4th Day of Christmas: Puzzle pieces of storming

At our last cardiology appointment, Dr. Luby said that since there were a few changes in Ellie’s storms in the last few months (what I’d describe as seizure-like episodes), she put in a referral to Neuro at Dayton Children’s.  She wanted to make sure Neuro was on board with what we were doing and since Ellie hadn’t seen a specialist in Neuro since she was 2 months old, I completely agreed with this idea.

So earlier this week, we went to see Dr. Kumar for the first time.  To explain Ellie’s storming, I had to recap her history back to when she was six weeks old and began all of this.  I explained the manifestation of storms at that time, what worked to calm them, and what state Ellie was in at the time.  Then I progressed through the changes in storms that coincided with the changes in Ellie’s health status… the medications involved for maintenance, prevention, and rescue.  We went through triggers, changes in manifestations, and theories that were thrown out all along this journey.  Then I showed video of the specific times when Ellie’s body shook (what I was thinking may be a seizure) in the midst of her storming.  I also brought up the photo I have of Ellie’s MRI and told her the results of the multiple EEGs we’ve had.  See, y’all, this is why I forget where I’m going sometimes… I have all this other mumbo jumbo in my brain (alongside the lyrics to most Salt-N-Pepa and 504 Boyz songs). 

She’d also collected a brief family history.  Once digested, she said, “I don’t think these are actual seizures here” (pause for a HUGE relief), then she went on to ask about other family history that I maybe didn’t mention in depth.

“You have migraines, right?  Do migraines run in your family?”  - YES (a big ol’ yes, that is)

“And you said fainting spells, too, right?”  - YES

“What about circulation issues?”

“Like Raynaud’s?  Yep.” I said.

She explained that the migraines, fainting spells, and Raynaud’s are all physiological signs of dysautonomia.  Dysautonomia can be genetic and can manifest in many different ways.  I’m sure the list above isn’t all encompassing.  She said that Autonomic Storming, or Autonomic Dysfuction, is another form of dysautonomia, but one that is almost exclusively caused by a traumatic brain injury.  We did know that.  However, she said with a strong family history of dysautonomia, she thinks that Ellie is having “an elaborate physiological response”.  So she takes this dysautonomia thing to the extreme (imagine that) and it presents as autonomic storming. 

She said it could be cardiac related – meaning her getting upset may be triggering something cardiac-related (though likely nothing scary) that then mushroom clouds into her autonomic system and sends her into a storm.  As crazy as that sounds… this is an explanation that finally makes sense… for the first time in over 38 months of storming, we have something that makes sense.  However, I told her that I was not telling my husband what she said because he’ll blame it on my faulty genetics.  Haha.  I have plenty of people (men & women) in my family that have suffered though unimaginable migraines… have several that experience fainting spells, and several that have Raynaud’s, too.  

Sooo… we already knew that there’s a 95% likelihood that it was MY extra 21^st chromosome that was given to Ellie… and Down syndrome is to blame for the heart defects… and now it’s almost certainly my fault that she storms… uhhh… I’m still in the running for mother of the decade, right?  Ha!  And Ellie’s dramatic, you say?  Ahhh… that cannot come from me.  Hehehe. 

So there you have it, folks – the first reasonable explanation for Ellie’s storms.  It boils down to me having fabulous genes that I’ve so graciously passed along and Ellie being a drama queen.  I like this, I really do.  Seems fitting!

All joking aside, storms still suck.  But at least we know it is unlikely that they are doing her body harm (as long as we control them quickly).  Dr. Kumar did suggest that maybe we need to go back on the beta blocker as a regular med instead of having it to dose out when a storm starts.  This would keep her from storming altogether (we hope).  She’s punting that call back to cardiology because it is, after all, a beta blocker.  We use propranolol, by the way. 

I would like to see the day when storms are no longer an issue.  We had about 15 months (between Boston and October 2016) when we did not have storming episodes.  Her first storm after that dry spell was epic and for the first time, she knew sign language.  The sight of her little body moving helplessly in that hospital bed, crying, and signing “all done” is forever burned into my heart.  There’s not much that prepares you for something that awful.  But once we recognized what was going on, we put a plan in place to treat as needed… and we’re moving on with that.  With this new explanation of Ellie’s storming (I call it “Ellie’s” because it’s unlike other storming), I kind of feel like these are things we’re just going to deal with.  I imagine that as we enter big changes in her life… like say, puberty… the manifestation could change again.  Who has energy to worry about something that far in advance, though?  Not this momma.  We’ll see what cardiology says about a daily dose of propranolol and we’ll go about our happy way… adding Neurology as eighth on the list of specialty clinics we’ll visit regularly.  Meh… could be worse.

And just like that, we’re a third of the way through the Days of Christmas posts.  And wouldn’t you know it… the more I write, the more I want to write.  But y’all… I’ve got to get some stuff done today.  I woke up extra early to crank out a post before I started working.  Have a wonderful day and thank you for following along. 

Ellie's only brain MRI - she was 3 months old.  She that pocket of fat on the back of her neck?  That's call a nuchal fold and it's another "soft" marker they use to determine if an unborn baby has Down syndrome using ultrasound.  This is the only image I have of Ellie where you can really appreciate it.

This is a picture of Ellie FINALLY sleeping after her 1st storm.  She was six weeks old and had been up all night frantically moving around, sweating, and had a high breathing and heart rate.  She also drove up her temp that night... but we didn't chart temps the first night.  After five hours of this "fit", she fell asleep as I packed our bag for the ER.  Luby had called me back and said that Ellie couldn't handle that kind of stress and I needed to bring her in.  We held off once she slept, but ended up in the ER the next night for the same thing.  We'd go on to have storms DAILY... through the WHOLE night until she went on propranolol at 11 weeks old.

These last pictures break my heart, but I'm going to let y'all in on the ugliness of storming, here.  These are from an episode about a year ago where Ellie started to storm and it went ugly fast.  In less than an hour, her body temperature was right at 104 degrees.  She was miserable and, as you can see from her pulse oximeter, sitting in the mid-80s in oxygen saturation (even with a few liters of O2 running) and her heart rate was in the 160s (it's usually around 100-110 when she's awake).  The picture just below it was taken about an hour after propranolol took hold.  The difference is amazing!  Storms usually wear her out completely because her body is essentially doing a crossfit workout for an hour or so, but this time, she was awake and happy afterwards for a bit - then went to bed for the night.  So this yuck picture, we see this regularly.  When she's sick, we see it daily - we hold her like this (sometimes with tears streaming down our faces) until the medicine takes hold and calms the storm.  Poor sweet girl.

 84% saturation while on oxygen... and a HR of 163... you can't see the temp of 104.

Is it any wonder why we're always amazed by her?

But she's resilient and bounces right back to her happy self afterwards.  And see... I complain an awful lot about my migraines.  #perspective  

Am I right?

On a super happy note (cause we can't end a post with a bummer), this is what we're trying today... skivvies.  She's a long way from being potty trained, but we're seeing how she does in these.  They were just too cute!

3rd Day of Christmas – Home Health Care

I really wanted to write about these folks last, but I just couldn’t wait.  When we found out we were headed out of the CICU and down to the TCC (Transitional Care Center) in Cincinnati, we were told that we needed to hit the ground running for home care.  We had no idea how much of a pain in the butt it’d be to 1) find a company that could support a child with a trach & ventilator w/in our insurance network, and 2) find one of those rare companies that met the 1^st requirement and actually had available nurses.  YIKES!  Literally, we spent three months working this out and only came home with 40 of our 110 approved hours/week covered.  It’d be almost five more months at home before we’d be able to fill in most those precious nursing hours.

When our nurses in the TCC spoke with us about home health hours, I told them how scared I was to have someone awake in my house around the clock.  Someone awake while you slept.  Someone in your home while you were gone.  That takes a ton of trust in and of itself… let alone adding the stress of them caring for your very fragile child and handling situations in a home setting that would send everyone else to the ER - all without losing your mind.  This was one of our biggest hurdles in Ellie’s care.  So let’s dive into the story:

3/20/15 – After months of work and tireless training, weeks of delays from insurance and home care companies, and meetings with a medical supply provider, we were finally approved to go home with Ellie.  We had already contacted our utility companies to let them know we had a vent-dependent child in our home and we made contingency plans for power outages.  We’d made calls to the Sheriff’s office and local emergency response teams to make sure they knew our situation, as well.  We had a plan in place if we needed to be squaded out and had more medical equipment in our small home than exists in some countries.  We were ready-ish.

3/21/15 – After sleeping in my own bed for the first time in four months, I was awake early to meet our 1^st home health nurse – Rae, from Highpoint Home Health.  My anxiety was in full force as she came through our door, looked down at Ellie, and tried her best to have some sort of composure as I trained her.  After about two hours, I was sobbing in the kitchen because I just couldn’t let someone else do this.  But I had to.  I needed to work – I needed to care for the boys – I needed a chance to take a break.  A while later, I told Rae about this breakdown I had in the kitchen and found out that she walked through our door, saw Ellie’s coloring and her oxygen saturation sitting around 70% and thought, “these people should not be at home”.  So on that first day, we each had our own private moments of panic.  She kissed a picture of Ellie every night and prayed that she’d be okay in the morning – then prayed all the way to work each day that Ellie would make it through her shift.  I’m telling ya, those first few months at home were scary!

We’d go on to have Rae in our house for 5 days, then have 2 days off, then 12 straight.  On her days off, we’d have subs fill it.  This was hit and miss.  We had one nurse that never showed up when she was on the calendar… and one that I had to call the company and say, “Never send that woman to my house again!”  She was inappropriate on all levels and should NEVER work in peds.  Fortunately, we had Rae.  We loved Rae.  She was always there for her shift, she played games with the boys, she worked on sign language every day, and she was overall a lovable person!  Earlier this year, as Ellie was getting closer to decannulation, Rae and I had many talks about her future.  In home health, when you’re taking care of a vent-dependent child, it’s rare that the situation has a happy ending.  And after experiencing many other tragedies, Rae wanted to get out of home care with a positive experience – Ellie.  So she moved on last spring to another job.  We miss her, for sure!

I think this is my favorite picture of Ellie & Rae.  Rae came to see Ellie in the hospital when Ellie was fighting a nasty infection.  We have a lot more pics below.

After that first day, and as we got more and more comfortable with having other people in our house around the clock, other concerns came up that we didn’t think about before.  If you have to yell at your kids – someone else is in your house.  If you fight with your husband (or God forbid, have any intimacy at all) – someone else is in your house.  If you want to go out with friends or family and do not want to leave Ellie at home, you’re bringing your nurse along.  They're there when your kids open gifts from Santa... as they do their egg hunt on Easter.  They hold your child while you watch fireworks on the 4th of July and through the craziness of family get-togethers.  You spend more time with nurses than with your spouse or any other family member.  They become family.  We’re used to this now – but it was a big adjustment.  There is no walking through your house in a towel and if you don’t sleep fully clothed, you’ll have to adjust for that before walking to the bathroom or answering that knock on your bedroom door in the wee hours (don’t judge me, folks).  As my friend, Julie, put it, "sooo... you can't walk around braless?  Deal breaker."  Haha!

About a month and a half later, we were finally able to hire another nursing company.  We now had a full time day shift nurse and a full time night shift nurse – each working for a different company.  Gone were our days of taking turns laying on the floor/couch next to Ellie as she slept.  We were finally able to sleep in our bed again.  Danelle was the only night nurse we’d ever had.  She came highly recommended from a family we knew and upon meeting her, I had no doubts that she was the perfect, trustworthy person to have in that position.  There were nights when Ellie would wait for Danelle by the back door, falling asleep there, even.  If Ellie was awake when Danelle would come on shift, Ellie would light up when she heard that door open.  She’d clap and slide off my lap, throw her hands in the air for Danelle to scoop her up.  Ellie loved Danelle (as did we).

I'm totally bummed that this is the only picture I have of Danelle & Ellie (although I love this shot)... I guess that's what happens when the shift is 10p-6a and I'm ready for bed.  They're watching fireworks here.  Sorry, Danelle.

It was at about this time that I started to realize that for as much anxiety I had going into this arrangement, nurses had at least as much on their end, too.  They walk into very questionable family situations and all levels of cleanliness.  They care for families who have the full spectrum of abilities and sometimes very little hope for a positive future.  Their job is hard, y’all.  REALLY hard.  But they continue to care, they continue to love, they continue to push forth despite all odds because they love what they do.  This has been our experience, at least – we’ve had an amazing experience with home care (aside from the few no-shows and impossible personalities that I had to call and take off our schedule). 

In August of 2015, we still had a part-time shift open on our schedule and if we didn’t fill it soon, the state would likely strip those hours away.  We needed some evening help and we needed some weekend help.  Ellie simply couldn't go out of the house for months on end, and because our two covered shifts covered bedtime and my work day, I would have to go grocery shopping in the middle of the night if I couldn't bring Ellie.  I reached out to our care manager with the Ohio Home Care Waiver and had her place an ad for private duty nurses.  This was especially scary.  Private duty nurses do not have a supervisor or company you call if things aren’t going well – I’d have to call our care manager directly.  I looked through several resumes until I found one that looked promising.  The nurse’s name was Olurin Olaleye.  I scheduled an interview and waited for her – Rae and I had prepared questions for her.  When a man walked up to my door, I was a little shocked.  He introduced himself and said, you can call me David.  Whew!  Okay.  David is originally from Nigeria and has a thick accent that was hard to muddle through on my end during the interview.  We were impressed, nonetheless, and David was hired.  Our boys were fascinated with his accent and his deep, dark skin.  They’ve asked many innocent (albeit uncomfortable) questions over the years – usually relating to his skin color or asking him to speak in his native tongue – which is amazing!  Ellie absolutely adores David, too.  She pats his head and dives into his arms.  She plays soccer with him and pushes his buttons with her ornery games.  Finally, we had filled 101 hours/week and we had a full staff – a regular staff.

David helping Ellie with her gingerbread house last year.

We’d have this same staff of three nurses (yes, we know how incredibly lucky we are in this regard) for about 20 months.  This is when Rae was headed out of home care and we’d be transitioning a new nurse into her position.  First, there was one who came part time and was absolutely terrified of Ellie’s complex needs.  In all reality, Ellie is MUCH less complex than she had been a few years ago, but this poor woman was stressed to the max on a daily basis.  Keeping up with Ellie was not what she was used to.  That nurse fell through and we’d wait a bit for Rae to train a new nurse.  When the new nurse came to the door (earlier than Rae that day), I opened it and said, “Hello, this is Ellie” as I showed her this two-year-old wrecking ball.  Later that day, Rae said that this new nurse, Melody, knew she was walking into a peds case, but had no idea how old the patient was or that the patient had a trach.  Nice surprise, I’m sure!

So, late this spring, Melody started at our house.  Ellie didn’t take long to warm up to her.  Melody works with Ellie on words, letters, numbers, and colors, helps her with sign language, and helped her with therapies until we were through Early Intervention.  Melody is my sanity most days – poor Melody – and she is absolutely amazing at making Ellie’s hair look adorable.  Seriously, if you see Ellie on a weekday, that cute hair is not a product of my talent – that’s all Melody!  Ellie’s mornings are full of laughter upstairs and she’s showing Melody just how sassy she can be some days.  The boys adore Melody, too… as they have all of our regular nurses.

Melody helped me (and by that I mean she did most of it) get 

Ellie ready for her preschool Halloween parade.

This journey is far from easy… having people in your home every day has challenges, certainly, but there is much beauty on this road, too.  We have had bumps along the way – near misses and close calls – trips to the ER with a nurse in the back next to Ellie keeping close tabs on her.  Days when bad news pours out of my eyes and down my cheeks and these nurses try to put me back together.  Trips to countless clinic visits where the doctor extends a hand and says, “and you are?” to my sidekick.  And then there are the looks in public I get when I’m out with the kids and David… yeah, shut it public... assume whatever you want!  While I look forward to the day when we no longer need nurses in our house, I truly love all of these people dearly and know that they’ve made an incredible impact on our lives.  They make my job possible (like… my actual job, but my parenting, too).  They make sure that Ellie gets the things she needs – attention, therapy, play, medical care, personal care – each and every day (well, except Saturdays), and I’m eternally grateful.

Don’t for a second think that I’m forgetting our newest nurse… but you’re all already super familiar with him.  Yes, in July, when Danelle left to pursue a career at Dayton Children’s (woot, woot - excited for her), Brandon filled the shoes of our night nurse.  He went to work for the company that Danelle worked for (Maxim) and adjusted the hours to work with his already busy schedule.  This meant that Brandon would now be doing more of the care than before (because, of course, Danelle was doing a ton).  Ellie happens to love her time with Daddy each week, too.  This also freed us up to stay out later some nights – since we didn’t have to be back home before 10pm.  And let’s be honest – I no longer have to worry that someone is going to knock on my bedroom door at 3am and I’ll answer in my undies and a t-shirt.  That’s a win, right?

How can she not love her newest nurse?

Thank you for sticking with these long posts… I’m hoping that Ellie doesn’t have as many caregivers as she gets older, and I know that when I look back on these 12 Days of Christmas posts, I’ll again remember exactly how big this army is that cares for her.

Let’s not forget the other staff that keeps this all running:

From High Point: Tiffany, Sarah, Carol, Kathy (this is who Rae and Melody work(ed) for)

From Maxim: Janet, Linda, Charles, Jen, Jennifer (this is who Danelle and Brandon work(ed) for)

Ohio Home Care Waiver: we’ve had several Care Managers through the years, but Jolene is taking care of us now and she’s fabulous.

Our pediatrician and specialists also work closely with home care to make sure we have all that we need.  Before we’ve even touched our DME, we’re already talking about at least fifteen people involved in this chunk of our care team.  And they all need updates on any and all changes in Ellie's care/status.  That alone make discharge planning rough on me.

Rae, Lance, and Ellie on the tablet at our old house

Ellie had just learned to sit.  Rae is admiring her progress!

 David and Ellie cuddling

 Rae & Ellie at a Halloween party last year

Melody and Ellie after lunch this summer

David taking Ellie on a 4-wheeler ride

David came to Children's one day to feed Ellie lunch and give me a break

Melody came, too... giving me some much-needed company.

A two-year old is much harder to handle in a cage/crib than a baby.

Ellie is learning how to use all of this thanks to her nurses.

Checking out David's stethoscope 

Daddy doing Ellie's bath, care, and hair on his first week on the schedule - of course this was NOT his 1st time giving her a bath or drying her hair... it was just too cute not to photograph!

2nd Day of Christmas – Pre-baby support

So I know I said I’d focus a good deal of these posts on Ellie’s care team, but the folks in this post were both Ellie’s team and my team, before Ellie was here.  They took amazing care of me and supported our family through a very stressful pregnancy.  I actually wrote about most of these folks in our very first blog post.  Since that’s been over three and a half years, it’s not a terrible idea to shine some spotlight their way, again.

After three years of hearing out other families and listening to hundreds of diagnosis stories, I’m even more grateful for this team than I was before I had Ellie.  Our diagnoses were given gently and with compassion – not to mention a very real sense that the doctors acknowledged that this was our BABY… and we loved her dearly.  We were never encouraged to terminate our pregnancy – but I’ve only met one other family who had that same experience.  This makes my heart hurt.  Just last month, the Ohio House passed legislation to ban abortion because of a Down syndrome diagnosis.  This is incredible, really!  While it likely won’t change how a parent feels about an unborn child, this bill will likely change the language used by medical professionals when they deliver such a diagnosis.

The diagnosis of Down syndrome, in and of itself, was hard to swallow during pregnancy.  If I had known just how hard our road would be once she arrived, I don’t imagine that I would have enjoyed my pregnancy as much as I did.  However, this team I’m going to recognize moved mountains to give us the best care possible.  We had a ridiculous amount of appointments between 18 and 40 weeks in my pregnancy… but we’re thankful for those crazy weeks and the attentiveness of this team.

1)     Dr. Dev – OB – Sidney

I vividly remember getting the call from Sasha (a nurse in Dr. Dev’s office) asking us to come in to discuss our quad screening results.  He went on to inform us that the results came back flagged for Down syndrome (we had a 1 in 93 chance instead of the 1 in 277 chance they expected for my age) and that we could do a non-invasive blood draw on me to determine with 99.6% accuracy if our child did have Down syndrome.  After ten long days, we were brought back in to discuss again.  Brandon and I were a mess of emotions, but Dr. Dev came into the ultrasound room and said, “Do you want to know the sex of your baby?”  “Of course”.  “Well, you’re having a girl… and it does look like she has Down syndrome.  Let’s take a look at her.”  He went on to do an incredibly detailed ultrasound.  When we heard her tiny heart beating, we completely lost it.  From here, we’d be referred to the next on our list and continue through our pregnancy with appointments both with Dr. Dev and Perinatal Partners.  Dr. Dev would finally release us from his care when I was 37 weeks pregnant.  When I came back to his office (when Ellie was about six months old, he’d ask about her and marvel at her when I’d bring her in.

I’ve always been in awe of how he handled our diagnosis (especially compared to other stories I’ve heard).  A few months ago I saw him at Shelby Hills for their anniversary celebration for Shelby County Board of Developmental Disabilities.  It’s no surprise to me that he supports the board of DD.  It was also no surprise to me when I learned several years ago that he spent time at the Women’s Center in Sidney making sure that all women had access to great prenatal care.  He’s an amazing man – a sweet soul – and the guy who delivered both of our boys.  We love Dr. Dev!

2)    Maternal Fetal Medicine – Perinatal Partners – Vandalia & Dayton

As I said above, we were referred to Perinatal Partners after our 18 week ultrasound where Dr. Dev found a heart defect.  We made an appointment for a month out.  As we drove to Vandalia for that first appointment, I sank my head back into the headrest in the car as Brandon drove.  Tears burned down my cheeks and my heart thumped hard in my chest.  I knew we were going down there to investigate Ellie’s heart and I was terrified.  We met Rachel, a genetic counselor, and she gave us the phone number of Lisa, a fellow genetic counselor who had a daughter with Down syndrome.  [Little did we know we’d come to know Lisa later through the Miami Valley Down Syndrome Association and through Dayton Children’s]  After taking an extensive family history and asking a battery of questions, Rachel walked us down the hall to meet Lara, our ultrasound tech.  Lara would spend about 45 minutes checking out our Bug with an ultrasound and fetal echo.  Half of that time was spent checking out Ellie’s heart.  Lara burnt our entire ultrasound to a DVD for us to take and spent a little time letting us see Ellie’s face, too. 

Remember this cute face from a much earlier blog post?  She was cute then, too!

Shortly thereafter, Dr. Sonek came in to talk to us about our tests.  He said that there were several additional markers for Down syndrome, but we could only know for sure with an amniocentesis.  We declined.  He then told us that he was referring us to Dayton Children’s Cardiology (insert Dr. Luby from the last post) and said it looked like our daughter had an endocardial cushion defect – also called an AV Canal Defect.  I lost it in the car because I’d done my research on this defect and was now saddened and terrified with this turn of events.

We’d go on to have many more appointments at Perinatal Partners (also called MFMUG – Maternal Fetal Medicine, Ultrasound and Genetics… I think I have that acronym correct).  We’d meet Dr. McKenna (who would later be there when Ellie was delivered) and Dr. Glover (who would admit me for pre-eclampsia when Ellie was 4 days old). 

The last time we saw the staff at MFMUG, I was back for my six-week follow-up and I came in toting Ellie and her brand new NG tube.  The staff went crazy over her.  Again, another office and another fabulous experience!!

3)    Dr. Josh Steinke – Steinke Family Chiropractic – Wapak

We started to get chiropractic care with Dr. Josh the same month he opened his office (May 2012).  I was very pregnant with Kaleb at the time and had a good deal of pregnancy-related discomfort that he helped me through.  He adjusted Kaleb when he was four days old and has been there for us through the past five years of our roller coaster.  When I was pregnant with Ellie, I had regular care.  When diagnoses started rolling in, he was there to support us and pray for us.  When we brought our tiny peanut in for him to adjust, he held her close to his chest and kissed her little head.  He’s prayed over our family many times and cares for our whole family.  We really need to get in to his office more often, that’s for sure!  He’s the first person to call Ellie “delicate” and it’s the perfect description for her.  She may not always be a fan of adjustments… but Dr. Josh is gentle and accommodating for her.  He’s also great at reminding us of the things he’s keeping a keen eye on because of the common troubles associated with Down syndrome (like tiny ear canals that are very susceptible to blockage and draining issues). 

I knew I had a picture of her 1st adjustment somewhere.  :)

You can call me crazy, but I really do believe that Dr. Josh is a HUGE reason why we’ve not had issues with ear infections for any of our kids.  I also totally credit him with the difference in my labor and birth experiences with Kaleb and Ellie.  He’s a kind soul and even when I know he has so many clients, he greets each one with enthusiasm and dishes out plenty of time and attention.  Man… I really need to get the family back there… like, now!

So yeah – this is the team that cared for us during our pregnancy and gave us endless support through the unknown craziness of that time.  This prenatal care team wraps up the group I want to recognize and thank on our 2^nd Day of Christmas post.  Thank you for reading along!  I know I tend to ramble on – sorry about that.

1st Day of Christmas: Our team (cardio & pulm)

If we were to list out all of the people who have cared for Ellie (from a medical standpoint) during her first three years, there's no way we'd be able to keep track.  She's literally had hundreds of people who have cared for her inpatient and at home.  One thing that I've learned along this journey, though, is that it truly takes a village of professionals to raise a medically complex child or a child with special needs.  In our case, we get to dip into both of those worlds.  As I was reflecting recently on how sad I was that our pulmonologist passed away, I was really disappointed that I'd never gotten a picture of him caring for Ellie.  In the countless appointments we'd had with this man, I never stopped to say, "Can I take a photo of you with her?"  Fail.  So during these 12 Days of Christmas posts, I wanted to take a bit of time to recognize the members of Ellie's team who are our core care team.  I know this list seems exhausting (that's why it'll be broken up into several posts), but we're just highlighting the folks who have spent considerable time with Ellie - enough time to really know her and to know us.  I'm going to try to incorporate as many photos as I can here.  I have so much love for these folks.  And I'm going to go by specialty here, plus highlight a few people who were part of her previous care and made significant impacts on our lives.  Here goes day #1:

Cardiology:  

1) Dr. Luby (Dr. Abdurrahman) - Dayton Children's Hospital
We met Dr. Luby when we were 22 weeks pregnant with Ellie.  She's been on our care team since May 2014.  During my first interaction with her, I knew she was the doctor for us because she explained our diagnosis, then gently placed her hand over mine, looked into my tear-filled eyes, and said, "I hope that by now someone has told you that this is not your fault.  You did nothing to cause this.  You couldn't have done anything to prevent this."  She went on to tell me how she would do her best to take care of us and our little girl.  That, she has.  When Ellie was born, the hospital had some trouble with the echo machine - it wasn't able to send files directly to cardiology at Children's.  As soon as I was out of recovery and allowed to venture down to the NICU, I was rolled into that room and saw none other than Dr. Luby performing her own echo on our 3-hour-old baby.  She was there... in a different hospital... after hours... checking on our baby.

When Ellie was little, I was in contact with Dr. Luby several times a week, and called her on weekends and evenings when anything seemed "off" about Ellie.  Dr. Luby was always quick to return my calls and thorough in her explanations.  When I saw signs of heart failure, she brought us into the office right away.  When we had different treatment options, she explained them thoroughly... then said, "option A is one option, option B is a better option, option C is the best option", but always gave us the choice.  When the presented surgical plan wasn't the absolute best plan for Ellie, Dr. Luby hit the ground running, compiling test results and records... sending them with a personalized reference to Boston.  Once our records were in Boston, she called the head of cardiology daily - I'm not kidding.  She said she'd even call him from different phone numbers and each time he'd answer and say, "Hi, Luby".  He knew that she was the only one calling him daily from that area code.  When Ellie was storming more frequently, she brought us in to check her out and reassured us that Ellie was healthy from a cardiac standpoint.  When Ellie was very sick, Dr. Luby called my cell phone on a daily basis, asking about signs of heart failure, asking how I was holding up... never letting on how concerned she really was.  We didn't hear about this until later.

On Ellie's third birthday, we got a hand written note from Dr. Luby about our "feisty Ellie".  Her care for Ellie is unmatched.  We love to see her and let her fill our room with laughter.  On days when I seem frazzled, Dr. Luby stops everything and says, "Are you okay, mom?  How are you holding up?" She also asks about others who have come to the office with us - the boys, our nurses, my sister.  She cares deeply and unlike anyone I've ever met.  We have so much love for Luby and her staff.  I could go on about her for days.  But... since she's just the first on my list of folks to recognize, I'll move on to Ellie's two cardiothoracic surgeons.

Ellie w/ Dr. Luby when Ellie was only 13 days old.

When I google her name, there are 4 photos of Ellie on the first page of image results.  Can you tell we post about Dr. Luby a lot?  We adore her.

#2) Dr. Morales - Cincinnati Children's Hospital Medical Center
We were set to meet Dr. Morales for the first time on 12/3/14.  However, because Ellie didn't recover well from anesthesia after her first sedated echo, we met him a few days earlier (the day after Thanksgiving) since we were already inpatient at Cincy.  Dr. Morales came in to describe Ellie's heart defect in more detail, informing us for the first time that her right ventricle was tiny.  We'd go on to have many tear-filled conversations with Dr. Morales in the coming month and a half.  He rocked our world when he had to break the awful news that Ellie wasn't eligible for the surgical plan that we thought she needed.  Her pressures were just too high in her pulmonary arteries to do the surgeries he'd described to us in November.  Even with a trach, Ellie wasn't able to reduce the pressures that needed to be in check for her first step in her repair.  In the end, this turned out to be the biggest blessing, but at the time, it sure didn't seem so.  To move her along and give her some time, he placed the PA band on the trunk of her pulmonary arteries on 12/31/14.  Without this surgery, nothing else would have been possible for her.  Ellie amazed Dr. Morales that day and I'll never forget shaking his hand and thanking him when she was headed to recovery.  He is a much loved man to the families we got to know in the CICU.  I cannot imagine the weight on such a surgeon.  While he didn't offer the plan we'd hoped for, he was in agreement with a whole team who had Ellie's best interest in mind.  He delivered the hard news gently, and operated on our girl when the odds were against him.  He certainly holds a very dear place in my heart... he was responsible for the surgery that limped her along and gave her a fighting chance.  He's the man who performed the surgery that protected her lungs from further damage... giving us a future off of the ventilator.

Dr. Morales - we don't have pictures with him - dang it.

#3) Dr. Baird - Boston Children's Hospital
We met Dr. Baird when Ellie was again in the depths of heart failure.  When we were discharged from Cincy and met back up with Dr. Luby in Dayton, she told us that we had about two months to get Ellie the surgery she needed.  Within a week of that two month mark, Ellie was in very bad shape.  She was rapidly growing out of the PA band that was placed five months before and spent her days with gray skin, purple lips, and fully dependent on the ventilator.  She wasn't awake much and tolerated very little.  We were desperate for a miracle when we walked into Boston for our pre-op testing day.  Dr. Baird met us and had this ease about him that was comforting.  He spoke about his plan with a comfort you'd expect from someone describing their recent golf match.  We only saw him briefly before her surgery on 5/27/15, but he spent a great deal of time talking to us after he was finished.  I wanted to wrap my arms around him and squeeze him until he could feel the gratitude in my heart that day.  That, of course, will never be possible.  In six hours, he used part of her pericardium to build a septum and her mitral and tricuspid valves.  He built the valves in her heart, y'all.  If that doesn't blow your mind... I don't know what would.  He then stopped in to see us every night before he left (after 8pm) and every morning as he came in before heading to the OR (before 7am).  He had Mt. Dew in the pockets of his lab coat.  He had an ease about him that was warm and reassuring.  He was not a man that was intimidating - even when you knew his mind was beyond fantastical!  I cannot wait for the day when we can bring Ellie to see him and let her shake his hand... maybe hand him a bottle of Mt. Dew.  I know nothing can ever express our gratitude to him, but we sure try by sending him birthday announcements and thank you letters each year.  I'm still in awe of this man.  Yet, I don't have a single picture of Ellie with him... but here is a video of him... it gives you a pretty good idea of his personality.  He specializes in valve repair (SURPRISE!).

If you have two and a half minutes, watch this video about Dr. Baird!!

We WILL get a photo with him when we get to see him again.

Pulmonology

#1) Dr. Cohn - Dayton Children's Hospital
This man is the one we've just lost from our care team.  He was someone Dr. Luby sent us over to when Ellie was sick one day.  We were in cardiac clinic and she said, "Yep, let's get her over to pulm... I have just the doctor for you".  She then walked us just down the hall (this was before all the renovations at Dayton Children's) and we met Dr. Cohn.  He was a tall man, but couldn't have been more calm and patient.  He was made for pediatrics.  When he was a child, he had an illness that required him to have a trach for a while, which was one of the biggest motivators steering him into pediatric pulmonology.  He was my go-to guy, allowing us to pull cultures at home and drop them off at the lab so that we didn't have to expose Ellie to all the hospital yuckiness during cold and flu season.  When we needed a new DME for supplies, he had just the right company for us (that'll be in another post).  When we came into clinic with a sick Buggy, he was often the one calling the floor to arrange for us to stay if we needed, or making lengthy phone calls with me talking about our options for home care to help us avoid the PICU during RSV season.  He worked closely with us through sleep studies and medication changes... battling through denials with insurance and rerouting medications we needed to make sure we had them in our hands.  In the past year and a half, we'd only seen him a handful of times because Ellie'd been relatively healthy and we could handle most things at home (with his guidance, of course).  Our last interaction with him was accidental.  We were waiting to go back to a pre-op room for surgery in July and Ellie was especially rambunctious.  I was trying to organize her things in our wagon quick when Ellie decided to bolt away from me and went running for the elevators.  As she neared them, the doors opened and she continued towards them like she was going to get on for a ride.  Dr. Cohn walked out of that open door, squatted down, and said, "good morning, Elizabeth, how are you today?  Are you trying to get away from mom?"  We weren't on his schedule... we weren't anywhere near pulm clinic.  We hadn't seen him in months, yet he saw that little monster running at him and he immediately recognized who she was and gently greeted her and kept her from boarding the elevator ahead of me.  He then greeted me and wished us luck in the OR.  That's just the kind of man he was.  He was always calm, always gentle, and was familiar with his patients even in unfamiliar encounters.  We will certainly miss him.  I don't have any photos of Ellie and Dr. Cohn together, but I do have a hospital image of him.

Our beloved Dr. Cohn

#2)  Dr. Wolf - Dayton Children's Hospital 
We had our first clinic appointment with Dr. Wolf earlier this month, but I only took a photo of how Ellie looked while she colored on the floor.  We've interacted with Dr. Wolf while inpatient many times.  During those interactions, we have been very impressed with him.  He's also very calm, but with a quirky sense of humor that I very much appreciate.  He came into Ellie's hospital room last month (after her lingual tonsils were removed) and said, "I hear we're taking this trach out today".  WHAT??  I think the excitement and surprise on my face caught him off guard.  He said, "Wait... let me talk with Elluru... he's the boss... I don't want to get your hopes up today."  So I'm looking forward to what the future holds as we work with Dr. Wolf.  In our clinic appointment last month, we had just gotten results from our trip to the ER and he saw that she had RSV.  This was the first time that Ellie's had RSV and he knew how scared I was.  Yes, Ellie is three, and yes, RSV is usually no big deal unless you're an infant.  Unless, you have a compromised immune system or respiratory issues - both of which Ellie has.  Dr. Wolf touched base with us through the week and assured us that the intensity of illness we were experiencing was what he would expect for Ellie.  He worked some magic and made sure that we had the medicines and treatments on hand to continue to battle it from home.  We look forward to working with him for as long as we need a pulmonologist :).

Dr. Wolf & Ellie coloring on the floor during her last visit with him.  He looked at her folded posture and said, "my legs would not still be functional if I sat like that".  Ha!

So there you have it - the first of my 12 Days of Christmas posts.  I'm not sure if they'll all be about medical specialists or not... we have about 50 people on our care team (that's not a joke)... but if I keep condensing these, maybe it won't take all 12 posts!  Thank you for reading along.  If you have some stellar medical professionals in your corner, thank God for them.  And make sure they know how much you love them, too.

Wrapped around those little pinkies

For any of you who have been living under a rock (when it comes to my posts) and do not already know about my obsession with Ellie’s hands… let me let you in on a few things, here.

First, Ellie’s hands are puffy – they’ve been puffy since the day she was born and I describe them like little peach pillows.  Ellie is squishy – I KNOW you’ve heard me say this.  I often describe her as pudding.  Because, honestly, she feels like a bag of pudding, folks.  She’s getting stronger as she gets older, but to squeeze on those little arms, hands, and legs… she still feels like she’s full of pudding.  There’s a delicate tenderness about her and all this squishiness stuffed into this little bitty body is just too much for me to handle.  

When Ellie was about a year old, we decided to make a craft that involved painted hand prints.  I painted and stamped the hands of the boys, then painted and stamped Ellie’s hands, too.  When I looked at the final product, I felt like I’d messed up.  I must have only painted two sections of her pinky… how in the world did I manage that?  I grabbed her little hands to repaint them and low and behold… noticed that I had not missed a thing.  It was Ellie that was missing something – the middle section of her pinkies.  WHAT?  How had I not noticed this before?  How was she almost a year old and I never noticed that her pinkies were just over half the size they should have been?  Maybe I’d been too caught up in her pillow hands that I never inspected the littlest digits.

I later discovered that this happens sometimes in kids with Down syndrome and it’s called clinodactyly – a very short pinky that often curves in towards the other digits.  Well… it kind of curves, too.  Hmmm… news to me!  I’d searched her little palms for a single palmar crease (another outward sign that’s a soft marker for Down syndrome).  She doesn’t have the single line – but I’d failed to notice those pinkies.  It’s thought that when babies with Down syndrome are developing in the womb, they tend to fold their hands at a ninety degree angle (instead of curling them in more).  This is due to low muscle tone and is thought to be the reason so many people with Down syndrome have a single palmar crease.  Ellie’s palms look exactly like mine – not one, not two, but three lines.  She must have been great at making a fist as she developed.  Maybe practicing shaking her fist at the odds of her success!  (Get it, girl!)

So in the beginning, I determined that she did not have the single line, and went about kissing those soft pillow hands for months – never noticing this cute little pinky.  Until the epic painting craft.  I also noticed that Ellie’s painted handprints looked huge.  Well, they’re not big at all… but once painted and smashed onto a page, all that squishy pudding flattens out to make a very boxy handprint.  I can’t get enough!

Ellie came home with this artwork in her backpack and I’ve been staring at it, admiring those square handprints… admiring those tiny pinky prints… and internalizing all these different little things that fill me with joy these days.  I mean, really… who gets excited about pinkies?  This girl.  That’s who!  Tiny reminders of how incredibly blessed we are to walk this weird, crooked journey as her parents.  Reminders littered through our normal days reminding us of how lucky these little boys of ours are to know in the deepest depths of their souls what compassion, hardship, and unconditional love look like.  For these things (and tiny pinkies), I’m so very thankful.

 

Her purple people eater is adorable.  Can you see the tiny pinky in the pic of her with my glasses?  Funny, right?

Side note:  I didn’t even attempt to do the 30 Days of Thanks this month.  I’d tried it in the past and failed miserably, so I wasn’t about to start and stop again.  Maybe I’ll try again another year.  It’s not that I don’t have a billion things to be thankful for… I certainly do… I just get side tracked when I have the best of intentions to post something each day for a month.  February is congenital heart defect awareness month.  Wanna know how many facts I managed to post?  Ummm… four.  Yep – and I had a short month to work with.  October is Down Syndrome Awareness month… I think I got about 5 of those 31 days, and at least three of those were just shares from someone else.  Eek – I’m terrible at this.  So instead of trying to stick with something for a whole month… I’m going to try to double my previous attempts and shoot for 12 days of Christmas (not even 12 consecutive days… just 12 days between now and Christmas where I post something).  How’s that?  You think I can keep up with that?  I don’t think I’ve made 12 blog posts all year… but why can’t I do 12 in the next month-ish?  Ha!  Today’s was even a bonus on top of those 12.  Keep me accountable, will ya?  I have a lot to be thankful for and a lot of people who have poured into our lives that I want to thank.  That’ll likely be the focus, btw, turning the spotlight on to the people who have cared for Buggy (and me) through this journey.  People who care for hundreds of kiddos each week, and do so with the intensity they’ve had in our lives.  Stay tuned!

Our elf, Luigi, hanging around.  The boys are so excited about this!

There's a house in Piqua that has more lights than the rest of the state, I swear.  We had to see it!

Took the boys ice skating on Sunday afternoon - B wasn't excited about my selfie.

I can't handle the cuteness.  We had sick kiddos last week, and again starting Sunday evening.  We're back to healthy now... whew.  No fun.  Keykob enjoyed the cuddles nonetheless.

Aunt Sherri bought Ellie a super cute sparkly shirt.  Ellie approves!

Yep, this is how we act for Thanksgiving dinner!

Surgery #14

Today was another day of surgery.  When I say this is surgery #14, it's far from our 14th visit to the OR.  We're only counting actual surgeries and heart caths here - not scopes or other sedated procedures.  I actually have no idea how many times this girl has been under.  Wanna know how many times I've been under in all my (I'm not telling how many) years?  Once.  I've been under anesthesia once.

Anyhow, at home we're preparing for Ellie's big annual meeting for home care services.  We meet with the care manager for the Ohio Home Care Waiver... and with our nursing companies and hash out how Ellie's needs have changed and how her health status has progressed/regressed over the past year.  To get ready for this, I always spend a good bit of time compiling notes and medication orders and lists of appointments/hospital stays/ER trips and all that jazz.  We need all of this to determine her need for care moving forward.  I was pleasantly surprised as I compiled all of this over the past week.  Did you know that Ellie has only spent 13 nights in the hospital this year?  That's drastically lower than her past years.  And we've only made 15 trips to Dayton for additional appointments.  I know that for most people, they never spend 13 nights in a hospital room EVER... (me included)... but for Bug, that's a heck of a year.  Now, that doesn't mean that she wasn't sick or didn't have storms and seizures in our house... it doesn't mean that there were never days when she SHOULD have been in the hospital.  Part of the reason this last year has been so good to her is that when we do have emergencies or pretty nasty illnesses at home, we're well supported and able to deal with them with the staff and equipment we have on hand.  The fact that we have such competent people in our house everyday has much to do with our lessening need for ER trips and hospital stays.  I'm hoping that part of it is that Ellie's immune system is starting to catch up with her and is starting to toughen to the things she'll be exposed to.  I really hope this is a big part of her progress, too... especially since we're in the thick of cold and flu season and she's around other little kiddos every day.  These last three years we've hunkered down at home with her... not even taking her to church during the winter months.  Let's hope that she's getting stronger all the time and that she can tough through this season without too much difficulty.  Anyhow - I thought you'd like to hear all that good news before we start to go into how things are going for her today.

So today, Friday Oct. 19th:
We packed bags for everyone last night and brought bags for the boys over to my parents' house.  Buggy wasn't allowed to eat anything after her bedtime snack last night, but we pump her full of pedialite through the night to make sure they can set an IV today.  (This is the beauty of a g tube and a feed pump).  Ellie's notoriously a hard stick for IVs.  Flooding her with pedialite before surgeries has been our life saver in getting her ready for this.  One, a full belly of pedialyte makes her less crabby in the morning, and a well-hydrated baby is much easier to stick for the nurses.  Win-win!

So we arrived at Children's early this morning, lugging around enough baggage for a family vacation, and Ellie went back for surgery around 10am.  She was out of the OR after about an hour and a half.  They went in and removed some of the tissue build up in her airway and removed her lingual tonsils.  They did NOT need to do a tongue resection.  Whew!  She was slumped over sleeping when we met her up in TCU.  She clearly has episodes of pain and normalcy.  She watched Moana and Frozen, ate lunch & dinner and just polished off some ice cream.  The only concern so far came from the dietitian... she said Ellie's BMI has dropped and she wants to ensure that we bulk up the calories so that she stays on a healthy BMI curve.  Can I donate my extra weight??  Pick me, pick me.  The dietitian probably wasn't impressed with my casual reaction to her concern... but really, if this is all I have to worry about post-op, then I think it's safe to take a nap. 

We're supposed to do a capping trial tonight, but Ellie's oxygen saturation haven't been real pretty... so she's on supplemental oxygen... we'll see what they say.  As far as moving toward decannulation, her surgeon said, "slow and steady wins the race here".  Ellie's airway tends to collapse just above her stoma because the trachea puts pressure on it and weakens that tissue.  She may need reconstructive interventions here again... but we'll have to wait and see.  If her airway coninues to be too small after she heals from this surgery, we may have to wait until she gets bigger.  We're not holding our breath.  We're just thankful for a successful surgery and a happy baby.

Our boys are going on camping adventures tonight - yahoo!  Until next time, friends... thank you for following along and for all the messages of prayers & encouragement today.  Love y'all!!

After running around the pre-op room, we finally got her to sleep before she went back.

Not fully awake yet.

After lunch... standing up, throwing toys out of the crib with a mouthful of goldfish while watching movies.

This was Mookie yesterday after the Running Club awards.  I think he wore both of his Running Club medals to school today.

When I picked Kaleb up from preschool on Wednesday, we took this random photo before having a short mommy & me date.