Saturday, December 28, 2013

Holiday Wave

Raqu has been very good for the most part during these weeks.
There was the day which was out of her control, where she limped quite badly and we decided to do a unscheduled dressing change.  There was a miss in the communication for what amount of background pain meds she was to have on board, as her bath takes exactly the required time for them to take hold.
It was a tough moment peeling back the visco paste bandage and the mushy purple balloon pushed the material unnaturally from her foot.  It covered about 80% of the top and instep of her foot.

After a couple seconds of picturing full on chaos and the obvious struggle moments away we decided to try the heavy artillery on her that had not been used since a Day Surgery visit at Children's Hospital.  I was nervous to try the 'Special K' as we are very aware of how strong the drug is.  I do admit for Raqu's sake it shouldn't have taken so long either as at least two other occasions surely required it.

After a 15 minutes wait I started the procedure of cutting at the large chunks of flaky skin on her somewhat good foot to test the stormy waters so to speak.

She was not impressed.  She kicked and squirmed despite moments later hallucinating that she was catching stars just above her head and giving them to us.
Once all was said and done she didn't shudder in pain and she wasn't bitter at me for making her suffer so it was a success for the most part.

We reduced the dose by 15% than what we'd agreed in the Hospital and I learned the soft way I should do as suggested as it was still not an ideal DC, considering she's to have many many many more of these.  The good part was that within the hour she was back to herself bouncing around regardless of how her feet looked or felt beneath the bandaging.

The next day (Christmas Eve) was rather interesting as we shared our dressing procedures with a new family still in hospital with a baby that appears to have EB.  Unfortunately for them our EB reality switch wasn't set to mute as few hard subjects came up that went a little too far for our new friends.

Ren walked into the middle of a conversation which was only intended as a note on Herlitz Junctional, but Ren went a little more into depth.
Coco hearing this went on even further despite me telling both of them to stop talking or change the subject.  Yet they went on and explored death and poor Tripp.  I was a little enraged at them for that moment, but knew it was my fault for bridging the subject in the first place.  I tried to keep my head down and let the tears well and fall from the young couple as undisturbed as possible.

The poor family.
If having your life shattered by the potential EB BS on what should be the proudest life altering day and or weeks of their life wasn't enough, we are the ones to unwittingly show them an even darker side of this disease.
I guess better us than the internet???  I'm hoping anyway.

Christmas.
The best present given and received in our household was the one Coco got from Santa.


I've always been a very big fan of Roald Dahl.  So much so that if Raqu had come out a boy she'd have been Roald.  Believe it or not it wouldn't only be about my devotion to the writer genius, but the fun of listening to Ren's ESL and my reappearing speech impediment struggle over Roald for a lifetime. 

Anyway, I bought her Matilda.
I hadn't come across this book before as my childhood and ongoing pre-adulthood was only enlightened by Charlie and the Chocolate Factory, The Great Glass Elevator and the Fantastic Mr Fox among a great many of his short stories more in line for adults.

As I read the first line and paragraph aloud to Coco the world seemed to tip.
Everything about how I saw the world made sense in a sadistic masochistic humour that must have spoke to me throughout my life time by those other books.

'It's a funny thing about mothers and fathers.  Even when their own child is the most disgusting little blister you could ever imagine, they still think that he or she is wonderful.'

After reading a few chapters I couldn't wait for Coco to nod off so I could indulge and go much further and not wait painfully for another day or week for the next chapter and page.




Monday, December 16, 2013

More expletives

More Expletives?
Not me however.
Although, it does make for some self awareness when considering a few of my buddies whom say nary a coarse word around their children and when hearing their children talk or speak it's clear that the clean path is rather shiny.

Raqu and her sister have a big infatuation with Katy Parry.  Ren and I are forced into listening to her songs every day off VEVO sometimes over and over and depending on Raqu's state or requirement to not move.
For the most part I now know nearly every chorus and many words and can keep up with the kids singing on a few of the songs for a moment or two.

I've always had a thing that most songs are more about a feeling and rhythms for me than the message.
I could never recite the lyrics to many songs I've listened to 100's of times.  Then if I do it's certain I hear things much different than what is sung. 

Creating my own reality that usually makes no sense to what the song is about.  I probably listened to too many Bob Dylan songs in my teens to think anyone actually sang about anything.
Speaking to some French girl
Who says she knows me well
And I would send a message
To find out if she's talked
But the post office has been stolen
And the mailbox is locked

Raqu's moment of fame comes after Ren starts to sing-
You change your mind
Like a girl changes clothes.

Then Raqu excited to join in carries on.
Yeah, you, PMS
Like a bitch
I would know

Ren and I just stopped the world seemed to go on pause. 

Not sure if I think she's the coolest kid in the world or I'm the worst dad in the world..

Anyway
Fixing her foot wounds from last week.
They were getting a little stinky and she was suffering from the itch....
Below is a photo doc of before and after today's DC.
As tough as I think I am with her wounds and dealing with new and old ones.  This was still tough, cleaning this up and edging all the dead flesh. 

After 4 days it's great that she's heeled so well and the epithelial tissue is looking healthy and strong.






Sunday, December 15, 2013

Wednesday, December 11, 2013

A little bump

It was so long that we sailed along with tiny blips here and there.
Now it's one after another, yet her resolve is really making me so proud of her I can't describe.

In the last month we've had a week or so of choking, four or five days of eye issues, three days of smeared face wound and some troublesome care decisions, with the bar set somewhere around a coming dilatation of her esophagus.

The most recent smeared her face incident miraculously healed in record time but for the strange piece of skin that healed very taut just under her eye to the base of her nose.  After the second day when the swelling subsided and my biggest fear that the fresh wound at her eye lid would attach to the fresh wound at her cheek while she was sleeping didn't happen, we noticed that the one large patch of skin that had stretched across her face reattached and maybe, too well.

In my mind, I pictured my big thumb smudging at it so it would release and not pull between the periorbital and cheek to deform the area forever...  Gladly reason prevailed and I impatiently waited it out, but it still looks like it's deformed, subtle, but I see it.

By the end of a week exactly, her face was as bright and pale as she would have been previous this injury.  The problem is that she has since found a way to smear a couple other marks on each cheek.

Back to the irritating smaller blisters and wounds that I can handle as an EB specialist.
Yet each one, still extremely troubling as they add up.  It's that damn Dystrophic word attached to her subtype ringing relentlessly in my head spelling fear.

I was recently scolded for my use of expletives.  I'd very much like to use one now to release the frustrating and unbearable sadness that wells at times.  Just a two letter acronym?
So much pleasure.  So much I don't have to write when typed in a millisecond.

Anyway.

Yesterday we noticed her limping earlier in the day.  Ren was busy, I had to work, our nurse was not able to do a change without me and of course Raqu had a Dr. appointment at Childrens in early afternoon.  By the end of the day in the time before the nurse was to leave Raqu topped it off with a long and extended nap.  When she finally woke our minds were not into a change, as I soon slinked off to bed, but as Ren changed her into a clean pair of night silks, she notice some large wounds at her fingers.
Laying in bed I attempted to tune out the conscious world, yet Ren's voice kept breaking the peace with uneasy demands for my immediate presence.  It took some time to mentally prepare for what awaited and it was a total put off that I wasn't either on a boat or in a river, even it it was 12am...

Raqu was very docile, extremely cute and talkative when I got to the scene. In fact but for the moments shortly after she awoke in the morning due to eye pain she was a delight all day long.
I saw the tops of her fingers poking through the mangled dressings and in the light I could see right through her ballooned skin to make out the silhouette of her finger below.

I can't begin to estimate how long those bubbles were growing below.
Other than her eye issues that kept her in darkness and crying in the nights till we caught back up to her pain we couldn't tell she was in discomfort.

Yes, the danger of medication.

But as we were up and slicing balloons it seemed a perfect idea to do the full deal and check out her foot.  I touched and squeezed it to see where she might be in pain.  It felt squishy, but I wasn't sure it wasn't because her dressings were a running past due for a change or if my metal state was to be trusted.  She didn't wince, only that she pulled away and told me to take a hike. This is very much a typical reaction to the prodding and poking of "blister or not to blister"?

After we took off the top two layers it was clear that we were in for it.  Buoyed by the fact we were not at all prepared on the medication front, but we continued.

Raqu continues to amaze.

Ren did many of our distraction games with puppets and bubbles while I poked, cut and sliced at her feet and hands.  We certainly had moments where she shivered and shuddered in pain, but she'd push her head deep into Ren's neck and close her eyes clenched out the pain and found her way through it without the loud screaming fidgety fight I expected and have had to battle through for nearly the entirety of her life.

Clearly Mama rules and her nurturing presence key to many troublesome moments.  I don't have much of the nurturing aura due to how I'm pressed to do the most painful care actions on her skin. 

Fluid and blood splattered the floor and once we'd gone over her body in near record time.  Moments after she was clothed and settled she was out for a long quiet and comfortable sleep, for us all.  I can't even begin to count how many times my expectations of a situation with her EB has left me scratching my head.  But any extra sleep and ability to relax the brain I don't for a moment take for granted or waste time wondering.

Really, expletives get in the way of a wonderful family experience?
I jest and yes I'll try not to swear,
much....


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.
Whatever.
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

Meander

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.

video 

 
video


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.

Eh.

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?

Wednesday, September 25, 2013

Rome 2013

In my role as a director to Debra Canada I was part of the Canadian contingent participating in a yearly Debra Congress.  I have already made my way to two previous Congresses, but for the most part on my own accord and for reasons directly related to my role as a parent for a child with RDEB.  This time was quite different and the information I obtained strangely enough was more directed to my role as a Director of Debra Canada and as voice being able to guide our programs and research.

It is very difficult coming into a rare disease such as EB with so many complex issues that require large teams of medical specialists and that understanding the science behind it has been a steep upward climb with very little traction till this point.  It has been clear up until this congress that asking somewhat simple questions to those in the science field has lead to very little extra understanding.  Medical terminology coupled with a vocabulary that I've never been a part of leaves me often in silence with a glazed dumb look on my face.  Frustrated at an inability to comprehend what seems second nature to these people is very hard to accept.  I guess not so hard that I haven't had a medical dictionary open learning the communication skills to keep up.

This time however was slightly different.  I was able to befriend a Chief Scientific Officer of a drug development company whom was able to impart some building blocks for me to question and in a way have a valuable bullshit detector from what we are told and hear.  Data is king of sorts.  There were certainly moments that he too made me feel my incompetence, but once I exclaimed my glazed over eyes in the middle of one of his sentences in a self deprecating manner we seemed to have got on very well from that point and the more we talked the less I felt like the kid sitting on the bench while everyone else played the 'GAME'.

The talk of a cure and beneficial treatments are shot at us in every congress.  Yet, the reality of these treatments impacting my daughter's life are few and far between.  We must rely on the Best Practice Guides to be sure our medical professionals understand the nature of the disease in terms of it's life curve.

It certainly helps that I'm certain the more I learn and the more I see we've made many of the correct calls in Raqu's care.  I'm also very cognizant that much of what we do still needs to be questioned and reassessed so that complacency won't one day find us a corner.

As far as Debra goes in a very clear manner I've been able to narrow down a plethora of issues and ideas to a couple that I think are attainable as a small member organization. 

It's a wonderful feeling coming away with an empowerment by understanding what I know and what I need to know and the proper paths through it in areas that I can with Raqu and a few others to control and likely make a difference for the future.

At the outset of this trip there was a bit of an internal ethical battle of taking a valuable spot at this Congress that Debra Canada can somewhat afford, but I'm now confident I made the right decision to go and will be able to soon add benefit to our national chapter if not the rest.

By the way.  Rome is something special.  Other than Ren and my two girls I don't think I've ever fallen in love so quickly.






Tuesday, September 10, 2013

No drugs. No problem.

It's been a big battle keeping Raquel comfortable through her all the ordeals she's up against yet we seem to be pushing through a new milestone of sorts.

No Drugs Required.
ha.
No not really, she still gets something for that damned itch.

A little child of age 3 with skin so fragile a nick with a finger nail peels off skin filling your nail as if it were butter saying 'No' you aren't doing it right, 'use your nails'.
She say this during a struggle to keep her heel from hammering the top of her opposite foot in every attempt to kill the itch.

This killing would more accurately be described as breaking down the last months healing.  Soon to follow the limp, then the ugly scream fest of a dressing change, then the anger at me for cutting blisters and manipulating her feet and then return to the itch.

I'm not sure who, of us two has more issues with loss of control on her bandaging?

As I so desperately want to dig and remove every last scrap of sloughed skin and her so dead against me even looking.  For her I'm able to apply a big goopy layer of aquafor, but not take a moment to look, inspect, ponder and touch in any way that is not directly related to me putting the bandages right back on.
TURKEY!!!

Some days of late have ended in a stand off, I've been able to do the bare minimum and she's stopped me from digging and the discomfort it creates.
Unfortunately it's the battle for her digits that I'm not in any way interested in losing.
Yet her toes are going going.....

No Drugs Required, right I was on track from something.  Got side tracked.

It's been a small grade down to today where she's had none.  We've done this before and I've regretted it, but she wasn't like she is now.  She has an awareness that I don't think I saw in her before and the start of her ability to push through pain and go for it what ever it is.

We walked out to a beach near here.
There are two dykes built up.
One to contain and push Vancouver's sewage way out into the ocean and the other to contain the great Frazer River's north arm.  The one that's the poop shoot is paved and has a parking lot full of people wandering it's high thin banks to it's terminus and back with the constant deep thuderous roar of jets making their way across the planet.  The other has a gate not stroller friendly, nor a path stroller friendly or a few hundred meters of river and ocean debris of piled boats and logs very view friendly, but once one breaks through, it's sand and stunning beauty.

After a KM or so pulling a stroller backward through soft sand with a lump of sleeping child we came to an area open and interesting enough that I could fish and the kids could play and Ren could, well not be concerned or irritated I was fishing.
Once the turkey woke it was everything 1 year ago we'd be losing our minds over.  Raquel playing in the sand making castles and so effective was she that her older sister had to join in.  In any event she was determined to climb on logs and balance 3' up from certain EB nightmares and she had a blast.  She didn't care her knees were crusty and raw or her feet either.  Only that she was part of the gang and making all of it.
Her typical investigative squat.  We're always on her not to kneel.

The castle before Coco discovered it.

haha you mac people won't be able to see this video.  Too bad, so sad.

video


My usual ten finger salute.  'get lost dad'

Tuesday, August 13, 2013

Animal Trainer

As reference to my last blog, I've had a few people giggle and suggest I might soon get my head eaten off by a lioness whom I may have pissed off.  Seeing eye to eye is something that is either just that or something like two magnets at odds or eye to navel. Who's navel is at what height is the question and obviously subjective.
Anyway, a psychologist has suggested to Ren to read the book 123 Magic. I think in relation to Coco's fear of death, which is nearly in hysteria. She'll ask questions and as they lead to her fear the only answer I can give is death and of course she then breaks down and starts screaming and crying. I've had to lie a few times, especially when asking about hypothermia and the sailing classes she doesn't want to goto, since there are only boys.
Ren came to me and read a passage from the book since my only reading is either architectural code, world news or fiction.  
Grownups who hold fast to the Little Adult Assumption will tend to rely on words and reasons when dealing with young kids.  Talking and explaining certainly have a place in raising young children, but kids are just kids - not little adults.  "Childhood is a period of transitory psychosis." Kids are basically bat shit nutty and to deal / cope as a parent requires a kind of dictatorship. The writer goes on to explain to break with the Little Adult Assumption try "cognitive shock therapy".  Imagine the little people as ummm, little lions or monsters making yourself the wild animal trainer.

I do see later in book (skimming) my stern zero to Mr Hyde isn't the way either.  But I was elated to see that democracy and council for youngsters hasn't become the new normal. 
Somewhere in there I'm likely looking at someone's naval. 
But
W
E

Kids are kids.  Till they can cook, clean, do laundry and be seemingly independent a democratic parent I will not be, especially when Raqu must have painful sores manipulated and cleaned near daily.  

Sucks to be my kids I guess.

Raqu had a bad day with the cornea.  Lost of crying.  Blood blister under tongue last night and one in esophagus too.  As she crushed and coughed it out she tongued the one at edge of lip.  Soo much fun needling children,  hahaha and parents too.


Friday, August 9, 2013

Lost the MOJO

Haven't been into writing or relaying the exploits of my littlest Princess at all.

It's summer and there are plenty of things to do outside being indoors with computer, outside of designing houses and arguing with city planners for the sake of a client and good design.

But yes, 'NO' news is 'GOOD' news.

Raquel is at her best right now.  Very little in terms of pain.  In fact we've not given her baseline pain dosing on many occasions as she's either becoming able to manage her own pain thresholds or who knows?
During our last dressing change, a nurse and I were cutting a ballon on her heel 2"x1" and about a half inch bulging.  After snipping it and watching the splash of fluid hit the floor and warning nurse to hold on to Raqu's appendages as I squeezed out the remaining fluid.  Raqu just looked at us and smiled a coy grin and said "what" with upturned hands.  A funny little gesture and questioning she's been doing a lot of lately, when we look surprised at something she's done.  Like an old wise person is locked deep inside that little girl and appears for a moment to explain, 'why shouldn't this have happened', 'why can't I be so clever', or 'what's the big deal'?

Her moods and determination are stronger by the week .  She's argumentative and will stand her ground for as long as it takes.  Which usually ends up as a long 'time out' for her.  Coco never had such outbursts or defiance that required us to stand in and lay down the law in order to step her back to reality.

I'm not say'n, but she does have a buddy that pushes the grounds, some in which I've not seen eye to eye with her parents.  There are certainly some carry over issues when time spent together is beyond an evening here or there.  I do admit she's a handful to begin with, but the entitlement that seeing her buddy go to places we're not at all happy with is something I worry about to an end I haven't quite placed or decided on.

We have another pain meet tomorrow which we'll try Raqu's meds so that we can get at her feet when needed.  Except, her feet are near perfect at the moment, well less the recent one on her heel.  I wish these invasive hospital meets could be arranged when we're in that suffering stage. But we don't have a 'pain team' of sorts at moment, so unfortunately not an option if it ever was.

Today while taking Coco to Playland and doing our best not to let Raqu in on what we were up to, so to avoid a melt down of being left out, yet again.  It's likely that if we didn't have a new nurse training we would have attempted to bring her along for the kiddy rides.

Anyway, I found out how OLD I am and that I have to retire from the twirly rides forever!!!!
All I'll say is that Coco was lucky she choose the inside seat on the swinging chair that took us up 100' spinning in a whirling dervish after we did the 'Corkscrew'.  I'm sorry it wasn't rain but corn and chicken that sprinkled those in the line waiting for a seat.  I'm also sorry that I was the reason the ride was shut down for 20 minutes as the young students summer employment extended to cleaning up for an old guy no longer with a strong constitution.
Poor Coco.  She got all of five rides before her dad had to be chauffeured home as I was unable to drive and 5 hours later still think it's not a good idea.
What was really funny was explaining to Andrew who ditched his work to rescue his puke covered, white faced, quivering buddy and upset and deprived niece was the part where I explained in full
detail about trying in vain to swallow my own vomit several times from splattering a larger area of attraction goers while under great spinning pressure.  In near hysterical crying laughter I watched him instantly start to gag and nearly filling his own car dash board on the way home.  Despite how weak I felt I don't think I've laughed that hard in years.
Gross?
Yes I know.
But who writes this anyway?





Raqu found lipstick after I pulled it out of Ren's seldom used kit to put a red dot on Coco's forehead. I was surprised she knew what it was and decided to look pretty all on her own.  Oh yeah, there was also a moment a while back where it was much less funny when Raqu and her buddy painted their faces purple like Mel Gibson in Braveheart while unsupervised for 10 mins with Coco's hair chalk. At first sight of her smiling proud face I had thought her whole face was going to shortly and eventually blister and smudge off.  There was a picture taken at that moment, I nearly gave way to inner rage and crushed the camera as any sence of fun and play against losing all the skin on her face needed to be understood by all.  But somehow I remained within myself and quietly waited to see if the biggest baddest blister of nightmarish EB horror would come to pass. Worse, was we were an 1 1/2hrs from civilization should she have been wrapped like a mummy.  Lucky, I didn't freak.

Raqu and her buddy A.KA. 'The Fang' at Raqu's BDay.

Showing off her new gloves and ability to point and show numbers.
Raqu being Santa
 Swim in the lake. I'm always surprised by how sturdy her physique is.

Tuesday, July 9, 2013

THREE YEARS OLD

Crazy.  Some say time flies.
But somehow the last three years have felt more like a decade.  Gray hair that was coming in one by one has come in swaths.  I'm too young at mind to look distinguished!!!!! (Some say immature).

This morning with a happy smile, Raqu standing at the corner of her crib says after calling for help says, "It's my birftday".
Yes it is.
Raqu is THREE.
The brightness in her eyes is evident and two weeks of being sick with a cough and snotty nose, fluid and crusties at her eyes is almost at an end.
Ren finally home after three weeks walked into a germ infected household and her plunge into the cold, dizzy, snot fest has just begun.
Some how Coco just maintained only a small cough through out.  Must be what three years of breast feeding does as that kid rarely gets sick and when she does it lasts very briefly.

Anyway, Raqu is three and becoming a beautiful, bright, stubborn and intelligent little girl.
She's loved and is thriving despite what she's been offered by her parents screwed up mixing of faulty genes.

Simona sent these from a little party made for her yesterday after our plastics session at children's, where we showed off her hands and questioned again what to do with her webbed and mangled feet.  (nothing)....
As suggested the bandaging regime that we've followed with keeping her mitted with splinting of viscopaste has only shown in the three years a 3mm webbing at one space on her right hand between pinky and ring finger.  The rest is great, but for her feet which is a losing battle if I've seen one.

Here's the princess.





I've never been much for birthdays and will not go out of my way for a third.  Coco on the otherhand at a coming 8 won't let that happen, but Ren made sure to give Raqu a present early.  She pulled out a Mahogany trike I made Coco for around this age.
With hard angled pointy parts and the trike being closer to a run about bike in stability isn't what a kid slightly too small child with EB should be playing with, unsupervised.
Anyway the result after a little crash when Ren walked to the door away from Raqu.
It was difficult not to turn into raging bull when I saw the crash and heard the cries of Ren looking at the smudged skin.  Thinking to myself.  Good blister or bad blister.  Then realizing that wasn't the question I should have going on at an internal dialogue.  In fact the best course of action was none and that I should at every cost not talk about it with Ren if can be helped.
Well. 
Is showing it off the the world a little passive aggressive?

Wednesday, June 19, 2013

Maintain the level.

After the dressing change ordeal the following day was up and down.  I think our newish new nurse doesn't fully understand the brunt of Raquel's drug requirements when her body is sore and what is required to maintain a level where she's not stoned or in pain.  This likely my fault for not laying out exact doses for day as I disappear for work for large stretches of day.
So when the drugs wore off and SH*& it the fan it was big time catch up and the stress levels and tension in her voice were alarming.  Then a few of the background issues that don't seem to be taken care of were pressed upon.

Such as Raquel's g-tube site, which has had ooze and blood coming from it.
I exclaimed it's been like that for a while. 
Weeks? 
Yeah maybe.
Should we do something about it?
Hmmmm.  What does that mean I think to myself?  Another hospital visit? ER? hahaha

I did mentioned it to a nurse at Children's the other day if they could find someone to come look at it.  She asked who was the Doctor responsible for it.  I gave the GI guy, but that wasn't who she wanted or thought should see it.  In the chaos that was a 4 hr dressing change of suffering by all, she stood in the doorway asking who??  I think I ignored her or was more focused on Raquel.  She disappared then as soon as we got Raquel's final hand wrapped so did we. 

We've been given silver nitrate or told to soak it to submission with saline.  Neither works as soaking requires her to lay still in middle of day when nursing help is on.
The silver nitrate is used to burn it off.  That being another one of the evil things I do to her which requires two people to hold her down while I dab basically a flame at her innards pushing the feeding port out...  I've been told it's not supposed to hurt unless we get it on her skin.  clearly that's not the case.  There always seems to be a tender part of it that makes her squirm and cry.  More of the bad daddy.  So the burning of her granulation tissue gets overlooked when possible.

Excuses.

Don't misunderstand any of this or from the other day.  I don't blame anyone for any misstep or moment of chaos we're just moving through the best we can and we are all learning.  I don't even think we're doing a bad job even as some things slip by that would make me shudder in fear should Coco have any of these issues, being of full of collagen 7.

I hate excuses.
Yes it's bloody.
Oh well... EB SUCKS.

But then last night, when Raquel's suffering was eliminated the three of us played our rambunctious nightly game of monsters under the covers at their usual 9:30 to 10pm bed time. 

Everything was perfect.

She was doing her somersaults and her backward free falls into the down-blankets.  The kind where team builders use as trust, but Raquel is more interested in stepping off and rolling the dice that Coco and I are by chance watching her and ready to be out of the way and ready to catch her should she have not lined up where the bed was behind her.

Last night she caught my knee on the bounce back of her blind daredevil maneuvers and sports a 25cent skin smudge in the middle of her forehead.
Oh well.  eb sucks.

Good blister or bad blister?

Good I guess, as we just carried on once I applied a patch of mepilex across the length of her forehead.




Monday, June 17, 2013

The Good, Bad and Ugly.

Asking, does the irritating person.
'So, do you want the good news first or the bad?'

That moment in a perfect world, has the person holding the information for effect also holding a handful of chicklets. 

The Bad.
Ren has gone away away for family reasons that are tough and difficult to say the least.  We're holding down day 3 of 21.
The Ugly.
Day 3 of 21 saw a 5am wake-up, a difficultly consoled then eyes shut till shortly afternoon by the reigning short person in the family.
In the mean time I had my first Heritage Commission meeting discussing a year old project that's seen many turns, breakthroughs and fall backs to amble in to a meeting of make or break it.

Ummm, can you spell stress?

It didn't break, so on we go!!!

Then a nurse and my mother with Raquel met me at Children's Hospital within the hour for a consultation that was to give us caregivers the upper hand at dressing changes when her feet in particular need special attention.

It was a bloodbath...

It kind of went like this.

Deep breath.

Wait for Big M to take hold.
Remove dressings on hands so she can play and be distracted while we go to feet where I could finally get at some really ugly areas under her toes trimmed and tidy.  So that, in my estimation giving her a chance at good balance and walking pleasure should an eventual CURE or not come our way - bad IDEA!!!
As distracted, she was.  But to at any cost not let us finish up her dressings anywhere on her body.
Start foot.
Nope, not cooperating.
DANGER...
Try hand instead of foot.
Nope not cooperating.
Wait a little for the drug to get in there some more....
Shit, she's got a blister on her palm from pushing around on bed while she battles us.
OK try bad foot as the bandage seemed squishy and likely had big blister.
Massive blister located.
Snip and the deluge, bloodying the bed...
Raqu majorly stoned and restless shuddering in pain while flopping all over.  Very difficult to contain.
Shit now she's nearly naked and out of control and how to minimize more blisters?
Do coverup on this foot and go to somewhere else on body to come back later when more drugs are on board.
Shit she's got another blister on her elbow from wrestling us over her hands or feet.
Fuck, who cares just get this kid back to her safe mode wrapped and bandaged....
Finally we finish a foot and one hand.
Dr. tries Fentenol.
Not really working in getting her to comply.

Takes time but she's still not giving in to let us finish her remaining hand.
Go back to bad foot snip more of fluid filled bloody balloon that gravity had previously pushed out of my sight. The foot still wasn't an easy bandage and there was still no way she was going to let me poke and prod at the giant scab, scar, pile of dead/living skin pulling her toes under in the EB nightmare that is Recessive Dystrophic.

A second Fentinol.
We finally do last hand with a minor wrestle and a little distraction, but still she's tough as nails and like her father, no sense of backing down when mind made up.

HOLY MOLY.

Back at home and it's 8 long after our day there and she's KO'd.
No wonder.
I was able to take a nap with her for an hour, but it was only one of us to rouse when her big sisters foot steps and giggles came stomping in the house from her day of school and play.

The Good.
Fathers day was bliss.
Took my mom and two girls sailing on Das Boot for five hours and the only (I realize now where it came from) issue was Cordelia kneeling on Raquel's foot while climbing around in the spacious cabin.  Raquel spent all the good windy bits where mom's eyes were soccer balls as a nice little breeze shuddered the little boat and rocketed us up wind to home sleeping upfront and in the shade.  Probably the only time I was content our saggy sails were closer to sacs than the carbon sails I'm used to seeing on Mpower (Farr30) while racing with the A-team.

Ready?

Waiting for wind.  Raquel sleeping in the cab.

shhhhhh! don't tell Andrew how messy a sailor I am when he's not there to observe.

Raquel not taking no for an answer about helming the boat.

My knees look a lot like Raquel's when my mountain bike works

Indian Arm.



Sunday, June 2, 2013

The hockey proverb

Lots going on.
Raquel is clearly becoming herself, between the moments she's attempting to off her sister by which ever method readily available. We often come running into a room with a child under duress and it's mostly Coco pinned in a compromising position with Raqu pounding at her. The terrible part, if that's not enough is that if Coco moves Raqu will likely be the one with huge wounds. It's surprising despite being beaten, she understands the catch 22. Is it she fears me or for her sisters well being?

We went to a little bday party and our friends made sure Raqu had her own private bouncy gym. The other kids had a huge air jungle gym which was an EB kids parent worst nightmare. 10 kids laughing and screaming running and bouncing about. Every few minutes the odd kid comes out crying because of this or that. Or then there was the evil child (not Raquel at this juncture) chucking the small plastic balls directly at the face of whomever, eventually hitting the right mark setting off a mini war of maleness. At that point one can only shake their head and contemplate perpetual world suffering. Well I guess one could do more as I contemplated and watched the bigger kid take aim and release with great precision time after time, it was a different parent instantly prompted with the need to break in on the violence and eventual tears.
Apple falling far from the tree, you might ask?
But as Raqu bounced in her little space landing on her bum yelling daddy watch this, as she further tested her agility and tumbled with joyous abandon, it was easy to smile and let her be free.
Hmmmm, as usual after some time it was clear her feet were sore. A little later on she refused to walk and hung off either Ren or I till we departed. The experience fell under 'worth while' despite potential pain and suffering. But as usual, time will tell how 'worth while' it actually was.

It's 2am and Raqu is on my lap after she was pulled from her bed screaming. Every few minutes as I type on her iPad her leg twitches or is kicked with some sort of suffering.

Once home we did a quick peek and of course there was a blister of various types developing on each foot.

The interesting one started off as a thin faint outline of red at the top of her arch. It was hard to initially decide if it was a colouration of an old one or something brand new. I gave it a quick EB safe rub with my thumb.

and Gasp..

It was shocking as before my eyes it started to grow and fill with blood. In seconds it was thick and worm like then started to push her seemingly healthy epidermis from the dermis.
Then the next struggle erupted between Raqu and I, as when I have a needle or scissor pointed to the demise of a blister on her foot.

I can't exclaim how effffing annoying it is for me to have only morphine in my arsenal to keep her calm in these instances.
I'm always sure she has enough on board to take away the shivering edge, as air hits large raw patches of flesh.
There is nothing for the anxiety even though we've had a meet with Dr.s and have solutions. The anger she must have for me after a needle's deep poke which missed the mark due to her ever stronger fidgets and or writhing.

But then there are worse things going on here.

Like the long emblazoned refrain in many a Canadian household. 'Keep your head up and your stick on the ice'.

Funny, the meat heads of the world had a deep thought or two.

Don't pout keep your head in the game.

Beware at all times for tragedy or opportunity.

These options best apply to this team as we jump over the boards from our slumbers to attend a suffering kid or our day once the nights have come and gone.


Thursday, May 23, 2013

Raquel's body 05/23/13

Webbed middle toes.  Her skin quite translucent and in bright light it almost looks like I can see through the onetime space.

This one in particular is very itchy for Raquel.  And she rubbed it with other foot and I did emergency change to realize the blood had transferred from other foot. It's clear here how the webbing takes place as under the toes becomes bridged to pad as toes are pulled and contorted by the pressure of tightening skin and scar tissue.
When we see her limping about it's either a bad one here or bottom.  A few posts ago it was both and she refused me to help. Imagine this, after a weekend hike in new boots.


What happens when wounds aren't managed promptly? The fluid sags and releases the epidermis, slumping further till we get scissors to cut away at edges to stop gravities evil work on her skin.  Red areas are scissor cuts.

Where I dropped her.  Amazing wound recovery.


Saw this for first time today, after what I heard was a scooter accident. Wasn't impressed that a scooter accident happened in the first place but it made me feel sick and terribly sad. 

I'm still extremely proud of her hands. Even though this happens once and a while.

Battered knees at 3 years old.  Milia are the pimple typed deposits.

The perfect foot.

 Perfect, except for the fact the big toe is being pulled under by scarring and the index getting pushed up and over.  Raquel and I battle relentlessly till I usually give up in fear of ripping skin off her foot or leg trying to hold her still while I cut at the thick scar tissue building up and pulling the toe under.  She's been getting her hands in the way when I'm about to cut fending me off with vigor.  An accident is going to happen!!  I can't wait for some M&M power!!!! (yes pain clinic - hear me)