Wednesday, September 26, 2012

She pushed off the whole palm

In a moment when Raqu asserts her independence, Ren watches from a far as she walks across a concrete play park.  Of course she falls and comes within a mm of pushing off her face too.  The cries intensify and it's clear her hand is in a great deal of pain.
Once home we do an emergency dressing change to screams and shuddering pain.  I had to slice many areas of the blister to waving hands and squirms.  One cut looked as though my fears of scooping a chunk of flesh had finally come true... Luckily it was just that this blister was very bloody to begin with.
Once the aquafor and zinc bandaging material and pain meds kicked she was her self again.
Although slightly more goofy than usual.

It's clear pain meds are for all.  Even if the rest of the family isn't on them.  The anxiety dropped very quickly afterward as our coping method of out of sight out of mind was able to kick in.

Today's change was rather PG 13.  Minimal blood, small cuts at the edges and only 60 seconds of suffering as Raquel looked deep into my eyes wondering -
'WHY is it always YOU, that make me feel this way?'

The funny part at the beginning of the change was her saying a word that rhymed with 'it' when she dropped a toy.  The splayed arms in questioning and look on her face well copied another member of this house hold.  We won't say who, but it wasn't me or Coco.

Wednesday, September 19, 2012

Hand contractions and bigger issues (not Raqu)

I can tell you it was very stressful.
The thought passed through my mind to find a scalpel quick and give the scar pulling at her palm a short slice.  Then when the image of a German girl who's mother exclaimed she cuts at her hand webbing when ever she thinks it's moving out killed the idea.
I do remember for a long while wishing I could cut at Raquel's two toes webbed and the skin under pulling them down and under.  Much like the area between her teeth and gums pulling her smile flat at her upper lip.


The signs are there.

I of course have spent the last 5 months ignoring these issues and enjoying all what has been largely unaffected by EB about Raquel.  Let me tell you when you are living high with your child there could always be this -

There was a little excerpt taken from a book Kidowed a mother of two babies with Junctional Herlitz now passed on gave at the Gala dinner.  I had the jolting pleasure to meet Jessica Kenley as she walked, shaken and stressed up through the lobby of the Hotel to the Congress.  This exactly 5 minutes after she was to speak to a room full of EB survivors and medical professionals.  Buses and the arduous travel from the US did not cooperate for her to make her entrance in a timely fashion.
Later in the evening and a reshuffle resulted in some hurt feelings and irrate behaviour from whom one would least expect.
I was talking to my mentor John and Leslie Rader (whom had once commented on this blog at it's very beginning).  There was a small exchange of hugs then John looks Jessica square in the face and says I've read your book, pause, then something to the effect - you were angry, I get it but you were angry.


At the Gala I found out exactly how angry and at whom and what.  During her reading I was taken back by a few cusses and hard pointed comments to the void of EB knowledge at the hospital her son's last were spent.

I thought I was upset when Raquel's hand was so badly bandaged from the NICU nurses, when her skin fell off like a wet latex glove when or plastic surgeon and burn specialist was finally able to present proper materials?

I found myself at one point listening to Jessica's dark sarcastic demeanor exclaiming the ineptitude of a person advising her on her sons care and did a snort of equally dark sarcastic laughter.  It was the moment of who's helping who?  Immediately I realized it was loud and looked quickly if anyone leered my way in disapproval. 
Like I didn't get the gravity of the situation. 
I got it, I just should have contained my internal dialogue, but I think the general horror for nearly everyone there overshadowed my little moment.  Although, I'm not sure her comment wasn't meant to be funny...  What else is there, when things become so desperate? 

She carried on and brought up a paper written by the group in Groningen.  I was in fact sitting beside the head EB nurse from Groningen and noticed her talk to her colleague.  As the rest of the evening unfolded I wasn't able to get a comment.  I remembered during last years Congress a presentation by W.Y. Yuen on Terminal phase in Herlitz JEB: experiences and data, (PAGE 10) which talked in similar themes.  It was shocking to hear them talk about once finding with certainty the child has the Herlitz version that care should move to palliative than interventional care.

At that time back in Groningen I couldn't deal with what she said or her mentor defending her presentation when an audience member cried foul.  I thought it was cold, callous science and showed little respect for those present suffering from EB, parent, patient whomever.

It took the year watching poor Tripp be overcome, to get it. Then listening to Jessica go on and on about the details of her son's condition as he got worse and worse and suffered incredible pain.  I was basically left shell shocked as a good portion of the crowd listening to her recount some of the final hours of her son's life and the guide with which she struggled through her second child's even shorter brutish life.

Of course, so far there is a great exception to the Herlitz rule.
Daylon - see at side.  I do notice nothing new for months in the families blog entries.  In this house hold that's a good thing.
I'm not so sure about others.

If anyone is interested in how hard and brutish Herlitz Junctional is, with the odd, no sorry constant cuss and sideways comment check out this amazon link.
Stand back and be punted.
But please don't find a free copy!!!!!
Pay for it dammit!
Jessica only gets a $1 from each copy sold.

Monday, September 17, 2012

Publication for Debra International Congress 2011

Publication of 2011 Congress

Now that I've seen the 2011 publication I realize the 2012 will take a while.

This years International Congress

Just back from the Debra Congress in Toronto.
I was able to split and shuffle dressing duty to Andrew and nurses while away.

Of course I was very nervous and was told everything went well without hitch.
Upon seeing Raquel this morning for the first time since last Wednesday she peered at me in disbelief and basically turned away and hid her eyes.  I did get a little smile while I tried through waving arms to get a kiss, taking the defensive karate chops off the forehead and chin.  I did eventually get to place one, but with was difficult with her dancing like a butterfly and bobbing to and forth.

Yesterday she took a few falls, so her face and elbows aren't looking that hot.  But what's under the daily preventative bandaging is what concerns me most and there I'm told remains perfect.  We'll see today.

Sitting through the Congress was very interesting.
It was geared slightly more toward the patient and front line care giver than I thought last year in Groningen was.  I'm not sure if that's a good thing?  I'm still struggling with what these congress' purpose is.  I look at the mission statement and can't figure out if it's science or care?
 Mostly I quietly observed and listened then got excited when an interesting conversation broke out on the direction of where things should go and what was being done correctly. 

The one time I did speak out in private conversation to the COO John Dart about the current writing of the Best Practice Guides and the inherent overlap of information due to the systemic variables of EB and all it's forms, I was left to feeling I'd best keep my ideas inside for the near future.  It's not that my idea was rejected, it's just that maybe it's uselessly ahead of it's time as much of the work has yet to be done and keying it all together into a singular form being a rather monumental task.

Having listened to the Doctors and nurses in the midst of writing the Complex pain, Nutrition and Occupational Therapy Guidelines just getting to where they are seemed difficult and time consuming enough let alone have to relay to one another where each of them were at that time and how to overlap and key into one another is clearly impossible at this stage.

I just hope once all these guidelines have been completed within the next few years that the second or third rewrite, Debra International can have them bound in some logical way.
The disease is just so unbelievably complicated as one starts from nutrition and branches out toward everything else and if one fails then it will invariably have consequences further down the line.

Confusing, yes - I digress.

What was amazing is the near cohesiveness of the Doctor and national groups.  I kept talking about divergent philosophies as it's clear many ways to work and make people comfortable with EB rely on the immediate care group.

Eg.  While sitting in on a nutrition workshop on the final day I spoke up about Raquel.
A Russian man was gathering information on G-tube care and best manner in it's placement.
The topic bantered around a bit till the question of what can go through the peg (her belly port).
Currently Raquel only gets Pediasure (a total nutrient concoction to meet her growing needs), I would very much like to get ourselves a blender and start mashing up our foods and feed her that way.
I've had some resistance from our nutritionist as to how complex Raquel's mineral and vitamin requirements are and the importance of maintaining this balance.  Plus I'm lazy to add another regime and time consuming part to her care.  Isn't it enough that we are constantly balancing her pain meds, with bandage requirements, wounds and total blister prevention attention to keep her safe..............

In any case, the UK people suggested that putting natural foods through the G-tube was possibly unhygienic and not advised, but the US people suggested that hundreds of people do do exactly that and it hasn't been an issue in most cases.  In fact as long as a good plan was created that it was advisable.
Of course I want to try it, but I'm somewhat nervous.  The UK nutritionist did say later once I showed photos of Raquel that it was clear we were on a good path of hygiene and that she did think we could try. 
But at it's core this type of divergent philosophy isn't just there at nutrition, it's at wound care, complex pain (ie. gasps of telling people Raquel has had a steady diet of Morphine although the Dr. Goldschneider did go on at length this year about it's advantages), dietary (ie gasps of people when I suggest Raquel only takes possibly 3-10% of her food through her mouth) etc.....
But then when the opportunity allows I'd show this video of her hands while sitting during a dressing change..

Or this full body shot sent via text from Andrew the other day of her standing in a tub with body sturdy and virtually unaffected with blisters and wounds.

Zero collagen I'd inform them.  As these shots highlight her pain, nutrition, wound care, clothing (Derma Silks) are clearly being met due to the obvious overall health of this little princess.

I did see in some eyes that this can't possibly be the case (zero collagen), as in some aspects this flies in the face of years of teachings and the various formats taken in the care of EB.

Anyway, despite all this, one can not question everyone that presented and witnessed this years Congress their unwavering devotion to the cause and that these forums are absolutely neccessary to be able to share ideas, therapies and techniques.

As a parent and a Board Member of Debra Canada and as the Adviser to the Medical Assistance Fund I'm doing my best to pick, question, and move through the evolving information and procedures to best care for my daughter and open up the lines for whom ever else is interested and in need.

Support your Debra within your country.
If you work or own a large company, please suggest to guide your dollars open for charities toward the Debra groups or become a personal member of the Debra Organizations within your country and our umbrella Debra International.

I understand in many cases companies send money through the United Way.  If so talk to your accountant and please advise United Way to send a portion to DEBRA.

There are many things need funding such as the Best Practice Guides as well as the researchers who are working for various treatments and here in Canada fulfill the many shortcomings of our healthcare system and give the EB sufferers the equipment and procedures they require from this extremely debilitating disease.

Yeah I know.  Raquel looks perfect, but trust me the imagery and case studies I was exposed to during this congress would shake you to your core.

In the coming days I'll link some of the speeches and workshops I found interesting as they are or will shortly be linked on the Debra International web site.

Monday, September 10, 2012

I didnt utter those words

It's a simple sentence spoken between Ren and I, often in a quiet moment over dinner or a ride in the car that basically reflects on the erosive suffering EB can have on all our souls.

"I can't believe how well she is?"

Virtually with in moments of us saying it, Murphy wakes up and lunges at our collective well being.  I'm far from Stupidstitious and on any occasion I do my best to poke fun at it's impossibility (Superstition).
Although for arguments sake, lets just say I'm doing my best to not remark and just enjoy 'it' while the good time lasts.

The cycle returns.

It started with a dressing change.
I'm not laying a heavy here, but I was in the middle of letting go my dressing change responsibilities, by getting Andrew and one of our longest nurses back into the process.

I could tell the hand was going to be an issue by the pressure of the bandage going on, but maybe going for a sail was more on my mind than trying to pull all that bandage off again and stress out my students.....
Unfortunately it wasn't too far into the evening before she pointed to her wrist, I ignored it and waited till the next change day as her irritation toward her wrist was short lived.

On that following change when the hands came out from the clothing and tube sock, a huge bump was pushing the bandages up.
I did what I should never do but it was a reaction to my instant guilt of not checking the area sooner.
I quickly reached out with my scissors and cut the top right through the bandage.
The moment I cut it, I knew it was a stupid move and had my repeating visions of the day I finally cut at a blister and take a chunk of flesh with it, but the bubble released and poured a few table spoons of fluid to the floor soaking the bandages.  Once I was able to start pulling the rest of the bandages off the sensitivity of the wound had her crying out in pain.
From then on it's been a wiggly painful experience with her.
Thankfully after a brutal night of listening to her cry every 20 minutes till 3:30am she slept and woke up a dream child.
We then went to a birthday party for our friends child all set up in their rear yard.
Most of the people there have a pretty good idea of what's up with Raquel, but I'm sure it's not in too much depth.
Ren was off at a bridal shower and within the first five minutes of Raquel using the small plastic slides set up on the grass, she does one of her funny little happy hops, folds her ankle on the grass and does a face plant with a whiplash effect of her face bouncing back up after the impact.

OH JOY,,,,

At first inspection I didn't see any of the giant patches of missing skin from across her face I expected so I figured I'd dodged a huge bullet and then picked her up to my chest to calm her.
Clearly one can imagine the looks of concern I was gathering from the people close enough to hear Raquel belt out her pain and frustration.  I smiled as if it was just a grumpy kid with normal collagen levels whom had a little fall.  Of course this wasn't totally the case.  After a second peek she had a bloody nose and a bitten tongue.  The best part is I was wearing a white shirt so for the rest of the party I walked around with a blood/slobber patch on my shoulder.
Ren did eventually make it and it was nice to finally get a beer and two slices of carrot cake to quietly smooth out the bumps in my brain.

I'm headed off to Toronto for the Debra International Congress.  It looks to be a great line up of speakers and best of all one of our EB mentors will be there from NY giving a presentation.

I need sleep, but can't as thoughts of total chaos with Raquel are plaguing my mind, while I drink beer late into the night on Young Street.
Oh the horror.

I'm mostly fearful that having taken some time for Ren to take her own turn of testy/grumpy I'll be right back to sleeping in a tent.  Except this won't be the warm summery pleasure of sleeping down by the lake.  It'll be the under the cold torrent that washes us Westerners from Fall to Fall amongst mud piles and sawdust.

Tuesday, September 4, 2012

Just a little EB and a whole lot of support

We were off to a friends this last weekend for a special birthday party.
Special in the way that when a party is undertaken with a band and lots of friends the center of attention puts her desires forward to support EB and Raquel by having a $25+ entrance fee (of sorts).
At the end of a georgeous late summer evening there was a big bowl full of cash and cheques equaling $1685.00.
So, when anyone asks, what can I do?
take our great friends lead when you hold a little social of various magnitudes.
Any and every thing helps.

Believe me, that money is going to help someone with EB who is left out from various funding shortfalls in our countries governments and municipalities.

It was an amazing party.  I feel so grateful that Raquel and our family has been able to have so many incredible friends to lend their various levels of support.  It really makes getting from one point to the next that much easier.

As far as Raquel is going, she's been great.  She did skin both knees yesterday and woke up at 5 with some discomfort or itch at her feet from wounds getting better, but really totally minor.

The vast areas of her body are perfect soft baby skin we tickle and kiss as much as possible and we certainly don't take it for granted.

Her mood and mobility are virtually unhindered.  She watches older children play and wants nothing but to jump off stairs and rock walls towering well beyond her head.  She basically only knows two speeds when trudging around.  Walk or run. 
I still can't figure out how she does it as I know how raw her feet are on most days. 
She's the true model of grin and bear it.

She Rocks

Merissa rocks!!!