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.

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.
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.

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!!!

Monday, November 29, 2010

All quiet on the Western Front.

It's like the night before Christmas.
Not even a mouse.  Well, there's me.

Raqu is in one of her best spells ever.  Other then the few small blisters that speckle her body , there is only one of any significance or irritation on her foot..
She's calm and happy despite having three vaccines today.
One in each thigh and an oral, which she spat most of it out.  I knew that was going to happen, which is why I waited so long to try.

So it's quiet none the less and everyone is relaxed and sleeping well.

I'm feeling a slight elation.
Could be that Ren let me go for a ski tour up our local mountain in a snow storm.
Mountain closed, but the lights were on.
Just a clean smooth path to top and a nice easy ski back down.
Funny, but I ran into our main nurse at Childrens 1/4 the way up.
Otherwise, there wasn't a soul.
Just like the night before Christmas.

Friday, November 26, 2010

Clothing EB

In the early days finding clothing and diapers was among the great issues we were to deal with.
While in the hospital Raqu was basically naked but for her diaper.  Two sizes too big and wrapped loosely around her waist.  Not to mention how slathered she was in Aquafor.  We'd go through one to two containers a day depending which nurses were on duty.  It seemed crazy as no spot was left un-greased. 
The Aquafor Glaze

Ren and I both fretted over 'how was her skin supposed to breath or expel sweat toxins etc'...  It's a great product but enough is enough!
On the other hand, if all the goop wasn't there our early clumsy handling could have been much worse in effect.  
Since day one her healing has been exceptional.  Less so on the original areas of her lower leg to upper foot.  There the skin was seemingly close to the bone and still seems nearly translucent, yet fortunately building.  Up until recently it didn't even look like any muscle developed in those areas.  Now, for most part those areas up the shin are getting much better and are mostly recognizable by a reddish colour and the little white Milia dots that seem to surround any of her larger blisters.
Ren's sister did some very good leg work in Czech and found the Derma Silk supplier or manufacturer in Italy.  Barely a week old and she was already having some Italian silks sent from Europe.  
My imagination runs often, as I had a vision of a mysterious woman (Raqu when she's older) in long white flowing dress and pants, long white gloves, a large brimmed hat and sun veil draping loosely down her front, her face barely visible, but for those big and beautiful eyes.  She had become a fashion mogul in this daydream and was walking elegantly along the Promenade in Nice.
It turned out we were sent a large package of outfits, tops and bottoms with some leggings.  There were also a few cuts of the fabric to make what we will, included was a large piece of a breathable bedding material, which unfortunately awaits it's final form.
I have to admit after reading about the derma silk and thinking it was the wonder fabric for almost any skin condition to underwear for skiing (it always comes back to skiing).  I was a little disappointed that it seemed coarse.  Far from the smooth satin image I was seeing Raqu walk down the Promenade in. 
We didn't dare bring it into the hospital for her to wear, as it would have been ruined in seconds by any number of care givers and the kilo a day Aquafor glaze.  
Only in our final moments as we were packing up and after our last bath and dressing change in the NICU, were we finally able to dress her in her silks.
Raquel in her Derma Silks

In a couple days it was clear the material was amazing. 
We choose not bandage the blisters she gets on her back or chest anymore .  This is due to the two times we were advised to put a full torso wrap, which of course creates more blisters in other area we really don't want blisters to appear. With the silks the wounds heal quickly as with the other bandaged places at her hands and feet. 
At the moment she has a big sore on her back larger then a toonie (1 1/2" in diameter), I copied what my cousin once did with mole skin for her feet while ice-skating.  
I cut a hole in the Mepilex to go around the blister then just pulled her Derma Silk outfit over top.
Another bonus is that no fibers make their way into the healing skin and within days the scab falls off naturally.
Once home, friends and family started the search for what to give a new baby of EB.
We tried to say to everyone, 'please don't, wait till we figure it out!'.  Besides, there is the fact Ren has four or five large plastic containers full of kids clothes making it very difficult to get into our laundry room. (I just counted, there are 8!!)
Not everyone got the message or thankfully payed attention to our request.  Due to this fortunate glitch in communication we were then put into contact with Jean from Little MoSo.  
Jean came over to our house on a few occasions, once with her beautiful little gaggle of kids keeping Coco happy, as we talked about the benefits of Bamboo.  She gave us a few pieces of sample clothing and some one of a kind pieces for Raqu.  Coco wasn't forgotten in this either as she has some nice little pieces she wears often.  It was great and a little overwhelming as we'd never met Jean before and she was taking plenty of her spare time between what appears to be a busy life to help out our little Raqu.  
Like our friend Simona (a retired OT) who was instantly after her mental development within a day of birth, Jean was focused on dressing Raqu comfortably, practically and most of all beautifully.  Ren and I certainly had other things on our mind.  Likewise , both women keeping us up to speed with normalcy in the best way available to them. 
Raquel in Little MoSo

We eagerly await what's next with Little MoSo.
For those in Vancouver, check out Little MoSo during the One of a Kind Show at the new convention center on December 7th weekend.  I'm quite sure you'll like what you see.
Great kids clothes, from exactly the kind of business and person most would love to support.

Friday, November 19, 2010

Coming out of our shell

We've been searching through the other families blogs and the issues a head of us seems steeper by each new story I come across.
I read over a blog then decide to show Ren for one reason or another.  Just now we have a waterfall spraying a mist over the place.  I play tough guy and pillar, but how do any or all of these parents keep it up?  I'm saddened to the core to all the pain and suffering out there.  Strangely, less so with our lot as it only takes a second to look at Raqu to realize my world has a new center and she's wonderful.
Well.  Coco is jumping on my shoulders, just now, 'Can we play, daddy!!?' So I guess the center is a little broader.  Oh and consoling the waterworks back there.
We had a little baking party happening at our place when I arrived from work.  Looks like a birthday cake for the guy just south of 40, but Coco exclaims it's just bread and nothing to get excited about.  She's run away and hidden the chocolate chunks I found on the counter.  Hmm wonder why she doesn't trust me with that??

Raqu was sitting in her chair quietly and patiently watching and being part of the action with her beautiful sounds and big big eyes!  Beautiful.... 
All the strength seems to be built up in those moments.  For exactly what will be The Blister Popping Experience this evening, on her lower gum.
Wait till the blister really starts to piss her off, usually around feeding time.  Quiet down the stress and noise of your own head, hold down arms stick a blanket/towel between her legs, steady her head with another towel (requiring another set of hands) all to minimize the nervous jerks,

Tomorrow is cake.
ahh right, bread with chocolate, if we can ever find where Coco hid those tasty chocolate chunks...

Tuesday, November 16, 2010

Dressing Change @ 4 months

Each dressing change consists at this point of renewing bandages and popping new blisters should they occur under the dressings.  Each foot and hand is redone after a bleach bath @ 5%. Our kitchen therefore smells like a public swimming pool every Monday, Wednesday and Friday.  MMM wonderful as dinner is made often shortly afterward.  

The web space of each finger or toe is kept apart with Mepilex Transfer gooped in Aquaphor and Viscopaste strips to preserve web space and range of motion for later in life.  We were first told that at about 2 years of age Raquel would be able to go free finger, free toe, as her dexterity would be better.  She would in theory be at a level of understanding of how to protect herself.  

Not so, as a recent dressing change with her Plastic Surgeon changed that dream to 4 years.  He asked the question of us and the other doctors and nurses present.  Four years of bandages or 54 years of no mobility?  I let the question of "only 54 years?" go, pick your battles, (I think he might be 54, he could take the O.L.D. poke but I'd probably lose concentration in the task at hand, as well it's hard enough to perform at top form when you have a room full of pros watching you and commenting one way or another about this or that, as a loose wrap at her instep will certainly produce a large blister by the next change).  Then came some comment that toe web space was of little concern. Another one I let go, but Ren didn't.  I listened to that conversation while wrapping the various layers, slipping in and out of their conversation.  Still I'm going to do my best to keep those little toes apart.  Two came out of the womb fused nearly to the top and a few others seem stunted and deformed.  

Raquel's feet look terrible on the best of days and some larger blisters are forming up her shin as her mobility increases.  We are actively trying to see how to keep her hands from retracting into a club, and have steady contact with Children's OT department, we have some small plastic splints made up to hold her hand in an L shape (safe position or something like that).  A larger one was made that goes most of the way up her forearm and was doing great amount of damage to her legs and face due to all the sharp hard edges, even under lots of foam.   

Inertia and the constant thrashing of a 4 month old.  Hunger, the itch, discomfort, bowl moments etc. can all create a sudden movement that will cause any level of blister and resulting wound anywhere along the body exposed to a flailing appendage.  The OT department had also made some fleece booties, which are now too small, but were effective for a few months.  

I've revised the cushion layers in her wrap with little pieces of foam.  They are wrapped into the upper layer of conforming bandage and have been good as a hold over.  I'm thinking of finding some of Coco's socks she won't immediately miss and sew some of that foam onto them.  Moon boots anyone?  

Anyway, this video above is a recent hand change of parts of the Viscopaste layer.  

Nice to see her relaxed during the changes.
Often enough it's anarchy.  Usually a three person job, screaming and enough pain of various types for everyone. 
We've only had four or five changes in this time where we've only had two to do a change.  Luckily those changes had been great.  It's hard to predict how a change will go, but the previous days events start to make sense. 

In general the changes have been trending in the right direction, one step back usually in any week period.  Rare that we'll pop a blistered area past two changes. 

Maybe we are all better equipped to deal with the situations.  

Maybe Raquel is just that smart to deal with it.

Raquel Bella 'The beginnings'

Where to start?
How about the battle we had with naming sweet Raquel Bella with our first daughter Coco.

Coco was incensed that we would call her sister anything other then Tinkerbell.  This went on for a few months, till one day she cut the Tinker and added an A.  Problem was our good friends had just named their child Ella.  Originality being high on my if not ''our" requirements for a name, doing the Ella-Bella thing at our weekly dinner parties with the Wolframs would be a bit much.  But as she was born and the circumstances of her disease were known, we planned to leave the name battle open.  The option of a child growing up with the issues involved with EB being called Beautiful every day of her life was quite intriguing. 

So, was it going to be Bella or Raquel first? We decided to see which name friends, Doctors, nurses and family would choose.  

Coco conceded eventually, but we must always remember to call her Raquel Bella while referring to Raquel Bella in Coco's presence.

Raquel is 4 months old now and time has both stood still and flown by.

Time did it's thing on July 9th, 2010.  We were expecting our second beautiful and perfectly normal little girl...  Why wouldn't we? Coco was and is healthy, we were and are healthy, the ultrasound was good and normal.

The labour was long and difficult.
36 hours of waiting and pushing.

In the final hour a large group of Doctors and nurses were assembled, as well a few odd looking devices and tools were also wheeled in.

A smallish South African doctor was the one called in to do the extraction as normal labour pushing was getting us nowhere.  I remember clearly looking at his hands and thinking it's best for women that I didn't become a gynecologist.  Not that Art School an a mild attempt at Architecture School didn't get in the way.

He did a little adjusting of Raquel to turn her into the right position and called for the Kiwi.  After all the people in our room I figured a tall lanky Kiwi was the last person we needed, but a small suction devise was passed under my nose with the actual words The Kiwi printed onto it.

After a couple pumps, pulls and pops, it was easily decided Raquel was having nothing of entering the world via the Kiwi.  So the South African asks for the next devise.  This thing had a large cylinder on the top and looked more like an auto-shop mechanical instrument for heavy machinery then a instrument created for birthing.  

In goes the suction cup, a few pumps and pop.  A couple more tries to where we were actually able to see the tip of Raquel's head.  

I was very close to the action, in that I was holding Renata's leg from kicking the extractor in the teeth.  Another nurse held the other leg.  After the 36 hours I was a little impatient too and just wanted it over and her out!!  So as he passed off the second suction device for the giant stainless steel spoons, I didn't feel all that faint.  Although as he clicked them together and did a mock test in air, my heart did flutter in nervous anticipation.  Of course he's done this many times before I had to assure my self.  A nurse took up a position close behind me half hiding behind the curtain.  I guess lots of dads hit the ground in these situations.  In went the over sized salad spoons and grasped over her head.  He pried his legs against the bottom of the bed and secured his arms for a big pull.  Very quickly it happened as her head emerged and adrenaline shot through my body, tears blurred my vision, I was floating and could hardly feel the weight of the world (till then it was just gravity - the weight of the world means something all together different now)

As she came out and the sight of her feet and legs came into view, something was wrong.  I looked around at the doctors and there was an anxious silence.  I went over to the little work station so see my screaming baby only to notice the bloody and raw look of her legs wasn't residue from the birth, womb or anything like that.  My floating feeling was gone, I desperately wanted the elation back.  The Pediatrician brought Raquel back to Ren covered up for a few moments and maybe a little suckle, we did our best to hide her apparent skin issues from Ren's eyes.  She did notice a few spots on her hands and mouth.  
The South African Extractor said it wasn't him nor the tools.  Our Pediatrician was very concerned and quickly pulled her off and down the hall to the NICU.

The Pediatrician was the only one who had any clue what was wrong and did utter the words Epidermolysis Bullosa.  Words I couldn't repeat for another week or more, I mean who wants to learn those words or even spell them, well other then a Dermatologist or say a Geneticist? 

Interesting in retrospect that the two types of suction devices that failed to pull her out or even the giant salad spoons which finally did do the trick, had little effect on her head.  Only a small circle of blood and broken skin was evident at the top of her head. 

I was struggling with a cold at that time and was only able to hold her from our room to the NICU down the hall.  I got a smile and a happy look up from her despite what was going on.  

She was mine and we were bonded.  My sweet, beautiful and lovely Bella Raquel.

Within moments I was told to leave the NICU. I didn't see her for three days till I was able to hold in my coughs and runny nose.

That evening Raquel was again moved off to The Children's Hospital's NICU in Vancouver via infant transport ambulance.

Ren, despite what she went through was able to go downtown to Childrens the next day.

During the few days I was held out I was only able to see photos.
Each day things were getting progressively worse.
New sores everywhere.  Not to mention the tape wound developed from an attempt to hold down a feeder tube through Raquel's belly button.  None of the nurses new how to deal with her, nor the doctors.  There was no protocol, nothing.  It was trial and error time and yes there were errors!!
Day 2

Scratches and big round circles of broken skin across Raquel's face and chest, legs, feet and hands were evident and painful to see from afar.

Finally I was allowed in and able to give Ren the rest and time to heal away from the tiny uncomfortable isolation room.

A few of our friends had shown up and provided an amazing amount and much needed help and support, including fantastic dinners and some strong advocacy for Raquel with people in the medical loop.

A few friends even stayed late into the evenings reading to her and advising on the proper care as it became evident.

As nurses moved in and out of shift work, everyone coming in needed to be trained a new, by either our private support group or the one nurse who actually had seen and worked with babies with EB before.

Feeding was an issue as the skin in her mouth and lips basically fell apart.  Finding the appropriate nipples became our largest issue for months as the only ones that worked were discontinued and we were depleting the hospitals supplies single handed.

In fact what was meant to be a single use nipple has lasted us for a long while now.  Which reminds me, I think we better start that search, again.

My first day with Raquel was the first day we were able to get the Plastic Surgeon into the room for a dressing change.

With Raquel in my hands the Great Doctor with a couple nurses for help pulled apart the poorly constructed bandages and I saw for the first time the damage that even bandaging could do.  In the room was also what has become one of  Raquel's most important advocates, who was able to procure a local nursing and medical supply program.
I can't imagine our lives now without this help.

The doctor, who works often with burn patients used his wonderful techniques using Viscopaste PB7 (zinc oxide bandage), Mepilex Transfer (foam splinting material), Aquaphor (Vaseline type lubricant) and Conform wrap.
I've never felt so helpless, sad, mad, etc etc with my new lot in life.
He did each of her extremities and then a full body goop of Aquaphor 

Day 20

and she was back into the incubator.

First bottle, day 2

Ren was home sleeping and resting getting to a point of getting back on her feet.  I went home grabbed my Mountain Bike and did my usual peddle up into our fabled North Shore trails to sweat it out.  I came to the realization that I'd better become the master of bandages and many other things with Raquel or else my helpless feelings will only compound.

The next day in the hospital, before our next dressing change I asked the doctor if I could take over.  How often does a non medically trained person get the direct supervision and training of a Plastic Surgeon?
The lesson worked out and within two more changes he decided I could manage on my own, but sent in some of the Intern Doctors as eyes, help and advice during the rest of our stay in the NICU.  The techniques and layers have been modified since, responding to areas of concern.

We've had our moments since, but all has been quite manageable.
Three days a week we have a nurse for 4 hours.  Mostly to help with the dressing changes that last up to two hours.  A little respite has been wonderful in letting Renata focus some quality time on Coco.

The pain control has Raquel on a constant dose of morphine, spiked at dressing changes.  The good thing is that only the dressing change dose has increased in the time since we left the hospital three months ago.  Raquel has grown and developed exactly on schedule as any other 'normal' baby in this time.

Blisters in the mouth are our largest concern as the rest of her body for now has been good. 
Try holding down a child that gets blisters from friction, while you put a needle to relieve the pain of an existing blister in the mouth of a moving squirming target.  
Ren had a panic attack the first time we had to do this at home.  Which was coupled by the fact we couldn't get any food into her in 16 hrs.  Dehydration was becoming a scary issue, bringing her into our local emerg. was out of the question as an unaware doctor and nurse can create a great amount of damage, as well we couldn't get any advice over the phone.  We'd been told not to pop blisters in her mouth, although I'd already popped one a week or so before.  Insert some of my coarse language and 4 am foggy stressed head.  No wonder.
This last time (last night, moments after above photo) was quite mellow by comparison.  She was force fed, by squirting the milk through her screaming mouth.  Half in half spat out, but enough getting in to settle her some. Although a few hours later Ren complained of being short of breath..  I had to giggle remembering her keeled over not helping too much in the first great mouth blister ordeal.  Glad she was able to hold it off this time till things were quiet and Raquel was on the up swing.  
No time for the weary in this battle.  
So, I say now in the safety of the present and a sleeping baby down the hall.

Making a blog has only just come to us as an idea.
The original idea was to share with Ren's parents in the Czech Republic.
But having looked today at the other families with EB blogs, maybe we should be a little broader in our scope.

We've been a little like the image of an Ostrich with our head in the sand.  Not wanting to know everything about the disease or anything about others.
Living day to day dealing with what's directly in front of us.  Learning what this disease actually is has been very very difficult on us both and seeing other children suffering seems even harder to understand and digest.  


Maybe, we can help another family get through the early days by our experience and knowledge.  

Maybe, a family further along in this battle with EB maybe drawn to provide advice in what lies ahead for us and paths through.

So, here we are.
Raquel is beautiful, she is loved immensely by all, she even rolled over today (wonderful and scary) for the first time, she talks to us in her coos and  bphhhps, and best of all she smiles and laughs at her big sister's goofy antics. 

Things are actually, quite normal, it's just this little issue with her skin.