Friday, October 28, 2011

Either Side of the World

How does one explain levels of frustration from an email where my wife uses a term I never hear from her as a first character guide of the orthopedic surgeon we saw today. 
Really, if you can't be slightly compassionate when a baby with skin falling off in your office then? 
Dot dot dot
Dammit Janet sent these.




I can't help.
Renata will have a terrible night.
Raquel will have a terrible few.

JOY.
From afar.


New Orthopedic Surgeon anyone?

I'm way to tired to write, think, socialize etc.
There are some amazing people here.
I'll have to make the most of tomorrow.



Wednesday, October 26, 2011

There's a collective THUMP

We're into EB awareness week and it seems many of the other blogs I follow are trumpeting the cause.
I'm no different.  I'm now safely in Groningen and wide awake at 4:30 am.  Yikes.
So pass us along to friends and family and if anyone want to help the cause look up Debra in your country and see what makes sense for you to help.

I'm still not sure what I'm going to see at this conference.  I think we're fairly progressive with how we deal with RDEB, but till I've been through this week I can't be sure.  I really hope outside all the invaluable face time I plan to get in with those who have gone before us there are some nuggets of gold to take home and apply to our lives.
Groningen.
What a beautiful old town, umm - the five streets between the train station and the hotel.
The Dutch beer has much to be desired.  Vancouver has a good thing going with it's beer.  Especially the ones that make it up from the Western US.  Lots of flavor, big assortment.
What?
This trip wasn't about drinking beer??


and taking pictures of old cities?

Tuesday, October 25, 2011

Stress and Expectations

We had Uncle Andrew come in for a try at a change with one of our longest nurses.
I told them as we were starting that I wasn't going to be around.
That I'd be gather my fishing gear and leaving for peaceful waters.
'Good Luck, live long and prosper'.
Of course that's not how it shook down.
I took the Third's duty (keep Raquel visually and mentally distracted at the top 1/5th from what was happening at the bottom 4/5ths).  Harder than I figured, I guess I should be a little more patient with the people at the top end.
It's hard enough lining up the cuts of visco paste to go smoothly between her digits and flat across the front or back of the hand or foot. 
Keeping her eyes and mind in focus while the two of them fumbled semi nervously with my piercing perfectionism and many comments of disapproval was interesting.  I felt the stress I once had months ago figuring out the methods I now take for granted. 
I've already scared Sasha and Dammit Janet from the job, which shows how great of a person Andrew is and his devotion to his buddies family.
People like that just don't come around that often.
OF COURSE Janet and Sasha are very eager to help and they do.
DONT worry. 
Back again.
Of course things happen and blisters form during the days between changes.
I have done some terrible wraps with a few not so clever ideas of how to get around certain issues, but I being the dad can live with my mistakes or the reality of what RDEB is regardless of what we do with prevention and healing. 
It's hard to behave and watch someone nervously trying to do the order and methods as I've created.  I did my best, but you have to sit back and let it happen sometimes, I guess. 
I think they did good. 
Time will tell.
I just wish it was only the same two people doing the changes each time while I was away.
It's easier to learn and adapt when you can see what happens from your technique.

The images of her heal I showed a few weeks ago.
It's finally healed.  I ended up putting some mepilex across her heal then cut some lines in it for a little more perspiration to help the healing on a bit.  We also had to use some bactroban and it worked quickly.
That was the first day there was no blood in the water and no yellow gunk build up on the white foam.
Ahhh relief.
One issue down, for the time being.

Luckily for Raquel the bandaging suites my personality of an ever changing procedure.
I think I'd go bonkers if every day's issues were the same.

That's not true.
If every day was the same as in 'perfect', I might be able to find some joy in that.

I'm a few hours away from my long flight to Europe.
Jo will be there to meet.
My Grade 10 german exchange student whom has like Andrew become more of a brother then a friend.
Some 25 years after the tall blonde German with red glasses walked in our door we'll meet for a few glasses of Dutch beer (not Heineken) and catch up on the three years since we last met.
We were set together as we had identical birth dates.  Except he's already 40.
Jaques (his father) suggests this year almost crushed a 10 year run where at least one person from each family has visited the other.
Before that it was a 14 year run I think.
It's been fun.
Wonder who travels the ocean next?
I think it's JO'S TURN!!!!  (Jo is my abbreviated name for Jojo or Joachim, I'm a lazy Canadian)


Monday, October 24, 2011

SIMONA AND MY GIRLS

RAQUEL PONDERING LIFE WITHOUT DAD FOR 8 DAYS
CORDELIA THE CAT AND RAQUEL THE VAMPIRE BAT.

Sunday, October 23, 2011

Debra & Halloween

There was a little function for Debra held at the children's hospital that we attended today.
Raquel is getting tough to hold and we had no choice to once and a while let her do the rounds about the auditorium floor in her tripod crawl.  I'm happy she didn't make a peep, just looked intently at what was going on.
It was very nice meeting a few more families that are scattered about the Lowermainland dealing with EB in many different degrees.
The world just gets smaller.  It's obvious to me and even from talking to another parent that just a few years ago the isolation was likely incredible.
No internet and little knowledge outside what you could assemble the old way, which seems odd now.
Now, we click on a site for EB and if you search hard enough you can find many things, although reading something from a medical styled site isn't anything like talking to those who have gone before you.  
It is odd that with one click, I can see people from Argentina, Bulgaria, Malaysia or as I can see in the stats page they us.
Today's conversations were light, but there were some nuggets of information that can prove to be invaluable if not matter of factly good to know.

More so, it was comforting, standing beside those few others and talking about nothing in particular.
A certain comfort in not being alone. 

Next stop Groningen and the DEBRA international congress.
How are my ladies going to be???? 
I bet the house will be quiet, clean and calm.
haha  not if Simona comes over. We know it'll be clean, but quiet and calm don't have a chance..


Oh yeah.
Raquel can say PURPLE.
I realize it probably doesn't sound like PURPLE to the average person, but my mother is very proud that the two of them were able to expand her vocabulary. 

Thursday, October 20, 2011

Getting What She Wants

A couple days ago.
Renata was in the kitchen and around the corner comes Raquel in her Tripod crawl dragging her jacket.
After a few moments of persistence from Raquel Renata realized she wanted her jacket on.
Not exactly sure why she'd want her jacket on Ren humoured her.
Within moments Raquel is off to the front door ummpfhing and pointing to the wild blue yonder.
The longer Renata watches in amusement the wilder Raquel emphatically gestures to go outside.
Next step is Ren carrying Raqu in the driveway, but Raqu is still pointing, pointing to the car.
Once in the car Raqu was smiling and ready for an adventure.  10 minutes later I'm hearing Renata climbing the stairs at the office with Raquel happy as can be in her arms.
Raquel is also into getting our shoes and setting them up at the door or bringing them to us.
It seems she has her daddies need for adventure and everything outside.

Last few days have been good.  Still the odd blood mixed with saliva dripping out the side of her mouth, The ITCH and some blood seepage at her foot has plagued the background.  Undaunted she tears around our place and gets into any and everything she can, loving her sister and smiling every chance she gets.
Just want to show off these beautiful hands

Wednesday, October 19, 2011

Hi Gabi

Hope you're well.
Love Ry.

Geneticist and Results

Renata told me she looked through a few things to confirm her fears and the worst seems to be it.
After Ren's talk with the Geneticist, next to Herlitz Junctional, Raquel seems to have the worst version of RDEB.
Not that it matters, in the way our lives will be.  We know (I) it's going to be hell.
The level of hell, will only be in our ability to avoid issues and think through the rest with minimal trauma.
It's the difference in how Renata and I deal with this information I think is the hard part.
For Renata it's often the finality of knowing the hard facts and the depth of sadness that follows.
For me it's a word, a name and certainly doesn't change what we are doing and how Raquel fares on a day to day basis.
We know Raquel's condition is not going to get better.
Like I said yesterday, issues, marks and trouble only add with Raquel.  There will be no subtraction.
I guess if there is a cure that isn't killing nearly half the kids that are trying it, then and only then will I get my hopes up.
It would be counter productive if I lose my focus and get blinded by the froth on a daydream.


Although I just have to get up once in a week long before the sun rises, take my canoe or kayak out and see something like this and the nerves are reset.  Could be shattered in moments on returning home, but...



Tuesday, October 18, 2011

Strictures and the Blending of Wounds

I've been thinking some of her issues are that she has strictures in her esophagus.  I just had an email from a friend that some of the call signs might actually be there for Raquel.  Although I'm still holding out in thoughts she's an immortal vampire.  You know, one of the good ones Hollywood seems to create in the movies of late.  Usually once a week we'll see a trail of blood seeping out of the side of her mouth or we'll wake to brown crusty stuff around her lips.  Again looking like she snuck out of her crib in the middle of the night with her bat wings and feasted on her sister.  We're always checking Cordelia for tooth marks, haven't seen anything yet, so unfortunately all the blood must be originating from a source unseen.
She's had troubles sipping water after a good cry.  Choking as if her throat were too swollen and sore to accept simple liquids.
More and more it seems the G-Tube was at exactly the right time.  Could have been sooner actually.
Maybe the underside of her lips wouldn't look like they have grown to her gums in a webbing like way that is famous with the Recessive Dystrophic EB on other parts of the body.
I'm sure it would eventually get to her.
That's the most stressful part of her disease, that every scar adds up.
Every blemish one for the ages.
I used to look at the many scar sites on my body with a proud and some not so proud stories. 
Like hearing that cats always land on their feet.  At the age of 6 or 7 I took our family cat Tassue to the balcony for the experiment.  As the cat was half way off the balcony from being heaved into the air, he realized that this wasn't a warm lovey embrace, shoots out the claws and tears a few deep wounds down my arms, then scampers quickly up and over my shoulder to safety.  The only good thing that came out of that over learning not to throw cats off decks was a deep scar that helped me in the early days know which was my left hand over right.
Raquel, will never get that quiet moment with a lover to show off this and that in quirky stories of bravery, shortsightedness or the funny stories laced with stupidity.  Her stories will be this is where my dad let me draw at my easel and wasn't standing behind me when my sox let go from the slippery tile floor and I hit the pencil tray with my cheek on the way to the floor.  Of course it'll be buried under many other nearly as benign momentary lapses of balance or watch.
The Safe Drawing Zone


Monday, October 17, 2011

Almost off her Pain, till.

In the last few days Raquel has been going huge stretches without any of her typical comfort medications.  We still do her Benedryl in the evening and she'd go for nearly 12 hrs before we'd give her the tiny amount of morphine to take the edge off.  I'm quite sure she's outgrown this dose altogether, but the last two nights have had another flare up of her fussing, kicking at her feet and clawing at her face in the middle of the night.
Again, it's impossible to tell what she's having trouble with, only that if we're not holding her in an improvised straight jacket made from soft blankets she'd be a mess.  This morning at 7am she was back at it again lasting for an hour. 
Once the food and comfort meds had worked their way into her system she finally calmed.  The result it took her a further hour to open her eyes.  I guess she stuck her thumb in there while we were trying to hold her down.  Hopefully the eye thing isn't a scratch, only dry eyes from using up all her tears.
Wow, Ren is in for it.  It'll be a long long 8 days if even three nights are a version of last night...
I'll get mine come December when Ren goes to Europe for 15 days. 


Friday, October 14, 2011

Unpredictable

Last night Raquel was hanging out and having fun.  Cordelia long asleep.
We were playing at a few different things and she was a joy.
It started to get late so I started to slow her down and hook up her late night feed.  About half way through she started to stress out and tried to crawl away.  I figured she was just trying to get back to crawling around and playing.  Certainly a no-no with a tube and pump attached to her belly.
Then she elevated her need to move, roller over, stretch out, anything, just not laying on the couch in my arms.  At first, I thought it was her strong personality and her new found sense of entitlement to do whatever she wants, when she wants.
Great.
A kid with EB, with an attitude.

Wonder who she gets that from??

Then the crying turned to panic with some choking.
Ren came running, we quickly turned off her pump and I did my best to hold her in a somewhat horizontal position with her face pointing down.  Similar to how we got past the collapse of her pallet way back when.
Nothing seemed to work until she ran through three or maybe five gags and spits.  Not sure I was counting, only that I was covered in slimy bloody gunk, so were a few blankets and her safe zone on the floor.
There was a lot of blood.
Ren and I were looking nervously at each other wondering how far this was going to go.
The thought of another ambulance ride flashed through my mind.
Assuming she makes it that far, considering how stressed she looks and the curious amount of blood coming from who knows where beyond her famous little uvula.

Then.
The screams and gags stop.
Raquel looks up.
Looks around.
A smile, then right back into playing, as she leaves Renata and I in a quasi state of shock sitting on the couch.

Just like that.
It was over.
Back to normal.

No, better then normal, if you consider for the second night in a row Renata and I were able to sleep right on through.
Not a peep.

It wasn't till the few moments after I was woken by my alarm clock that became a little unnerving.
Dark thoughts flashed through my mind as we rarely experience a quiet night.
In fact we never have a quiet night after an evening episode.
I wanted to run and check, but.....
There have been a few of those, moments you want to run, but....
Within seconds of me standing in the hall studying her crib from a far she was up and crying for food.

Ren really has her work cut out while I'm away.
4 days is one thing.
8 days flying solo quite another.

It's fairly easy to get through a good 4 day patch (for Ren).
8 days and there will certainly be at least one moment of high end stress.
Could be internal, could be during a dressing change, could be anything.



Thursday, October 13, 2011

EB awareness week is around the corner

It's settled.
I'm going to see and learn what I can for the Debra International Conference in Groningen Netherlands during the EB awareness week.
Renata pushed me so much that I had to go.  Plus I had been hoping to meet with the family on the East Coast who have helped us out in a few urgent phone calls and many emails.  As they have helped us get over the hump in deciding to go ahead and have the G-Tube inserted.

At first I wasn't sure what to expect from the conference.  I read the line up, but maybe wasn't able to concentrate enough to see what was likely in our interest.  Better put, interesting enough to go all the way to Europe and of course be away from Raquel & family and  for a week.  
I'm certain now the trip will be well worth it.  It took a few emails shot off across the globe to realize in many cases travel will be the only way to meet many of these people and for the man who's kept Raquel fully clothed in Italian Silks, an in person thank you. 
My German buddy better return my email!!  As it'd be great to see him for a night or two.  I can promise his wife it won't be the same trip as we did at age 16.

I talked about isolation before.
It's a desolate landscape when you only know of four other people suffering with the severe forms of EB within the Lower Mainland.  And when many of the nurses and doctors we meet give long blank stares as we describe what they can or cannot do when treating or inspecting Raquel.
I'm starting to realize the importance of such conferences.
Especially in how much I've taken off the net from Debra, EB Nurse and other sites to help with Raquel's care.

Anyone reading this will know what EB is about and probably too much info on our little family. Maybe you've told others about this girl, Raquel, whom goes through a daily battle often full of pain and discomfort.  There are many others scattered across the globe and often living in much worse situations. 
This is the fund drive for Debra to get out information and help sponsor more research.
If you can find their sites for your country, please donate.
There's as many causes as the world has issues, hopefully this year many of you will think of dEBra.


Her fall last night into her drawing easel, (need some grippy socks that aren't too hot)
 As bad as those marks look.  She was a nut today.  Goofing about all day till dressing change time.  We had to call into action a new helper.  She did fine.  Raquel only protested and twitched during the popping and rewrapping of a new blister across the whole top of her left foot.  Experiment in wrapping wasn't so good I guess.  I know her husband, one of my main fishing partners would have  passed out and been more of a problem then help at the sight of the blood soaked sheets. Maybe she was close too, as I noticed her eyes were doing their best to keep looking in other directions as I was snipping at the edges of her blister with my special scissors our plastic surgeon gave me.  I'm glad the change wasn't as frantic as Monday and the Friday before.  Ren and I could have done today together, but when Raquel disintegrates, so do Ren and I.  Very helpful to have people say yes so Ren and I don't have to test our vows.
Although, I don't remember the part .till death do we part. before I said 'I do'.  Or 'ano' (Czech)
Maybe that was why I agreed to get married in Czech.   I wouldn't understand much, and maybe not accountable, haha.

No, not true.
Her Grandfather did a wonderful job for us and much was spoken in English.
But still I don't think we're bound till death.  What a relief.  haha.
My ears are burning, I can already see and hear what Gabi will say to me poking fun at Renata again.
I miss having Gabi scold me, it was almost a daily exercise while she was visiting from Czech.  It's just not the same now..
woops.  I should delete that, probably not a good idea for her to know how great I think she is, err or how much of a pain in the ass she was.

This is the smile and peering eyes we've had since yesterday

Wednesday, October 12, 2011

Push Kart


Raquel yesterday.  Seems she's back on the climb out of the three or four days of hell.

video

So it's happy times all around right now.  Not exactly like the hockey players cliche's talking about not getting too high or too low for the victories and defeats.  But a good sleep (Raquel up only once and for a moment or two) and a trip abroad to hopefully learn a bunch more about EB and meet some more people that have walked down our path does wonders to the psyche.

Tuesday, October 11, 2011

Down Time

It's terrible how nearly every time we have a team meeting with our doctors immediately afterward it goes down hill and fast for Raquel.
She has been miserable for 3 or 4 days.  The nights have been crazed when she wakes and the last two dressing changes have been all about pinning her down and going as fast as possible.
Then yesterday, being a holiday (Thanksgiving) we had no nurse for the dressing change.
The afternoon was bad and there were signs of blood seeping through at her heal so I warned Andrew (our stand-in nurse) that it wasn't going to be fun.
It was hell in fact.
We basically had to yell over Raquel's screams to hear each other.
Worse was if you were the one holding her and closest to her mouth it was all about lip reading.
Since none of us lip read, we had to speak louder and louder to hear each other.  Of course when things are difficult the third time you say something because the others can't hear you, a sense of irritation flashes up at a sentence as simple as 'Please pass the water!!!!'. 
It was impossible to figure out what was bugging her.  Her heal was about as ugly and raw as I've seen one of her blisters and has showed no signs of healing over the last two weeks.
October 7th

Joy.
October 10th (see popped blister under right eye)

October 10th

She has also scratched her face up pretty good in the last few days and a few nights ago we forgot to pop a blister just under her eye while she was asleep.  Of course it grew 4 times the size by morning and while she was laying back the bubble would start to fall back over her eyelashes into her eye.
I thought popping blisters in her mouth was a chore while she was fighting.
Wow, I don't want to do that again.......
Had my mom with me to help on that one.  This is the second time I've seen her look rather queasy while in the middle of an event.  She was certain I'd stick that needle right through Raquel's eye.
Glad I'd been out for a few hours that morning and had just come home happy, accomplished and with a refreshed set of nerves.

Today Raqu and I are off to get a custom foot brace to maybe correct her foot structure.

I also started an experiment with her foot dressing, which is stressing me out.
Hopefully it works.  A new sense of layering.
Once she gets a shoe I'm sure it'll be fine.
Just hope she gets to that stage before all that skin falls off again.

On the good news front.  45 minutes after the change yesterday -
She started back to her good old self of hamming it up.  She wouldn't go to sleep and kept playing and playing.  It took Ren and I to finally put her down.  I'd hold her still while she tickled her face with a blanket till she finally relaxed.
I figure she was making up for the lost time over the previous few days.
What a kid!!!
Miss Ham and Hammier


Thursday, October 6, 2011

Healing Under Derma Silks

I understand that infection is such a big deal with children with EB.
We've been very lucky so far that we've maybe used bactroban 6 or 7 times in the last year, on Raquel's foot and the fungal cream at her bottom twice.
I was recently checking out Debra Canada's web site and read about a boy who died of septic shock at a very young age.
YIKES!!
I felt a heavy heart reading about it and am very uneasy that such a demise is such a real possibility for Raquel.  Among other things...
There is this new found sense of community I feel to those who have EB themselves or within the family unit, where their plight and good times affect me more and more.
It's a new feeling for me as I often read of wars and social unrest the world over to almost junkie status.  There are so many ways for life to be complicated and there are unfortunately enough people imposing their will that there are rivers of despair everywhere.   Anyway it usually culminates in a few cross words in front of the computer screen.  If my irritation pushes on past that to the point I have to bring it up in conversation with a friend or whom ever will listen, it's often met with blank stares.  The isolation of prosperity, I guess.
EB is an obscure disease wrought with the feelings of isolation, it has wicked consequences and the deeper we go with Raquel's age the potential issues pile up.  By seeing the older families and even the new ones dealing with it, it's getting tougher to sit idle and swear at my computer.

OH how the mind wanders.
To delete or not to delete?
I promised a friend I wouldn't, but I'm not sure that applies to this BLOG.
Oh well.
October 6th

September 26th
Back to bandages and her silks.
We've not put one bandage or wrap on this area.  It oozed for a couple days and we were (Renata was) diligent in keeping clean silks on and the wound has been in the above state for a few days now.  With Raquel a blister of this nature that gets good air circulation will heal to a sealed state (last night I actually read what it's called but like with other descriptive medical terms - a sonic boom is heard as it passes over my head).
I'd better get a useful grasp on these terms as I'm planning a trip East to the Netherlands for the Debra International Conference.  I've already been called out on my comment the other day of me being the 'normal gene carrier' and Renata the clear 'recessive carrier', and not explaining that I likely have clusters of the recessive gene that we're not found in my sample tested.  It was too much fun to poke at Ren for me not being the father then to actually be factual.
How the Recessive Gene is transferred
http://www.ebnurse.org/index.php?id=28
Thanks Gena!
The Conference does not have the line up of issues directly facing us, or issues that I spend a lot of time thinking about.  I also wonder if much of the information can't be found out on line or by asking the few EB families I keep in contact with.  I'm thinking the people I may meet in person will be the invaluable part.  Just hope Ren, Cord and Raqu can handle a week without the man who turns the household upside down on a regular basis?

Tuesday, October 4, 2011

Banking Hours.

This morning I awoke and felt incredibly rested.
Like one great sleep can solve all the angst on the planet.

Off to work and a bit of the regular - one part brain, one part routine, one part harass the grumpy guy on the other side of the wall.

Dressing change was planned for 2pm, due to nurse availability.  I wasn't able to get back anywhere near that time, so Ren and I called in my mom for a late evening third's duty.  Forcing Ren to take the dreaded #2 JOB.
3rd is keeping Raquel calm by what ever means and holding down free appendages.
2nd is holding the appendage being worked on and working in seamless conjunction with the wrapper.  The problem Ren and I have is she can't turn off the nurture part and hardly every pays attention to the next thing she's supposed to help with while bandaging.  Which often turns me sour.
I don't really have an excuse to be sour, but I'll try - as it's often necessary for the dressing change to be over quickly and when feet flap, arms wave and positions aren't maintained....

It went reasonably well till the last few moments on the last hand.  Raquel was getting bored and tired and maybe a little claustrophobic and started to lash out.  As much as my mom and Renata can't stand doing dressing changes with me, well....

It's like two candles shinning light, love and devotion around a powder keg with a little Brat with a good reason kicking up a dusty volatile storm.
Anyway, we survived.
Put Raqu to bed clean and newly wrapped with minimal effort.

Then,
just an hour ago (1am) Raqu woke in her typical nightly grumble, which lately involves a Gfeed and Benedryl.
It's a chore, as we both are eventually forced to get up and take care of the little tasks involved in expediting the procedure so we can all go back to sleep.
Tonight was different.
I pushed Ren to start the response and off she went.  (I'm on first response after 3 or 4)
But with the words 'What's this?' coming from Raquel's room turned groggy sleep to peaked attention.
By the time all three of us made it to the couch it was evident with all the blood soaking through her bandages and clothing at her feet, immediate attention was required.
Great.
1:30 am and a dressing change with the possibility of giant bloody blisters running up her leg or pushing off the remainder of healthy looking skin at her feet.
Why us?  I guess the painfully small odds of genetically handing down this disease is making it's rounds in our thoughts of late.
Ren and I don't ask to be a normal family, but what we wouldn't give to have a typical, maybe even slightly difficult 1 year old whom wakes in a noisy unanswerable fuss, then with a little rocking and a bottle eventually settles and sleep can be sought by all.
Hmm
How boring is that?
Banking hours for when we finally reach 'the great sleep'?
For some reason it seems Ren and I don't have enough character to slip into an easy life.
I used to say before Raquel was born when Renata fretted about giving birth.
There's billions of people on the earth, what could go wrong?

There was a lot of blood.
It got exciting.
People that love each other didn't use many phrases with meanings pleasant, warm and or fuzzy .
There was also one of the many times I've looked down at her wound surveyed the situation and asked myself.
'How can I wrap that, not cause more pain, and create a slip layer that won't continue to wear away at the red bloody oozing area with no skin?

We'll see, won't we.