Tuesday, November 16, 2010

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.


  1. Awsome Ryan. Raquel Bella we love you!

  2. This is so touching and such a good idea! I hope this blog helps you and others living with, and understanding this rare disease. Looking forward to following your journey and learning as much as I can from it.

    Paula Neufeld

  3. Hey Cuz,
    This is really beautifully told. Thank you for sharing.

  4. Hi

    Raquel Bella is precious! I am the person that sent your friend the care package and fuzzi bunz diapers. I am here if you have any questions, etc. and you can find me on Facebook. EB Resource is my blog there is a link to it if you search EB Resource on Facebook.

    You are doing a great job of caring for her. She is lucky to have you as parents!

    Take care,

    Leslie Rader, Louisville, KY

  5. wow, you have done a great job starting off this blog, Ryan. Anyone reading it can feel the pain and difficulty you all face, but also the immense love and beauty.
    Raquel is so precious and she and your family are in my thoughts every day.

  6. wonderful blog Ryan. your girls are beautiful children. putting up the links is a great idea - when my son was born (24yrs ago)I had nothing like this to readily access. any advice/help/support I can offer to you and Renata please know that i am just 'a nurse' away. they can give you our info and perhaps we can all meet up when you are up to it.
    have a wonderful Christmas too - may be a little different from before but that is all.

  7. Hi Renata and Ryan,

    I have been thinking of you a lot since Sasha told me the news. You are amazing, courageous, strong parents. I would love to come and see you some time.
    Love to everyone.