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.

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!"

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.

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.