Tuesday, March 12, 2013


The gloomy days of late winter put me in a foul mood.  I just can't peak out from under the blanket of gray that covers everything these days.  And while it is a very chic Benjamin Moore paint swatch, it isn't the color I want outside my kitchen window day after day.

Michael's ongoing fight against Pseudemonas lingers.  After he wraps his 28-day cycle of inhaled antibiotics and 14-day flight of oral antibiotics we find ourselves in February.  And ready for to have him "recultured" to see what might be lurking in the recesses of the tiny airways of his lungs.  And the positive culture, slaps us in the face and confirms that we must continue additional therapies to control the lung infection.

Then, Michael gets sick on top of his positive culture.  The afternoon before his special Valentine's Day party, he complains that his ear hurts.  Our pediatrician whisks us in and confirms a raging, pussing ear infection.  Groan.  Aaaaand back on oral antibiotics on top of the inhaled antibiotics.   My only relief is that we catch it and start treating it quickly so that he is able to enjoy his school's Valentine's exchange.  Two years ago, he wasn't so fortunate.  The smothering weight of that ugly winter blanket sits on top of me.  A big heap of blah.

Somewhere under the blah, Valentine's Day comes and goes with lots of adorable red construction paper heart projects from Kindergarten and preschool.  The many ways our boys tell us that they love us.  My favorite expression of love is one of the many times Dylan asks the question, "Mommy, you know how much I love you?"  I play along.  "How much?"  He pounces on the end of my last syllable in his deep, lispy voice without missing a beat. "All the way."  Yeah, me too buddy.

Love you.

All.  The.  Way.

A few days later, Michael marks another celebratory day in Kindergarten -- the 100th Day of School.  I realize this is a big day.  He reminds me before he heads out the door that morning, "Mommy, it is HALF-WAY to first grade, you know."  I chuckle at the gravity of his tone.  I am in charge of snacks for the party, where we send baggies of goldfish with the kids and a poem marking that they are "O-fish-ally" 100 days smart.  It's fun but it's also a marker for them on all their time spent learning and exploring in Kindergarten.

Later that day at the bus stop, Michael is charged with energy when his feet hit the pavement off the bus.  His eyes are dancing against the blue sky.  He and I do our same hug as any other day as we walk up our front yard.  We tumble through the front door as the dog is doing her welcome home mambo as she whines and cries that Michael has arrived home.  Dylan is a chatter box trying to explain to Michael all that he has done in his day so far.

Michael says nothing, plops his bag down with urgency and hurriedly unzips his bag.  After shuffling through this bag he tells me that there is something he must share with me.  "Look, Mommy!  I drew something for you at school today."  He shoves a worksheet with a drawing toward me.

I take it in my hands and see a red crayon stick figure with a beaming smile on its face holding a brown crayon cane.  The assignment title at the top of the page, "This is what I will look like when I am 100 years old."  My eyes well with tears.  I start crying and cannot stop.

100 years old.

Old age.  That's old for any person, but for someone with CF (where today's average life expectancy is 38 years), well, that's a miracle.

My tears stream down my face.  Michael's face wrinkles and he aims his confused expression at me.  "Mommy, why are you crying?  It is because I am such a good draw-er, isn't it?"  I laugh as I am crying.  "Yes, Michael that's exactly why I am crying."

Sure enough a few days later like all moms can attest to...  my other kiddo starts with his own version of sniffles.  A fever that lingers.  And lingers.  And five days later still LINGERS.  I finally take Dylan to the pediatrician to find out that he has a yuck nose and looming sinus infection.  We agree that something might be brewing and with a negative flu test and a negative strep test, she hands me another script.  She and I both concur that waiting might be a good idea, but this way he is covered if it turns ugly.  Let's see if he can tackle this.  So we wait.

But not long.  The very next day we lounge around as a family.  More gray outside.  So we enjoy a cozy day at home inside.  My husband and I have the same thought that a nice family dinner out would be the perfect end to the day.  Something to get us out of the house and some good hot food.

We all get ready late afternoon.  A hot shower, a little eyeliner, and diva black mascara help me feel a bit better.  It won't erridicate the gloom, but at least I look, er, brighter.

After some bickering with Michael, he agrees to put on the dress clothes and we head downstairs to get Dylan dressed.  We find him slumped at the kitchen breakfast bar fast asleep.  We realize he is really sick and not quite himself.  Fever and fatigue.  I head out to fill a script that had been safely in my purse knowing we'd cross this bridge.  So much for a family dinner out.

As Dylan starts improving and his cough that has emerged with this round of illness begins fading a bit, I settle into the week.  Within days, I realize that I am also getting sick.  Standard.  By Sunday morning I find myself at an urgent care center with a prescription for antibiotics in hand.   The upcoming week we are expecting my husband's parents, Papa and Nonna, to come into town for a visit and to watch the boys for a couple days so we can attend the Cystic Fibrosis Foundation's National Volunteer Leadership Conference.  After starting to rebound from feeling lousy and a canceled flight because of a storm making life difficult, they arrive safely in New Jersey.  We are thankful for a little time with them and by evening, we make our exit to head to the conference in DC.

My husband and I head off into the night through a chilly mix of rain and snow.  In between the squeaks of the windshield wipers, we reflect on our boys' journey with CF and anticipate the two days ahead...  seeing old friends who are also fighting the same CF fight in their homes...  raising money to help find a cure for their loved ones...  hearing medical updates, advocacy strategies, marketing insights and more.  What is it they say...  ?  "Knowledge is Power."  It sure is.  Especially in fighting a chronic, devastating disease.

The conference is an emotional one, but filled with love and hope.  And remarkable science.  It becomes clear as the Chief Medical Officer and the Chief Executive Officer/President share the medical update that there is tremendous progress. They spend considerable time explaining the state of CF today as a disease and the expectation of where we are heading to a CURE.   The massive screens in the ballroom reveal a bar graph that shows a trajectory of medications treating 95% of the CF population to control and potentially CURE CF by 2017.  95%. Completely and totally astounding.  My brain almost can't process it.  2017.

I begin processing the genetics and the medicine.  My eyes slowly track each bar and adjacent note.  One bar, then the next.  And it hits me.  Our boys are in the remaining 5%.  The 5% NOT on the bar graph.  Here's the catch.  We have always suspected that the drugs would help our boys because in theory the CF protein is broken for the same reason (or a similar reason) as one of the most common mutations.  But the reality slams into my brain.  The truth is WE JUST DON'T KNOW.  The anticipated medications might not help them or might help them considerably.  Michael and Dylan are a rare group in an already rare disease.  (To read just how remarkable their gene story with CF is, read blogpost "Cracking the Code").  There aren't enough patients, or possibly any patients in the world, with their exact genetic match to research these amazing medications on the horizon.

My hands shake under the table as I realize the magnitude.  I shift in my seat reeling from my conclusion.  My cynical voice snorts inside my head, "5%.  Right, of course we're the 5%".  But my trust and experience with this amazing, results-driven organization shouts in my head, "Keep listening.  Don't freak out just yet."  And I am glad that I take pause and force my heart to be still.

The bar graph disappears and another slide emerges, "GOAL:  100%  We must not stop until ALL mutations are treated with success!"

Got it.  100.


No wonder 100 is a milestone.  100 days of Kindergarten.  100 Years Old.  Of course, 100%.

My hope, my trust, my belief in this journey do not fade.  My heart swells as my brain mulls this over.  I find peace and would find promise in my subsequent conversations with the Chief Medical Officer and the CEO/President about our boys over the following day.  Terms like "personalized medicine" and "methods of individual testing" are discussed.  But the most amazing part of all this...  they are BEING DISCUSSED.  To know that we are close to a cure or controlling CF is one thing.  But to be discussing individuals' experiences with life-saving medications and possibly in the next four years, this is the miracle in and of itself.

The people fighting for this cure...  The volunteers in this room...  The friends and family, co-workers and neighbors raising money to find a cure...  The scientists in the lab...  The leadership of the CF Foundation.  They get it and they are absolutely behind us.

Or as the salty boys would say...