Chaos and our first ambulance trip

Came home from work, started my dinner as Cordelia was taken out to go get her Beau Jakob (from across the street) with his mom.  Renata was about to go to the airport to welcome another Czech beauty to Vancouver.  As I started to eat, Raquel in my arm she started to fuss and Renata closed the door and gone for the evening.
oh, back track a few moments.
About half way through cooking my dinner (heating up Renata's cooking - mmm) I walked into the living room and Renata was on all fours head down in some strange breathing pattern.
'Having a panic attack?' I asked, not in the warm soft caring mode I'm sorry to admit.
Yes she was.
So I asked her about her day and all was well.
So then I gave her a nudge and a brief pep talk. 'buck up my dear'.
Foreshadow maybe.  Mothers intuition?

In goes the bottle, out comes a mouthful of blood then directly into a choking frenzy.  A couple seconds go by and no gasp.  She was able to get in a few then back into it with deep cries between gasps and the death gurgle you hear in war and horror movies.
Luckily my mom was near by to help out.  I'll not throw her under the bus, but it's really difficult thinking of how to care for a child with breathing difficulties when you are wondering if you'll have to call another ambulance for the two of them.

After four of the long breaks in breathing I called 911.

Basically I was thinking I could get some advise on the line, not exactly a fire truck and an ambulance and five guys in my house in 10 minutes.
As they walked in I started my speech about the rareness of her disease and the latex gloves they had on un-lubricated would create blisters the size of their fingers, nearly instantly if she was handled in the wrong way.
They each were extremely respectful of her situation and listened.  By that time we had (mom) calmed Raquel, Mom was doing fine she was back in my arms,looking about wide eyed.
In a few occasions she'd cough and this strange bubble would come out her mouth.  I realized early on it was a blister that had broken and blocked her airway, but I wasn't sure how big by that point.  The weird bubble coming out her mouth freaked me out to say the least.  I can also say with firm belief even the hardened ambulance medics and firemen were in uncharted territory and had an uneasy look on their faces.
We decided we'd give it a go and try to pull the blister out with some excellent tweezers they had (I definitely have to get me some of those), the medic suggested I be the one who do it.

Gladly.

Mom and and the medic held her steady while another medic shone a light into her mouth while I pulled at the massive skin flap that was her ENTIRE MOUTH!!!
The part that really got to me was the neat little hole that appeared to be where her uvula was.
It was shocking to see the entire layer of skin in her mouth including her tongue come off.
While I pulled it was attached very firmly at her lips and I felt a weakness deep inside me, as I tugged and watched it tear at the firm skin at her lips from the inside outward.  I did my best to twist the tweezers to rip the bubble to create a bigger hole and maybe stop it from covering her blow hole, since it wasn't coming out in pieces easily. Problem is that skin is way thicker then the skin elsewhere on her body.

We agreed it needed to be cut off.

I suggested scalpel.

geesh no wonder I'd not get into medical school with half baked thoughts like that.  A firm C+ average and a passion for art not anatomy wasn't going to make me much of a doctor.  Clarify, inner body parts and their workings didn't used to be all that interesting and chemistry diagrams very very boring,  I like anatomies just fine.  Brains are interesting too, but still way too much schooling.

The medic asked or suggested that we best take that trip to the hospital.
I agreed.

I tried to quickly assemble my 'go box' or 'butterfly box' (red box) but was dazed and basically forgot most of the important contents as I'd not yet replenished it from our ski trip.
I did have time to have a vision of doing this procedure in the gas station along the #1 highway at Lyton, with a few boxes of -40 window fluid as a table with the many insightful slogans written everywhere to keep us company.

Once in the hospital I text Dammit Janet, to tell her I was at her place of work and sent a pic of Raquel with her mouth open, clearly in the ER intake hallway. 

the circle is the hole of the uvula fallen forward, The white part isn't her tongue but all the skin from her pallet and tongue

It took some time for her to reply, but she was in the hospital before long to help out.  Great considering they are her colleagues. I didn't ask, but knew I didn't likely have to, the image was probably enough to maybe motivate one of Raquel's personal nurses to some unpaid overtime.  I was very grateful she came. I had to also lean on mom to bring over some of the things I forgot in my moment of haze.
After going through the spiel again about her skin and the dos and don'ts the doctor figured out we give her a shot of a morphine and midazolam to calm her before we tug around on that flap.  M&M's

hehe Renata just showed me the tweezers the medics forgot.  Wonderful, the silver lining.

Back to it.
We held her down while the doctor pulled out the skin flap and cut it off in two tries.  There remains a big flap at the back and it still flutters with her breathing.  I can hear the flutter now while she sleeps beside me on the couch.  She's on her side so that the bigger flap lays flat to the cheek.

The choice was, make more damage trying to get at it or let it go.  We let it go.
The other part of the silver lining was I was able to bring up the fungus and smelly foot from our last dressing change.
We set up a tray and did a foot change there in the ER, while a nurse took a swab and handed me the bactroban type ointment and we were on our way.
Dammit Janet you rocked!
I'm very grateful I didn't have to train a new nurse how to hold her or take up the ER nurses time either.

Although she did text me later to poke at my resolve saying she saw me waver when the Doctor had the scissors and was cutting the skin flap very close to my crying babies lips.  He was young and agile so it passed quickly.  But it was still interesting to see him slump his shoulders in relief when he realized we were done and he didn't have to go back in and it was a seeming success.

By tomorrow morning at 10 am I'll judge if it was a success and that I didn't have to see him again.

I have those wonderful tweezers now, so just as long as Renata doesn't go into another panic attack we should be able to nip it.
We just don't have the M&Ms in our repertoire for sedation.
I have to admit. I haven't seen Raquel that stoned since one of the first morphine doses she got while a couple days old in the NICU.

It's wearing off now though.
I foresee very little sleep tonight.

hmmm where's Cordelia??

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.

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.