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A Seventh Birthday

AUTHOR: | POSTED: 03/29/15 11:41 AM
CATEGORIES: Blog

Seven years ago today a little girl bounced into our world at a whopping 2 pounds 15 ounces.  We learned immediately that what she lacked in size she made up for in spunk!

Mary Kate, 2008 at a whopping 2 pounds 15 ounces.

Mary Kate, 2008

Seven years later Mary Kate continues to amaze us.  When not reading her favoritie American Girl book she loves making her baby brother laugh and playing Calico Critters with her two little sisters.  In addition to loving ballet, soccer, running and acting Mary Kate is always up for a snuggle with a side of sass.

Mary Kate met Kit Kittredge, American Girl, on her 5th birthday compliments of Grandma Kate, Grandma Linda and Papa.  They are inseparable.

Mary Kate, 2013

Mary Kate, 2014

Mary Kate, 2014

Tonight is her final performance in CTW’s production of the Unprincess and never has their been an “Annie the Inn Keeper” who nailed her part with such enthusiasm.

Annie the Inn Keeper getting her make-up!

Annie the Inn Keeper getting her make-up!

MK with Aunt Jo Jo

MK with Aunt Jo Jo

 

DSC_0126We are so very proud of our girl.  It is a pleasure to parent her!

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DSC_0144DSC_0147DSC_0151Happy Birthday, MK.  We love you!

 

He Laughs in His Sleep

AUTHOR: | POSTED: 03/26/15 2:22 PM
CATEGORIES: Blog

I could probably leave it at that because he really does.  The first time I saw him do it I thought it was gas or a fluke, but I’ve now witnessed the phenomenon repeatedly.  It truly is laughter.

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Of course, he has reason to smile.  He is four months old and thriving.  Weighing in at a healthy thirteen and a half pounds, Nicholas continues to slam dunk all of his milestones.  With the exception of a run-of-the-mill ear infection, he has also managed to remain healthy.

Thank God.

As for the rest of us, we are adjusting to our new normal.  Being the mother of six is a lot.  A lot of chaos.  A lot of laundry.  A lot of crazy.  And, a lot of love.  Every time I find myself dragging due to sleep deprivation I remember where we stood only a few months ago and mutter a prayer of thanks giving.  We are so very grateful.

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Amidst the gratitude there is still a little bit of angst.  Because Primary Ciliary Dyskinesia is genetic we were encouraged to have our other five children tested for the disease.  Sean and I were genotyped last month and we know now that we are both carriers of a mutated CCDC 40 allele.  (Admission…the only reason I can remember it is the CCDC allele is because CCDC reminds me of AC/DC. You’ve got to love a little heavy metal word association to keep one looking smart.)

Anyways, Sean and I were very confident that none of them had it.  We’ve never seen any symptoms that mimic what Nicholas has been through.  Unfortunately, our confidence was misplaced.  Turns out two of our other children have PCD.

When we learned of the test results we were stunned and stumped.  Stunned because our other five are generally pretty healthy.  Stumped because we weren’t sure how to best proceed while respecting our children’s privacy.

It’s their lives.  It’s truly not up to us to divulge that kind of information without their permission.

So, after spending a few weeks discussing it we’ve landed in a comfortable spot.  It’s okay for me to write about PCD.  It’s okay for me to talk about parenting three children with a respiratory illness.  But we’re going to leave it at that until the other kids are old enough to tell me if it’s okay for me to write specifically about them.

I think that’s how I would’ve wanted my mother to handle it.

Hence my silence.  It goes without saying that we were shocked by the additional diagnoses. There’s been a lot of meetings where we’ve been trying to learn what this means for our children.  And there is sadness.  Sadness so profound it’s paralyzed me.  I know myself well enough to know that when I get like this I kind of shut down.  All I want to do is take care of my family.

So, that’s where I’ve been.  Taking care of my family.

As time progresses I am sure I’ll snap out of this funk. Winter has given way to spring and summer can’t get here soon enough.  RSV season is officially over mid-April and Nicholas will get sprung from his quarantine.  I can’t wait for him to learn about the world outside of our home and his doctor’s offices.

In the mean time we are taking a doctor approved trip south to warmer weather.

Stay tuned.  Some sunnier posts are coming soon!

And Then The Doctor Called…

AUTHOR: | POSTED: 02/18/15 4:30 PM
CATEGORIES: Blog

I’ve noticed that many of you tend to worry when I get quiet.  Most of the time your concerns are unfounded.  My lack of presence is due to being trapped under multiple loads of laundry or being sequestered to my car depositing children at their various activities.

Unfortunately, this most recent silence isn’t that simple.

Last Tuesday Sean and I were summoned to a conference call with Nicholas’ pulmonologist.  As we waited on the line for his doctor to join the party we were both silent.  We knew the genetic test results were back…and we knew that our required presence for an explanation probably meant we were about to get some disconcerting news.

Neither of us could talk.  I can’t speak for Sean but I was quiet because I feared if I opened my mouth I just might throw up.  In fact, I was so sure of what I was about to hear that I spent a minute or two trying to decide where I wanted to be in my house when I heard the doctor say it.  Which room did I want to forever associate with bad news?

I chose an upstair’s closet.

Now before everyone gets too upset let me put you at ease a bit.  There is nothing life threatening going on with our little boy.  Nicholas continues to eat like a champion and slam dunk his milestones.   If you looked at him you’d never know there was anything amiss.  That is…until you heard him cough.  Then you’d wonder why our sweet little three month old sounds like he has a ten-pack-a-day habit.

Spending some time with Reagan...his twin.

Spending some time with Reagan…his twin.

We were hoping his cough and occasional wheezing were from the remnants of his pneumonia.  Some of it may be…but it appears that it is more likely that Nicholas’ continued respiratory symptoms are as a direct result of his new diagnosis.

Nicholas tested positive for primary ciliary dyskinesia.  

I’ve written before about how I think bad news has a sound.  For me it’s like the most offensive alarm I’ve ever heard.  It’s constant.  It’s piercing.  And it’s distracting–almost as if my brain is trying to protect my heart from breaking.  I guess it’s a defense mechnism of some sort.  Perhaps it’s purpose is to soften the blow of a new and unwelcome reality.  I don’t really know.

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MK read his valentines to him!

MK read his valentines to him!

What I do know is that when the alarm lifts and the news starts to sink in I’m often left somewhere between mental chaos and numbness.  My mind is racing trying to figure out how to assimilate what we know now into our daily lives, while my heart is left breathless…trying not to think of the worst.  And because I’m a mom…and I’m hard-wired to worry…I have to shut it all down, reverting to that whole one-step-at-a-time strategy.

During my pregnancy with Logan, Sean and I swore by the one minute…or one step… at a time strategy.  It’s how we survived.  We employed it again during Nicholas’ hospitalizations.  I was hoping we were done having to cope in this manner.   Fate had other plans.

The other day my mom asked me how I was feeling after having had a week to digest the diagnosis.  I told her the truth.  I really don’t know.  I guess I’m fine.  It didn’t really come as a blindside.  We knew PCD was a possibility.  We were just hoping that this whole mess had truly been caused by a monster infection.  That would have been temporary…something we could have left in the past.

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Instead we have to accept that our future–Nicholas’ future– is going to be different than we thought.  And we have to remind ourselves that, that’s okay.  At least he has a future.  Hopefully a future filled with a lot of normal…and a lot of extraordinary.

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Now it’s our job to provide him every opportunity to grab his life by the horns.

Diagnosis be damned.

*****

As a result of this new diagnosis, it was important that Nicholas had the status of his lungs checked.  We were hoping to see clear open lobes on both sides.  Unfortunately, that pesky right upper lobe is still problematic.  As a result, Nicholas is headed back in for a bronchoscopy tomorrow.  The goal of the procedure is to suction as much mucus out of his airways as possible allowing air to flow freely resulting in an inflation of his upper right lobe.  

Please keep him in your prayers.  I will update on Facebook tomorrow evening.

A Little Smile with A Side of Good Health…An Update on Nicholas

AUTHOR: | POSTED: 01/26/15 3:17 PM
CATEGORIES: Blog

We’ve been home now for nine days.  Nine days!  Nine days without listening to the alarms of hospital monitors and hearing the cries of sick children.  Nine days of waking up to the whimper of a hungry baby instead of waking up to the whimper of a baby being examed by a resident before morning rounds.  And, most importantly, nine days of continuously improving health for our baby boy.

Dare I say we are turning a corner?

I’d love to report that Nicholas is completely better.  I can’t.  I had no idea his recovery could take months. That being said, he looks and sounds better than he ever has.

Thank God.

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I’d love to also report that Sean and I are feeling more comfortable regarding his diagnosis.  I guess we are…kind of…but there is still a lot of uncertainty.  That makes us nervous.  We have been told multiple times that we may never know exactly what caused Nicholas to get so sick.  The only thing we know for sure is that it wasn’t just one thing.  It most likely was a conglomeration of little things that came together creating the perfect storm.

Here’s what we think may have been the contributing factors to Nicholas’ illness….

  • Nicholas has a grade one laryngeal cleft.  It’s not significant enough to warrant surgical correction but it probably was a contributing factor to his illness.
  • Nicholas has a silent aspiration causing him to aspirate breast milk into his airway while eating.  It wasn’t severe in that he only aspirated one out of twenty swallows but it was enough to warrant thickening his expressed breast milk in order to prevent further aspiration.
  • Nicholas has a possible immunoglobulin deficiency of IgG.  This diagnosis is a bit uncertain because even though his IgG results were low, they could have been suppressed by the presence of infection.  We are retesting his IgG levels in two months, but if he does have an IgG deficiency his immune system would have been less able than a normal newborn to fight off an infection.  (If he does have low IgG it is easily treated.)
  • Nicholas took a giant gulp of amniotic fluid while exiting the womb.  It was aggressively suctioned but *may* have started this whole mess by causing the pneumonia.
  • Nicholas was a c-section baby.  The significance being he didn’t have the opportunity to squeeze through the birth canal which helps a neonate rid their lungs of fluid from the womb.

Given those five facts our medical team has determined two things…

1.  Nicholas has an under treated pneumonia excerbated by repeated premature withdrawal of IV antibiotic treatment during multiple hospitalizations.

2.  Over time, the pneumonia morphed into bacterial meningitis.  Yup.  You read that right.  BACTERIAL MENINGITIS.  This determination is supported by the fact that Nicholas had three lumbar punctures over the course of a month that all came back with elevated white blood cell counts…which IS meningitis.  The question was whether it was viral or bacterial.  The infectious disease team at Rainbow Children’s decided it was indeed bacterial because of the severity and duration of illness.  They admitted that his case of bacterial meningitis was not typical…apparently Nicholas forgot to read the medical books inutero…but nevertheless they were going to treat it as bacterial meningitis.  (Technical information…Usually bacterial meningitis is diagnosed when bacteria from the spinal fluid grows in a culture.  Nicholas’ cultures never grew anything and that stumped the infectious disease doctors.  After five days of trying to figure out  why Nicholas was so sick but the cultures were negative the chief of infectious disease  decided he didn’t care any more and treated Nicholas for it.  He told us we’d have a more definitive answer about the bacterial meningitis if Nicholas responded to treatment.  And…he did.  Thank God!)

There are a few outliers as well.  We’ve written a lot about a condition called Primary Ciliary Dyskinesia.  Up until our hospital stay at Rainbow Babies and Children we were operating under the strong suspicion that Nicholas had PCD.  Now that he is responding to his  current regimen of antibiotic treatment we aren’t so sure.  We did have him undergo some genetic testing for the condition.  Hopefully those results will come back soon.

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As for right now we are thankful that Nicholas seems to be developing normally.  He weighed in at 11 pounds 7 ounces last week.  The fact that he’s managed to gain more than four pounds since birth is promising.  Hopefully we can stay on a positive trajectory.

In the meantime we are trying to restore some normalcy around our home.  Slowly but surely our other children are adjusting to having their baby brother home.  Of course, PICC line maintenance, around-the-clock aerosol treatments (Nicholas gets ten different nebulized medications a day), combined with the beeping of his pulse oximeter and the hum of his oxygen concentrator (he’s only on a wee bit of “blow by” oxygen at night)  don’t exactly add up to the typical newborn experience.

I guess this is our new normal and it’s a heck of a lot better than where we were for the first two months of his life. We’ll take it.

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Sean and I continue to be incredibly humbled by the sheer number of people who’ve held Nicholas and our entire family in their prayers.  I don’t get out much these days but in the few outings I have had I have encountered all kinds of people who have been thinking of us and our sweet boy.

We are so thankful to everyone.  We wouldn’t be remotely close to where we are today without the love and assistance from so many.

Hopefully, some day soon this little corner of the internet will return to what it used to be…or something close to it.  In the meantime, thanks for sticking with me.  I’m sure I’ll be back to story telling and laughing at our family antics in no time.

In the meantime, I’m settling for a little smile with a side of good health.

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**The images in this post were taken by my very talented friend, Jennifer Mott.  You can see more of her photography on her website here.  

Nicholas Update and A Dose of Perspective for This Crazy Time of Year

AUTHOR: | POSTED: 12/22/14 1:41 PM
CATEGORIES: Blog

This morning when I turned my laptop on I had a moment.  I’ve had the very same moment a few times before and trust me when I say when it happens it’s weird.

It’s weird when I’m signing onto my email account and my own face pops up as a “top story” for the day.

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It always surprises me when my pregnancy with Logan resurfaces in the media.  Usually I blame it on a slow news cycle.  Why else would a freakish story from what seems like a life time ago be front and center for people’s reading pleasure?

Today the incident struck me as different.  More significant.  A reminder of perspective.

You see, the headline says our pregnancy with Logan left Sean and I ‘terrified’.  I guess, at the time, it did.  Being pregnant with another couple’s genetic child after a medical mistake is scary.  But not as terrifying as what we’ve endured the last six weeks.

Not as terrifying as not knowing why our seemingly healthy newborn son’s lungs keep collapsing.

Not as terrifying as being given a laundry list of catastrophic diseases that could be causing our son’s health issues.

Not as terrifying as watching our beautiful baby boy screaming in pain after being poked so many times I’ve lost count.  

A few days ago I was on the phone with a friend when I told her, “I’d take being pregnant with the wrong baby any day over this.

My statement left us both quiet for a moment as we thought about the absurdity of the sentence I had just spoken.

I guess that’s why this morning’s  reaction to our pregnancy mix up headline is so different now.  Characterizing my experience with Logan as ‘terrifying’ seems dramatic.  I don’t want to down play the emotional turmoil Sean and I experienced as a result of the mistake, but I always remind audiences when I’m speaking that we consider ourselves lucky.

There are worse crosses to bear.

We are bearing one with Nicholas.  Watching our child struggle to breathe.  That is terrifying.

*****

Update on Nicholas’ condition…

Nicholas continues to improve.  He is off of CPAP and breathing with the assistance of “off the wall” oxygenthrough a cannula.  (“Off the wall” means all major breathing aparatus have been removed from our room.  He’s just getting a little oxygen from the built-in pump on the wall behind his bed. He will be coming home with the equivalent of “off the wall” oxygen.)

Today’s goals are to secure home delivery of a vest to help clear Nicholas’ airway.  When we came home from the hospital the last time Nicholas was prescribed chest percussions four times a day.  I manually percussed him with a little handheld “thumper”.  The purpose of percussions is to help him loosen mucus in his airways and lungs.  Turns out manual percussions aren’t enough.  So, we’ve been prescribed a vest that percusses him with the help of a machine.  Today we are jumping through the redtape of insurance in order to get the machine shipped to our home.  Hopefully it will arrive tomorrow, but until it does…we have to stay in the hospital.

Other than that, Nicholas is doing much better.  Hallelujah.

Mr. Chubby Cheeks.  Obviously eating is not a problem!

Mr. Chubby Cheeks. Obviously eating is not a problem!

Getting Closer to An Underlying Diagnosis…

Many people have asked (mostly through our Facebook page) what his underlying pulmonary issue is.  The answer is we still don’t know for sure.  We are getting closer, though.

As it stands right now it appears Nicholas may have a rare, genetic condition called Primary Ciliary Dyskinesia (PCD).  In short, it appears his cilia–the tiny hairlike structures that line the lungs and airway and help move mucus up and out–do not move properly.  What this means is he is at risk for airway obstruction when mucus gets stuck.  Hence the need for percussion therapy.

What this also means is when Nicholas gets sick and there is more mucus than normal he is at risk for respiratory distress.

That is what caused our most recent hospitalization.  Nicholas contracted a virus –>The virus caused the creation of more mucus–> The mucus blocked his airway–>and because he couldn’t get air to his lungs his lungs collapsed.

PCD is a lifelong, genetic disease that will improve as Nicholas grows and his airways get bigger.

Last week we took Nicholas to Rainbow Babies Childrens Hospital in Cleveland to meet with the chief pediatric pulmonologist, Dr. Ben Gaston.  Dr. Gaston drew blood from Nicholas to test for the genetic markers of PCD.  We will have the results in a few weeks.  In the meantime, we are treating Nicholas as if this is his diagnosis.

So, that’s where we are at.  It’s not a great diagnosis.  It’s not the worst diagnosis.  We are choosing to look at the diagnosis through a half full cup.

Which, brings me back to the gift of perspective.  Would I rather not have experienced a pregnancy with someone else’s baby?  Sure.  Would I rather Nicholas not have PCD?   Of course.  But, there are people in this world who have much larger crosses to bear.  That doesn’t mean I can’t feel sorry for myself and my son.  We are all entitled to have our moments of sadness and grief.  It’s just my way of dragging myself out of those moments of self pity.

We all have unwelcome curveballs thrown at us in life.

This is how I’m dealing with mine.