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.
Quiet.
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.

Thursday, November 25, 2010

Proactive

     We had a Physiotherapist in to see Raqu yesterday as her hands are basically bound 24/7, from about day four or five.  Typically we spend a little time stretching and playing with her hands during the dressing changes.  I don't think it's enough as we've already noticed a little finger drift.  The splits are helping a bit and her hands are flawless without sores.  The issue is if she's  fussy during the Visco paste layer it's tough to get her fingers where they should be within hand splint.
It can get a little chaotic as I demand Ren to give her the bottle during her these moments.  Sometimes it works, other times there's milk everywhere and the resulting skewed wrap.

Ren and I have a steady battle over the use of a soother.  I say NO> She says we should look at it as an option.
Well, for a kid that gets blisters from even the lubricated mouthful of milk, putting an semi sticky object in there where her instinct is to suck it dry would be pure carnage in my estimation.  Followed, quite possibly by a repeat 22hr freak session by all.  Except for maybe Coco, she seems well insulated to our plight in almost every aspect.  She's actually the true pillar of strength in this family, hugs as a true lightness of being given at random.
I was very proud of Ren last night as she did her first needle to blister in mouth routine without my help.
While our nurse was looking out for Raquel, Renata was able to do some grocery shopping (Coco and I were again off skiing trying not to miss any jump on either side of the run).  When Ren came home Raqu was in a fit of pain and screams.  Inconsolable.
Ren noticed a blister on her lower gum, the two of them with the help of some reinforcement (our neighbour across the street) held her down and lanced the blister.  Within moments she was quiet and feeding.  From what the house was like when I arrived home an hour or so later, there was no waterworks, no shortness of breath, just a really proud MOM and content baby.

We are also looking at getting ahead of her eventual rolling over and crawling.  Our first daughter started crawling at 7 months and rolling at 5 months.  Raqu rolled over again today.  I keep thinking when she does it is going to be full time.  Luckily this is not the case.  Knee pads.  Elbow pads.  Special padded socks.
I've yet to sew into a pair of Coco's socks some foam.
It adds a half hour to the change, cutting special little pieces and wrapping them into the conform bandage for both her hands and feet.
Efficiency Rye!!  You'd think I'd take the 2 hours one evening to make up a few.  In just over a week we'd be earning extra time for hand calisthenics.

Then there is this wonderful round of support that keeps popping up.  Our good friends The Nels sent out a note to many of their extended community suggesting help for a family in need.  I went through the email list and only recognize two names, which makes it all that much more amazing. The result, dinners on dressing changes till sometime in January.  WOW!
Eating late isn't an issue for Ren and I since Raqu is on the EBM, but feeding Coco late has been the issue with the dressing change evenings.  I hear stories of children of Coco's age getting to bed at 7.  I can't imagine.  I'll not say when we get around to feed her on those days let alone put her to sleep.  Could be why some of her grades seem a little lower then I'd expect.  Should be that better planning is in order on our part.  These dinners do the trick now for certain.  We've had two dinners and they were wonderful!!
Lots of left overs for today too.  So really THANK YOU ALL!

I could get philosophic here and on to my run on sentences and ideas, so to be brief cause I gotta work - when you do your best for the people you love and respect, the people you love and respect will surprise you back.

Tuesday, November 23, 2010

Ella Bella and the first taste of Winter

So you see calling her Bella didn't have a chance at working.

Today was about Coco.  We did what has become our yearly ski tour up Seymour Mountain for our first taste of powder for the year.  Coco is a dynamo on ski's.  I tried to urge her down some steep and deep but she stayed with the not so steep but much more technical descents.
My thoughts were very much of the tri-ski contraption I'd attach to my waist to drag Raqu up the mountain when she's four or five.  Some where between a recumbent bicycle, and a dune buggy.  I realize the Chariot we have for Coco might work with the ski attachments, but too bulky and not efficient enough. With little doubt she too will be urged to sled ski down any variety of slope within reason of course.  But when you don't push the envelope then what's the point?

This is Raquel in her car seat bungied to a wooden sled with the Wolframs, Coco, Ella and Ren.

Coco and I went on ahead for some serious fun in the powder and barely covered rocks.  We put a bicycle inner tube around her waist and attached that to a rope that wrapped around my waist.  With my skins attached to the bottom of my skis, bindings un-clipped off we went.
 Beautiful...

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,
Aim
Lance.
Joy.

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.  

But

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.