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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.

Plowing Through – Our Latest Update

AUTHOR: | POSTED: 11/30/14 1:50 PM
CATEGORIES: Blog

As most of you know this isn’t our first trip to the NICU.  We’ve had a 30 weeker (Ryan);  a 32 weeker (MK); and a 37 weeker who acted like a 34 weeker (Isabella).  You’d think after a track record like that I would have been a little more realistic about the odds of us ending up with a NICU admission for Nicholas.

I wasn’t.

I don’t know where my cockiness came from.  I guess I thought God was throwing us a bone with the entire pregnancy.  Given everything we’d already been through I figured we somehow earned a healthy, uneventful delivery.

Am I ever going to learn that life really doesn’t work that way?

There is no score card getting punched for every challenge someone faces.  There is no cosmic being magically balancing the strokes of good fortune with the hard stuff.  God doesn’t sit up on a thrown deciding who gets sick, who gets to face unimaginable loss, and who gets to breeze on through with minimal hiccups.

We all live in a world that we cannot totally control and sometimes we pull the short straw.

The trick is in surviving the short straw.

Over the course of my 45 years I’ve learned a few things about how I operate under stress.  First off, I know as much as I don’t want to be, I’m snippy.  My patience is almost nonexistent which requires me to apologize…a lot.  I also know that the peripheral crap I normally occupy my mind with gets dusted when I’m under duress.  The need to decorate my house to perfection for the holidays…gone.  The need to meticulously dress my children…ditched.  The need to straighten my frizzy head of hair…whatever.  Is it clean?  Are my teeth brushed?

Fine.  Done.

The rest of the journey consists of an approach that requires me to lower my chin to my chest and plow on through.  I comfort myself by knowing that a year from now, no matter how this turns out, I will be in a different place.

Hopefully it will be a better place.

*****

Nicholas is doing really well right now.  He still has a nasal cannula, but his oxygen requirements are minimal.  We even gave him a three hour stint without the cannula yesterday to see how he tolerated breathing without it.  He did well, but even though his saturations stayed consistenly healthy he looked like he was working really hard at inhaling.  So, in the interest of not allowing him to crash and burn, the cannula was put back on.

I was okay with that.  He needs a little bit more time.

The really good news is that his lung x-ray was clear this morning.  How about that?  It is the first time we’ve seen him with clear and open lungs since the day after he was born.  He’s making progress.

The doctors are more convinced than ever that this is an upper airway problem.  So, tomorrow he is going in for another broncoscopy.  The pulmonary team is going to take another look at his lungs and bronchus and then the ENT team will check his upper airway and most likely dilate it again.  (Remember, we were told that they may have to dilate his airway a few times before it actually stays open.)

There is a good chance he will return to us intubated…again.   In order to keep him comfortable they will have to sedate him during the intubation.  I hate that,  because he’s been so alert and eating so well.  When he’s sedated it’s like our baby boy is gone.  But I understand he has to be sedated while he’s intubated in order for him to be comfortable.

Double edged sword.

Please keep Nicholas in your prayers tomorrow.  We need him to come through this next procedure without complication.  We want to continue down this road of healing.

*****

I’ve met a few other parents in here and I’d like to ask you to pray for their children as well.  The first is baby Samantha.  She is undergoing open-heart surgery (her second in five weeks) tomorrow.  Her mama is scared.  They need our prayers.

Also…

Please pray for baby Emma and baby Madalyn.  They are twins born at 29 weeks after suffering from twin to twin transfusion syndrome.  Emma is a little more fragile than Madalyn.  Hopefully with a lot of prayer and expert medical care their mother, father and big brother will have them home…healthy and happy.

*****

As always, Sean and I are so grateful to all of you who are holding Nicholas and our family in your thoughts and prayers.  We will really need them tomorrow.  I will update our Facebook page in the afternoon.

Uncle Andy came in from Tucson to meet Nicholas last week!

Uncle Andy came in from Tucson to meet Nicholas last week!

Papa also came along for a cuddle.

Papa also came along for a cuddle.

And...drum roll...our first official family picture.  Not to shabby.

And…drum roll…our first official family picture. Not too shabby.

An Update and A Statement about How Much You Are Helping…

AUTHOR: | POSTED: 11/25/14 6:49 PM
CATEGORIES: Blog

Nicholas underwent a broncoscopy and laryngoscopy this morning.  This was his second time being “bronc-ed” by a pulmonary team but the first time an ENT team joined in for a good look at his upper airway.

Here’s what they found…

Pulmonary Findings

The pulmonary team found nothing abnormal.  His bronchus, lungs and all other parts of his pulmonary system are anatomically normal.  They were able to biopsy some cilia (your lungs are lined with cilia…little hair-like fibers that help move mucus and foreign matter out of your airway).  The results will be available in a couple of days, but they were able to confirm that they were moving…which is good.  (One of the possible diagnosis was a disorder of the cilia and although most ciliary disorders are manageable, they are debilitating.)

So…in a nutshell…the pulmonary team found nothing remarkable.  (Same finding as the pulmonary team in Toledo.)

ENT Findings

The ENT (ear, nose and throat) team did find something abnormal.  Nicholas’ airway right underneath his vocal cords was narrowed by more than 50%.  What that means is every time Nicholas tries to inhale, he has to exert 16 times more force than normal.  That’s a lot of work for a seven pound baby.  It also explains why Nicholas uses his whole upper body to inhale resulting in a lot of shoulder movement and a head bob.  Even better…it could mean that he hasn’t been able to take a deep enough breath to fully inflate his lungs.  Hence, random wandering lung collapse.

In order to correct the narrowing the physician inserted a breathing tube to manually remove the scar tissue that has formed around the opening of his airway.  He called it dilating the airway.  He showed us before and after pictures.  The after picture definitely showed a widened airway.   This is good because if Nicholas was born with the narrowing, it could explain all of this.

Now, before anyone gets too excited, there is a catch to this theory.

Nicholas was intubated last week for 36 hours.  During that intubation period he was very, very agitated and couldn’t stop thrashing about.  It’s possible the tube running down the back of his throat irritated the airway and caused swelling…which caused the narrowing of the airway that was found today.  If that is the case the reason behind the random wandering lung lobe collapses is still unknown.

We won’t be able to confirm anything until Nicholas is extubated and begins to recover.

A Few More Details…

  • Much to our dismay Nicholas returned from the operating room intubated.  He will remain intubated and on a vent for at least 24 hours (and three doses of steroids to help prevent swelling of the airway.)
  • He is comfortable, however.  Last week he was only mildly sedated during his 36 hour intubation.  Mott does it differently.  He is currently more adequately sedated to keep him calm.  He looks like a very peaceful sleeping baby burrito.
  • We will be repeating the laryngoscopy next Monday to remeasure the airway that was dilated today.  We have been told that it may be necessary to re-dilate the airway multiple times before it actually stays open.

Tomorrow Will Be Scary…

Last Friday, when Nicholas was extubated and had trouble breathing afterwards, was probably one of the most stressful days of this journey.  Seeing as how we are facing a second “extubation day” there is a lot of anxiety on our part.  Things *are* different this time.  We already have the steroids on board to prevent swelling and stridorous breathing, but there will be a period of the day where the sedation will be lifted in order to extubate him properly (he needs to be awake enough to breathe on his own when extubated).  That will be a rough couple of hours because he will be very uncomfortable.  That’s hard for us to watch.

What You Can Do for Nicholas…

As you know Sean and I have begged everyone and anyone to keep Nicholas in your thoughts and prayers.  We believe in positive energy and divine intervention and are convinced it is working (in conjunction with some pretty kick-ass science) to cure our little boy.  Tomorrow we need to storm the heavens with more prayers for a smooth extubation without complication.

So, if you wouldn’t mind….we’d be most appreciative.

Oh…and About Your Unwavering Support…

I want to thank you. You know, for years I’ve watched pleas for prayers and positive thoughts come across my various social media feeds.  I don’t think I’ve ever scrolled by one without commenting and throwing up a small prayer for the person in need, but I’ve always wondered if it made a difference.  Now that I’m standing on the side of the begger, I can honestly tell you that reading your comments really does provide a tremendous amount of comfort.  I would equate it to a reassuring hand squeeze…over and over and over again…and it helps.     It lessens the feeling of being alone in this.

For that, I am thankful.

Our sleeping baby burrito.

Our sleeping baby burrito.