Ruby Hope McKee Starling

( UPDATE: After writing this post Sunday night, Holly pointed out this morning that they actually did move her morphine back to a 6 hour interval, not 4 hours like I originally wrote.  That was just a temporary step.  So really, everything was a step forward this weekend.  It was just a very hard-fought step forward.   I also failed to mention that Holly spent the night with Ruby last night so she could be there to hold her when she cried.  I wrote this late last night on the couch and I fell asleep halfway through.  So that's my excuse! )

Yesterday we weaned Ruby's morphine to 0.2ml every 6 hours, down from every 3 hours.  This is similar to the most recent Versed wean.  However, Ruby wasn't quite as accepting of this wean as the other.  When we went home at 11pm last night, she was sound asleep.  But when we came into her room this morning, there were 4 or 5 pacifiers strewn about the floor, and the IV for her antibiotics that had previously been in her left foot for her was gone and a new one was in her left hand.  Immediately, we knew Ruby had been misbehaving.  Apparently, she woke up at midnight and was inconsolable until about 4am when they gave her extra morphine and Versed to calm her down.  This morning at rounds, it was decided to keep her Versed at every 6 hours, but split the difference on the morphine and administer it every 4 hours instead of 3 or 6.

She continued to have withdrawal symptoms throughout the day today, including sinus congestion, crankiness, and vomiting.  We went through about 4 different outfits because she kept spitting up on them.  Unfortunately, I didn't have the camera ready.  The vomiting wasn't great quantities, only frequent, and so she got plenty of calories today and managed not to spit up any of her medications.  Her PO volumes are down a little, but she's still very eager to take the bottle or breastfeed.

Meanwhile, she has made big progress with her respiration support.  As of yesterday, she has been on 21% oxygen -- "room air".  And today they lowered her airflow from 2 liters per minute to 1 liter per minute.  So basically the nasal cannula is just blowing a little extra air in her nostrils.  We didn't really put 2 and 2 together until later this afternoon, but these changes are in preparation for sending her home -- to see if she needs to go home with oxygen.  We don't know how soon she could go home, but it feels like it is getting closer and closer.  We think it will be 2-4 more weeks, but it largely depends on Ruby and how soon she's able to get rid of the Morphine and Versed.  But even through all of the withdrawal today, her oxygen saturation remained 100%.  This is huge, because the medications she's having so much trouble weaning from are a large part of why she's still here with us today.  The morphine and Versed weren't just for pain management, but to keep her calm and sedated in order to keep her pulmonary hypertension in check.  It was to help keep that SpO2 at 100%.  So the fact that the amount of oxygen in her blood remains normal even as we are removing all the support mechanisms really gives us confidence that she is truly cured. 

This is Ruby at a happier moment yesterday, sitting in Mom's lap, seeing what a finger tastes like or perhaps seeing what the inside of her mouth feels like.  Her hand is glowing red because the pulse-oximeter is on it.  This is what measures the oxygen in her blood, telling us that she's doing great.

Yesterday was a Versed wean day.  She was already at 0.2mg every 3 hours, but administering a 0.1mg dose orally is a little tricky.  So we kept the dose the same and started giving it every 6 hours now, instead of every 3 hours.  The Versed weans are always easy, but we took today as a rest day and didn't wean sedatives.  She is still getting over Wednesday's morphine wean from 0.4mg to 0.2mg.  Weaning the big M is always difficult and that was a 50% reduction, so it definately hit her hard.  But this time around was a little different.  Instead of being fussy and crying frequently, she's been WIRED for the past 48 hours.  She was up late Wednesday night and then Thursday she woke up at 5am and never went back to sleep.  She took a few brief cat naps, but was awake pretty much the entire day and well into the night.  She wasn't agitated or irritable.  In fact, she was downright happy and cheerful.  But she just had absolutely no interest in sleeping.  She just wanted to play and watch what was going on in the NICU and interact with people.  We tried leaving her alone with no mobile or toys, thinking she might relax and sleep if she had less stimulation, but she just quietly laid in her crib, happily playing with her fingers.   We finally gave up and went home around 11pm, since we were having trouble staying awake.  The nurse said she finally fell asleep about 1am.  Thankfully, she slept all through the rest of the night and into the morning.  She napped frequently today and caught up on a lot of her missed sleep.  Sometimes babies in  the NICU get their night and day flipflopped, but we can deal with that later and mroe easily once she's off so many drugs. 

We postponed her PO feeds until later in the day, since sleeping was more important.  But at 9pm tonight she took her biggest bottle ever -- 39ml !  I thought we werne't going to get to do a bottle feed because she'd beel asleep in my arms for over 2 hours.  But she woke up right on queue and was ravenous!  She did that 39ml all in one go without stopping.  

There was some concern earlier in the week because the Infectious Disease doctors came by and painted a very grim picture of the infection situation.  It's their job to track infections in the hospital and to ensure that the right antibiotics are being used for the right amount of time and to prevent the spread of diseases between patients.  They wanted to draw out some of the fluid from the tiny pocket of fluid to identify what bacteria it is.  They also said that they think we might need to cut Ruby open and replace the Gore-Tex Patch on her diaphragm because it might be infected.  Needless to say, this really upset Holly because it'd mean she'd have to be intubated and we could lost so much of the progress we've made on feedings and sedation.  However, today at rounds the surgeons and neonatologist basically blew them off and said they were taking their suggestions into account but that because her white blood cell count and CRP levels were back into the normal range, they were going to assume the antibiotics she's currently on are working and she will just continue them until a full 7 day course is complete.  We switched her from Gentamycin back to a Keflex, I believe, because Gentamycin has ototoxic side-effects, meaning it can cause hearing loss.  So no sucking out the fluid and culturing it, no unnecessary surgeries, no replacing the patch, no being intubated again and no loss of progress.  Needless to say, Holly was pretty frazzled by it all.  I like the idea of having the Infectious Disease doctors helping us fight Ruby's infection.  They're basically like a quality assurance team that plays devil's advocate and ensures we're proceeding in the best ways to fight and prevent infections.  And it's great that they have differing opinions on things sometimes, afterall that's what drives science and medicine.  But I really wish they'd talk to Ruby's attending doctor before they start spouting off about replacing patches and extra surgeries in front of the parents. 

I don't have any new pictures at the moment, so here's another good photo from Wednesday's bath.  Holly thought this photo really highlights how much Ruby looks like her grandma:

Ruby had her CT scan yesterday and after rounds this morning the neonatologist and nurse practicianer came back around and told us that it looked good.  The CT confirmed that the pocket of fluid we can feel from the outside and that we saw on the ultrasound is isolated and is not any larger than we thought.  It is approximately marble-sized (less than 3/4" cube)  and they expect it will go away on its own.  She will continue to stay on the antibiotics until Monday, since they don't want to take any chance that the Gore-Tex patch on her diaphragm could become infected.  The incision is looking less inflamed and isn't oozing pus anymore.  Her white blood cell count and C-reactive protein level are back down to normal, indicating that her body is no longer fighting an infection.  So the 1-2-3 punch of Keflex, Vanco and Genta nuked whatever it was that was there.  We're happy that this isn't going to be something that'll keep her in the hospital any longer than she has to be with the sedation and oxygen wean.

Speaking of which, Ruby had two big wean days yesterday and today.  Yesterday she went from 0.4mg of Versed to 0.2mg, and today she made the same jump on her morphine.  We're not entirely sure, but we think they'll just go from 0.2mg to 0.  That doesn't mean she'll be off them in 2 days, though.  She was VERY cranky tonight after having had the lower dose of morphine a couple times.  She fussed and cried a lot and was sweaty and sneezy.  She was having her happy moments too, and she had a very enjoyable bath tonight.  We're not sure if they'll take her off the versed tomorrow or if they'll let her rest a couple more days until they wean her again.  We'll see how she does tonight.

Child LIfe services came by yesterday and said that it had been discussed and that Ruby was getting a mobile for her bed.  They usually want a baby to be at least 3 or 4 months old before they allow them to have something as stimulating as a motorized, musical mobile.  But they said Ruby was so alert and attentive and seeking stimulation that she could handle it fine.  And she loves it:

At first, she was amazed:

Then she was delighted:

I think we may need to get one of these particular brand of mobiles for home!  With the IV in her arm, we didn't want to get her too wet, so she just has a sponge bath tonight.  Ruby loved it:

Well, the tube count is up -- Ruby had to have an IV put in tonight. 

The repair incision is still inflamed and they did the ultrasound today and there was indeed a small pocket of fluid in there.  They are doing a CT scan tomorrow to see exactly how big the fluid pocket is and whether it's just a little infection at the surface or if it's something more extensive.  They started her on vancomycin and gentamicin, which should cover all the bases for any sort of infection it might be.  We're hoping it's just a localized infection, rather than something systemic or sepsis.  The Vanco and Gen will kill just about anything it could be, so even if it is something bad, we've already started the treatment.  We'll know more tomorrow after the CT.  We're worried, but this is far from the worst thing we've dealt with.

Ruby, on the other hand, isn't worried at all.  In fact, she was downright cheerful today.  She took 25ml with me from the bottle, and then 32ml and 30ml from mom.  They weaned her Versed down to 0.2ml and her morphine is still at 0.4ml, but she's was very comfortable with the Versed wean today.  Last night she slept soundly from 11pm to about 6am and she was happy and alert much of the day today.  They increased her feeding volume to 70ml every 3 hours, but tonight her reflux seemed a little worse.  Over the weekend, they switched her Zantac to Prolosec, which seems to be making her reflux better overall. 

Ruby and I had a great Father's Day.  To celebrate, I replaced her NG tube by myself for the first time.  She wasn't quite as excited about it as I was.  It went well and you can see my handywork with the duoderm and tegaderm tape below.  After our pajama party, we went to the Fetal Surgery Center Reunion.  Ruby couldn't make it this year, but maybe next year.  We did, however, get to meet some other CDH kids that have graduate from CHOP.  It was so encouraging to see other CDH kids and even kids who had been on ECMO, running around and playing just like any other kid. 

Here was Ruby after this morning's 25ml bottle feeding, quite content and snuggly, sporting her new NG:

It's late and we're tired.  We'll post a porper update tomorrow.

Ruby is still doing great.  We might have hit  a bit of a bump in the road, we just need to wait and see.  it's nothing too serious. Part of the incision from her repair surgery is red.  One of the surgical fellows drained and she's taking keflex to head off any problems.  If it doesn't clear up in the next couple days they'll do an ultrasound to make sure thier isn't a pocket fluid under her skin.  I've been told this nothing to worry about and it happens.  We are worried and do hope that it's othing serious.

We had a nice father's day.  Chris spent the night with Ruby last night becasue she was so cranky from the sedatoin weaning.  From what I understand they had a pajama party in the NICU.  Ruby hung out Dad and surfed the net until almost 4am.  She usually sleeps through the night. So, we knew the wean was hitting her pretty hard if she was up all night.  Her doctors gave her another rest day today, thankfully.

The good news is that she took 29ml from the bottle this morning. We also got to add another PO feed, that's a total of three a day now.  We're doing one bottle and two breast feedings.  We are so happy she progressing with the feeds.

This afternoon I laid Ruby in her crib on her back.  i went the restroom and when I came back I found her like this. She's catching up developmentally, which is a really good thing.

Here's Ruby saying "Up up and away!" Actually she's most likely saying, "What is that thing at the end of my arm?"

Ruby gets another break from the sedation weaning today.  She had a much better day yesterday than Tuesday, but she still had some withdrawal symptoms.   The craziest one was projectile vomiting.  I was sitting the chair next to her crib and all I saw was a fountain of milk.  It could not have been a pleasant experience for her.  Her feed volume was raised 70 ml on Tuesday.  So, today they are going back down 65ml and uping the calories to see if that helps with  the vomiting. She also lost 25 grams yesterday.  We're not concerned just yet because she hasn't been sleeping well, vomiting, and having really big watery stools.  Diaper changes have been fun lately.  She was one stinky baby yesterday.

Grandma bought her a pretty pink bath tub and last night we put it to good use.  We still can't submerse her in water due to the steristrips on her broviac central line dressing.  While I can't wait to give her a good soaking, we don't need an infection.  It's healing and hopefuly the steristrips will fall of soon.   Ruby hates the water part of her bath, but loves the lotion part at the end.  Her OT has taught us some baby massage and Ruby really responds to it.  After bath we wrap her up in a fuzzy towel and give her a massage.  Last night she even tolerated a back rub with some very important tummy time. 

Notice that her repair is visible in this picture.  She has stitch working it's way out on one end, it's nothing to worry about.

While it seems as though we aren't making much progress we really are.  Everday her lungs are growing and getting better.  Thats what's important.

The nurses and doctors use terms to describe a babies status that are a little miss leading.  When one of the nurses says her baby is "misbehaving" as many many nurses said Ruby during the first 5-6 weeks of her it means they are not fairing well.  As in medically they are doing poorly.  Sometimes Chris and I would use the same terms here, without realizing that those who didn't have a front row seat to everything might not understand the extent of how close we came to loosing her many many times.  Ruby is a fighter.  She's proven herself more than once.  She has fought the big bad pulmonary hypertention monster and for the most  part is winning.  She still requires some respiratory support and only time will tell how long she will need that.  She defied the odds by being born sicker than expected and then did the same again in over coming so many things. Chris and I are still a little shell shocked.  I imagine this is what post tramatic stress feels like.  Chris and I have daily conversations about how to go about not spoiling her.  I think it's going to be impossible.  Plus she's over  come more in her 10 weeks than most do in a life time.  I'm just not sure where we are going to keep a pony.

We were able to aggresively wean her morphine and versed over the weekend.  She's now down to  0.6 mg/kg oral morphine and 0.4 mg/kg oral versed every three hours.  She was at 1.0 and 0.8 respectively last week.  So, we have made great strides in weaning.  The down side is that the with drawal caughtt up to her yesterday.  Yesterday afternoon she was very cranky, restless, sneezing, and in general not a very happy baby.  She did not nap all afternoon or evening.  She finally fell asleep around 2 am after two versed rescues.  We give her rescues to help her get through the with drawal, it's more effective than going back up on the doses.  It is very hard to see her so uncomfortable.  The best thing to do for her is to hold her.  Luckily we had one of  our favorite night nurses last night and she was able to hold her for long periods of time.  I have a feeling Chris or I might be sleeping at the bedside a couple times during this process.  Sometimes the nurses are too busy to sit and hold a baby for hours on end.  Really though, that's our job as parents and we are more than willing to give up a good nights sleep to get her through this.

We have also switched the timing of her sedation and feeding.  We were giving her her sedation and feeds at the same times.  This caused issues with the oral feedings.  She was either too cranky because she needed her meds or too sleepy because she had recieved them.  Last night while she was so cranky we took advantage of it and moved the timeing of her sedation meds to an hour and half before her feeds.  Hopefully this will help make some progress with the oral feeding.  Having a tube in  your nose and taped to your face can't be fun.  We are more than prepared for her to have this tube for many many more weeks.  The volume of her feeds have been increased to 70ml, too.  Hopefully she can handle the extra volume resperatory wise.  Her little lungs can't handle any extra fluid in the body.  It's a fine line between giving her enough calories without giving her too much fluid.  This is why she is getting fortified breast milk, that way she gets more calories without the extra fluid.  She's already taking a diruetic to help her body with the fluid.

Now for the most important thing.

As you can see I had plenty of knitting time while she was on ECMO.

Ruby's grandma came to visit again this weekend.  This is the first visit she's had since Ruby was extubated and could be picked up and held.  It is very obvious that Ruby remembers grandma, but it was hard to tell who was more happy:

Ruby has been handling the reduced amount of morphine fairly well thus far.  She hasn't been terribly cranky, but her nose has been stuffy.  Oddly, this is one of the side-effects of opiate withdrawal.  So she has been coughing and sneezing the last two days.  It's really cute when little babies sneeze, but the reason is not so cute.  We went ahead and weaned her Versed today.  So she's at 0.8mg morphine and 0.6mg Versed every 3 hours.  The next step will be to take morphine down to 0.6mg, but we're not sure when we'll do that.  One funny thing about the oral morphine is that it is dyed BRIGHT BLUE so it's easy to identify to avoid accidental overdoses.  When they first switched her to this, we were in for a big surprised the first time we changed her diaper.  I'll spare the details, but lets just say that what goes in bright blue comes out bright blue.  Holly was worried there was something wrong with her bowels until we realized what was going on.  Now that she's taking half as much as she first was, the hues in the diaper are more of an emerald green.  Don't worry, I won't post pictures.

She's till taking two feeds per day through her mouth.  She only got 5ml from the bottle with me today, but Ruby got 10ml straight from the source with mom this afternoon, which made us all happy.  Her reflux wasn't as bad today, so I think the increased zantac is working.  We're holding her steady at 2 liters of airflow with 25% oxygen for the time being.  We're confident she can come off that when the time comes, but it still really seems to help with PO feeds and when she having an withdrawal tantrum. 

She made good progress on her torticollis today and was willingly and happily turning her head to the right to see us and toys and even to just relax and sleep.  The nurse yesterday got a toy out of the NICU baby toybox to help entice Ruby to turn to her right side when we're not around to police her head position.  It's some sort of plastic box thing that plays music and jungle sounds and has lights and dancing monkeys and frogs that is scientifically designed to mesmerize infants.  It probably has a proper name, but we call it the Baby TV.  It works wonderfully, as you can tell:

Grandma brought Ruby a whole new wardrobe and bought her a big girl bathtub.  Once the steri-strips fall off her broviac central line wound, she'll be OK to immerse in water for a proper bath.  Up until now it has just been a sponge bath.  It should be grand fun to watch her splash around for the first time!  I'll have the camera ready to take some naked baby pictures to embarass her with 15 years from now.  Until then, one last photo of Ruby in Grandma's lap, wondering why dad is pointing the camera at her again, instead of paying attention to her:

Ruby had a good day today.  The fever she developed after her vaccines remained under control and she seems no worse for the wear.  She got all the standard 2-month vaccinations -- rotavirus, DTaP, Hib, PCV, Hep B and IPV.  IPV is an enhanced version of the original Inactive Polio Virus developed by Jonas Salk and the University of Pittsberg.  They no longer give the oral live polio virus vaccine that previous generations received.  This is cool because Ruby's Grandmother was a Polio Pioneer in the Francis Field Trial when they mass-tested the vaccine on a quarter of a million children in 1954.  So Ruby is benefitting from her grandmother's own childhood medical efforts. 

She stayed up until midnight last night happily playing with her octopus and it was a major source of entertainment today too:

All the play really wore her out by this afternoon and during her weigh-in before breastfeeding, she fell asleep on the scale:

She's finally starting to put on some baby fat and look like a healthy baby, instead of one that didn't eat for the first several weeks of their life.  You can really see the chub around her face here:

They had to weight-adjust her zantac today because she no longer weighs 2.8 kilograms -- she' s 3.3 kilograms now.  with the increased zantac, she seems to be having a little less trouble with her acid reflux.  Her oxygen flow is still at 2 liters and about 25%.  Today's progress was on the sedation wean.  THey lowered her morphine down to 0.8 -- the same as her Versed.  So we expect some crankiness over the weekend. 

It's been a busy couple days for Ruby.  We are working on three things these days, weaning sedation, weaning respiratory support, and feeding by mouth.  The one thing she is excelling at is the weaning off the respiratory support.  The problem is she handles lower percent oxygen and flow until her sedation is weaned or we ask to eat by mouth.  Yesterday she was on 23% oxygen and a flow of 1.  When we tried to bottle feed her she started breathing fast and her oxygen saturation levels dropped to the low 90's (anything within the 90-100 range is OK).  We have been unable to wean her sedation for the past couple days due to withdrawal symptoms.  After talking with one of the neonatologists we decided to bump her back up to a flow of 2 and 25% oxygen.  The thinking is that we know she can handle lower amounts of respiratory support, so why not give her more support to help get her through the sedation weaning and learning to eat by mouth.  We are all for this approach.  We can take her home on the amount of oxygen and flow she is on.  We can even take her home with an NG tube (Chris and I learned how to insert one).  We can't take her home on the amount of sedatives she's on.  We need her weaned off the morphine and versed.

The good news is Ruby gets to experience some "normal baby" things.  She got all of her two month immunizations today.  She also spiked a fever because of them.  We were told to expect this and I was ready for it.  What I wasn't ready for was that when a baby has a fever typically their heart rate goes up too.  I noticed Ruby felt kinda warm and that her heart rate was in the 180's.  Over the next half hour her heart rate went up to 210, and set off the the alarms on her monitor.  I promptly got really scared.  After all that we've been through, any time the alarms go off for Ruby it's pretty stressful.  Luckily her nurse knows me and did her best to calm me down.  The nurse practitioner and neonatologist both told me this was all normal.  It might be normal, but "normal babies" aren't hooked up to a monitor that makes horrible beeping noises when the heart rate goes above 200.   They gave Ruby Tylenol.  When we left tonight she was comfortably sleeping.  Lets all hope Mom can do the same.  When we say Ruby had a quiet day, we mean just that.  No beeping is a good thing.

Now for some fashion show pictures!

No post is complete without the duck!

The Duck now has competition in the form of a singing octopus.