister in mouth at uvula = pain, suffering, irritation, no sleep and a grumpy morning and Ren and I pissed at each other.
Wow, aren't we setting off on a good foot for a long weekend..

Hopefully I'll have happy images of Raquel in a canoe with expressions of joy and wonderment at a new environment by next post.

Sunday, May 15, 2011

Back to ER and Corneal Issues.

Our nights are usually of fussy kid.
Usually it takes Raquel an hour once up in the morning after she really opens her eyes.  For a few days we've figured it was that she was still asleep considering no one in our house less Cordelia actually gets a full nights rest.
Today, she didn't open her eyes till 9pm.
Renata took her off to Childrens to find out what was up while I was off enjoying the rain and placid waters of a regatta that was barely able to finish one shortened race. 

Since you don't pry open the eyes of a baby with EB to see what is going on, nothing happened, nothing was resolved.  And again we had a Dr who looked blankly at Ren and could only offer he's heard of EB.  I love the honestly at least.   It was only suggested we use some drops once she opened her eyes. 
Renata had a tough day keeping her calm and in darker rooms and basically dealt with the emotionally draining day of wondering what was going on.

Once home and seeing the note about her eyes and no one to greet me, I was quickly able to catch up in draining stress about our daughters eyes while I waited for my crew to come back home.

Again I'm freaked out about her sight and having continuous corneal abrasions scar her sight to nothing but shadows and light.  Or worse....
It's one thing that I've barely gotten over, the fact I'll never be able to chuck her in aerial somersaults into mass pillow cushions Cordelia and I set up or take her skiing/mountain biking off into the deep back country and roughing it, in typical jumping, crashing fun. 
It would be tragic not to at least spend some quiet and just as fulfilling time next to the paintings of Goya deep in the Prado, or the texture, sculpted shapes and volumes of the Guggenheim in Bilbao. 
We all have our strengths and passions and sharing them with your child should be goal one.
It is mine anyway.  Of course we'll have to adapt to what comes should this continue in the worst possible avenue.

We still don't actually know if she's scratched her eye yet or not.  It's only occurred to me now that it could be something else. 
Really, EB sucks. 
 "'Excuse me, but someone forgot to give my daughter her Collagen 7, it's kind of important, can we have it - now!"

Friday, May 13, 2011

Ups and Downs.

I think we have a vampire for a daughter.
During the day she's as content and fun as can be.
Then early evening, strangely the time I come home from work, things start going sideways.
My first grasp and kiss early evening is shortly followed by distress.
I'm fearful that it's related to the dressing changes that happen in those hours.
We threw a monkey wrench in that today.
We did the change at 9am.
She was a pleasure.
But last night was terrible.
Up on the hour every hour till we gave her some Tylenol.  She's cutting her top two and bottom two teeth all at once.  How come they grow at night?  This is why I think she's a vampire.

Anyway, at 4:30 am was my turn to take over tending her discomfort.
The only thing that helped was holding her in my arms with her in an as upright position as I could.
This carried on till 7am.  I got a good hour in on the couch till 8.

She had no blisters anywhere, but she's developed a great number of small ones on her face.
I currently blame Simona as she spent the day on Wednesday with her and let her go great stretches of time with her thumbs out of her silks, not fully supervised either.
Not exactly in my approved care criteria. 
But what do you do?
She sent Ren and I off to a romantic dinner and a movie that Renata actually stayed awake through.  Crazy!.
Next weekend we go to our cabin with a crew.
We usually do Easter up there and for probably the last 6 years in a row.  It didn't fall on our month timeshare so we'll have to deal with May.
The weather is better anyway, but hard to mess with tradition.
I'm excited to have these people share in with our late night/early morning scream fests.  Our cabin is a log very rustic with no doors.  Every vinyl slip in your sleeping bag can be heard in every point of the cabin.  The mountain air helps keep most people dead asleep.  We'll test this though.  Actually, I attempted to rent the neighbours cabin for Ren and I but it's full.

Wow, are a few people gonna love us.
I've warned to bring ear plugs.
I'm quite sure by the third night tempers will flare from lack of sleep (Maria and I in a dual to the death as it usually turns out).

Andrew, Raqu (hiding) Renata, Ella, Cordelia and I for a walk in the forest.

Tuesday, May 10, 2011

Some Blister Areas Healing well under silks (No bandage required).

Week old, had some reoccurring blisters. Mostly to do with lifting her and forgetting about the wound below.

Two week elbow

I think Raquel will have to live through many indignant times such as having dandelion seeds blown all over her head.

Monday, May 9, 2011

Raquel is testing our stamina

Nights of suffering for all, then morning comes and things are almost normal.
We had a blister on her tongue that was too small to attempt a pop last night, as well she had the scabby skin flap gurgleing at the back of her mouth.  So every once and a while when laying back we'd hear the sound of drowning baby.
It's actually quite frightening looking at this weird white bubble thing flapping deep down her mouth while she's screaming at you and thrashing, up, down, sideways, up, down, kick, stretch, forward.  Gasp Freak. Lunge back in the opposite direction your hands are protecting her from.
One of these times I'm going to be talking about why she had a giant face blister smear.  It'll be because she jettisoned herself from our grasp in the middle of the night where my or Renata's faculties weren't exactly functioning at full levels.  Her core strength is actually so strong that both Renata and I are at the limit of our capabilities in keeping her safe from herself while attending to this or that blister in danger zones. 
I have to admit, this morning I couldn't get out of the house fast enough to go to the sanctity of work.
Even if I'm trading one situation for another in a nearly 50 year old balding, disgruntled Czech who complains and laments nearly any and every issue facing his little daily circuit from parking tickets to Czech Hockey.  Maybe not every issue, I bring up world politics he's a little out to lunch, unless it directly impacts him.  He's not a citizen yet, so when he complains about even his parking tickets I cut him off tell him to become one, vote and then maybe the airwaves coming out of his mouth will become a little more then hot air.
Then if he wins at darts?  arggg.  What an annoying sound of primal monkey chest beating I must endure.

Why do I know so many Czechs?

This my goofy half Czechs with enough Canadian 57 to save them.
Check out the size of that cranium to the right.  It's got it's own atmosphere.  Hopefully it's all brain.

Friday, May 6, 2011

Yup another night of joy.

Every hour on the hour till two ish when I had to take over from Ren.
Same deal.
Two hours of on - off on - off.
Great kid all day, monster at night.
One of her back blisters bled through her shirts, so at least there is something to point to this morning that begins to make sense.  She started at it again at 7am, great.
My mom came over and I disappeared for work ASAP.

Now to design and draw.


Thursday, May 5, 2011

The 24/7 dose is gone.

This is Raquels first day of free pain.
Free to experience it like everyone else.
It has been a little while now I had suspicions she's out grown her dose.
Only her Tylenol and the higher dressing change doses seemed to help in the extreme cases.

Last night we had a violent fit that was ear shattering.
I had one of Renata's extra pillows firmly pressed against my upward ear while Renata attempted to calm her.  It was when I heard Renata crossly yell "Raquel stop".
This instantly got me out of bed and down the hall bumping off the walls in disorientation.  We (Ren and I) have a good thing going.  When it's clear the other can't cope, you gotta step in.  Renata knows I've handed screaming baby off on plenty of an occasion.  I'm just happy I went to bed at a decent time and was able to spend two hours holding her till I was sure she was down.
It's amazing how strong she is in her fits.
I was holding her with hands down and legs apart while she thrashed for 45 minutes.  It literally took great effort, to be firm but not overly tight that I damage her skin.  It was great when the Tylenol kicked and what ever that was passed.  If I had her strength in the body ratio from her to me, I'd be The Incredible Hulk(man).
She wasn't really awake, but makes me wonder if it's just the normal baby colic?  I once lived below a family where the baby screamed night after night after night.  I couldn't stand it.  I can't imagine the mom, as I only ever heard her songs and voice.
Hard to say or know, even a doctor yesterday mentioned that she once phoned her colleagues at night asking what's this then holding the phone in her screaming childs direction.
Just baby being baby.
Hard, cause we've had no experience such as that with Cordelia.  She just cried for attention.  Which is really easy.
Go to bed!  Then ignor her till she finally figured out we can listen to her yells and cries for attention all night.  Of course Raquel has a leg up on Cordelia in the attention department to begin with, as we're screwed when Raquel starts to figure out the manipulative areas to get attention.
She'll get it. 
Side note a friend forwarded me a very funny story 'Go the Fk to bed'.
Worth a peek should any of you be interested in crass humour.

Anyway, we've reached the no morphine 24/7 miles stone.
The other little thing that's happening now is her opposable thumb grasp.  I'd forgotten what it was like to be holding Cordelia when she was dudu (soother) sucking and holding and squeezing my finger in her grip.  Raquel does that now like a little bandaged crab.  It's bliss feeling her grip.  In so many ways.
Love her,
Love it!

I'm preped for the 2-4 am scream fest.  Yesterday was three in a row.
Do I hear a forth?

Wednesday, May 4, 2011

Derma Silk - A Testimonial. (Recessive Dystrophic Epidermolysis Bullosa)

I've been told by our local Derma Silk representative that Customs Canada is sticking a 17% duty on Raquel's clothing.  This is insane!!!!  Why do we have to battle on every front for the care of our child?  To steal a quote from Daniel, "This isn't Prada" wear!!
Anyway this is what I wrote.

    Our baby was born with Recessive Dystorphic Epidermolysis Bullosa (RDEB).  It’s is an extremely rare genetic skin disease, where collagen 7 is missing in the bottom layer of the skin.  The absence of collagen 7 means the skin has no anchoring fibrils which results in blisters and sloughing skin from only the mildest forms of friction.  Our daughter’s skin is so fragile that even the slightest miss touch with a finger nail will damage her skin.    
We first became aware of her disease when she was born without any skin on her feet and a few spots on her hands and mouth.  Her condition rapidly deteriorated even with aware handling while in the NICU.  Her skin basically started to fall off in huge blisters all over her torso and extremities.  Poorly constructed and extensive bandaging also had a terrible impact. 
When the skin heals with this form of EB it does so in a scaring fashion.  Without proper and extensive preventative care many of children with RDEB end up with clubbed hands and feet due to strictures that happen as a result of the scarring and tightening of the skin.  A host of other issues relating to constant and continual raw and open skin areas are also a concern, the main being; skin cancer, which is among the leading causes of death and concern for people with RDEB.  It should also not be over looked the constant pain involved in having open and blistering skin where a dime sized sore can expand rapidly to 5x in area when over looked.
After a month in the NICU and confident in a few bandaging techniques directly related to protecting her web space (hands and feet) she came home dressed in her Derma Silk clothing.  The difference between how she was able to heal and the level bandaging required was drastic.  Only in rare occasions are we forced to bandage any blisters that are present on her torso, elbows and knees.  Her Derma Silk clothing has been instrumental in letting her wounds heal and breathe.  The clothing also acts as a valuable slip barrier, so that the friction of picking her up happens between the silks and her outer clothing layer, preventing countless abrasions.
Our child is 10 months old and since her 3rd day she has had her hands bandaged 24/7.  We have just started to let her thumb loose within her Derma Silks and with great results over the last 2 weeks.  This is something we’ve been advised against till the age of 4 when she becomes more aware of her surroundings and body.  She lives in a very controlled environment, but this is a great breakthrough in allowing her to better experience the world with her hands.
It has also been a great advantage having the antimicrobial agents within.   Our daughter has had many sores on her body to date and has yet to have any type of infection in areas open to her Derma Silks.  She is able to heal quickly with none of the issues we have when adding more bandaging.  I have been very active in researching other cases of RDEB and can’t stress enough what a help this product has been in every level of her wound care and prevention.  The advantages are so great that I cannot imagine putting any other product next to her skin, ever.
Ryan Hultman.

Tuesday, May 3, 2011

Crawling or some form of it is now inevitable.

She's rolling over and stretching out.
Our soft safe zone isn't large enough anymore.
Until now we've been able to control her environment to the n'th degree.  We are quickly losing control of that now.
We used to have moments were we take a look and notice she's content enough that we could go quickly around the corner to make toast or pour a glass of water etc.
Not too much longer, as those moments seem to be near blips now. 
Exciting and stressful.
Exciting that your kid is developing somewhat normally, I think the stress part is understood.
I remember 4+ years ago my buddy Ken made hard jokes about kicking the feet from his kid to slow him down from walking.  At the time I was shocked that he not be excited his child could and would walk early.  How was it something not to be proud of?
Cordelia was getting close and I was hoping she'd be up by 9 months, as my mom suggests I was.  Now that I'm further down the road of parenthood  -
Kick the feet out from your kid (gently of course, especially if it's going to create a raw puss fill pocket you'll have to lance).
There is a life time of walking, running and all that (hmm for many I guess). 
What's two or three more months?

It's two or three more months of a little peace for the beleaguered parent, is what it is.
What are we supposed to do with an creepy crawler with EB that just doesn't get it?
Doesn't get that she's so fragile, that she's so fragile, and lastly THAT SHE'S SO FRAGILE.
Almost every lunge she makes in an attempt to go somewhere ends up flat on her face on the coarse wool carpet 6" from her padded play area.
Her elbows already are two giant sores.
Oh well.
I was just starting to worry less about her hands.
Kind of lost track that soon would be a whole host of other issues.

Last night was many sleep interrupted fits of pain.
No idea why.

Back down and descending the North Face I'm thinking. 
Down river without a paddle or up a mountain without a rope? 
Ski's would be nice, just point em and go! 
Down river?
Why go back?
Just keep going, you'll find ocean sooner later. Problem is, I've heard my ancestors trudged across Eastern Canada in canoes looking for Beaver.  I guess it's in me to portage right back up to the start.
Glutton for punishment.

At least it'll only last a week or so.
Deep breath....

Monday, May 2, 2011

What was it I said last night?

Renata fell asleep while feeding Raquel.
The result a big bubble of blood sticking out of her mouth when I went to feed her at 6am this morning.

It seems like chaos, but things leveled out quickly, all I had to do was replay this video to her and that was the end of it.  Seems misery loves company.

Sunday, May 1, 2011

Tip Top.

I wonder, can this get better?
What a week Raquel and us have had.
Her hand bandage has changed so that I've allowed for her thumb to be free.
Not free to the world free.
Free to move within a couple layers of Visco paste and under her silks.
It's been great.  Many of the hand issues that were pestering me through Renata's questions and suggestions have seemed to have rapidly corrected themselves.
There is still the stricture at her palm, but I'm hopeing that will get better as with the rest of her hands.
She's been rapped like this for almost two weeks now.  I can tell she likes it too with how she can hold and grab things.
I've been anxiously awaiting some blisters on her thumbs or within the web space of the thumb, but it has yet to happen.
Seems we might be able to brave further steps now.

Saturday Renata was out all day, so Raqu, Coco and I did our thing.  We were gone for the better part of the day going from this place to that and it was a freedom with Raquel I've not yet felt.  We stress every time we get her into a car seat and going to where ever.  The day couldn't have unfolded better.  We're planning for our summer trip to the cabin and it seems more and more likely should this trend continue.
She has quite a few new blisters on her chin and cheek from the buckles even though they are wrapped in lambs wool.  Minor.
I say minor, but it's meaning is bizarre.  On Wednesday while cutting her remaining finger nails I was able to pull off another.  Renata and I we quite happy to see it go.  Some parents I'd think, well me should Cordelia loose a finger nail I'd be quite upset, but Raquel loosing one means one less hassle.  Major issue to some, benefit to us.  Minor.

She has been amazing during the dressing changes of late too.  It was getting so bad I asked our Pain team to look into what we might be able to use to get her into a calm state while doing various dressings or getting into her mouth for blister popping or skin flap removal should it be necessary.
No fuss, no problem.  I know it's just part of the roller coaster ride, but seems so far away I was forced to consider such drastic measures.  Although we are supposed to be going in this week to do a supervised run in hospital.  Minor.

Tomorrow or at one point this week, now that I've said this, all will go south.
It's happened with shocking regularity, it's like climbing the Eiger.  From what I've heard.
One side is a reasonable hike, the other - the North Face a straight plummet to the bottom.

But for now we'll enjoy.

Not the bear I carved back when, but the hands are suspiciously close.

Last day of organized snow fun, irritated they closed the fun part of the mountian with the 6 meters they are boosting  so that we were forced to ski with the Jibbers on the flat side.