Magic Space Pod

Bonnie had a good day today.  After a couple days of fussiness and acting like she just didn't feel good (along with high heart rate and respiratory rate), they decided to try tweaking her feedings a bit.  They had added a protein supplement to increase calories, but apparently that can be hard on little baby bellies.  Today was her first day without it, and she seemed *much* happier.  She also got to try out her special chair from outer space.  Not really.  It's a Mamaroo (check it out), and she seems to like it.  (This one belongs to the NICU.  We also have one of our very own to use at home.)

She did like it, I promise.  This is just her fussing before falling asleep.  I included this picture because it's kinda funny and when Henry saw it, he said, "Bonnie mad!" Hehe.

Just checking in.

Still doing well in Bonnie Land.  Her medications are still being weaned with minimal problems on her end.  Hopefully within the next week or so she'll be off her drips (fentanyl and versed) and can be on scheduled doses.  Her occupational therapists are still working on some oral motor exercises to help overcome her oral aversion (due to being intubated for 5 weeks) so that she can eat by mouth eventually.  Still on a tiny bit of oxygen.  (80% of the sentences in the preceding paragraph contain the word "still".  We're in the slow progress phase of our journey.)

Her heartrate has been a touch on the high side for the past few days with no other obvious issues.  The only thing it seems to correlate with is temperature--when she gets too warm, it goes up.  They checked her hematocrit and it was good (36), so that wasn't the problem.  No signs of infection or anything, so nobody's too concerned.

Her cry is definitely getting stronger these past few days.  It's probably at 75% of normal baby volume.  Way to go, Bonnie!

This week Jonathan's folks have been keeping Henry while we're at the hospital, but of course they got some snuggle time with Bonnie as well.

Bonnie and Buddy (Jonathan's dad).

Smiles for Buddy.

Snuggling with Nan (Jonathan's stepmom).

Pretty girl.

Smiling at her friends (i.e. the mobile).

Sweet girl.

Sweet. Not salty.  Bonnie passed her sweat test with flying colors.  No cystic fibrosis.  I can't even tell you what a relief that is.  Praise God, and thank you all for your prayers over the past few days.  And *that*, friends, is the end of this ridiculous little chapter of Bonnie's CDH adventures.

On to better things!  Bonnie had a big ol' time this morning with her occupational therapist.  She's learning to hold her head up a little!

We also had our daily dose of snuggle time.

Mmm, tasty baby.

Not a lot happening with Bonnie since my last post, medically speaking.  She's still on a very low amount of oxygen (0.1 liters of flow and 30% O2) and is doing great with that.  She's getting 20 cc/hour of breastmilk, and today they (the speech therapists) attempted to get her to bottle feed a little bit.  

The whole eating thing is complicated.  We found out a few days ago that Bonnie's right vocal cord is paralyzed.  This is probably due to some combination of being on ECMO and being intubated for so long.  The vocal cords (or vocal *folds* as they say in the biz) help close off your airway when you swallow so that you don't aspirate.  Inhaling food (or breastmilk) into your lungs is clearly not an ideal situation, so we have to be super careful when feeding Bonnie by mouth.  So careful, in fact, that they don't really want to do it until she's had a swallow study and can prove that she can protect her airway.  The swallow study requires that she be able to swallow (imagine that), which, until today, she had never in her life had to do.  The speech therapist came today to give her some practice sucking and swallowing.  Short version is that she's not quite ready to eat by mouth yet.  She didn't throw up or anything, but she was sort of gaggy (common in CDH babies) and not very interested in sucking.  After about ten minutes of trying (during which she consumed a whopping one milliliter of milk), she promptly conked out.  Oh well, gotta start somewhere.

So we won't be having a swallow study for at least a few more days.  What I'm hoping is that by the time she's ready for it, she won't need it anymore.  Her cry has been significantly louder the past day or so, which I'm hoping is a sign that her vocal cord is coming back to life.

The big event tomorrow is her sweat test to check for cystic fibrosis.  I've been assured several times that no one thinks she has it, we just have to do the test to rule it out since her newborn screen came back positive  (twice) for a CF marker.  The test measures the amount of chloride in a person's sweat, and if it's higher than a certain number, it indicates they have CF.  One little tidbit that I vaguely remembered from nursing school and was then reminded of by one of Bonnie's nurse practitioners, is that if you kiss a baby with cystic fibrosis, they tend to taste salty.  She said that's *almost* as diagnostic as the sweat test.  So it should come as no surprise to anyone that I've licked my child about five times over the past couple days.  She doesn't taste particularly salty to me, so I'm taking that as a sign that all is well.  Nevertheless, please pray for a couple things tomorrow. 1--That she is able to have a successful sweat test. A lot of times it's hard for itty bitties to sweat enough for a valid test.  2--That she, for the love of pete, does not have cystic fibrosis.

And now, some pictures!

Pop Pop (aka my dad) got to hold Bonnie a couple days ago.

Lots of pink.

Sweet sleepy girl.

An artsy shot.

In 20 years, we will be able to tell that this is Bonnie and not Henry only because she has all this stuff on her face.

*Now* what?!

Bonnie is still doing well, y'all, but now we've got something completely out of left field to worry about.  Her initial newborn metabolic screen (which they do on all babies--tests for a variety of diseases) came back abnormal.  I knew about that one because they had mentioned it in passing one day in rounds.  Didn't think much about it because there are a billion obscure diseases that can show up, and nobody seemed worried about it at the time.  I didn't even ask which test came back abnormal.  Well.  Anytime a metabolic screen turns up something, they send a second one to confirm the results.  Bonnie's second test was sent about a week and a half ago.  

We found out today that this one was abnormal too.  It came back positive--for the second time--for the marker for cystic fibrosis.  All I'm going to say right now about cystic fibrosis is that it's a really scary disease.  In the grand scheme of things, I'm probably more freaked out by the possibility of CF than I was with the diaphragmatic hernia.  Maybe that's crazy. Whatever.  *Anyway*, Bonnie had an elevated marker for CF, (which is called IRT--immunoreactive trypsinogen).  This doesn't mean that she has the disease, only that more testing is needed to definitively rule it in or out.  Various folks have assured me that it's quite likely she doesn't have it (no symptoms, for one thing), and that there are a variety of things that can cause an elevated IRT.  One of the main ones is stress, the type that might occur when you are a brand new baby with a diaphragmatic hernia.

Sooo Bonnie will be tested for cystic fibrosis.  The initial test will be a sweat test, and if that's inconclusive they'll do some genetics labwork.  The sweat test involves some kind of complicated procedure, and there's only one person in the hospital who can do it, and they only do it on Tuesdays.  Today is Friday, so we get to wait four days for any sort of information. 

We would really *really* appreciate your prayers that Bonnie doesn't have cystic fibrosis.  By the grace of God, she's made it through the scary phase of her CDH.  Not sure we can handle another life-threatening diagnosis.

Here are some fun pictures to cheer us all up:

And then we have Bonnie and Jonathan. I was out of the room when these were taken (pumping, of course), and I'm so sad that I missed out on this smile-fest!  So glad our nurse was able to capture it on film, though.

NICU fashion.

The big Thursday event this week is that Bonnie got to wear clothes for the first time today!  We came in to see Bonnie today and found that her lovely nurse Meghan had done this:

Isn't she cute?!

The update from the ENT evaluation is that she has a paralyzed right vocal cord.  This should resolve eventually, but it could take several months.  Her cry is getting a little stronger but it's still really soft.

She's back to continuous feeds through her NG tube (20 cc/hour).  She had been getting 45ish ccs over an hour and then off for two hours.  She had some throwing up last night, so they backed off to the continuous feeds.

Bonnie started getting a little puffy last night (nowhere near as bad as when she was on ECMO), so she got a dose of Lasix to help depuff, which helped.  They decided to give her Lasix daily to help with her tendency to puff.

We're still enjoying the heck out of snuggling with our girl.  She's a cuddly bundle of squishiness that I can't get enough of.

Hard to believe that just a month ago we were here:

So many praises to the One who has gotten us through.

Turn up the volume.

Bonnie has been working on finding her voice since she was extubated last week.  When she was on the ventilator, her breathing tube went between her vocal cords, preventing her from making any sounds.  In theory, now that she's extubated, we should be able to hear her cry.  The first couple days they didn't necessarily expect her to be very loud because her vocal cords could have been swollen from being intubated for so long.  By now, though, any swelling should have gone down and we should be able to hear her...but we really can't.  We can hear her cry occasionally, and it sounds like a normal baby cry but with the volume turned way down.  Anyway, it's gotten to the point now where her doctors are wondering if there's something else going on, and they've gotten the ENT (ear/nose/throat) folks involved.  It's definitely not a big deal in the grand scheme of things, and we should know something more in the next couple days.

Meanwhile, in Good News Land, Bonnie has been doing awesome with her feedings.  She's still getting them via her NG tube, but she's up to 35cc given over 90 minutes, every three hours.  So far no problems with spitting up, which can be a big issue with CDH babies.  I figure the reflux will kick in eventually, but we're really happy with her progress so far. Yay Bonnie!  They're waiting to try feeding by mouth until her vocal cord situation is sorted out, to make sure she can protect her airway and not aspirate while she's eating.

They've been slowly weaning her pain/sedation medications, which so far hasn't been horrible.  They did take a couple days off of weaning because she was showing some signs of withdrawal, but today they were back to weaning.  (For any CDH parents reading this and wanting details, she's currently down to 3.2 mcg/kg/min of versed and 4 mcg/kg/h of fentanyl.)  At some point they'll switch to scheduled doses of her medicines, but at the moment she's still on continuous drips.

This is the part of the journey where things have started going very slowly.  It's going to take a long time (i.e. several weeks) for Bonnie to learn to eat and wean off her medications.  She's doing *great*, but it's still going to be a long time before we bring her home.

Thank you all for praying us to this point.  God has been so so good to our family.  Please continue to pray that Bonnie would do well with her drug weaning and learning to eat.  Please also pray that her vocal cords kick in and she finds her voice.

Here are a few pictures of our sweet girl.

And because he's cute and awesome, here are a couple of Henry.

Bonnie smiles.

As the title would suggest, Bonnie smiles.  And just generally looks super cute.  (That's an IV on the side of her head.)

Bonnie also enjoys being held by a variety of folks.  Grandmomma got to hold her for the first time today.

Christmas in July

That's what it felt like when I walked in today after a day at the hospital with Bonnie.  I came home to a package sent by the folks from work (Developmental Pediatrics and Pediatric Neurology--hi y'all!).  Opened it up to find all this:

I was totally blown away.  So much love and support from my work family, even though they're 3 hours away and haven't met Bonnie yet.  A lot of this stuff was for Henry, which he was super thrilled about too.  His birthday has been going on for about a week now :)  Thanks work folks, you made my day.

Meanwhile, back at the hospital, Bonnie continues to amaze and astound.  She has started giving the briefest flickers of a smile (which I have yet to catch on camera).  I thought I saw a smile yesterday, but it was confirmed when I walked in today and Bonnie's nurse said that she had seen a few smiles today too.  After all those days of ECMO when it almost didn't feel like there was a baby in there, Bonnie has decided to make up for lost time and make her personality known.  She's still on a tiny bit of oxygen via her nasal cannula, but that was being weaned today.  She got her arterial line removed this afternoon, so one less tube to be attached to. Hurray!  She's up to 2 ml/hour on her feedings and is tolerating that well so far.  Her versed was weaned a bit today and so far so good, but it can take awhile (12ish hours, I'm told) before withdrawal symptoms show up after a wean.

Here are a couple pictures from today.

Redeeming Thursday.

Back in high school, the running joke amongst my friends (shout out to Ruth and Nicole) was that if something bad was going happen, it would be on a Thursday.  Our teachers seemed to like to give difficult tests on Thursday, I guess.  Maybe one of us got our heart broken on a Thursday, I can't remember. Thursday should have been great because it's only one day away from Friday, but we were almost superstitious in our Thursday phobia.

In the "several" years since high school, I've pretty much overcome my issues with Thursday.  It's often a downright pleasant day.  In the five weeks since Bonnie was born, I think it may have become my new favorite day of the week.  Here's a recap of some recent Thursdays:

Thursday, June 7:  Bonnie is born.
Thursday, June 28:  Bonnie comes off ECMO.
Thursday, July 5:  Bonnie has her hernia repair surgery.
Thursday, July 12 (that would be today):  Bonnie is OFF THE VENTILATOR!

That's right, friends.  Bonnie was extubated today around 10:00 a.m.  She is breathing on her own and doing a stellar job of it.  Praise the Lord!  (Seriously, take a minute and say thank you because this is nothing short of incredible.)  For the first couple hours, she didn't have CPAP, Vapotherm, nasal cannula--nothing!  Nothing at all on her sweet little face.  First time that's happened since she was about 2 minutes old.  They did end up starting her on a nasal cannula on extremely low flow oxygen (0.1 liters/minute) because her doctor didn't want her O2 sats to be below 95%.  She was mostly keeping them up that high anyway, but would dip down in the high 80s occasionally.  Blood oxygen levels that are too low can exacerbate pulmonary hypertension, and we definitely don't want that.  So I'm totally fine with a nasal cannula.  Compared to that terrible ET tube, it's downright adorable.

Here is a photo recap of Bonnie's best Thursday yet.

This is the ventilator, not ventilating.

Bonnie with *nothing* on her face except leftover adhesive from all the junk that *used* to be on her face.

Yes, I had held her before, but we had some serious snuggle time today.  Couldn't have done this very easily on the vent.

Sleeping peacefully with a minimal amount of oxygen flow ("less than a whiff", as Bonnie's favorite respiratory therapist Joe says) through the nasal cannula.

And one more thing I'm super excited about.  They started giving Bonnie some breastmilk today through an NG tube.  Super tiny amounts at first--1 cc an hour, i.e. less than an ounce a day--just to get her belly to wake up.  There are likely to be several speed bumps on the way to learning to eat by mouth, but I'm so excited for her to get started on the process.

So much to be thankful for today.  Thank you *all* for your faithful prayers for our sweet Bonnie.  They have been answered over and above what I even could have imagined.  The big prayer requests at this point are for her to tolerate feedings (and learn to eat by mouth eventually), and for her to wean off her pain/sedation medications with minimal issues.  The drug weaning I am told is a very long and difficult process, and Bonnie is on high doses of some pretty heavy stuff (fentanyl and versed).  She basically has to go through baby rehab, which is not going to be enjoyable for anyone.

Happy Thursday, everybody!

Still doing great, with pictures to prove it.

Bonnie is doing great, y'all.  There were several people that walked by her bed today (our beloved Dr. Jenkins and a couple nurses) who hadn't seen her in about a week that were shocked at how good she looked.  Remember, just one week ago, she was still really puffy from holding onto so much extra fluid, she was on dialysis, and she hadn't had her repair surgery yet.  *Today*, she looks amazing.  No more hole in the diaphragm, kidneys are back, and there has been some serious discussion about taking her off the ventilator.  My understanding is that the only reason she's still on the vent is that she's on some whopping doses of fentanyl and versed, which may decrease her drive to breathe.  The plan is to get those weaned down a bit and then extubate in the next few days.

Here are a few pictures from the past couple days.  Jonathan got to hold her today for the first time. So sweet.

Here are a couple of me and Bonnie from yesterday.

And then the girl you *really* came here to see...