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.

THE DAY THE GENERAL CAME TO TOWN



Not unlike the rest of the CF world, we have our quarterly clinic visit etched in gold on the kitchen calendar that hangs off the side of the fridge.  It's like a date with the Pope; nothing gets in the way of it and come Hell or high water, Joe and I are both in attendance.  We've been at this for three years now.  Four visits a year times 3 years makes for a dozen trips down to the hospital to meet with our team.  It dawned on me a couple clinics back, that I have a ritual that has preceded every single clinic visit:  BitchFactor 10 - á la PicklePits of course.

The closest thing I can think of is a severe case of PMS.  You know, the kind when your husband throws out an innocent, "Gonna run today?"

And you bite his face off with, "You know, I really don't appreciate your snide comments about my ass, Mr. Man-Tits!  I've birthed THREE of your children.  I've earned these curves..."  and then you run in the bathroom, slam the door and cry your eyes out because your jeans are too tight (again).



STEP 1:  RADIOLOGY

Each x-ray is carefully

scrutinized for any 

changes from one year

to the next.  Changes

mark lung damage &/or

progression of disease. 

The effects of lung

damage in CF are

irreversible.



STEP 2:  LAB

Results from the annual

blood draw are used to

chart vitamin  levels. 
This data guides us in 

better understanding

the current status of
the kids' GI tract.   

So BF10 starts to brew about two weeks prior to clinic and always ends in much the same way:  a fit of tears.  Once the tears come and go, I'm good.  The storm pushes through and The General is back in town with her game face on in time to greet our team as they march through the revolving clinic door. We had a lot riding on this April clinic.  It would be our annual visit; the wide angle shot at the kids' health across the scope of the past year.   Scheduling the annual visit takes a bit of choreography since I like to get the x-rays and labs done in advance but as close as possible to our visit with the team.  It's my way way of feigning control in the face of so much uncertainty; a snapshot of the here & now so I can heave a sigh of relief knowing that we left no stone uncovered and our team is seeing the whole picture for what it is as opposed to what it was. 



STEP 3:  PFTs

Pulmonary Function Tests (PFT's)

are new for Charlie.  He does 

PFT's prior to each clinic visit

now.  PFT's measure how well

the lungs exhale.  We are working

with Charlie to help him in

perfecting his technique in hopes

of providing our team with the

most accurate picture possible

of his current lung function. 

 Yet there was one thing missing:  my meltdown.  I had kept it in check when the respiratory tech took a phone call in the middle of Charlie's PFT's.  I even threw a bone to the phlebotomist, smiling when she hit vein on the first try, as if that were even an option after the last time.  I was so busy chasing appointment times all over town that I hadn't had a second to fear, let alone think about what was looming. 

At exactly 7:48AM, twelve minutes before heading out the front door on our way downtown, the phone rang. The coordinating nurse from clinic was calling to announce the absence of two team members at our annual.  Would we like to reschedule?  Like a puss loaded zit begging to be popped, came the rumble of Mt. Vesuvius from within.  Of course I wanted to reschedule but guess what, I'd already taken the entire day off of work so it was a little bit late for that invitation, thankyouverymuch.  By the time we were out to the car, my heart was racing, my stomach was turning and I was seconds from blowing.  Poor Joe, he was about to be blindsided, oblivious as to what was about to hit him. 

"What in the hell are you doing?  The interstate is that way!  Oh no you're not!  There's no time for Caribou...Jesus, Joe.  Come on!  We've got Clinic in less than 30 minutes for God's sake.  How can you even think of coffee right now?" 

My husband didn't have a prayer.  The next 7 miles would be the longest of his life.  Kids in the back fighting for the remnants of the last blueberry bagel, Henry babbling nonsense at the traffic whizzing by and me unraveling in the passenger seat, tears streaming as he sped down I-80. 

Welcome to BitchFactor 10, my love.  Buckle up it's gonna be a bumpy ride.

I'm sure the 10 minute drive felt more like 10 hours for the poor guy; yet he took it like a man, a decaffienated man at that.  I guess after all these years together he's used to my neuroses.  Well that or he just knows better than to try to stop a train wreck with his bare hands.  By the time we got the car unloaded and stuffed our fivesome into the elevator, I was back in operational mode, just in time for the party.

Nurse

Pulmonolgist

Respiratory Therapist

Social Worker

Geneticist

Pharmacologist

As always, hindsight is 20/20 and in looking back, I see the elements that made up that perfect storm:

1. Charlie's PFT's had dipped since his last clinic and I was stressed out about it.  Was he growing a bug?  Were we going to see evidence of scarring on his chest x-rays? 
2. Vitamin levels at our last annual were a bit off - nothing to warrant a GI consult but something to keep an eye on.  With no dietician or GI present at this clinic visit who was going to interpret the lab results?  Leave the lungs to the lung doctor and the vitamins to the dietician.  How was I going to cover this base without her or our GI guy?
3. I hadn't had a good run in a while which meant I hadn't had a good cry either.  I usually have my preclinic breakdown between miles 3 and 5 of any given run.   So not having a good, long one messed that up big time.

Yet in spite of everything, it all worked out.  Our pulmonologist was pleased with both kids' xrays.  I leaned in (probably a little too) close over his shoulder and made him give me a personal tour of each lung.  No scarring.  No striations.  NO CHANGE.  I didn't believe him at first and pestered him for more detail, more proof that my kids were okay for now.  The smile, the reassurance; that something no one else could give me but him, not even my husband.  Those couple of minutes we spent pouring over the x-rays meant everything to me.  He chuckled saying, "Really, they look clean.  These are great, look at all that black, all that air space.  It's really good, I promise."  I reached for him, wanting to wrap my arms around his neck and just hug him tight.  But I didn't, my fingers left instead to just squeeze the cold plastic arm of his chair.

The pharmacologist read vitamin levels and concurred with the pulmonologist that all levels except Vitamin D were well within normal range; the vitamin D low most likely due to the dark winter months so more whole milk for now. 

Just prior to leaving some three hours later each kid gave up a nice juicy pflegm ball that would be our ticket out:  throat cultures.  We would get the call four days post visit that all was well.  No pseudomonas.  No B.Cepacia.  No funky bugs. I guess in the end that's all I wanted:  the assurance that comes with clean bill of health.  Isn't that what we all want for our kids? 

So I'm good now, good for the next three months. 
Until the next clinic visit.
Until The General is back in town.





TIT FOR TAT

It would appear that my daughter is much more like her mother than I would care to admit.  She has learned the art of dangling the carrot.  Not quite three yet full up to the top with the cunning ways of a 37 year old.  God, help me.  In spite of promises to never utter the telltale threats echoing throughout my childhood memories, I have ever so sadly done just that as evidenced by our most recent confrontation: 

ME:  Come on now, Lola.  A couple more bites, you're just about done.
LOLA:  One.
ME:  One?  Whaddya mean, one?
LOLA:  One bite.
ME:  No, not one.  Just a couple more.  Come on, let's go.  You don't wanna miss Dora, do you?


A lone middle finger stands erect
 mocking &counting simultaneously

LOLA:  ONE.



ME:  Listen here, Missy.  If you don't finish there will be NO Dora.



Pause.  She hedges her bets.



LOLA:  You not 'vited to my birthday party. 



Pause again. 
Her brow furrows and she glares at the spoon heaping with the next bite. 
And her slow, deliberate whisper taunts, 

No Dora cake for you.

SPRINGTIME IN PARIS

Yesterday was a first for me.  Against my better judgement and cloaked in my contraceptive nightware (sweatpants and a thermal long sleeved t-shirt), I called the kids into the master bedroom closet. 

"I need your help, guys.  I'm not sure what to wear to work tomorrow.  Whaddya think?"

Lola looked up at me bewildered as if  to say, "Clothes, dumbass.  Clothes,"  while Charlie ventured a bit more trepadatiously, "You mean we can pick it out?"

"Sure.  Why not?"

Lola eyed my shoe rack, eyes scrolling from summer to winter and back again as Charlie made a beeline for the pants hanging at eye level.  "Here.  These."  Nice.  My black fatties.  They hung too loose and I'd be tugging them up all day but they were far better than the alternative hanging two inches to the right.  Definitely dodged a bullet there.  Phew.  "And THAT one!"  A finger shot up to a silver sparkly top with a plunging criss cross neckline. 

"Ooooooo, " purred Lola, "I like shiny."

Conservative on the bottom and a little rock n roll on the top.  It's not like I had a choice at this point.  Besides, staying true to the rules of my Before 40 Bucket List, I would not try to sway the kids one way or another.  This was their choice.  I'd have to redeem myself at work another day.  "I like it Charlie.  Good choice.  But what about my feet?"

Charlie reached for my black pumps with the gray speck of buckle on the toe and I felt a wave of relief wash over me.  Apparently he had remembered these shoes as part of my standard "look" when wearing anything black.  That and I'm sure he was sick of the game and just wanted to get back to the pile of race cars strewn all over the living room floor.  I would go to work the next day having completed my first bucket list item and no one would be the wiser.

Well, Lola was all over that shit.  "No!  I want these."

So much for discretion.  She had selected a pair of brown strappy sandals with a wedge heel.  A mistake I had made three years ago and had forgotten to purge.  I briefly contemplated how to play this card as Charlie examined them.

Clunk. 

The pumps hit the closet floor with a hollow thud.  It was decided.  The brown sandals would be going to work with me in the morning. 

"You're gonna need some socks with those, Mama."

"Ya think so, Charlie?"  Oh boy.  Now I was starting to sweat.

"Oh yeah.  There's snow outside.  Your toes might get cold with these holes."  He wove his fingers through the straps of the open toe to emphasize his point.

"Ohhhhh, yeah.  I hadn't thought about that, good point." Shit.

He blew past me through the closet doorway and headed for the socks packed snuggly the bedroom's armoir.  Before I knew it a pair of gray ankle socks were thrust into my ribcage.  Thanking him, I managed two additional thank yous; one for the fact that he had pulled a pair with no holes and two that he had somehow, perhaps by Divine Intervention, missed the drawer full of white running socks.

And as fast as it had started, it was over.  My outfit for the the next day of work was laid out and ready to go.  The kids took off, content at having accomplished said chore so efficiently and I stood there taking inventory:

• 1 pair of black pants
• 1 silver, long sleeved shimmery top with a plunging neckline
• 1 pair of gray ankle socks
• 1 pair of strappy brown sandals on a wedge heel 

A smile crept past my lips.  And I remembered Paris, a city I have visited just four times in my lifetime and loved each time more than the previous.  The Parisian women with their silky scarves blowing behind them and their designer shoes clicking with delicate precision against the sidewalks crowded with foreign tourists in dirty tennis shoes. 

My foot.  My shoe.  My sock.
My Mission Complete.

Tomorrow I would be one of them - Parisian. 

Not in fashion but definitely, most definitely, in attitude.

Beauty and the Beast

Lola is by all counts, a Tomboy.  She'll take a dump truck over a tiara and happily sport a pair of Incredible Hulk underpants any day.  When we found out that we were having a girl I ran out and bought dozens of pretty pink dresses with matching tights and shoes.  And much to my dismay, they hang to this day, up in her bedroom closet like last week's special at the deli counter.  Untouched. 

But yesterday was different.  We had a turn.  A turn towards femininity as my diamond came out from the rough.  I pulled up in the driveway to a little girl clad in a matching pink short outfit and dripping with good taste.  As I cut the engine, I heard the hollow jingle jangle of 35 multicolored plastic bracelets slide down to her elbow when she threw up her arms to greet me.  A crooked bow pinned down a chunk of hair unwilling to be tamed and a gangle of baubles dribbled down her chest, each one tangled in the next.

My Tomboy now a Beauty.

Clinic, and by that I mean CF Clinic, was just two days away.  We had to race over to the doctor's office to get her chest xray and labs drawn so that they would be ready and waiting on Wednesday for the Good Doctor.  I let experience park the car in front of radiology and we practiced saying "Cheese" as we strolled through the parking lot - me with a purse full of medical orders and she with a purse full of Matchbox cars.  The lab would take the backseat.  I knew better this time.


Jingle, jangle
Jingle, jangle
Jingle, jangle


We made our way to the waiting room and sat alongside the grimmacing woman in the foot cast; Lola accessorizing her right, then her left, then her right arm again and again as the clock's giant red second hand swept around and around.  I worried that they wouldn't get a good, clear image of her lungs.  And that she'd be pissed about having to take off her "jewelry" for the shot.  Lucky for me, the technician was a mom too and knew just how to stroke Lola's budding fashionista ego.


"Love the snazzy jewelry you have there lil' lady," she cooed.  "Wanna make me look pretty too?"
Hook, line and sinker, Beauty took the bait.  We got the lateral then frontal images in one try.  Next stop:  THE LAB.


I dread going to this place, not because I fear needles but rather the people wielding them.  I rank phlebotomist right up there with "roadkill removal crew" and can't imagine doing that grind day in and day out.  Add to it the nightmare that is taking a small child into this place and you feel my pain.


"It'll be okay honey, they just need to get a little sample from you."  It didn't even sound reassuring as it fell out of my mouth.  I offered a lame, "Mama's gonna sit with you the whole time.  It'll be quick, I promise..." but by then I had lost her.  Beauty's eyes were now off of me and darting back and forth from the cheaply framed prints of deer poised at an unnaturally blue stream in some mystical looking forest to the red letters of the exit sign blaring ESCAPE HERE against the institutional beige walls of the waiting room.  One door in.  One door out.  She was trapped and she knew it.


Jingle jangle
Jingle jangle
Jingle jangle


A little hand, dusted with remnants of sidewalk chalk, reached up and grabbed mine.  I gave her one squeeze for confidence and another just because.  The woman who checked us in must have forgotten her teeth that day because she didn't smile once.  No "Hi!  How are you?" from her.  No siree, she was all business.


"Take a seat.  We'll call you when we're ready."


"Thank you very much," I replied.  As if that extra "very much" would buy my daughter a get out of jail free card for what was to come.

The tech approached as Lola's name was called.  It was like Vanna White stepping forward to turn a vowel.  She was a young one that tech - which I thought could play out one of two ways; maybe she would be one of those twentysomethings who are great with kids and win them over right away or...or...she would be the new hire who couldn't hit a vein on the first try to save her life.  Try as I may to be the optimist, I began to hedge my bet on the latter as opposed to the former.

Her loud snap of the rubber glove annoyed me.  So cliche, was that really necessary?  Beauty was now up in my lap and hoping climb into my shirt to hide.  "You're gonna hafta hold her arms and legs while I do this," were this gal's opening words of wisdom.  Yep, she was a new hire alright.  And obviously barren.  Was she crazy?  Had she gone out to her car and smoked a joint during her lunch break?  Did she really expect me restrain a bucking two year old AND keep the site steady for the draw?  Sure, I could pin a leg or two but there was no way I could keep her still enough to get the needle in and keep it in.

I sat there, with Beauty in my lap, contemplating how I was going to pull this off.  A black padded armrest saluted us at ninety degrees before it dropped like a guillotuine down in front of us.  We were locked in.  After that I heard nothing but screams pouring out of my daughter's mouth.  Her head thrashed wildly back and forth and too soon her legs fell into synch. Her frantic rhythm mimicked that of a caged wild animal desperately trying to free itself before the slaughter. 

Jingle jangle

Jingle jangle

Jingle jangle

The plastic bracelets bounced off of each other as she wriggled and fought me.  Such strength my little girl has, I thought.  I love that she has this energy inside of her but hate that it's a needle drawing it out.

"Hey, Brenda...I need a hand with this one."

Huh?  A what?  You're in the middle of the procedure.  You're asking for help now?  I looked down at Beauty's arm and saw the needle plunging in and out again and again hunting for that vein.   After about the fifth plunge, it swam to the right, then back to the left, then right again.  THE NEEDLE IS IN HER ARM YOU STUPID IDIOT, WHAT IS WRONG WITH YOU?!?!

Brenda arrived in time to get kicked in the hip.  At this point I was so upset with everything that I was secretly cheering for Lola to land as many blows as possible.

"It sounds bad but she's not in any pain.  She's just mad that we're holding her down."

My vision, blurred from my own tears, was lost but my voice was not.  Through gritted teeth I managed, "Yeah, right.  Draw the frickin' blood!"  Had her whopping two weeks on the job really desensitized her that quickly?  I briefly contemplated grabbing the needle and lunging at the beast, then stabbing her repeatedly in the eyeball.  "Oh, it's not painful.  You're just upset about not being able to see, right?" would be my condolence as she rolled around, curled up in a fetal position on the cold linoleum floor. 

When it was all said and done, the tech collected 3 vials of blood from my little girl.  End to end they would have stretched wrist to elbow up my arm; each one of them filled with the deep crimson specimen ordered by the Good Doctor.  The contents of these vials would be scrutinized later as we assessed functions of the tiny organs that lay tucked so neatly inside her little body.

The draw now complete, we left the office not hand in hand but head to shoulder.  I carried my Aching Beauty out through the lobby and into the parking lot with promises of a better day tomorrow. No one accompanied us out to the car that afternoon.  It was just Beauty and I walking away from the beast, her bracelets clumsily acknowledging our feat.

Jingle jangle

Jingle jangle

Jingle jangle

    

A Snowman in April

There was a time when we had arguably the best yard on the block and with the exception of the old retired guy one cul-de-sac over, possibly the entire neighborhood.  We weed wacked, edged, mulched, sprinkled and fertilized to our heart's content.  It was pristine.  An oasis of emerald green, it beckoned bare feet from as far away as six houses in either direction.  One step into that yard and your toes wiggled in orgasmic delight.  

Then we had kids. 

The weedwacker disappeared behind a team of strollers and the edger was overtaken by a gang of rogue Fisher Price toys.  Diamonds, criss crosses, wave and circle patterns were replaced by the same thoughtless horizontal back-and-forth-as-fast-as-you-can-before-naptime-is-over patterns that blanketed the rest of the neighborhood.  Our perfect oasis of green had become a desert peppered with crab grass and bald spots.  Not even the ChemLawn man could save us.  We had reached the point of no return.

This was and still is my reality as I stood before my withering lawn yesterday afternoon.  With spring in full bloom and a hint of summer in the air, I decided that a miracle would happen on this day.  In the span of an afternoon, I would nurse this baby back to her glory days and all order would be restored.  So as soon as Henry went down for his nap, I raced into the garage and wrestled the lawnmower free.

After a few passes, I settled into the task, confident in my ability to undo the damage of the past four years worth of neglect.  Charlie was busy across the street constructing a bike ramp worthy of at least one broken bone.  And Lola was...wait...where was Lola?  Normally her brother's shadow, she was nowhere to be seen.  I cut the mower off and headed into the garage.   Salvation for the yard would have to come another day.

"Lola?"

No answer.

"Lo-Laaaaaaah.  Where are youuuuu?"

Still no answer.  Had she wandered down the block in search of cooler toys?  Was she small enough to squeeze through the mouth of the sewer drain?  Christ, had she been kidnapped?!  My mother's panic button was seconds from going off.

 

"Do you wanna a popcicle?"

A faint rustle came from the back of the garage.  Checkmate.  I had the rabbit in my crosshairs.  Carefully, I maneuvered the obstacle course before me keeping close sight of that tuft of blond hair peeking from behind the parked stroller.  What was she doing back there?  Was she hiding?  Was she okay?  Had a paint can fallen and rendered her unconscious?  I couldn't get back there fast enough and in my haste, I tripped over 2 plastic grocery carts, a dumptruck and a half eaten tennis ball.  Note to self:  time to clean out the garage.  And then I was upon her. Oh shit!

Oh, shit.

Her big round eyes fixed straight ahead at the plastic blue police car, she wouldn't break the stare to look at me.  I knew that look.  I had seen it many times before, usually at the dinner table.  That was the look that gave her away.  And then.  Then the smell. 

"Lola Geist.  What.  Have.  You.  Done!?!"  As if I even needed to ask.

"Lola go caca."  One point for bilingualism.  Too bad it was all over my garage floor. 

  

But where most mothers would be pissed, annoyed, or dismayed, a CF mama is forever curious.  Was it going to be a CF poop; greasy, bulky and foul smelling? Was this to be the end of our journey down the road of pancreatic sufficiency?  How long would it take me to teach her how to swallow all those enzymes before every meal?  I crouched down to get a closer look.  Au contraire mon frere!  It donned the same shade of brown as my batch of chocolate brownies baked not too long ago and was sprinkled with...was it remnants of green peas from last night's dinner?  Not a trace of grease that I could discern and it was beautifully formed.  A smallish round head rested upon a portly shaped belly, it had the the shape of a snowman!  It was indeed The Perfect Poop.

All jubilation was interrupted when my husband's head popped through the door to the house to announce the end of naptime.  Never one to mince words he bellowed,  "Henry's up.  He wants a BOOB."  Aaah...yes, honey.  I'm coming.

The sad lawn would wait another week, her crab grass sprouting and her bald spots widening but alas, with the arrival of the snowman in April, a new sense of order had been restored.