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.


  1. Thanks for the book recommendation "Kidowed". I bought it a couple of hours ago and am halfway through. Should be required reading for all humanity.

  2. Thank you for the post. Any exposure is good exposure. Keep spreading awareness!!

    Jessica Bonczar Kenley
    Author "Kidowed"