Friday, November 29, 2013

Fragile 'this side up'

We've had such a good run.

We are constantly aware that Raquel is one step from total disaster.
That step came yesterday at her school.

Raquel has been at her Montessori class now for some time.  She's adapted well and there are a few kids whom have enjoyed her company as the teachers suggest. Finally, she's not hanging off and clinging to her buddy Ella and creating some new friends of her own.
Ha, nervous laughter... I'm reminded of a situation her teachers recounted wondering if or if not to interject with Raquel when she smudged herself between Ella and a new friend.
Elbows, knees, buts and butterfly wings.
How difficult will that become, will it get worse with age?  The jealousy and possible feelings of inadequacy.
Maybe that's just me projecting my awkward teenaged life onto a situation that has the potential to be even more awkward adding trauma laden disease.

Anyway, yesterday Raquel was at school with one of her prized nurses and simply was walking and didn't quite pick up her feet, as she tends to shuffle about anyway.  The bottom of her shoe skids and stops and face first she falls into the arm of a wooden chair.  Apparently the chair even had a coat protecting the arm.

After the screaming and a little choking session due to her rant Raquel was whisked home to tend to the missing skin and a very upset child.
I happened to be working from home and as I hear them come though the door early I go to see expecting a sniffling nose or some other random event.  Not that a portion of her face looked like a wet towel on concrete squished up in clumps as if someone was running down a hall and jumped on it to slide then left wrinkled and clumped in the corner.  It's just that the corner on Raqu's face was her nose and the shiny patches were of raw flesh and an eye nearly swollen shut also missing most of the skin in that area.

Of course I went directly to her to cuddle and give her my best, but up came the straight arm any Sunday rusher (American football reference) would covet.  I got a nose full of her silk covered palm as she blocked my hug/tackle attempt and bolted away.  It took a while to figure out she was totally fine mentally and coping with the pain well, if it existed at all in this moment.  She was simply content to play and not be crowded.

It took a while for me to figure out how this might be?  But it's really quite simple.  She didn't have a wound that would effect her movement and dexterity so why should she hang out and whine about it?

My day was shot.  I still have a very hard time coping with such a blister especially when I haven't a fking clue how to dress or manage it.  I'm a pro at anywhere else on her body as we can for the most part bandage it and keep it clean and safe.
But every time she goes to touch the one at her eyelid I get angry and scared beyond belief.  She's destined for an eye scratch and that four day nightmare, add in when this gets to 'the itch' and this could be a good long go.

But, this kid is soo tough.  Who knows.
Smiling and goofing about within the hour. She can't even see out of her left eye??

Nearly everyone that was in the war zone on this one was impacted, I even broke down talking to my uncle and had to finish the conversation.

Maybe the big problem with this, is that I know, this is really only a small smear and wound compared to many with RDEB.

That's the really scary part. 

Thursday, November 14, 2013


I struggle in part with a few of the decisions we've made for Raqu.

I'm always assessing her pain needs and her nutritional intake against almost everything.
It's hard when the warm blood and soft meat of a lamb tastes so good and Raqu is sitting beside me with fork in hand making a fuss that she feed someone.  I have to admit out of pure curiosity I've put a loaded fork in her face and asked if she wanted some?  She looks at me like a big joker and says the same every time.
'I can't eat that daddy'.

Is it fine to finally stop the morphine?
We're on to a smaller dose now and I quietly had her dose stopped completely for a few days.   In my recollection she's been without for maybe 6 - 10 days in her life.  I noticed no signs of withdrawal, but it turns out we met with our Pediatrician shortly afterward and she pointed out that babies due to their fat content can actually hold the drug in their body for some time and that in a few days it might start to effect her.  So Ren had her back on a minor dose.
It didn't end up mattering as within a day or so she was back with choking and night time breathing issues.
This latest round was fun as it sounded as if a skin flap was at the edge of her epiglottis.  Every minute for hours she'd cry, fuss or squirm to make sure it stayed open.  While I listened helplessly in horror to the fluttering sounds with a vivid and 3 dimensional imagination.  Every fuss I'd wonder if it was going to be an ER visit or worse.
Eventually the sounds subsided and sleep came to us both.
Ren and I were kind of able for the next part of the day forget we had EB.
Well, maybe that's because she was left with a nurse for the day and it wasn't our problem (of sorts).

Cruel, but in a way the only way to survive as I see it.  Separate as quickly and for as long as possible, so that the strength is there for when it's really difficult and needed.

I am getting that this attitude and wander to water isn't marriage friendly even if it's a useful tool in the EB battle.
Between the canoe and that pile are a few hundred salmon very interested in my fly presentations

A few giant blisters and back we go, the routine in full swing.
Or will we?  It seems Raqu has become so much more aware of her body and suffering and ways to manage it.

We did a little Emergency change today on a foot
When I know there is something like this lurking below due to either her telling us or finding it by pressuring various parts under her bandages, hopefully to find a little pain or an unnatural softness.
I can tell you that this is definitely easy to spot under two socks, her silks and 4 layers of bandages.

This time we didn't have the time to dose, so we worked with our various distraction techniques.
The main one is that she blows bubbles in the air (pretend) and her favorite is to blow it at me so no one can hear me once the bubble has covered my head.
I then give orders and directions but nothing can be heard by me unless she pops the bubble.
She giggles and laughs and asks 'what you say?'
This one was fairly easy.  She didn't fight like she used to only a month ago as this would bring out max anxiety and max screaming.  That's not to say she didn't look me straight in the eye while her little body shuddered for a moment but to ask in silence 'why the hell are you doing this to me!?!'
Do I tell her it was her sister's fault for not paying attention - AGAIN!!! Kneeling for the second giant blister in a row on her foot.

The questions.
Feed her some food other than Pediasure and yogurt and run the risk of plugging her up and scratching/scaring what is likely the most important tract in her body and last bastion of mucous membranes likely fully intact?  I'm starting to understand that if we screw that area up for her, then retaining nutrition will be a battle tougher than protecting her from her sisters knee and life's abrasions.
Remove most of her underlying pain medication and possibly make her old before her time?
Dunno.  I see something in tortured souls and the eyes showing the weight of the world.  I'm of a philosophy that pain is dystrophic to.  She's full of feist and really the only time I see her break down for large stretches is when she can't use her eyes from some type of abrasion.  Like a jail.  Locked inside her body she goes dormant and simply waits it out.
But there are the other reasons to do the other options that many do in the world.
Time will tell and I guess this is the really evil part of EB.

Monday, November 11, 2013

Tough enough

Yesterday, I was planning a quick excursion up Seymour to see the little skiff of snow and show Coco, Raqu and Jake.  It's a yearly event for me since I could get myself up the mountain.
As I was asking Jake's dad for permission to take him with us Raqu happy and overly excited about such an excursion forgets to look down and basically in her run, trips on my foot and does a face plant straight onto a paver driveway.  The sound of her skull making the impact with the concrete was very distinct and very much concussion sounding.

I look to him and say "Nope, not going." I quickly picked her up and off to home and see what damage has resulted.
I was rather sick to stomach wondering how much of her face was missing skin as I didn't dare check till I had all the materials and tools necessary to deal with such a crash.
In the moment I picked her up she finally let out a cry and it wasn't the usual screaming that accompanies this type of accident but something surprisingly low key.

We make it to our place and sit on the couch as I do my best to console her and check her face and hair line.  After a couple moments she finally puts a few words together explaining why she's so upset and it's not what I expected to hear.

'I want to see the snow' in a quivering pout.
How tough is she?

As Jake's dad shows up at our door to check in on Raqu, I have to explain that the mountain hike is on.

We don't get far, but were able to have a snow ball fight, play with ice shards and then there is the part where Jake found some frog eggs in a giant puddle.  I had to giggle as he figured someone had dropped a jelly bean in the water and was looking like he was going to give it a try.  It's clear to me how very lucky Jake is that I am not his father or else I quite sure I would have kept quiet to see what happened during his inquisitive moment of what a jelly bean from a pond might taste like.

That being said.  I am a dad of a Coco-nut.
The death thing that she was trying through a while back has morphed into something much larger and gaining a wicked amount of speed.   I haven't a clue....  I think the void of heavy stress from the EB front has finally started to crack the family.
I can handle EB and all it's shit.  I can't seem to handle a burgeoning OCD, clean freak, and unrealistic expectations on top of it all, add sensitive mother (not mine). Shit yeah, her too.

Just when EB was somewhat licked - meaning it's a good long stretch where we have been able to handle it with authority and grace, all the hidden psychological effects of everyone but the center of this family are bursting at the seams.

So who's the same around here?
Raqu, that's who.  Even while up at mountain this painful skin puddle was growing within.

The video is from her after we've gone through the pain of the popped blister.
I guess I'm still a bad daddy and Simona is best.



Friday, November 1, 2013

Long time hit.

The weeks and months breeze by.
I think at 3 years EB is old hat now.
Warn in and stable.
That's not to say coming home to notice four finger scratches and a thumb scrape at the edge of her stoma site from a care giver doing a minor bandage change doesn't get my blood boiling.
Good blister bad blister?

She's simply been exceptional.  She fights every day to be who she is and what she wants.
This of course sets her will against mine in complete turmoil and a quiet resolution is rarely accomplished.

In fact where I thought as a parent, at some future date I'd worry how the older child teaching the younger bad habits and attitude.  This has clearly not been the case.  Straight out fits and stands in fierce defiance has almost become a daily occurrence and where it was only the young one I now have to patiently wait Coco out in various occasions.
Again I thought wrong, in those quiet moments pondering a life with EB - wouldn't it be great if Raquel was strong and had all the fight to battle this shit in a fierce defiance....

Yeah then there is
Raquel notices for a second that a cuticle has started to reform which looks more like a scab than anything and she proudly shows who is present in the fleeting moments I allow her hands to be free and she gleams that she'll soon have enough nail to have Simona come and paint her nails like she does Coco's.


Whatever.  It's EB week or the end of it.  I'm not interested at the moment.  For the most part of the last four months or so we've been extremely good at forgetting it.
Of course less the moment she coughs up a chunk of esophagus or a blister balloons from the back of her knee or the moment she keeps us up for the 5th or 6th night in a row or an eye episode.

Really it's actually mostly positive right now.  She runs, she plays, she's fierce and for many moments we forget just how fragile she is.

Like the moment I had both Coco and Raqu on a swing the other night pushing them just past the point where they loose that pendulum swing and go too far and have a short jarring vertical drop as they begin the next motion.  They giggled and giggled as they flew higher and watched their sister panic at that moment yet still wanted more.  Even after Raqu's swing wobbled and she scuffed her head on the metal cable smearing a small patch of skin.
Good blister bad blister?  haha I guess it wasn't even a blister.  Just literally a skin smudge.  Like one would try to remove rubber cement from a glossy wet surface.
Instantly feeling terrible that I'd forced that wobble from pushing the limits and then attempted to stop her swing.  She quickly went from crying to 'No daddy I'm OK!! Look I'm smiling. Push me, I don't want to stop".

Crying down the hall.
Time for a rare night time 'Give Ren a break'.
Good husband Bad husband?