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.

REINA SOFIA HAS CF

 

I'm sorry but I just wanna tuck those puffed out
curls behind her ears each & every time I see her.

In case you're not up to speeed on your Who's Who of reigning monarchs, let me intoduce you to Reina Sofia.  Reina Sofia means a lot in this country. First off, she's the queen of Spain and a pretty likeable gal from what I can gather although I do think it's high time she updated her hairstyle. But let's face it, when you're the queen you get certain perks no matter what your hair looks like.   As you probably guessed, Reina Sofia has a lot of things named after her: streets, parks, airports, and tons of buildings. Even the hospital that we go to in Cordoba for our quarterly CF clinic is named for Her Majesty. Hence, the title of this post. No, the queen of Spain does not have cystic fibrosis however the hospital named for her does~an entire unit in fact.  And that is where we found ourselves earlier this month.

Two weeks post visit and I'm now cursing myself for not having taken better notes because quite honestly, it's all kind of a blur.  Three squabbling kids squished into a doctor's office has that effect on me, ya' know?   But there were some noteable differences that are worth a mention.

1.  THERE IS INDEED AN "I" IN TEAM  


Dr. Cowboy caught the wheeze and prescribed
one puff of Seratide before each vest session
over a three week period.

Dr. Cowboy is our pulmonologist.  That's not really his name but whip out your Spanish 101 notes and I'll bet you can figure it out.  Anyway, he's like the captain of the CF team for us; the main guy we see and the one who runs the show.  He interviewed us and logged copious notes into the computer about the kids' health history and then he sat down with each of them to take a listen.  When he was finished he handed me two sealed medical packages:  the swabs for their throat cultures.  At first I was a little taken aback.  Me?  I have to gag them?  You can't be serious, I'll screw it up.  I'll puncture a lung or pierce a vocal chord.   Thoughts like these raced through my mind while my brain was trying to translate, that's your fucking job, buddy minus the obscenities into Spanish.  Alas, no translation needed.  We were to take the culture kit down to the lab.  The kids would get their throat cultures done there.  Hmmmm.... interetesting, I thought.  And then we were given a two inch stack of medical orders and told where to go to get each one done. 

So much for getting out of the
'homework' assignment. 

Our visit with Dr. Cowboy was a quick one.  But our time in the hospital, not so much.  The time we would have spent waiting in our old CF clinic routine was spent walking long, white corridors looking for faraway laboratories and hidden specialty offices.  I still haven't figured out how all the players come together - but for the time being it appears that technology links them.  When we entered the office of the GI doc, the first thing she did was pull up Dr. Cowboy's notes from twenty minutes prior.  She did her own dog and pony show, entered the new data and gave us our homework: collect three day's worth of fecal matter for each child and bring it back so labs can be run.  Oh but Good Doctor, they're pancreatic sufficient!  Best check those notes again....
    

2.  TAKE A NUMBER
Spain will forever be the country of 'manana'.  Why do today what you can put off til tomorrow?  I'll never get it, being of good German stock this mindset drives me nuts.  As we were running all over Reina Sofia (the hospital not the Queen) there arose a common theme:  la vuelta (the return).  Every stop was just that, a stop.  We took our number, waited the fifteen minutes to be seen and when it was finally our turn we were able to go in to make an appointment to come back.

Labs?  See you on Friday morning!
GI?  Catch you after labs!
Radiology?  We'll be there..how's Friday for you?

On one hand it was frustrating to me that clinic seems to be so compartmentalized here; each entity doing it's own thing on it's own schedule and I had to remind myself that it's not wrong, just...different.  Yet on the other hand I left each doctor feeling as though we were in really good hands, dealing with the experts in their field which helped me to swallow the inconvenience pill that comes with living in a pueblo 45 minutes away from Cordoba.

3.  OUR FREE LUNCH
Dr. Cowboy was very interested in how CF clinic was run in the States.  He asked about the frequency of our visits, which turns out to be the same here - every three months unless something comes up.  He asked about who made up the members of the care team, which again is much the same except there is no pharmacologist on team here.  And he asked about medications and the protocol in using them.


Then, very slowly he leaned over and said, "I want you to understand something very important.  Very important."


I was on the edge of my chair.  Charlie and Lola were taking turns jumping off of the exam table and Henry was unrolling gauze pads by the meter.  We were about to be denied, banned or exiled I wasn't sure which but it was serious from Cowboy's tone.

"You will not pay for medication here.  Do you understand?"


Uhm, no.  Actually, I don't because it's like a billion dollars back where we live...you know, in the Land of Milk and Honey where everyone is blond haired and blue eyed and drives a little red Corvette.  "Excuse me?" I managed.


"Cystic Fibrosis is a chronic and life threatening disease.  You will have a standing order at the hospital pharmacy for each child's prescription needs."

"How much is the copay?"


"The what?"


"Copay,"  I repeated.  "The part that we are responsible for."

"There is no copay for these medications.  As you run out, you will come in to Reina Sofia and refill your order.  We're not here to make a profit.  We're here to treat a disease."

I was speechless but managed to kick Joe's foot, my signal for him to confirm that I hadn't misinterpreted anything.  We would later learn that prescription coverages vary depending upon which region in Spain you live in.  In Andalucia, the regional government has funded a healthcare plan that covers prescription costs for chronic diseases such as cystic fibrosis.  Not funded in Andalucia's plan however is The Vest.  Patients here use other pep devices such as the Acapella and the Flutter in combination with manual cpt. 

Having access to free medication almost seemed too good to be true.  Was Hypertonic Saline available?  TOBI?  Pulmozyme?  Azithromycin?  When should we expect to start Charlie on the next batch of drugs?  I asked Cowboy what our protocol would be under the team's guidelines.

"Your children are uniquely yours.  Charlie is different from Lola just as Lola is different from Charlie in spite of them having the same genetic make up for this disease," he began.  "I do not feel comfortable starting either Charlie or Lola on a new drug just because they have a birthday.  I want to know first how their bodies are handling this disease, what it looks like for them.  I want to study their chest xrays, review their labs and get a baseline of where we are now before we jump into a change of plan."

I was silent, liking what I was hearing but still a bit paranoid that we might miss out on a more preventative approach to their care.

"If you're concerned about the availability of these treatmenet options, don't be.  We have access to them when we're ready for them," he continued.  "Let's keep a few things in our back pocket for the time being while we figure out where each child is.  Is that fair enough?"

"Yes.  Yes, it is."  We nodded in unison and explained to Cowboy that while we want to be very aggressive in a proactive approach we struggle with the possibility of over medicating the kids and as a result are very much interested in trying a more holistic approach before jumping immediately to drugs.  Cowboy listened, we talked some more and closed things by confirming that clinic had our contact information for the pending lab results.

By the end of our first clinic visit, I felt good with where we had landed.  I felt like Cowboy had listened to us, heard us and in the end it just felt like a good fit.  As with every clinic visit though, I was completely fried.  Clinic visits take a lot out of me physically, mentally and emotionally each and every time.  There's the early rising, the anticipation, the planning - so much going into it all to make it as worthwhile and thorough for the kids as possible.  And in spite of the differences, which were really just that - differences - no better  no worse, there was a constant.  That is to say, the constant of knowing that the team members who are dedicated to helping us fight cystic fibrosis for the sake of our children want to give us The Best.   Make no mistake, I'm not talking the best American care or the best Spanish care but rather The Best CF Care. 

And for that we are very, very grateful.

DREAMING IN TECHNICOLOR

It was cold.
It was wet.
It was windy.

How fitting that the weather decided to shit in my face for my official return to running at Saturday’s 10k run for Great Strides. I left the house knowing that Joe, my dad and the kids would be staying at home that morning, filling up on a warm pancake breakfast without me.

And I was okay with it.

Wading through the swell that was fast overtaking the parking lot I lamented the likelihood of a sparse turnout. Running in the rain is nice. Running in a typhoon, well, not so nice. By the time I was standing under the park shelter, the rain was coming down not in buckets but in sheets. It was going to be a long and lonely six miles.

And I was okay with it.

The route was two loops around the Raccoon River Lake. It’s a beautiful trail, much of it shaded by towering deciduous trees whose branches tickle the clouds before arching towards one another to form an overhead canopy. I swear every year that I’ll run the loop in the fall, my favorite time of the year, but true to form I always forget.

Rain pelting, I committed one foot in front of the other until I found my stride. After the first mile I didn’t even notice the rain. By the second mile the wind felt good. And by the third…by the third mile my miracle had arrived.

As I was rounding the second curve of the lake and coming up on a small clearing in the tree line that bordered the gravel path, it was as if an invisible hand was peeling back the layers of cloud that covered that miserable Saturday morning sky. Then, little by little, the hole in the sky began to fill with the most amazing sunlight. It was a Technicolor tribute; a buttery warm yellow pouring in from behind a wall of solid black. It very nearly stopped me in my tracks. In a word it was breathtaking.

As fast as the hole in the sky seemed to open up, the storm just dissipated; overtaken by that flood of sunshine from the heavens. After you’ve read stuff like this and this and this and this from me, you know as well as I that I am not one to write about my relationship with God, religion or anything even remotely spiritual. It’s just not my comfort zone. That said, the Technicolor sunshine that shone through over this weekend’s Walk was far beyond my wildest dreams for the day.

Des Moines, one of just 600 cities to host Great Strides, raised nearly a quarter of a million dollars for the Cystic Fibrosis Foundation and while the totals aren't yet official, I know that we exceeded goal for this year's walk.   It's true that we don’t yet have our cure, that we're still amidst a brewing storm but this much I do know: the sunshine is coming.  It's not here yet but it is coming.

And I’m okay with it.

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.





SILVER BULLET x 2

It feels like forever since my first CF related post even though it's only been about three years.  I dunno anymore, is three years a lot?  A little?  Just enough?  I guess I've quieted some because we're still sailing far enough ahead of the brewing storm.  If Charlie and Lola are good, I'm good.  And lucky for us, they've been doing really, really well for a long time.  I was telling my amiga Susie that when it comes to CF, I tend to go in cycles.  I'm cool until about 2-3 weeks before our quarterly clinic visit and then I morph into Sybil.  I just bitch-out if that's even a term.  Every little thing sets me off.  Everything.

And it's all because I've got that leech called cystic fibrosis waiting to suck me dry at every turn.

Well, last week, something big happened.  I'm talking big as in The New York Times big.  It's taken me a full week to even get my brain around it let alone be able to rattle it off on the keyboard.  So here's the 411 after a week's worth of marination in the void that occupies the space once reserved for my brain:

There's a wonder drug out there called VX770.  I wrote about it once here but as quick as it posted to my feed I let go of it; let it fade back into white noise, just another prelude to a dream.  Well, last week this same wonder drug made front page of the Foundation's website.  Did you hear about the earthquake in Arkansas this week?  Yeah, well the press release for VX770 is like an 8.0 on my Richter scale.  I shit you not.  You see, this drug is now in Phase III, the final phase, of clinical trials.  You have no idea how tough it is to get a drug to this point.  I've often wondered if winning the lottery would be easier because for all the drugs that make it into the drug pipeline in the first place, it's very few that make it to market.  Very, very few.

So last week's news from the CFF that they were planning to apply for FDA approval of VX770 sent shockwaves through the CF community.  I was actually sitting in my first block class when I read the release.  At first I just sat there, staring at the monitor not believing what I was reading. 

"Patients who took the drug, compared to those on placebo, showed a marked improvement in lung function at 24 weeks, which was sustained for the duration of the 48-week trial."
WHAT?

"Patients also showed improvement across all key secondary endpoints in the study, including reduced likelihood of experiencing a pulmonary exacerbation, decreased respiratory symptoms and improved weight gain. Each of these areas is critically important to the health of people with CF."
NO FUCKING WAY!

"In addition, average sweat chloride levels of patients on VX-770 dropped toward normal levels, while those on placebo did not change — indicating the drug is impacting the underlying defect in CF. Excessive sweat chloride (salt) is a key clinical indicator of CF."
OH.  MY.  GOD.

I jumped up from my stool and clapped my hands together half a dozen times and just kept repeating, "YESSSS! YESSSSSSS!" over and over and over again. And then the tears came and I had to pace around the classroom fanning my eyes so my mascara wouldn't start running down my cheeks. My poor students, bearing witness to the freak who was their teacher.


But here's the catch. 

The VX770 will NOT help Charlie and Lola.  They don't carry the same mutations that this drug is targeted for.  It will instead help just 4% of the CF population.  What's 4% of 30,000?  Anyone got a calculator?  Like 1,200 people?  Hell, let's talk globally.  Let's take 4% of 70,000.  What's that make...2800 and thensome?  I know what you're thinking.  Yeah, she's totally lost it.  Crapping her pants for a drug that's gonna help 1,200 maybe close to 3,000 but not her own kids?  What a dreamer.  No, strike that.  What a moron.

Well hold on.  I'm not done. 

You see, there's another drug by the same company - the VX809 - and they're working on the theory that giving the VX809 along with the VX770 will help CFers who carry at least one copy of the most common CF mutation (the Delta F508).  In essence not one but two silver bullets.

Why hello there Mr.  VX770, I'd like to introduce you to my fucking amazing children, Charlie and Lola.  No, we didn't name them from that stupid cartoon from Disney Channel.  They're nothing at all like that cartoon.  They are two beautiful, wonderfully chatty, hilariously stubborn, lovey-dovey,in your face kids who happen to have CF.  And they also happen to have

a copy of that nasty Delta F508 gene that you're gonna fix.  Got it?  Good.  Oh and by the way, would you mind terribly passing this on to your pal, Mr. 809?  Thanks!

The VX809 is still in Phase II, so it's a little bit farther back in the drug pipeline but so far, things are looking good, very good.  Still, there's a catch:  getting a drug all the way through these trials not only takes good science but Megabucks.  I'm talking gazillions of dollars.  The US Government does not fund fund these clinical trials.  They are funded through donations.  The gala dinners, the WineOpeners, the charity golf outtings, the foot races, the walks...all the way down to the bake offs and can collecting.  In short, all of those fantastic donations.  That's what funds this research.

So I can't be all polite anymore and ask you to consider making a donation.  Now I'm knees to the floor begging you:  Help me Help the Foundation Help us.

And here's how:

1. Go HERE and click Join My Team.  This will register you as a walker on our family's team.
2. Once you register as a team member, you be given a login and password.  Please use it to make your own fundraising homepage 
3. Wanna really be a hero?  Go to www.CFF.org, click on the Great Strides logo and create your own  Charlie & Lola Team and recruit walkers under you.
4. Last but not least, if you haven't yet made a donation, please, please, please make one by clicking HERE.  Anything you can afford, I mean ANYTHING, would just mean the world to me, to us.  

Yesterday, as I was packing a box of dishes, Charlie came up and kneeled beside me.  "Mama, how much longer do I have to do treatments?"  God, what I wouldn't have given to have been able to tell him the answer he was fishing for.  It took everything I had not to snap that dinner plate in two, I swear.  When your child comes to you with hope in his eyes and you have to be the voice of reason, of right, of truth...well, it just so totally sucks.  

So until then, until the press release that makes me own up to No. 15 of my Bucket List, I just have to stick with, "...until we've got the cure, Charlie.  Until we've got the cure."

THIRTY SEVEN

THIRTY SEVEN.

That number just blows me away.  Has it really taken thirty seven years for me to love my body?  To admit that I'm no longer a natural blonde and probably never was?  To own up to the fact that a size 10 is my version of 'petite'?  Simply stated, it's taken me thirty seven years to accept myself for who I am instead of worrying about who I am not. 

In less than six months I'm going to be turning 38 years old; dangerously close to 40 though a milestone all the same.  It dawned on me the other day that this, my 37th year, is especially poignant in light of the fact that 37 is actually the most recent the life expectancy released by the Cystic Fibrosis Foundation.  I'm no stranger to this statistic, in fact it was one of the first things I Googled when we got Lola's diagnosis: life expectancy and CF.  Where the answer to my search used to make tears well up in my eyes, I'm pleased to report that this is no longer the case.

I've accepted it.  It's there.  It is what it is.
And I've got roughly 32 more years to change it.
Period.

Five years ago, the same year that Charlie was born and long before we even knew he had CF, a pharmaceutical company out east by the name of Vertex submitted to clinical trials this little ole' drug by the name of VX-770.  Shortly thereafter, it submitted a second drug, VX-809, to clinical trials.  The aim of both drugs was to target the basic defect of cystic fibrosis at the cellular level.  Fix the cells, fix the problem.    Evidently, there's a bit more to it than my peas and carrots brain can articulate but that's the nitty gritty.  For those of you who want it spelled out, you can click here for a mini science lesson that does a pretty good job of explaining things if I do say so myself.

As I turn the corner on January and sail into these last six months as a thirty seven year old, I am looking at new beginnings. My glass is full up to the top and spilling over.   I am as hopeful as ever that VX-770 will gain final approval by the FDA and that VX-809 will continue to show promising results in its testing so that it too can move on down and out of the drug pipeline.  I often think about what it will mean not only for those battling CF, but for anyone battling a monogenetic disorder.  Getting a drug or combination of drugs that can punch a disease - can knock it out - at the cellular level is huge.  HUGE.  Could this pending breakthrough by Vertex eventually impact those fighting sickle cell anemia?  Huntington's Disease?  Hemochromotosis?  I can't get my head around it - my heart yes, but not my head.  Not yet.

So on May 21, 2011, five pairs of running shoes will be laced up in Des Moines.  We are participating in Great Strides, the biggest and most important fundraiser for the Cystic Fibrosis Foundation.  I would like to invite you to do the same, to walk a three mile stretch with us on that day, wherever you are.  Donations made to this event go towards funding the research so crucial to finding a control for CF.  Of you I ask not one, not two, but three simple things:

1.  WALK WITH US
     Wherever you are on May 21, 2011, lace up and walk those 3 miles with us.

2.  MAKE A DONATION
     $10, $20 or $200 - every single little red penny will fill the bucket.  Really, it will!

3.  SPREAD THE WORD
    Pass the link to my blog to your Facebook friends.
    Forward this link in an email to everyone you know
    Talk about CF to your colleagues at work, to your friends at church, to your buddy at the gym, to your cashier at the grocery store - tell them about the crazy picklepits lady who can't catch a break from her Fabio, who wrestled a pair of skinny jeans (and lost), who swears she's part Gitana and who would walk to the ends of the earth if it meant a cure for CF...tell 'em all...I don't care...
JUST SPREAD THE WORD!

2011 is here. 
2011 is now.
2011 is it.

Now let's get out there together & grab it!

A Glass Half Full

Dear Blog,

Please accept my most sincere apologies. I've been absent lately, lost in life's routine of nothingness. And in this absence I have found myself thinking a lot, too much probably about The Beast lurking in the shadows: Cystic Fibrosis. Sometimes I get so annoyed, so entirely miffed at its constant presence. I've often pondered how I would describe its location to a medical team, in hopes of cutting it out of me like a random yet festering cyst; somewhere between the grisly knot in my throat and the right hand side of the crown of my skull. It bounces back and forth between these two places haphazardly, like a rogue pinball. Some days I don't feel it as much - these are usually the days I'm consumed with the busy-ness that is motherhood and a full-time career yet other days it ricochets back and forth on its own accord, laughing at me as it splits me from the inside out.

So, after a too long hiatus, I'm back and this time with a plan. I know the glass set before me is not quite full yet not quite empty either. And with that, I remind myself to make a conscious decision to look at raising 2 CFers from BOTH perspectives: Parenting CF Kids from a Glass Half Empty or Full Whatever the Case May Be.

Top 5 Cons of a Glass Half Full (Part 2 to follow next week)

#5: The "What-If" Bandit

The rules of the parenting game changed drastically way too early as far as I'm concerned. My worries about Lola getting enough breast milk and her refusal to sleep on her back like the good pediatrician ordered were cast aside instantly upon confirmation of Cystic Fibrosis. Ever since that day, I notice the physical manifestations of my worry; a few more wrinkles, a few (more?) gray hairs, and yes, even a few more pounds. There is at least one CF related worry on my mind per day, I kid you not. What if the neighbor kids get too close with their colds? What if that cough I heard in the middle of the night was more than just throat clearing: a coming cold? the arrival of The Beast? What if the teachers at school don't catch the sick kid who showed up to class because his parents had no option for daycare? What if my kids end up hating sports and instead prefer endless games of Parcheesi to a good cardio workout? Whatifwhatifwhatif...

#4: Tick Tock

Let's face it, staying on top of ANYTHING requires time and a concerted effort. CF is no different. Currently we do 30 minutes of chest clearance PT twice a day. Our CF care team has only prescribed 20 minutes for the kids but my Type A personality usually gets the best of me and I set their vests for an extra 10 minutes. Honestly, I don't know if it does any good for their lungs but those extra 600 seconds of therapy set my mind at ease, at least for that day. It wasn't until recently that we got the second vest machine. Treatment for the kids prior to the arrival of machine #2 took up to 2 hours of each day minimum. I still shudder at the thought of what it would've been like had either child been sick and needed extra vest time. Still, it was a challenge to keep a regular routine due to bedtimes, naptimes, appointments, school, work...aka LIFE.

And like many other diseases, CF is a very expensive one to manage. We are fortunate in that we have excellent health insurance coverage but even so, we are not exempt from the occasional bureaucratic "miscommunication" as was the case with the 2nd vest machine. Persistence paid off and my husband deserves ALL the credit in the world for his hours upon HOURS of waiting on hold, filling out paperwork, and writing letters to mysterious VP's so high up they don't even answer their own phones, only to being told, "No, a second vest is cost prohibitive." I often joke that not only could my husband sell ice to an Eskimo but he could get blood from a turnip...and that he did, to the tune of $16,000 (the cost of the new machine).

PT time aside, we can't complain much and spend our energy trying to keep the kids as active as possible without completely destroying the house. Not an easy feat come January when cabin fever reaches an all-time high. From late March til the first snowfall, "It's an outside day!" is my catch phrase, so much so that Charlie now greets each sunny morning with the same observation. It's great to hear those words come out of his mouth and I'd be lying if I didn't admit to feeling my chest puff up when he runs after the big boys of the block but still I wonder, what would we be doing together if we had those extra couple of hours to piddle away together every day?

#3: DENIAL. IT'S NOT JUST A RIVER IN EGYPT.

"Well, as far as I'm concerned, they don't have it. I mean they don't even cough!"
-family member who shall remain anonymous

Ugh. I've heard this repeatedly from those who, despite my best efforts at educating them, just don't get it. I've made videos, sent literature, extended invitations to clinic visits, and yes, in my worst moments, screamed like a maniac into the phone everything I know about this disease:

• CF IS A PROGRESSIVE DISEASE- it gets worse over time


• CF HAS NO CURE (YET)- do I really need to explain this?


• CF IS LIFE SHORTENING - the average life expectancy of a CFer is about 37 years old


• CF EFFECTS MULTIPLE ORGANS - "Lungs, pancreas, intestines, liver, you name it! People hear CF and automatically think lungs however, it's much more complex than that. CF is a disease of the cells. It boils down to the cellular level because the cells can't regulate the proper amount of water and salt. In short, when the cells don't work properly, a good portion of the rest of the body is thrown off track too." - definition by Ronnie Sharpe, a CF warrior

It's aggravating, if not downright insulting to think that some of the closest people to me view my diligence and dedication to my children akin to crying wolf. Believe me, I can think of a million and one other things to exaggerate about and CF is definitely not on the list.

2. THE CLOCK

Imagine 17 or 18 years old as being the new "Middle Age". That would make me practically geriatric. Ridiculous? Scary? Well, it's both in my opinion. Science has come a long way baby, but not far enough for my comfort zone. Currently, the average life expectancy for someone with CF hovers around 37 years of age. That's great news considering where we were 20 years ago but it isn't good enough, dammit and though I've never spoken these words aloud, my greatest "what-if" bandit remains clear: What if Charlie and Lola turn out to be just average, or God help me, below average?


1. WALKING ON THIN ICE

Imagine setting out on a stroll across Lake Michigan in the dead of winter. You start out feeling pretty confident; one foot in front of the other and you're thinking to yourself, "Yeah, no problem. I got it!" It's not until you get past the no turning back point that you start to notice the fine lines in the ice, the ones that look like they're going to split apart at any minute and swallow you whole into the the bone chilling black waters that await below. A quick glance over your shoulder to get your bearings and you realize that your starting point is a mirage at best. At this point you can do nothing...you've already gone too far to turn back yet you just don't know if, pardon the expression, you have the balls to take another step forward. What do you do?

That's how I feel about CF. Sometimes I'm walking along, kids in tow, and I feel like yeah, it's not so bad. We CAN do this, we'll make it through. There are other times however where I'm as uncertain as the idiot out on the middle of the lake. How in the hell did I end up here? And the point is, it doesn't matter, I AM HERE. When you're the kind of person who thrives on control, being dealt a deck that you can't read is a real problem. You do the best that you can possibly do and pray like hell that it's gonna be enough to get you across that lake. There is no turning back - you have no more choices. One step in front of the other until you reach the other side and if you're lucky, really, really lucky, when you do finally make it you'll be able to look back and say that it was all worth it.

Shadow in the Night

If I were to ever get a tattoo, my Battle Cry would be the one. Since Lola's diagnosis of Cystic Fibrosis, I feel like I've been drafted into a war against an invisible enemy, a shadow in the dark. It's the first thing that scrolls across my mind when my eyes pop open every morning, the last thing I think of before my day bleeds into night and many, many times it lurks in the shadows of my dreams just to let me know it's still there. There are no weekends or holidays off for this disease. It's 365 days a year, 24-7, in your face. It's like the CNN ticker that crawls along the bottom of your screen. It just never, ever stops...an infinite reminder of sorts. Already our first year of living with CF has come and gone. We came through battered but relatively unscathed and for that I am grateful. Yet, it never fails. Just when I think I've got a handle, a good firm grip on it, that shadow reaches out from within and shakes me to my core. It grabs me by the neck, chokes me with fear and screams it's own silent roar, "Not so fast, Kelly. Not. So. Fast."


"Your daughter's newborn screen came back abnormal. We would like to test her for Cystic Fibrosis." That was our introduction into the wonderful world of CF. Two inconclusive sweat tests and a genetic test later, we were told that she had Cystic Fibrosis. Excuse me, but where was this in the fine print of raising children? I don't recall signing up for this. And twice? Definitely not twice.


Three weeks ago, we got that same phone call. The one that begs your heart to stop as you drop to your knees and try to gasp for a breath that isn't there. That's the one. It was the same, even voice, "Charlie's sweat test results are in. The numbers are high." My Boy Wonder, the protector of his baby sister, has Cystic Fibrosis too. Since that call I often fantasize about redoing that moment. It would have been great to have replied, "I'm sorry. You must have the wrong number. We've already had our fair share of trauma at this household and besides, my pot roast is nearly done. Thanks for calling though. Have a nice day!" Isn't that how June Cleaver would have handled it? Too bad my quick wit never seems to surface during times of crisis.


At this point, the wound is still fresh. It's painful to the touch and still seeping tears. Just this afternoon I had to swallow a lump in my throat the size of Mt. Rushmore as a neighbor child pried a little too deep. "Yeah, I know about the vest - that it helps their lungs but why do they have to wear it? What's wrong with them?" Gulp. Didn't see that one coming but knew it wasn't too far off. I'd rehearsed this moment several hundred times on my way to and from work as if an Academy Award depended upon it. Regrettably, I don't know how convincing my deer-in-the-headlights delivery was.


In spite of it all though, I know how lucky I am. Charlie and Lola are doing very, very well with CF; much better than I come to think of it. I remind myself daily that things could be far worse. And so I make a conscious choice not to ignore but to coexist with the infinite CF ticker running its loop across my brain reminding me that yes, it's still there, lurking in the shadows.