Thursday, March 31, 2011


"Mommy!  Where are you going?!" an exasperated Michael yells.  I take a heavy sigh and explain that I am just leaving the room to make a phone call and to get the basket of clothes I need fold.  Dealing with his insane attachment issues and anxiety about not having me in his line of sight at all times has been very stressful...  for him and ME.

I am exhausted just thinking about how many times this week I have reassured him that Mommy is not going anywhere, he is stuck with me.  But no matter how many hugs, cuddles, kisses on his porcelain forehead I give him, he is still not buying it.  At almost four years old he is has full blown separation anxiety.  It has become extreme since he has been staying at home so much the past two months with our quarantine to get Michael well after his hospitalization and fallout in recent months.

He has become my shadow.

It is sweet, almost endearing.  There are moments that I know I will miss that he wants to be two steps behind me.  I certainly know that I should cherish these moments.  And believe me, I do.  But there are also the times it is...

(WARNING:  bad mom comment coming, turn and look away if you can't bear to read this)


Like the times he just has to check on me in the bathroom.  Or the times when I go to quickly spin around and rush into the other room and in doing so completely level him to floor.  Or the times he starts to get anxious when I walk out of the room to check my email on the computer in the next room.  I know, I know.  I realize it sounds cold.  But on some level, a person needs a quiet minute to themselves.  And quite honestly, until my kiddos go to bed, I don't get it.  Not one minute.

Nonstop energy leaks from the windows of our home.  Playful activity spills over from the boys' baths.  Extra globs of blue gel toothpaste smeared on the counter reveals the overzealous workings of a four-year-old.  The pace around here is a million miles per hour.  And in all of it, to simply have some personal space shouldn't be all that much to ask for, but in this circus it is an impossibility.

My boys are loud and rowdy and full of life, which I love.  But it's plain draining.  The kids jump off my sofas (even with all the scolding in the world!) in a heated firefight with other robots.  As I lunge for the ringing phone, the robots in the other room start yelling at eachother.  I clearly miss the phone call in the commotion and our answering machine picks up.  Over the loud volume of someone's voice leaving a voicemail about a check up on the boys' respiratory vests, there is toddler shrieking and crying (Dylan).  Then, there is growling from the older robot  (Michael).  Then I hear a pitiful response from Dylan, slurred behind his pacifier firmly in his mouth, "I saaaaa."  (Translation:  "I'm sad.")  He comes running in to me with his dramatic scowl and asks for me to pick him up.  "Uppy?  Upppppy!!!"  I have been having chest pains lately probably from constantly having to break these brawling boys apart and navigating the complicated waters of toddler negotiating.  Or maybe the chest pains are from lifting and carrying around a hefty kid.  Either way, I am living in a circus... or a madhouse depending on what you call it, where chest pains are normal.

Michael is also now the biggest tattle on his baby brother.  Maybe it's a sense of obligation since he believes he is my wingman, but in this moment, Michael purses his mouth.  He looks down then up again at me with his meanest robot face he can drum up.  I appeal to Michael's sense of brotherly play and love.  "Please play nicely with your brother.  Okay?  Mommy is going to start counting and if I get to 3 that means no more Transformers (his favorite show for CF treatments, so much so he ASKS to do his treatments.)"  I don't know if this more of a threat to him or to my sanity as we lead up to treatment time without his favorite show cued up.  Michael rolls his steel gray blue eyes and heaves a sigh.  "But Maaaa'm..."

I walk away.  No sooner am I heading up the stairs in our foyer holding Dylan, than Michael is at my heels.  He races around my even paced strides and stops directly in front of me.  I stumble and he starts moving his feet faster, "I'm going to win, huh huh.  I am going to beat you Mommy."  Great, all I need is a fall down a flight of stairs because of a foot race with my four-year-old while holding my hefty two-year-old.  I am convinced that kids his age don't understand cause and effect.  At least, they don't understand their own behavior as the causes and the resulting effects to other people.

It's not until later in the day when the dog starts her booming bark and aggressively heads for the front door that I realize Michael's constant patter behind me is a much deeper issue.  The pooch's ears are perked forward attentively and her tail is straight out.  Her fur is standing  up as she lurches forward in her barking ferocity.  She hears something on our street (probably the UPS guy) and her mania startles even me.  Michael whines with a dramatic squeal, "Oh no!" and follows it with a "The are strangers outside are coming to get me."


I immediately stop the clean up of the dominoes that I have been furiously tossing in the metal bin.  Every domino that lands in the bin is deafening.  I am surprised that we could hear the dog's booming warning over these stupid dominoes.  I walk over and pull Michael into my arms and hug him.  In all the loud chaos, we find a quiet moment.

It all crystallizes.  Even though I am around, I am not always present for him.  And his concern and fear are completely real.  You can feel them in the room.  The laundry can wait.  The email can wait.  The calls can wait.  The mail can wait.  Dinner can wait.  Treatments can wait (a few more minutes anyway). 

Even that personal space I so need can wait.  This little boy just needs his mommy.

Minutes later we are playing on the floor when the trippiest show ever, "Yo Gabba Gabba" radiates from the TV underneath our playtime.  Happy songs, psychedelic characters, and Jack Black all parade across the screen.  I am convinced that some dudes in a dark room tripping on LSD created this cheery show, but obviously they did their job since I walk around regularly singing its tunes.  Dylan drops his head and declares in a distressed tone, "I no wike him."  He wails.  He clearly is not a fan of the Jack Black.  I turn and view the chubby man stuffed into an orange suit with a scraggly beard and beady eyes.  Jack is now dancing and singing, swiftly moving around the brightly colored TV set with the screwy characters dancing around him.  I look at Dylan.  Sparkling tears well up and his eyes turn pink from crying.  I scoop him into my arms and Dylan clutches on and he continues to sadly share his distaste for Jack Black.  "I no wike him," he says shaking his head looking at the floor pathetically.

The only supporting response I can give him is "Neither, do I, Dylan, neither do I."  Afterall, Dylan is right.  Jack Black is a little...  off.  I can't blame the kid.

I glance over at Michael who is now lining up Thomas trains meticulously, one by one, preparing them for an imaginative playtime attack of train robots.  He is sprawled on the carpet of our family room floor concentrating on the impending attack.  I hug Dylan and I plop on the floor with the boys.  "Which train can Mommy have?"  I put my hand out and Michael drops a train in my hand and explains the battle landscape that is developing.

My shadows.  They are more pronounced some days and even downright independent.  And other times they are more reserved appearing only when the timing is just right.  But when the light has shifted, my shadows cast beautiful shapes.  They show up and are playful, these shadows bring joy.  The revelations of my shadows in each appearance teach me new things every day.  I don't ever want to miss my shadows two steps behind me.

My favorite shadows pictures from last year.

Monday, March 21, 2011

Cracking the Code.

N1303K.  and Delta I507.

Pretty meaningless code to most people, but this combination is the one, two punch of Cystic Fibrosis in our house.  These are the two genetic mutations that reside on the boys' Chromosome 7 and misdirect the protein that causes CF.  It is these two bits of data stored in Michael and Dylan's double helix that causes us to spend tons of time cleaning up after it.  One mutation from me and one from my husband.  They were meaningless to me at one point in my life.  But are no longer.

I barely remember high school science classes...  earth science, biology, chemistry.  I vaguely recall my monotone, white-haired teacher talking about recessive genetic disorders.  The most I can recall from the topic are X-chromosome disorders like hemophilia, and that hemophilia typically is more prevalent with males than females.  I can also visualize the 4-square diagram that the teacher would demonstrate the probability of 'carriers' having offspring with genetic disorders.

There it is.  That is pretty much the extent of what I absorbed.  That's all.

I was more likely swooning over my latest crush or visualizing my performance at my volleyball game after school.  I never really embedded the science in my brain.  Why would I need it afterall?  I blew it off as I got older as something that I needed to learn for school and would never need to 'retrieve' from my brain's file folders again.  Let's face it, as an adult, I worked in sales and marketing for a media conglomerate.  My job was brands and persuading people.  My brain's filing system had tucked any science that I did once know way back in a dusty old manila file somewhere near the files for trigonometry (groan) and French rolling jeans (double groan).

There are moments in my life that are completely unreal.  There are the times that I reflect on these moments and start to connect the dots, even back to that unnecessary science lesson.  It is then that I realize the astounding coincidences and ironies around me.  They transcend explanation.

After college, I became close to a friend who was getting her Masters Degree in nutrition.  She and I lived together for a year and we both stood up in each other's weddings.  Our husband's both graduated with the same degree from the same esteemed university and went on to work (and still do 11 years later!) for the same big accounting firm.  To say the least, I have trusted her from the very first day I met her.  She is a true, caring friend, who is also now the mother of two little ones.

I recall sitting at my desk at work in between conference calls and sales planning meetings when I receive an email from my friend.  She had become a dietitian at Children's Memorial Hospital in Chicago, and on this particular occasion, the email is not about dinner plans or a bachelorette party.  Instead, she was requesting donations to support her participation in a fundraising walk.  It was for money to cure this thing called...

Cystic Fibrosis.  

Humph.  Shrug of the shoulders.  What's that?  It sounds pretty ominous, I think, but I have no clue.  I just know that my friend is doing good and I decide instantly that I will support her.  My phone rings and I swivel my chair around for my next marketing call from New York.  I gaze out my window at the quintessential Chicago view.  The Wrigley Building dead center of my office window emanating its beautiful architecture.  It's iconic clock staring back at me.  The void of the Chicago Sun-Times Building now starting to be filled with Donald Trump's ego, still only a few stories tall.  As voices join the call, I peer out at the western view of the Chicago River and Wacker Drive as it bends around South to travel past more colossal skyscrapers like the Sears Tower.  "Is everyone on the phone?  Who are we still waiting on?"  And the call surges forward focusing on whatever marketing plan is necessary or whatever fire drill is scorching from our executives. In my work distraction, my friend's email remains quietly in my inbox.

It isn't until weeks later that I remember, "Oh crud!", I forgot to make that donation...  for whatever it was that she was fundraising...  what was that again?  It sounded medical, sort of familiar.  I open the email to refresh myself and click through quickly to make the donation.  I don't want to forget again or get distracted from this task.  I click through the menus...  I don't know.  Do I "join the team" or do I "make a donation"?  I think it's a donation...  oh, I don't know.  After I make the donation, I call my friend to ask her to make sure that I did it correctly.  I curiously ask her how she got interested in this cause and she casually mentions that she has a colleague who treats kids at the hospital for this condition...  Cystic Fibrosis.  "Oh, cool.  Sounds like a good cause," I respond.  Then I follow it up with, "What nights can you guys catch dinner next month?"

A meaningless memory.  I have many of those that are buried deep in my brain never filed in my archaic system where the history notes and the high school gossip now reside.  These memories are in unorganized stacks, piles of different colored papers, scribbled with notes.  Some typed neatly, some are memo-style, and some are faxes.  But this donation memory gets lopped on the to the top of the pile and slowly gets buried with time under more useless information.

I never think about it.  I don't even hear that term again...  well until about a year later, when I get a call from my doctor early in my pregnancy to tell me that I am a genetic carrier for this thing called Cystic Fibrosis.   Again, I don't even recognize the term when he says it.  I am juggling my new promotion, which throws me back into the world of sales.  I am recalibrating my career and have a lot going on.  Oh, yeah, and now I am pregnant.

"It's the most commonly carried gene among Caucasians for genetic disorders, but it's rare and unlikely that your husband is also a carrier.  It's probably nothing, but you should have your husband tested, just so we are sure.  No hurry."  I hang up and have a lump in my throat.  What was he just talking about?  I call my husband and am starting to cry.  His positivity and no-worry attitude puts me ease.

When his test results a couple weeks later come back, I get the call.  He is also positive as a carrier.  I just don't know what to do with this information...  and after some Googling, is when the high school probability table comes back to me.  25% chance that our baby with have this thing called Cystic Fibrosis.  Where in the world have I heard that before?!  After a few days of heartache and my brain going to worst possible places, my husband reassures me that everything will be fine.  "There's a 75% chance that our baby won't have this... CF," he points out.

Huh.  75% huh.

For whatever reason though, this is still unsettling for me.

As time passes, there are more intersections and ironies.  After Michael is born, there are things passed over.  Doctors who dismiss the CF carrier conversations.  There are glaringly obvious symptoms that we wonder about, but as new parents don't know better.  But my gut keeps telling me to continue asking and pressing for more answers.  It is when Michael is 10 weeks old that we finally proactively have him sweat-tested for CF.  Not a real shocker for me, since as many moms can attest to...  you just have instincts about things.  Forget probability.  Maternal instinct trumps probability.  He tests positive, and that's when I choke down my ignorance.  Quickly, it is replaced by heartache.

We walk into the small medical exam room with sweet Michael in the baby carrier.  I am washed away in tears and my husband is quiet.  We spend time talking with the doctor, who explains everything about the clinical aspects of what this this diagnosis means.  She provides the comfort and support we need and also reveals the immediate steps that need to be taken to ensure our baby's best days ahead.  When we are through talking to the doctor, we speak with nurses, a social worker, and finally, a CF dietitian.

"Hi, I hate that we are meeting under these circumstances," the dietitian says as she comes in and sits down.  What did she just say?  I am confused.  There is both sadness and heartfelt kindness in her eyes.  "I am friends with your friend..." and states her name.  I am stunned.  That email...  I stammer in my head.  That donation from last year...  She is my friend's colleague.  And now, we are all connected in an inexplicable way.  My friend had walked last year to raise money for her patients who have Cystic Fibrosis.  And now, my baby boy is one of her patients.  It is all too much, along with the fact I am shell shocked purely from the diagnosis.

She sees my blank expression on my face as the wheels turn in my head.  I explain that I donated last year to the walk.  She smiles, but I can tell that she is already knew the foreshadowing of our connection.  "Everything will be okay.  Michael will be fine.  There are some things that we should discuss.  I know the doctor had mentioned to you that he needs to start enzyme pills with his bottles and I want to show you how to give them to him.  We will have a plan to help gain weight back.  Everything will be okay."  She can see that I am now past the inconceivable connection that we share and I am now drowning again in my grieving.  She realizes she needs to work fast to ensure that I get all the critical information before my head goes below the water level as despair and coping wash over me.  I can barely tread any longer.

It's these moments peppered throughout my life that make me do double-takes.  There are so many of them, I can't piece them all together.  I wonder what special, insightful moment is next and how the the surprise will be wrapped?  Will the the next moment tell me more about my future or more about my past?

Back to that code, within the immediate weeks following Michael's diagnosis, we learn more about these genetic blips.   First, our boys have rare mutations for a rare disease.  The odds are just crazy, but I guess I am not surprised.

  • There is a CF gene called Delta F508 that accounts for about 70% of all CF mutation gene copies in the world.
  • About 85% of those with CF carry at least one copy of this mutation.
  • Approximately 50% of the CF population have both copies being this mutation.

To take a step back, our boys do not carry this mutation... at all.  Neither of their two copies are Delta F508, making them unique in an already unique group of people.

Since my basic science class from sophomore year of high school, I have since learned so much more about these CF mutations.  These bits of genetic code have run through history and along family lines for many generations unnoticed.  That is how it went in our families.  A silent gene carries on through the centuries.  Now as I study and learn more today, I can share a couple specific things about our code.  It has given me wisdom and insight greater than me.  We know that the N1303K mutation tends to follow Mediterranean lineage.  Not shocking since my husband is Italian.  And as I start to uncover information, the only thing I can find published in multiple academic medical journal abstracts shows a a higher concentration of CF population with this mutation living in Lower Normandy France.  France?  My family is Scottish and German.  I can't for the life me process... French.  My mutation is French?

Years pass, and we refocus our lives away from genes that we can't change, but we work on how we live every day to stay healthy.  Michael becomes a toddler and then a preschooler.  We have another baby boy, Dylan, who is a busy body and 'active' doesn't even describe his inability to sit still ever.

Just before Michael turns three, I decide it would be a fine idea to start to untangle our ancestry.  Nice, neat books filled with artifacts and information for our boys to understand their ancestors' stories.   I start with my family history on my father's side.  I spend days combing ancestry websites and doing web searches looking for information.  I begin compiling documents and the family tree where some generations span as many as 13 siblings.  As I research, I become intrigued with my ancestors further up the generations, those hard-working people who came to America and I want to understand why they chose to come here.

I find a blurb by someone who also carries my maiden name and has researched our family clan.  He tells the story of our Scottish clan that moved to Ireland and then on to America to start a new life away from religious persecution.  But the author goes beyond and takes even further back.  It is this that immediately catches my attention.  "The surname (my Maiden name) is one of great antiquity. It originated in the area of the Picts, the eastern portion of Scotland, where they (Picts) were allowed to settle on condition that all their Kings agree to marry an Irish Princess. The Picts are considered to be among the most ancient of the founding races of Scotland. Bede, a respected historian (born 673), estimated that they came to Scotland some fifteen centuries BC...

from France."

The hairs stand up on my arms and back of my neck.  There is my answer.  I have no doubt that this little genetic blip has traveled from France before Christ's time all the way to Scotland, to Ireland, to Philadelphia, to Virginia, to West Virginia, and then to Chicago.  I read further and find the my ancestor's motto was "J'ai bonne esperance."  In English,

"I have good hope."

That says it all.

I understand that this gene has passed through the generations since an estimated few hundred years before Christ.  It has been part of my family and been there all along.  It is part of me.  It is part of us.  Remarkable and unmistakable.  I now look very differently at this teeny tiny gene.

My brain has since refiled this information about genetic disorders and how the work.  I have pulled them out of the filing system, dusted them off, and planted them at the front of the filing cabinet.  I have also added to the file more information that spans the centuries.  These special bits of information I will no longer need to sift through piles of memories to retrieve.  Instead, they are burned into my brain, my heart, and my soul.  Forever.

These two strings of letters and numbers have led us in a new direction in our lives and given us beautiful context of survival and life.  And HOPE... or direct translation, "good hope".   They certainly make our daily lives tricky and staying healthy for the boys but there are blessings hidden inside.

Meeting new friends and caregivers that have forever changed our lives with their wisdom, support and grace...

Connecting with incredible stories of heroism of individuals battling against their own genetic blips, which we would not have truly heard otherwise...

Uncovering family history about ancestors who also carried this genetic code deep inside of them.

Most of all, these two genetic mutations are challenging us to look at the before, the now, and the some day to learn more about ourselves.  Without this code forever altering our lives, my story just would not be quite what it is today.

Monday, March 14, 2011

The shift.

It's five o'clock in the morning and my husband jumps out of bed to get to work early.  I am not at the top of my game at the crack of dawn and not at all attuned to things.  On this particular morning there is one thing that fully catches my attention.  Muffled by the walls of our home and blankets in bed doesn't make it any less apparent.  Michael is coughing in his bed this morning.  It doesn't last too long.  Once it's quiet again, I drift back to sleep, satisfied that he is comfortable.

I awake to some commotion and conversation in the hallway between Michael and my husband.  I hear my husband explain to sweet Michael that he has to head out to work.  Seconds later, I see sweet Michael scooped in Daddy's arms next to our bed, then Michael climbing into bed with me.  Daddy kisses each of our foreheads and departs for work.

"Can I play a game on your phone?" he asks.  Michael is the ultimate negotiator.  He capitalizes on moments in his favor.  I fumble around awkwardly with my phone on the nightstand in our dark bedroom and gently thrust it in his hand.  I am too tired to argue, so the mini litigator just won his case.  I hear him boot up "Fruit Ninja", where the primary goal is to slice and destroy as much flying fruit as possible.  Mangled pineapple.  Bleeding watermelon.  Splattered kiwi and broken bananas.  I hear the acrobatic swings of the Ninja sword as the fruit massacre ensues.

I pull the soft comfort of my covers up to the tip of my nose.  My eyes are buried beneath my tired lids.  The sounds of the fruit slaughter are dulled by the ominous cough I am hearing from Michael.  A gravely, junky cough .  Slice, whip, crack, and splosh!  Then sounds of a watermelon cracking.  Michael painfully clears his throat.  He works to gain control, but can't quite seem to stop coughing.  After a breath pause another wave of coughing overcomes him.  He begins to get frustrated since his cough is affecting his ability to decimate brightly colored, oozing fruit.

I hear Dylan's wake up call from his doorway.  "Mommy?  Ma-meeee!"  I hop out of bed and my mini fruit-hating ninja shadows me.  After a wake up diaper change, pitstop at the potty, everyone is fresh and ready for morning CF treatments.  We usually spend more time lazing around and starting everything around 7:30, but this morning we are revving up the machines an hour early because Michael's cough is very much present.

It's a cough that has been plaguing Michael and he can't quite shake.  This is the cough that ebbs and flows.  It's the one that has kept Michael at home from school for over a month.  These are the moments that those squeaky wheels grinding in my brain slow and halt altogether.  My mom brain shuts down.  I just don't know what do with this cough, or rather the shift in his cough.

It's subtle, but my ears have gotten attuned to it.  Airy and dry.  Lately airy and dry.  Sometimes infrequent, sometimes frequent, regardless always an annoyance for Michael.  Other times is becomes spasmatic and completely uncontrollable.  But it's the shift that catches my attention on this morning.  No longer airy and dry, but now wet and junky.  Clearly menacing.  Rattling.  All of this layered onto the original version.  I guess you could call it now Cough Version 2.0.  Also, layered in is constant throat clearing.  Because it was the very first thing in my senses this morning and hasn't left us for a moment, it warrants a call to the CF Team.

It's a Friday morning and I have fallen prey too many times to doubting myself on a Friday and then realizing I should call someone on the care team when it's 4 pm and the staff is ready to head home for the day.  I just hate to bother docs on the weekends if I don't need to... on this morning, I decide early that I need to reach out and get the ball rolling.  I leave a message at 7:45 a.m. strategically.  If it's nothing or they want me to hold off to monitor the cough through the weekend, they will just let me know.  But, if they want to tweak any meds before the weekend, I will have the opportunity to hit the pharmacy or make adjustments throughout the day.  Done.  Decision made.  Now to hear back...

Within the hour, I get a response from the team and assessing begins.  Details detail details.  I go through every detail of our recent days.  I have learned what seems meaningless can often be the important to the doctors.  The coughs highs.  And the coughs lows.  But I share with the nurse, the most important reason for my concern...  he is now coughing in his sleep and first things when he wakes.  Satisfied that I have covered everything, we agree that she will check in with me once she has conferred with the doctor.

I wait for the call amidst the scramble of finishing treatments and making breakfast.  This morning on the boys' breakfast menu are gooey, fragrant cinnamon rolls and salty bacon that cracks and crumbles with each bite.  The only thing I need is a good cup of coffee.  Then the phone rings with the verdict...  the doctor wants to see him...  today.  A little surprised, but also relieved, I hang up.  I unmistakably see my day shift right before my eyes.

I immediately dial Nonna to ask if I leave my other monkey with her as I plan to take Michael to the doctor.  The next phone call I must make, makes my heart sink... Michael's preschool teacher.  I have been keeping Michael at home for over a month during his continued battle with this cough.  He has fought this junk since November when he was hospitalized.  As he recovered in November and December, we tried to establish normalcy with school and through the holidays.  But it was at the January visit with his CF Care team that he had a recurrent cough and shocking weight loss.  We were told to hibernate at home avoiding germs and viruses for a while.  His overall picture of health had, very clearly, shifted.

His caring teacher had offered to visit him once a week at home to help him stay connected to his classmates and their activities.  I feel gray even with the rays of sun pouring in the house, because now the doctor's appointment is trumping a fun visit from his thoughtful teacher.  Not only is this troublesome cough preventing him from going to school with his friends, but it is now altering our Plan B for him to still have access to fun and learning with his teacher.  I have to cancel for today and try to schedule with her next week.  Just how do you explain this to a kid who doesn't really feel sick?  I catch her briefly and we agree to talk on Monday to reschedule.

The next hour is a scramble to get dressed and get on our way.  As I help Michael into his pants, Dylan grabs the potty seat, puts it on his head and runs out of the bathroom laughing.  I lock eyes with Michael.  He smirks and starts laughing.  I just shake my head and snicker under my breath.  As I finish helping Michael with his shirt, I see a flash of Dylan run by and then he slowly turns the corner around the staircase.  He begins by hanging on the banister clutching the spindles and starts walking along the unsafe side of the staircase.  Foot over foot and hand over hand.  I dart out of the bathroom seeing this, fly around the banister and grab him.  This child seeks out the most dangerous things possible and tries them.  My heart in my throat, the acrobat in my arms, I am relieved.  Then, I firmly reprimand him.

Some black eyeliner, a good pair of jeans and my favorite red trench coat cinched at the waist, and I am ready to roll.  Nonna arrives and Michael and I head out.

Typical CF clinic routine...  the paper face mask...  the hurry up and then wait...  the checking in process.  This time we must wait a few minutes before they can usher us into an exam room.  So, with no one else in the waiting room, Michael and I decide to hack open some fruit with our trusty digital ninja sword.

Once in the room, we are greeted by our favorite nurses, who immediately comment on how great Michael looks.  It is evident that he has gained weight like a rockstar.  I had noticed this week the little pudge that has formed under his chin.  His face is fuller.  He is heavier when I pick him up.  It is when he stands on the scale when the collective gasp fills the small white room.  He is 40 pounds!  What?!  40 pounds?  He can't be.  But it's true.  Here it is, before my eyes as the nurse slides the metal markers over on the clunking scale, there has been a shift.  I am bursting with joy.  I have to swallow my relief and emotions down, so as not to completely embarrass myself.  He has rebounded from his weight concerns from seven weeks ago.  My kid has porked out.  And I love it.  I fidget with the tie of my trench coat for a distraction.

We power through the remainder of the visit with the nurse and the doctor discussing the next steps with this pesky cough.  Same drill...  culture, antibiotic in the meantime, and lots of albuterol (you might recognize it from the puffer device that asthmatics use during an asthma attack).  Nearly two hours later, we have our marching orders and head for the door.

We exit the pleasant medical campus through the dull lobby.  We pass under the overhang where dark shadows live and step out into the unbroken rays of sunshine.  I welcome the light as we climb into our familiar car.  When I suggest we pick up food to bring home for lunch, Michael explains that he doesn't feel like eating.  The irony of the 40 lb. kid has had his fill of eating.  He then asks, "Can I go home and do my vest and mask?"  I pause.  I know my little negotiator is angling to watch his favorite TV show, since he knows that we let him watch his favorite show to reward him for doing his treatments.

Our day is thrown off.  The food can wait, especially since he is not hungry.  And how many times is he going to ASK to do his CF treatments?!

Of course, I agree.  Michael has been such a good kid, who overachieves every day.  I shift my thinking.  He doesn't have negotiate to win his position this time.  He's already won.

Tuesday, March 8, 2011

Digest this.

I am pouring the creamer in my coffee when out of the corner of my eye, I see Dylan not eating, which in and of itself catches my full attention.   I glance over at him to witness his hand slowing lowering into the remaining milk of his cereal bowl.  The Cheerios are nowhere to be found, presumably in his belly, but the milk has become a watercolor scene across my table.  Dylan deliberately sweeps his hand in the white liquid and gently carries it across the table.  Then he lifts his hand, now in the position of a downward claw, where five continuous drips of milk fall off, one stream from each fingertip.  He watches this messy phenomenon of gravity for a few moments and then, SPLAT!, slams his hand down into the lake of milk on my kitchen table.  Deep sigh.  "No!  Dylan, honey we don't do that.  That's naughty and you know it.  That's 1."  To keep my kids in check, we usually do a count for each offense, when they arrive at 3, that means a stay on the naughty step in our foyer.  He looks at me and grins with a white sparkle in his black eyes.  He responds, "That's 2!" and laughs.

The day then continues with Dylan finding more trouble, this time centered around the fifth member of our family.  Our chocolate lab is a lovable, anxious dog.  She is probably best summarized by saying she is completely insane.  She is has done everything from destroying a friend's checkbook to shredding two Bibles (she must not care for religious texts, since she did these offenses on different days too).

As if I don't have enough on my plate, this dog is also a load of work.  There's a shocker...  more things to handle every day.  As a puppy, within weeks of bringing her home, she started getting sick, which didn't bode so well for crate training.  I believe this is probably partly why she is the way she is...  NUTS.  The other part being her squirrely disposition and unbalanced psyche.

To add insult to injury, she has considerable digestive problems and requires prescription dog food that can only be purchased at our vet's office over 30 minutes away.  After years of dealing with this, and our boys joining our family, I will never forget the vet, who is also a friend, telling me that there is a good chance the dog would possibly need, get this... DIGESTIVE ENZYMES with her meals.  Seriously?!  You have GOT to be joking.  As time passed, she in fact didn't need them, but you get the point.

The boys shove the dog out of the way when she kisses them.  They try climbing on her to ride her.  And they frankly take her love for granted.  But there are the quiet moments when I have seen Michael not knowing anyone was watching, walk over and gently pat her head, "Why you sad?  Good girl, good girl, Haley."  Or Dylan stroke her back saying loudly, "Niiiiiice.  Niiiiice."

The one thing that I will give this dog mad props for is that she has always been loving and sweet to two little boys that have terrorized her from the start.  She stood firmly and patiently when as babies they would pull themselves up to standing onto her, their tiny fists full of her fur.  She didn't wince or growl.  Although, she did look at us with a stare that said it all, "Can you please pull him off of me?  It doesn't feel so great."  She sprints to greet them outside their bedroom doors after naps as they stroll out, dazed with rosy cheeks.

I am grateful on this morning that she is the sweet dog that she is.  Ting.  Ting-ting-ting.  Bap, Ting.  I am hearing noises as I stand in the laundry room that I immediately recognize.  Oh no...!  Dylan is in the dog bowl.  Each "ting" and "bap" are kibbles hitting our ceramic floor in the kitchen.  "Dylan!  Out of there!  No no no!  We don't do that."  I dodge around the counter to find him with handfuls of the dog food ready to throw.  "Dylan...!"  He instantly starts returning the food to the bowl.  "Ok.  Ok, Mommy."  Well, at least he listens.  He begins collecting the kibble around the kitchen with his tiny forefinger and thumb.  He knows that my frustration is boiling over.  "Ok."  Then he loudly starts singing the "clean up" song.  Cheerily, he has rectified the problem.  Mommy is happy now and he toddles off to find more trouble.

Dylan always seems to find trouble.  Quite the converse, though, trouble has really plagued Dylan the past few weeks.  My little guy has had some digestive highs and, well I guess to put it nicely, lows.  

As with everything else in his life, Dylan does nothing in moderation.  He is a garbage disposal when it comes to food.  He eats when he is hungry and he eats when he is not.  The child shovels sausage links in his little mouth with his cheeks bulging.  He can down 5 of them in a sitting with other breakfast foods.  He also loves cheese and I have witnessed him eating 4 slices of gourmet deli sliced cheddar as if it was the last food on earth.  But the one in the record books was when he was shortly after he turned 18 months, he ate 9 and a half McDonald's chicken McNuggets and a half of an order of large french fries.  It makes my own stomach hurts just thinking of it.

A ha!  You say.  This is clearly good for kiddos that need more calories.  And right you are.  However, with every good thing, there is always another question to be answered that leaves me scratching my head.

Imagine that your physician tasked you with starting a high-calorie diet.  You begin cramming food in.  To put it politely, at some point, the food must exit.  And my guess is that your metabolism hasn't suddenly sky-rocketed since you have started eating more food and your pace of digestion hasn't increased, if anything is excruciatingly slow.  At a point, you're full.  Packed full of food to the top, until things, uh, get moving.

Now imagine that you are doing all of this with a system with less than ideal plumbing in your body.  You must rely on medications to help manage the entire digestive process, but are still tasked to sustain high caloric intake and good nutrition.  In the end, you wait...  you wait for nature to happen.  But it just isn't happening.  You continue eating and continue waiting.  Your belly hardens and the meds work furiously.  While a normal part of human nature, what seems so simple, in CF terms is actually highly complex.  All meds must be working on optimal performance to continue aiding and promoting regular digestion...  for the boys this includes digestive enzymes every time they eat, laxative therapy, antacids, digestive aids to help open the stomach to help food pass through and appetite stimulants.  Complex to say the least.

To put it nicely, after the massive necessary binging, what goes in, must come out.  And most importantly, as parents, we must be vigilant and observant...  is everything, well, working?

Lately for Dylan, it has not.  He has been struggling with "backing up" issues lately, where he is not going potty regularly, but not entirely "backing up" where a kiddo ends up throwing up, which is often the case.  This can happen because digestion is so slow that the kid is packed with food that has no where left to go.  I am simplifying it.  There are even more significant fall out from all of this like menacing bowel obstructions and lower GI complications.  Surgery is not uncommon.  We even know families where their child was born with a bowel obstruction and immediate surgery was required.

In the recent two weeks, Dylan keeps downing food, but his belly is managing the process ineffectively.  We keep adjusting his meds and then Saturday comes the blow out.  Phew!  His little belly finally relaxes.  It is softer, almost fleshy if you rub it.  The way a chubby kid's belly should be.  But it is the next morning that sends a flash of worry through me and my husband.  By afternoon our fears are not alleviated.  Because of the intestinal distress and other apparent symptoms, we are afraid that he has had a hernia.  And his symptoms are not subsiding as the day continues.

By late afternoon, we confer with the on-call CF doctor and share the ongoing situation and our concerns.  Panic sets in and my mommy instinct tells me that we need a physician's eyes on the matter and fast!  The talk with the doctor confirms my worries.  He immediately suggests we head down to the ER.  It is late Sunday afternoon when we drop Michael off at Papa and Nonna's house and start our trek downtown Chicago.  It is a cold, rainy evening and the monotone sweep of the windshield wipers are nothing short of annoying.  Dylan drifts off to sleep in the warmth of his carseat.  My husband and I are frustrated that our little guys have to endure such trips.  We just want everything to be okay.

Because of concerns of the boys being exposed to germs and viruses, the doctor has called ahead and we are whisked quickly to one of the ER rooms upon talking to one of nurses in triage.  Little Dylan is now awake and angry that his face mask that keeps getting in the way of his pacifier.  He is also upset that he can't swill his sweet, cherry Gatorade, since the nurse has advised us that he needs fast until we know next steps.  Disgruntled, exhausted, and uncomfortable...  a perfect mix for a toddler nearing bedtime and out of sorts.

We arrive in our tiny hospital exam room and we dress Dylan in the kiddy gown they nurse gives to us.  We all try to relax.  Quickly the attending ER physician and a fellow doctor come into our room and quickly begin with a barrage of focused questions.  Their tone is warm and there is a reflection of kindess in their eyes as they assess Dylan's situation.  They decide that an ultrasound is definitely in order and based on my context about the recent digestive issues they also order an x-ray of his belly to ensure there is nothing impacted in his intestine (i.e. an obstruction).  Now we wait.

Tick tock.

Tick tock.

We play with bubbles. These bubbles are fantastic!  They don't break when they land on things.  Dylan still frustrated that he can't have his "Gay-or-ay" (translation: Gatorade) is distracted for the time being.  He giggles and claps his hands.  He inspects them as they land on objects in the room, and SMACK!, he pops them with his tiny hands.  He loves these bubbles and they are completely a Godsend.

Tick tock.

We also play with Pla-Doh.  He loves making little balls and pretending to tee them off with a straw in his best golf swing.  Periodically, someone knocks on our door and my husband takes Dylan to have a test done.  Minutes later they arrive back at home base in our little dimly lit room.

Tick tock.

Exhaustion and hunger are wearing on us.  I look at my watch.  It's nine o'clock.  I am hoping that Dylan will take a nap or at lay down and least rest since it is getting way past his bedtime.  We flip through the only 5 channels, all with kids programming, on the TV and distract Dylan with a movie, Shrek.

No Academy Awards for us adults.  My one indulgence of the Oscars is the red carpet.  I enjoy watching the gorgeous celebrities strutting around in glitter and glitz and my questions always percolate...  what designer dreamt up that gown?...  What jeweler sculpted those stunning earrings?...  And, oh God!, what were she and her stylist thinking?!...  Instead of the flash of paparazzi and pirouetting socialites on the screen, we are subjected to watching a green ogre in ghastly hospital lighting as we wait.

My husband kneels next to the hospital bed where Dylan is laying.  Sweetly, Dylan takes his hand and starts rubbing my husband's head as if comforting him.

I swoon.  What a gentle, adorable little boy.  He is soothing my husband even though he is the one laying on the white hospital linens.  He carefully runs his hand from my husband's head, down his shoulder, down his arm, and rubs his forearm.

I am in awe.  How selfless.  How brave.  No one teaches these things to children this young, at least explicitly.  Dylan then quietly slumps back against the bed and refocuses on the TV.  Warm tears well up in my eyes.  My husband turns around, looks at me and grins, "Did you just see that?"  We both know how incredibly special our little boys are.

After a few more minutes, I make my way out into the shocking, bright lights in the hallway of the ER.  My eyes are screaming as I gently blink to ease in the blur of doctors on phones, doctors on computers, and doctors talking with more doctors.  The head doctor recognizes me and says she will check on the results of the tests that have been run.  Within minutes she pops her head in and says things are expected to look fine and we will likely be heading home.  She just needs to wrap up the final notations on the ultrasound.  Phew.  Crisis averted.  Within the next 20 minutes we do our routine check-out paperwork, prescriptions, and repack our bag to leave.  We are heading home.

The saga still continues throughout the week with Dylan's belly.  I spend repeated time on the phone with the CF Care team and dietitian trying to sort through his current state of digestion and next steps.  We adjust medications and continue to sleuth through the problem together.

While I still have no answers and certainly no consistency with how he is doing, I am strangely thankful this morning when I end up cleaning one of the biggest messes of my life.  After some trusty bleach and a load of laundry, things are back on course for the day.  All mothers have been there...  waiting for a kid to "go".  Or changing that diaper that is completely incomprehensible.  But even in these most stressful situations, I inevitably thank the Poop Gods and give a sigh of relief that everything has returned to normal...  at least for now.

As I finish the clean up, I contemplate the next time I can pick up a gossip magazine to thumb through all those Oscar red carpet moments.  It's better this way, anyways, I decide.  Maybe next time I can bring the magazine with me.

Wednesday, March 2, 2011

Questions and Coffee.

A reliable, steaming mug of coffee gives me comfort.

I love when tan, creamy coffee is fresh and greets me in the morning.  I never end up making it all the way to the bottom of a cup of coffee, since it usually gets set down in my distraction and goes cold.  Icy, disrespectful coffee is my nemesis.  There are also the times that I brew a cup of coffee in our Kuerig, only to forget about it entirely.  I easily average making 3 cups of coffee daily, but I am lucky to drink one whole cup.  I just have too much I am juggling these days.

For whatever reason, this is a morning that I can actually sit down with my dependable friend and watch the morning's headlines.  With a sigh, I flip on the TV.  The kids are eating breakfast pleasantly at the kitchen table.  Our morning is already upside down, but I figure we'll rearrange our routine and get right back on track.

We normally greet the boys every morning in the same fashion.  Dylan flings his door open and if we haven't heard the thud of this act, we usually hear his sweet toddler voice call for us.  He stands at his gate with his favorite thumbie blanket, "monkey, and his two pacifiers, his "boppies".  We enter his room, first with a diaper change, then gift him a cup of juice spiked with Miralax, and we collectively crawl back into our bed with the melodies and spinning scenes of a kiddie show.

Next, Michael will usually stroll into our bedroom, now as dark as a cave, with bed head.  Not just a little disheveled, but completely messy hair in all directions.  Michael's azure eyes blink slowly.  Dylan yells in a perky tone, "Hi, Mike!"  Michael rubs his eyes and climbs into our bed.  Our family together, every morning.

Usually, our immediate laze is soon contrasted by a flurry of CF treatments and medications.  There are nebulizers and the airway clearance sessions, but not until Michael and Dylan have had their first medications of the day like antacids, appetite stimulants, steroids, digestion aids to help move food through the stomach.  Then there is a barrage during breakfast of vitamins and probiotics.  I have to constantly run through the checklist of all that needs to be done, and more cumbersome, and when it needs to happen.

*   *   *

It is Friday night and one of my best friends from college comes to visit.  My husband picks her up at the airport and once she walks through the door, it is just like old times.  A chatty catch-up paired with beers (now Blue Moon, not stale keg beers served in Solo cups).  My taste has improved a little since our days of 5 cent beers at our favorite bar where bands played.  We are happy.  You'd think we are 21 again.  Well, except for my two sleeping angels upstairs tucked their slumbers.

The next morning I shove my husband out of bed to go deal with the kids.  He is always good about letting me sleep in and this morning, honestly, the few beers are having fun trampling on my tired brain.

After a short while, I roll out of bed and stumble upon the kitchen.  The loud whir of the medical machines in the next room advise me that the boys are doing their morning round of CF treatments. My friend is sipping her cup of coffee, and my husband is task master for the morning's to-do's already starting breakfast for the kiddos.  I take a prepared breakfast casserole out of the refrigerator and set it down with a plunk! on the counter.  I turn oven on and brew a hot cup of coffee to help me wake up.  There is nothing like a good talk over coffee, or beers for that matter, with an long-time friend.

As the day progresses, my dear friend witnesses all that has been weighing on me.  As we go along our routine, she presents really good questions.  Questions that I have never been asked.  I have never had someone who isn't already intimately familiar with our drill spend almost 24 hours in our house to see all that we do.  She wants to know so much...  why we do certain things, how we do them.  I am really touched by her curiosity and her heartfelt sadness.  From my good friend who is also a mother, I can also read what she wasn't saying aloud.  I don't think she has realized how CF dominates every aspect of our lives.  Completely.  Small children require lots of care, of course, all mothers can relate.  We just have a crazy amount of extra steps.  There is simply a lot that goes on in one day in our home and I have become desensitized.  Her sparkling eyes and inquisitive sentiments got me thinking...

What would it be like to be able to wake up and not think this way all day?  My brain has been completely hard-wired to think differently.

I have no idea what it is like to simply hand food to my kids without pills or without thinking.  Without thinking... anything.  Normally feeding my kids is a mental exercise.

I am constantly doing the medicine math in my head...       How many pills does he need with this?...
When was the last time he took enzymes?...      I swiftly grab the box of you-name-it and study the fat content...      How much fat is in this?...      What is a serving size?...      How much will he probably eat?...      When was the last time he took his digestive medications?  Is he on schedule so his hunger will peak now?...      If he has a huge snack now (applause!), then will he eat his meal later?...

I actually have moments when I wonder if I have taken my enzymes before I eat something because it that ingrained in my brain that this task precedes eating.

Besides some thoughtful questions from a close friend, it is also happens to be a natural time of year when I reflect on this journey since we are on the brink of fundraising for our annual walk in May.  The coming two months mean preparing, planning, creating, educating and fundraising.  It is a time of reflection and insight into our lives and not just for our circle of supporters, but also for our ourselves.  What are doing and why are we doing it?

I relive Michael's diagnosis.  I remember it vividly.  It's a bright Friday afternoon and I have arrived home from Children's Memorial Hospital where we had brought baby Michael for a test.  He is now quietly napping in his crib after the exhaustion of the morning.  I am standing in my kitchen, summer sunlight flooding through our windows when the phone rings.  I grab the phone and quickly glance look at the caller ID and see it is the pediatrician's office.  "Hello?"  I am confused since we weren't expecting a call until after the weekend.  The senior pediatrician introduces herself since we have never met in person.  She clears her throat, "We just got a call from Children's Memorial with Michael's sweat test results.  They were 112 and 110.  This indicates that he most definitely has CF.  I just spoke with the director of the CF center and she is prepared to talk to you right now and even see Michael this afternoon."  There is urgency in her voice.  Silence on my end of the line is broken by sobbing.  I brace my weight with my hand against the kitchen counter.  And the phones starts to slip from my hand.

I also experience the remarkable highs of this journey with all other individuals, families, friends, and supporters of advances with treating Cystic Fibrosis.  Last week, I am laying in bed for once before the boys wake up and I am scrolling through apps on my phone.  I see the headline breakthrough on my social network news feed from my mobile phone.  I dive out of bed and hurry to the blaring white light of our computer.  "Announced positive results from Phase 3 Clinical Trials...  improved lung function...  fewer pulmonary exacerbations... weight gain..."  A drug that we have been watching like a hawk since Michael was diagnosed four years ago has achieved favorable results in the final Phase 3 trials before it will go to the FDA for approval.  Wait, not only favorable, but OVERACHIEVED expectations.  This oral pill, over a significant duration of almost a year, helped CF patients' key markers that define CF are all notably improved.  Even the sodium chloride in their sweat shifted significantly toward a more normal range...  translation:  patients are less salty when they sweat!

A miracle.

I start crying.  My heart swells into my throat.  Then an email pops up from my brother-in-law about the CF drug and the company's stock is trading up.  We exchange emails and I then share the news broadly with friends and family.  While this drug alone will not cure Michael and Dylan because of their genetic code, this pill combined with another one in Phase 2 Clinical Trials are hoped will be the key.  The key to fewer questions in my days, better health for my boys, and overall a better quality of life.

Our fundraising journey and creating awareness about CF is often met with questions.  Questions that I am always, unabashedly, glad to answer.  If I don't keep telling our story, no one will for us.  I think of all the other parents before me who continued their vigilance in sharing their kids' stories.  It was these pioneers who have brought us to a remarkable place in medicine.  A pill that alters the body's cells to CORRECT the dysfunctional CF protein.  I silently thank these men, women and children who have changed the course of days for my two sweet children.

I take a long, controlled sip my coffee.  It's hot and burns as it rolls down and warms my dancing soul and my busy brain.  "Mom!" Michael shouts abruptly from the other room.  I set the cup down on the table and walk away to see what today's adventure may bring.