DANCE WITH THE DEVIL part 3

As it turns out, a little thing happened between our clinic visit and Dr. Cowboy's request for the CAT scans.

Her name is Veronica.

Yeah, as if three rambunctious kids, a too big dog and a husband wasn't enough insanity, I was now pregnant.  Pregnant and careening towards 40.  Holy shit.   And the pregnancy was horrible in case you wanna know.  I was in and out of the hospital, on and off bedrest, and not at all sure that Little V as she has come to be called would make it.  But she did.  And she was/is perfect in every which way.  She would arrive some two months early, about the same time as her two oldest siblings would have their CAT scans and she would live her first month and a half in the NICU right up until those CAT scan results would come back.  There was so much going on for us as a family that I don't remember much only that it was chaotic as all get out, we were separated with me staying in Cordoba with Little V and Joe trying to manage the rest back in the pueblo and I had missed my very first clinic visit in four years.

I was busy feeling sorry for myself, alone in my hospital room when Joe called. 
"They've got nothing.  Nothing.  He's shocked."

"Who?  What?   Huh?  What are you talking about?"  I was annoyed.  Why wasn't he visiting with the kids more often?  And I needed food, real food.  By real food of course I meant chocolate.  The hospital had  fast become a prison and I was losing it.

"Cowboy has the results.  The CAT scan came back clean..."

I heard nothing after that.  Nothing but me trying to catch short, gasping breaths between sobs. 

Soon after that phone call Little V and I were parolled released.  We went back home to the pueblo, I reassembled/disinfected the home that had housed the pack of wolves for the past two months and I waited anxiously for the next clinic visit, my chance to hear it from Cowboy himself.  In the meantime, we enjoyed a new treatment protocol:  nothing.

Upon reading the CAT scan results, Cowboy threw down the emergency brake on our crazy train.  If the kids were getting a hardcore daily dose of physical activity (running, biking, swimming...) he would count that as physiotherapy and we could eliminate our twice daily vest sessions so long as there were no respiratory issues going on.  If the kids had a cold or anything that sounded even remotely junky, we'd have our choice of throwing in a session with the vest or using a handheld device (Acapella or Flutter).  Once the weather took a turn towards winter (I still snicker when I say that here) and the kids were less active, we would do one treatment session a day (vest or handheld device) until spring showed up.  The Albuterol that the kids had been inhaling with every vest session was axed.  No more daily inhaled meds.  We would monitor this new action plan visit by visit and he made clear that he would reserve the right to amend it should  future lab results warrant it.   And so our dance with the devil had begun.

When clinic finally rolled around again, I was all over it.  This would be the day that I would finally get to sit face to face with Cowboy and get it straight from the source.  It had been fine to hear the instant replay from Joe but I needed to hear it from Cowboy for it to be real.  Just what had he seen on those CAT scan results?  What was he thinking?

He sat perhaps a little too comfortably in the rolling desk chair.  With a cock of his head and swivel of his hips, he leaned in.  "Que no hay nada.  Nada."  And just what was that supposed to mean?  How could there be nothing?  He pulled out up the results from the CAT scan and read off a laundry lists of No's.  

No bronchiecstasis.
No mucuous build up.
No sinus polyups.
No sinus drainage.
No ghosting.
No scarring.
No inflamation.
No-no-no-no-no.  There was just, just...nothing. 

He went on to tell me that the image that he had studied was not one of a cystic fibrosis patient.  Unsatisfied, I pushed him a little further.  "How can you say that?  What about the genetic side of all this?  The one that has told us that they have cystic fibrosis."

"I'm not saying that they don't have the genetics for the disease because they do.  What I'm saying is that for whatever the reason, they are completely asymptomatic.  It is quite possible that one of your husband's less common genes is acting as a corrector and functioning enough to get the job done. Take your son's case.  How old is he?  Six?  By this age we should expect to see something to link him to the disease; something beyond his genetic profile yet there is nothing."

It was overwhelming.  I couldn't speak.  And then the exclamation point,

"If it weren't for the genetic side of all of this, I would never believe that either one of your children had cystic fibrosis."

______________________________________________
And that's how we left it. 

We still go to clinic every three months.  The kids still get swabbed, cultured and pricked.  Levels are checked, cultures are run and we're more than aware that things could change down the road.  The main difference is that we have cut back on vest treatments to nothing in the good weather months when they're swimming and running their brains out to once a day in the off season.  Our plan is to purchase two Flutter devices so we can retire the vest at least for the time being.  While this turn of events has been nothing short of miraculous, I'd be lying if I told you that I've fully embraced it.  I worry a lot about the what-ifs, not just because I'm a mom but because that's how I've always been.  I want to enjoy every second of this reprieve but I don't want to be sailing down the river of denial either.  It's a fine line and I'm confused about which side of it to anchor into.  Who knows?  I'm beginning to think I'll be forever adrift between the two.  And that's okay, right?

At least for now, in light of it all, I'll try sleeping with one eye open instead of two.

DANCE WITH THE DEVIL part 2 of 3

Joe, like it or not, is not one to get his panties in a bunch over life's what-if moments like his crazy pickle wrangling wife.  My news was met with barely a raised eyebrow and a lackluster,"Yeah, we'll wanna look into that."  Annoying? Uhm, yeah. 

So we waited.  And waited.  And waited.  Remember, we're in socialized medicine land here so there was no speed dial to Dr. Cowboy with this epiphany.  At our next CF clinic, which happened to be a painful three months down the pipe, I put it right out on the table for the good doctor in my Lone Ranger (it's actually more Tonto than Lone Ranger) Spanish, "There'sthiswebsiteputtogetherbytheCFFandHopkinsthatsaysthekidsmightnothaveCFwhaddyathink?" 

Well, she just may have been
on to something.

And as he's so good at doing, Joe translated my babble.  "What we mean is that there have been some recent publications specific to the kids' mutations and we've got some questions.  I guess we just want to be sure that we're on track with their treatment plan given this news."  Pretty generic, yes but probably better than the emotional babble that spewed out of my mouth. Cowboy listened, asked a bunch of questions that now, barely a year later I don't even remember and then said, "I'm glad you brought this up because I've been scratching my head over your kids for some time now.  To be honest, I've never seen anything like their cases before.  Here's what I want:  I want a CAT scan.  I want a CAT scan of each child.  I want to see beyond the collection of yearly x-rays and the lab reports.  I'll order it today but it won't be for a few months.  You'll be assigned an appointment time for later this summer.  Let's start from there and play our hand accordingly.  Until then, we keep our action plan as-is:  no change."

I nodded, wide eyed.  He didn't laugh at us or call us crazy.  He didn't hmph about us being in denial.  He listened.  And it appeared that he too was unsettled about our Cystic Fibrosis.  Maybe not as unsettled as I but unsettled enough to order a CAT scan for Charlie and another one for Lola. 

The can of worms had been officially opened; a lot would be riding on the results of the upcoming CAT scan, at least in my mind.  If it came back clean it might mean a whole new way of approaching and ultimately managing our CF.  If it came back showing evidence of the disease it would be reliving the diagmosis all over again, a chapter in our lives that I was none too keen to revisit.

My walk out to the parking lot that day was both a half ton lighter and a half ton heavier. 

Hmph. 

All in the same clinic visit.  

DANCE WITH THE DEVIL part 1 of 3

D0 you ever find yourself stopping dead in the middle of a conversation with a friend and having no clue as to a) what you were talking about or b) what you were going to say?  I do.  And sadly, it happens a lot.  I used to worry that it was early onset Alzheimer's now I just blame it on motherhood.  I also have a hard time sitting still and staying focussed on the task at hand which is precisely how this trainwreck of a blog reads.  One minute I'm hi-hoeing my way off to CF Clinic and the next I'm making an ass of myself in the pueblo.  So to set the record straight, Life in the Pickle Jar was born out of desperation.  Sorry, but there was no altruistic intent to connect with others who were dealing with Cystic Fibrosis.  If you've ever dared to wade through my early posts you'll see a woman on the verge of unravelling or hell, maybe I was unravelling.  Point is,  I was scared out of my mind; in a full-on blind panic, my mouth wide open and a scream so shrill only wild dogs could hear me.  It was a desperate place, a desperate time and this blog some way some how, became my outlet.

Then, in the midst of it all, there we were, packing up to get the hell outta Dodge.    We launched our little family across a big, blue ocean and started to settle into life all over again.   I  made a conscious decision to covet the 24th hour of the day for sleep, not worry and definitely not fear of the unknown.   And surprisingly,  the world didn't fall apart as I feared it might.  Actually, if you really wanna know, nothing happened.  NOTHING.   No cough.  No pseudomonas.  No funky cultures.  No phlegm.  No greasy stools.  No tummy aches. Nothing.  Piecing it all together, four years of nothing is a lot of, well, nothing. Don't get me wrong, we were still doing our vest treatments twice a day with the inhaled bronchiodialator.  And we made it CF clinic for our quarterly visits like clockwork.  But now I was starting to feel a little unsettled.   Why were the kids doing so well with this killer disease?   Were we missing something?  It weighed heavily on my mind but not enough for me to change anything.  I was just thankful that we were still flying under the CF radar.

So fast forward to last year when while surfing the internet as I usually do because there's only so much Spanish Wheel of Fortune a girl can take, I came across this link put out by the Cystic Fibrosis Foundation and John's Hopkins University.   It's basically a one-stop-shop database of hundreds of CF causing mutations.  Exciting, right?  Well for me it was.  Three of the four mutations that the kids have are pretty rare.  That, coupled with the fact that they have not two, but 4 CF causing mutations is like winning Publisher's Clearinghouse Sweepstakes.  Anyway, my interest was peaked so I started to dig up what I could on each of their mutations.  And not even five minutes later, my heart was in my throat. 

R74W
This mutation has varying consequences.
Some patients with this mutation, combined with another CF causing mutation, have CF.
Some patients with this mutation, combined with another CF causing mutation, do not have CF.

Come again, son?   Blah-blah-blah-blahblahblah-blah with this gene do NOT have Cystic Fibrosis.

Oh, but wait, it gets better.

D1270N
This mutation has varying consequences.
Some patients with this mutation, combined with another CF causing mutation, have CF.
Some patients with this mutation, combined with another CF causing mutation, do not have CF.

And they say lightning doesn't strike twice.

After a few hours worth of reading through the site...okay, that was bullshit on my part, it was more like 45 minutes of reading and 2 hours of walking around, biting my cuticles (yes, and I bite my nails too if you must know) and thinking Oh.My.God.  Oh.My.God until Joe got home.  Was it possible, was there even the slightest chance - or even just half a chance, that these two mutations, two of the four that I've sworn up and down and any which way that I'd annihilate if given the opportunity, weren't killing Charlie and Lola but saving them?   My mind was racing, yes.  But my heart...well my heart was ten million miles ahead of my head and picking up speed.

DEATH BY CHOCOLATE

While I'm workin' the espadrilles and throwin' down what are sure to look like some heavy duty gang signs, one thing's for sure: no matter how many ways I tie my scarf, I'll never be European. This fact evidenced by the purchase and consumption of one single chocolate bar.

After nearly three weeks of hospital food, I broke down yesterday and high tailed it to the supermercado nearest the hospital under the guise of post c-section rehabilitation. What I was really doing though was hunting for deodorant and chocolate, two such things one cannot live without postpartum. Ten minutes and two euros later I had my fix, one of those really big chocolate bars, not at all like the American jumbo sized candy bar but more like the 'could be a tennis racket' size.

I will admit to being more Snickers than I am Nestle or Hershey but being that there are no Snickers, Whatchamacallits or even Milky Ways on this side of the Atlantic, the Nestle brick would have to suffice. It all started with my roommate, a Euro-waif, who not even a week after giving birth is back to wearing her size zero jeans. I'm not kidding. Size ZERO. Anyway, there she was her scarf draped casually over one shoulder and her chocolate bar resting in her lap, the tin foil folded neatly back like you would a fine linen bed sheet. Carefully, and what appeared to be in slow motion, she snapped off two little pieces along the perforated line in the chocolate bar. Until this moment of watching her fingers break those chocolate pieces away from the mother-load, I had naively assumed that executives in the chocolate industry had designed those perforations specifically to aid in my consumption. They were, in my small world, tracks for my teeth to rest in, leverage for the task at hand. Any notion of opening a chocolate bar but not eating it whole was unfathomable, dare I say sacrilege. Yet there she was, in her chic Euro scarf and size zeros wrapping the remaining 34 million squares back up for Armageddon. That's just about when I decided that a year's worth of Spanish living owed me the same luxury and so I set off for the supermercado.

As fast as my swollen little ankles could carry me, I waddled on down the road, entered the store and made a beeline for the novelties aisle. I'll admit, the deodorant I threw in the basket was just a decoy, and a pretty lame one at that. The cashier, fully capable of calling my bluff, thankfully did not. I'd convinced myself that I too can enjoy chocolate in moderation, one, maybe two squares at a time thus making it last until well into the following week. You know where this is going, don't you. [sigh]

So what happened once back 'home' at the hospital? Yeah, you know what the fuck happened. I snap off my two pieces, wrap the remaining bar neatly back up and put it to bed in the mini fridge for safe keeping. Fingers trembling ever so slightly in anticipation of what's to come, I sit down and pop the first perfect little chunk into my mouth. Heaven. The second piece chases the first, a sweet fountain of milk chocolate all the way down the back of my throat, almost pornographic. This, the European Way, should satisfy me but of course it doesn't. Instead it's like teasing a lion with a chicken wing from KFC. Not ten minutes later and I'm up for another two pieces, wrap it back up, mentally chastise myself for the indulgence and put it back in the mini fridge. Ten more minutes go by, same thing. Two more squares gone. The fourth trip I just say fuck it and eat the whole damned thing.

Sadly, the punishment for my crime is not the absence of the Nestle bar but rather the roommate's Cadbury sitting untouched since the day before between the strawberry and vanilla yogurts on the top shelf of the fridge, his wrapper snug as he hisses disapproval in his haughty French accent because European disapproval always comes with a French accent, “Go on now, Fat American. No more chocolate for you!”

Yes, moderation it appears is proof positive that I'll never be European.

No matter how many different ways I tie my scarf.