Tuesday, December 15, 2015

Little Trucker

We've been enjoying life and getting along very well. 

I will qualify that by judging it against all the other kids and families I know with EB and how difficult it was mentally with Raqu in the early years.

There are still many days where the smallest piece of food lodges in her (AGAIN/ALREADY!) restricted esophagus and she hacks and chokes till she dislodges it.  Usually we have piles of saliva and blood mixed it with the minuscule food particles.
It's not like we feed her anything really.  Pieces of pancake/crepes (depending on continent of origin of parent) or processed turkey.  This is absolutely the extent of hard foods if you can call them that.

Her hands and feet have been very good.  I consistently cut away at her calluses anywhere they form on her body.  After a hot long bath is the only ticket.  I've watched a new add of this little contraption that sands or cuts off normal peoples thickened skin at hands and feet and wonder if that might be the ticket for her in some places that the skin is pulling back.  Might be my EB kit present from Santa if he reads this blog.

Still the same layers of visco paste at hands and feet.  This is prior to adding the toe flap, mepitel and conforming bandage.  Raquel didn't want me to photo so she kept blocking my shot with what ever she could find on the DC table.  As one can see at 5yr 5 months she's still got great dexterity on her hands even though I mitt them in layers of zinc paste bandage and conforming bandage.  Those remain her only fused digits however the big toes on both feet are losing the gap at a good rate.  She's probably got another few years before it's closed up.

Personality wise....  Raquel and I have been like two rams on a mountain top. Bashing heads till one falls off the cliff or submits....  It's rare that either wins.  It usually ends in stale mate.  Like Russia and US in the era of nuclear deterrent, big war of words and posturing.
She'll charge off to her room crying, with a big slam of the door to make the point everyone in the neighbourhood knows there's an earthquake coming and it's her wailing her case in tears till her dad, mom or whomever comes to her.  Sometimes she's just too angry to reason with we wait till she burns off all that agro energy and comes out normal.

Her school has been an incredible experience and she's handling full days of kindergarten quite well.  We miss days for eye issues or other EB things and usually miss diagnose normal maladies for EB and send her to school anyway.  The nurses watch and help keep her out of most trouble so that too has been great.  I can't imagine sending her alone or with a school aid in which our health care provider was hoping to do.  Crazy to think someone off the street is to needle mouth blisters, clean huge butt sores or fix large sloughs of skin from a slip of a fall while or at school or in transit.  So that four hours later when home they could be three times the size and all the issues Raquel requires trying to heal all that extra missing skin etc. etc.

This is from a few days ago.  She's telling daddy how strong she is and not to mess with her during a dressing change.  She can't stand me spending the 15 minutes I spend obsessing over her dry skin flaps and bumps every change.





We just came to 5 yr and 5 months where we started marking Cordelia's height on our wall.  Raquel is a couple inches shy of her sister's mark at the same age.  However, I still think Raquel has a tankish little form, good for bulldozing her way through life.  Might just be that big skull she has with that equally big brain.

One of my EB little heros- Anton is close to passing.  It's a sad day and one to reflect on EB.

If you have it in you to help try to support Debra Canada or your national EB association this holiday season, please do so.  As Vice President of Debra Canada I know we're all doing our part to make awareness, find a cure and everything else it takes to make these families and kids lives better.  Hopefully for our sake it's well before my little Raquel (same age as Anton) takes a turn.
 

Friday, August 7, 2015

Keeping on.

It's  been very good for a long while.

Yes, the typical blisters at feet, knees and elbows are weekly.  The monthly corneal abrasion sneaks up and shuts things down for a day or two.  Had a few big mouth blisters that haven't been seen since the early days.  But all in all Raquel has been extremely resilient to her EB suffering and her basic pain threshold is something to be in awe of.

The only big procedure has been a dilatation in June.  I sensed her esophagus was getting closed off, but because she eats almost no foods through her mouth it was difficult to tell the scope.  It turned out her opening was reduced to the same size as the first time we had the procedure.  I'm still amazed that after the few months preceding her first dilatation we were up nightly wondering if this would be her last.  As some of the choking sessions became extremely dire.  In this time around I think we had maybe two to four nights that were of any concern.  Never mind the days and weeks of minor choking episodes and spitting out her saliva for up to a week because she couldn't swallow, just not the prolonged choking and breathing issues of the last time.  I'm guessing this is mostly due to her being a pro at EB and not letting it get in the way of much.

I'm fairly certain because we've established stringent parameters to her play and daily interactions with kids, toys, adults, nurses we are where we are.  She wears bandages at hands and feet daily, knee pads and silks under her regular clothing with a blanket close behind for chairs, ground, travel and so far one of the main reasons she has been able to flourish.  I do have many nervous moments as she has only developed one pace.  Running!  Her style of it anyway and is jogging anytime she gets out of the house.

Ren and Coco have been away for the greater part of the summer and Raquel & I - Babi and the nurses have been the team in charge.

It has been amazing to have much of this one on one time as Raquel has finally come around to thinking that after all the pain and suffering that her daddy puts her through, he is still a good guy and maybe - that she loves him!  I honestly wasn't sure this would ever come.  Raquel has reverted a bit since Ren has returned from Europe to say a sad goodbye to her mother.
One of the few kisses I get from her that wasn't at the end of some intense bargaining.


Raquel and I have had a few amazing trips this summer without Ren and Coco, included are two camping trips for two days and a night each in a tent.  Both were managed extremely well.  The first, a trip by ferry and bicycle to an island camp site near here.  The second a sailing trip in a small 19' sail boat deep into our local fiord and camping at an old mill site with her cousin, uncle and aunt.

Raquel in her typical jog trying to track down her cousin

Raquel and super nurse Louise at Saltspring Island

Raquel and cousin loving the sailing

Home for a few nights.


Sealynn Bowl moments before we flipped the Chariot

I guess one of the not so great moments was entirely to do with me and taking her into the skateboard bowl in the chariot and after about 10 laps snaking back and forth I finally flipped it with her inside on my final exit from the bowl.  I got away with a small blister to her thigh and another to shoulder.  Man she was pissed at me.  She's become very expressive with a decent descriptive vocabulary.  Enough that she knows how to scold her dad for being a dummy then tell everyone in detail how I managed to crash her buggy.


Canoeing, well being blown into shore.  She carried that paddle around for two weeks before her wounds healed enough for me to allow her into the lake and go up and down beach in that boat.

Her hands are our greatest achievement in her care so far.  Her feet another matter and getting closer and closer by the wound from losing her toes.  MEh..  As one can see, we keep them covered wrapped and cared for to the ninth degree in hopes someone figures out how to get her some collagen and those hands can one day pick any and everything she wants without barriers and her daddies restrictions.