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.

Friday, November 21, 2014

So good

It's really been an amazing run and it keeps going.
Yes of course the blisters are more and cover new and greater areas on her body than when a baby, but all the things we had to work out with her basic needs and the philosophizing if this decision was correct or that has really gone by the way side.
Raquel is in amazing spirits and health.  She has a spark that was evident early and hasn't gone away.

I let nurses do dressings sometimes twice a week. GASP>...

There simply isn't much to report other than she's a pain in the ass for being too much like me.

Friday, September 5, 2014

Summer time rolling

It's easy to tell when things are going very well for us.
Raquel has been so good and we've not had any kind of health concern outside the regular blistering that is and will always be her life.  Feet, face, knees, elbows, and the odd finger and arm print on her torso from a miss placed poorly timed interaction with a wiggly hell bent child on her own agenda.

Yes the attitude from this one keeps climbing.  Still not a fan of her dad too much, unless we are isolated from her mommy for any predetermined time span.

We recently spent a good time at our family cabin and the first while Raquel piled on the blisters and due to letting her have walks deep into the lake almost nothing healed.  Once it was obvious untreated lake water was the issue despite still having her bi-daily dressing change with chlorine/salt bath we nixed her deep water play time.  Obviously , she was not a fan of this decision as her sister and cousin were basically in the lake at all times and the reindeer in her made for a few cries in protest.  We'd often catch the top of her head sneaking under our view from the deck, as she headed down to the lake on her own.  As soon as you'd call her name she'd do her version of a sprint down the EB hazardous path to her favorite place of blazing sun, gritty sticky sand, sharp uneven rocks, bacteria filled water and everything semi cautious parents of someone so fragile fear most.

Of course for the most part we let her go and do as she needed.  The laundry and attention to her detail was quite intense at times, but when she had buddies staying with us, we'd often forget she wasn't just as the others and often let her out of our sight for who knows how long (?).  In the end it was very rare we heard screams from a fall or other and maybe she had less problems then any other kid there.  As one received a poorly thrown rock in the temple, another found playing with the sharp end of a knife, another stabbed by the flaming end of a marshmallow.

All the true fun and games for kids at cabin and what life is all about.

In fact I was the only one to make the long trip to the town's ER for a tetanus shot for stepping on a 5" rusty nail.  So creepy having enough conscious time to actually visualize the nail puncturing through the three layers of my runner before entering my foot just back of the pad of my big toe.  After stubbing almost all my toes at one point walking around in bare feet it was kind of nice that for a week or so I had no feeling in that toe, especially since I was limping and in most cases less stable than my littlest princess.

My little phischorre - Twice Raqu and I were alone for a few days.  Only time she was into whatever I suggested.

Coco and cousin enjoying an evening swim at sunset. 
I keep hearing from other parents with older children with RDEB that she's young, she won't be that healthy forever.  Enjoy it while you can.
HA!  Yes we are and we are going to keep this ball rolling with every bit of focus and energy we can funnel her way.

Wednesday, May 28, 2014

An EB Normal

Life is good.
Raquel is holding steady.
We've cut most of the issues that might arise with getting blisters outside of sticking her in a bubble and it's working.  There are still giant blisters that envelop most of her foot once every week or so.
It's literally the moment it heals that it falls apart again.  It's almost to point where we might think of doing daily dressing changes.
Problem with that is we'd have to do daily dressing changes.
I like the days off I get from seeing and dealing with all that crap. 
Currently we have nurses that are doing great in taking away up to two days a week, but still it works out that there is no one else around to do second and I have to be there.  I shouldn't whine, I know many families that do once a days for 20 years. 

The JOY we must look forward to.

Raquel has totally gotten a hold of her pain within for the most part.  She knows how to cope and it's amazing.  Of course there are times during changes and the blisters are cut and the air gets in or the zinc paste that make her little body shudder.  It's just all the other time when awake that she is very good at either hiding that she's in pain or getting past it.

Her personality is all of her own now and that there is no doubt.  If she sees weakness or indecision by anyone in her care or her daily planning she's all over it and does everything in her developing brain to manipulate the situation for her advantage.  This is with everything from the moment she wakes till the time she goes to bed.

Thankfully we have a couple nurses that have been around for so long now that in their company she tries little of it or gets away with none of it. 

During the last dressing change I taught her how to do a finger war.  Was I ever in fits of giggles as her will and tiny fingers would accept nothing but total domination of my larger than normal man hands.  Yes we practised safe war and of course my hands were lathered in Aquafor and zinc as was hers.  Hopefully we can continue this little game long long into the future.
I kind of doubt it, but I'll keep the dream alive.
Not really sure why she's allowed on our friends island and gets whip cream every time we go over. Notice her knee pads.  We started that from moment she's awake till after she sleeps and her knees have been amazing!  They are wrapped with conform between her silks and pants.

She's getting big and looking less alien more human

We're painting on zinc nails before rewrap

What's a blog post without some gore

Friday, April 25, 2014

EB kick in the %$&s

Ren and I have been thinking a lot lately of Hannah.
A girl who is linked at side bar that recently lost her life to EB and all it's complications and issues.
Losing a child at 11 it's eff'n scary.  Coco is 8!
I can't imagine or at least I'm trying not to. 

At the moment Raquel is doing very well.  She still of course has all her typical issues with eyes, blisters and everything, but she's super healthy, growing with a robust frame.  I often giggle with mixed feelings as I watch her -she kinda runs around like her dad - more of a rhino than the gazelle her sister is shaping up to be.  Cool to see a mini me, but then she's a girl - so sorry Raqu.

Raqu's school time has been great, but at home not enough is rubbing off.  She's ever more demanding and in some cases takes it out on nurses, then at increasing frequency attempts it with Ren and I.  When her attitude that the world was born to her servitude conflicts with her engagement with me, Raqu tends to very quickly be alone, in a room protesting in screams. 

I have always said, she's got the fight and personality strong enough to handle this disease, but dammit we still have to live with her day to day.

Couldn't she be just a little tamer at times?

Raqu in class- notice Grandma's sewing and fluffy chair Raqu specific?
All is good, because of everyone and everything that is going well.
No need to vent.

Saturday, February 15, 2014


Not much action around here.  Raqu for the most part has been exceptional.
There are the occasional sleepless nights for one reason or another.  It seems we are well past the effectivity of her itch meds.  She wakes wildly crying that something hurts, but after a few moments and asking her what's up she admits that her foot is itchy.

No real problems anywhere else other then her attitude. 
She's a handful to say the least.  She knows what she wants and is starting to figure out the levels of manipulation required for getting her way.

Some nurses have great difficulty making any ground with her when she has her mind set.

We are fast approaching Bella's Ball up on Grouse Mountain.  Hopefully it goes without hitch.
West Jet and Hilton have teamed up for an impressive silent auction item.
Flight anywhere they fly and 10000 Hilton points.
That will be an impressive trip for the winning bid.

If anyone in the lowermainland wishes to volunteer email the contact link and we'll see what we can do.
Go to the Debra Canada website to donate.

Here's some of the good and bad of Raquel of late.

Probably from when we were jumping on couch

After bath, getting ready to put the bandages back on
Raquel learning how to tell time
Ice cream - let mommy alone