ABCs

Have you ever caught yourself visually tracing the contours of your child's face in the wee hours of the morning while they slept?  No?  Me, neither... well, until Monday morning.  Who has the energy to wake up before the kids and watch them sleep?  Not this girl.  However, Monday morning, I could hear Ellie jabbering through the monitor before 5am.  By the time I made it up to her room, she was sitting upright unraveling her pulse ox probe and had turned off her cool mist machine.  I scooped her up, changed her diaper, and headed back to bed hoping to get her back to sleep in my arms for a while.  She fell back asleep quickly, I, on the other hand, started reeling through what my day held... and couldn't sleep.  At some point, I stopped daydreaming and looked down at her sweet face, snoozing away in the crook of my arm.  I whispered a prayer thanking God for this crazy little girl that I never knew I wanted.  I mean... I wanted a girl... I just never thought I needed one so wildly different.  But she's changed every ounce of who I am and in that early morning moment, I was eternally grateful.  I'm glad I had these few minutes with her as just a few minutes later, I was alerted of school closings and soon realized that a day of chaos was about to ensue.  On to the topic I really want to lay out, though...

Elz was originally diagnosed with severe obstructive sleep apnea when she was just under three months old.  Since then, she's had nine sleep studies and a boatload of procedures to help this and fix the myriad of other wonky things she had/has going on.  First, there was the nasal cannula to increase oxygenation, then the trach and ventilator.  Then we weaned off the vent and onto bipap, then onto just a cool mist collar around her trach at night.  We still need oxygen bled into that line a few times a week, and she still sleeps with a pulse oximeter on her foot to make sure we know when her oxygen saturations dip lower than we want them to.  Her saturation goal is 90%.  Have you ever had an oximeter reading of 90%?  I haven’t had mine taken often, but when I was really sick and felt like I had a bag of cement on my chest, my reading was 93% in urgent care and they gave me an albuterol treatment that finally made me feel like I could breathe.  So a saturation of 90% feels awful to the rest of the world… but Buggy has always been a bit different.  When she was little, her goal was to stay above 70%... but that was a cardiac thing and we’re not living in that world anymore (thank goodness).

Anyhow – back to my story – her sleep apnea was always an issue, but it didn’t take center stage until after we tackled the most life threatening issues she had.  Raise your hand if you’ve taken a CPR course.  How many of you remember that you had an acronym to remember the priorities you check (okay… this is before the hands-only CPR method… I’m old school)?  You checked your ABCs, right?  Airway – Breathing – Circulation.  For Ellie… her ABCs have been jacked up from the start.  We addressed them in reverse order… but we’ve addressed them all.  Circulation trumped all others in her case, so we had those issues fixed in her first 9 months of life.  Breathing was the next hurdle… and we were pretty sure we had cleared that one once we were able to put the ventilator on standby.  And airway, she’s had a ridiculous number of surgeries to stabilizer her airway and has had that handy dandy trach in place for three years as a crutch until we get to a solid place for her airway. Once we have her airway on solid ground… I feel like we’ll smash A and B out of the park.

Now that we have some background, let’s jump into the present.  Ellie had a sleep study done in mid-December where she slept with a capped trach.  So all of her breathing had to happen from her mouth/nose while she slept (remember that she hasn’t had to do this since she was two months old).  We made it through the whole night and anxiously awaited the call from her specialist to give us results.  They hadn’t rushed in through the night and uncapped her, so I knew this sleep study had gone better than her last.  I was cautiously optimistic.  Her ENT called me on Monday to go over results.  Here’s where we’re at now:

•       Ellie’s shown marked improvements in her ability to breathe while she sleeps.  In the past, her apnea-hypopnea index was in the 60s (double the threshold for severe sleep apnea).  Now, it’s measuring 26.  So she’s only half as bad as she was in April 2017.  That’s good.  But 26 isn’t great, either.  We’re getting there.

•       Ellie had no episodes of complete obstruction – this is fantastic – removing three tonsils and her adenoids and some tissue from the inside of her trachea has helped tremendously here.

•       She had 1 episode of central sleep apnea (this is where the brain forgets to breathe).  Sounds awful… but 1 episode isn’t bad.  The crap part about CSA is that there is no way to fix it… it has to resolve on its own.  Her doc wasn’t concerned about this, though, so neither are we.

•       She had 231 episodes of incomplete obstructive apnea.  Ugh.  So 231 times during the night, she stopped breathing.  That’s roughly once every two minutes that night.  The length of her apnea episodes lasted anywhere for 17 seconds to 44 seconds and dropped her oxygen levels between 82-89%.  This means that she's still in the "severe obstructive sleep apnea" range.  Frickety frack.

Needless to say, with results like those, this trach isn’t going anywhere right now.  When she was inpatient last week, we also got a new diagnosis of reactive airway disease (RAD) [think: asthma].  So we’re kind of bummed out about it all.  It’s not horrible.  We’re realistic… and we’re used to this trach life… but I’m not over here throwing a party, either.

Her doc said, “How do you feel about this, Mom?  What is your breaking point for the timeline on the trach?”  Ha!  I told him that we’ve had the stinkin' thing for three years now, so it’s not like it’s anything new and it’s not like we have a huge adjustment to keeping it.  And while, OF COURSE I want it out… I fully realize that she has to be able to breathe AND sleep… so we’ll hang out here for a while longer.  He said we’re probably looking at a solid year yet – maybe two.  And in response to his question about my breaking point, I said, “Right now we’re okay.  We have a plan that works.  She has the care in preschool and home that is good for her and a staff that’s very capable of taking care of it with us.  I really don’t want her to go to kindergarten with it, though.  That’s 2.5 years away and I REALLY don’t want to do that.”  Of course… if that day comes and we’re still in this position, we’ll do it… but I’d rather not.  He said that seemed pretty reasonable and we spent the next long time talking about the things he thought might be causing distress still… and interventions we could work on to address them.  Some things we can do in the next year:  a sleep endoscopy, a tongue resection (if needed), fixing the suprastomal collapse in her trachea.  Some of the ideas (if they’re the culprit) are things she’s just going to have to outgrow: a small nasopharynx and tracheomalicia.  So from here, we plan to go back into the OR for a sedated sleep endoscopy to help evaluate her airway during sleep with a camera.  This would help us determine the next steps… and we’ll have this scheduled sometime in the late spring/summer time frame to keep this trached kiddo away from the hospital if at all possible during cold/flu season. Dr. Elluru (her ENT & surgeon) is also bringing in Dr. Kalra (pediatric sleep medicine) to help handle our case.

During our stay in TCU last week, along with the RAD diagnosis, we were referred to work with our pediatrician on getting answers to some new questions on storming, too.  We like to keep Dr. Smith busy, I guess.  So we’re diving into the possibility that storming (in Ellie’s case) may be related to cyclic neutropenia.  This would mean that Ellie’s white blood cell count would dip in a cyclic fashion… and that, in and of itself, would throw Ellie into a storm and also make her SUPER susceptible to sickness.  This might make sense… but is all new to me.  We’re bringing the Infectious Disease team on board to see if they have any suggestions or ideas about this. 

For now, we’re just taking things as they come and bouncing around to see if we can come up with plans and such that make sense for us… that make sense for Ellie.  I can’t say enough how fortunate we feel to have a team that is crossing all Ts and dotting all Is as they try to do what is best for our Bug.  She sure is a complicated little booger.

And for following along through my vomit of medical jargon, here are some recent pictures of our crew.  Thank you for continuing to care, for continuing your prayers, and for drudging through this weirdness alongside us.  We love you! 

Some light reading as we checked in for the sleep lab

Sleep study #9:  I'll never understand how anyone can actually sleep like this.

Have you tried Kiwi Crate?  It's super cute and your kids can do projects each month.  This month, the boys made a stethoscope (that works), and sewed these plush organs to put on their body... and made glow in the dark x-rays.  Cool stuff!

Buggy on Christmas - it's not often we get a still shot of this busy body

Dressed as Moana, holding Moana, watching Moana... can you tell it's her favorite?

Christmas with great grandma Shuster

Christmas with great grandma Ward

Big cousins are the best - Andrew cuddling with Kaleb

Please tell me that other people's kids steal their phones to take silly face selfies while going potty.  He took about 42 photos and 3 videos of him singing me songs.

Grandma & Grandpa Schroer on Christmas Eve (at grandma Shuster's house)

Mamaw and Papaw Maier on Christmas

Ellie decorating her hospital bed

And watching Moana... for the 8th time in 3 days.

Papaw Ward & Stacy visited for Christmas, too!

We got a movie projector for Christmas.  Best. Gift. Ever!  Now we have movie nights and project a 12' screen on the wall.  So worth it!

Mook was drawing pictures in the lobby of Dr. Smith's office.  He said, "These are birds.  This is a hurricane."  I said, "Like two sparrows in a hurricane?" and chuckled as the song played in my brain - yeah, Tanya Tucker!