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.





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.

LET THEM EAT CAKE

Two birthdays in the span of less than a week. 
One anticipates kindergarten.
The other, walking.

It's a HUGE deal that five year old birthday party.  So why then did I wait 'til the day before to pull out great grandma's homemade chocolate cake recipe and the KitchenAid?  Uhm, I mean, order the cake?   A HUGER (yes, my word) deal still that first birthday.  And I don't have a single present wrapped yet?  Uhm, okay, I mean bought.

What's up with that?

Fine.  I'm a crappy mom.  But truth be known, I don't even remember my fifth birthday party let alone my first.  While some might think me putting the store bought cake on a pedestal and passing it off as my own akin to plagiarism, I think it's sheer genius.  But hold on a second.  Before my regulars (all 5 of you) jump to my defenses with, "...but you're not a crappy mom...you're really not..." I invite you to raise your glass (hand if you're foregoing) and toast ALL the crappy moms out there like me.  And by 'crappy' I mean:

1.  You're running around half the time like John Bobbit; your dick in your hands focused on getting that thing sewn back on ASAP but failing to notice that your house is burning down, the dog just ate a pooey diaper and is licking the baby's face and your bra's on inside out. 
translation:  YOU'RE BUSY

2.  You're just a smidge grumpy from that four hour cat-nap most call 'nightime'.
translation:  YOU'RE TIRED

3.  You pass off the bedtime story because it's easier to snuggle your kiddo tight in your arms as he lays transfixed by (insert cartoon).
translation:  YOU'RE SCARED HE'S GROWING UP TOO DAMNED FAST

Cheers ladies, here's to the next 365 days. 

May they be worth every single candle on the next (store bought) cake.

Dear Son,

I've dreamed many things for you; college, the first real job, moving out, marriage...so why did yesterday throw me for such a loop?   One minute you were cruising down the sidewalk at lightning speed on a borrowed Big Wheel and the next you were sailing by on a bike.


A bike without training wheels.


Um, excuse me but when did you learn to do this?  You did not ask for my permission to reach this milestone.  Weren't you supposed to fight gravity for a week or two?  Wasn't Papa supposed to be worn ragged as he followed you up and down the block, his hand glued to the back of the seat?  Where are your skinned knees?  Your bloody knuckles?  What's next?  Writing your name?  Reading a book?  Tying your shoe? How dare you grow up on me!


Time is passing too quickly.  I go to sleep on Monday and wake up to Friday. Spring turns to fall before I've had a chance to even smell the first perfect blooms from the flowering pear tree out back.  And you, you are growing up at rocket speed, racing away from me to do those Big Kid Things that are now just fond memories of my own youth.  I watch, no,  I study you.  Is it possible to trick a lingering moment and trap it into a memory? 
   

Every beetle is a gazelle in the eyes of its mother, so says the Arab proverb.  And you are no exception, my son.  No matter how far you ride away from me, you will always be to me my little boy...my Boy Wonder.