Ruby Hope McKee Starling

Ruby had a good day.  They weaned her versed and her flow.  During rounds we found out that her sedation weaning schedule is 21 days long.  That's if everything goes perfectly.  We've been told to expect it to take longer.  The good thing is that there is a plan.  Ruby's flow is currently at 2.5 on the High Flow.  She needs to be at a flow of 2 to switch to the low flow.  This could happen in the next  couple days, if she continues to do well. 

We've had nice weekend spending time with her. As she is less and less sedated we are able to interact with her more.  The withdrawal does make her cranky, and she's definately starting to get her voice back.  Her cry is still pretty horse, but it's getting louder and stronger by the day.  Soon she'll be screaming like any other 7 week old.

Now for some pictures!

Please check out Chris's post from last night for a cute picture of Ruby on the high flow.  Ruby had a good night and was only breathing hard (tachypnic) after her feeds, which is normal.  Tachypnic means she is taking more than 70 breaths per minute for more than twenty seconds.  It's very normal for CDH babies to do this when physically stressed, which could be caused by eating. Ruby has been peaking in the 70's which is good for a CDH baby.  Last week when she was taken off high flow and put on CPAP she was consistently taking over 100 breaths per minute and getting really tired.  We just have to remember that Ruby doesn't have full sized lungs and will breathe a bit faster and shallower than a baby without CDH. Here's Ruby letting us know how much she hates the CPAP mask.

Here she is letting us know how much happier the high flow makes her.

Now that she's back on high flow the next step respiratory wise will be low flow oxygen cannulas then room air.  She can go home on low  flow if absolutely necessary, and some CDH babies do.  We are hoping that Ruby won't need too, but we'll just have to wait and see. 

The morphine and versed are being weaned pretty aggressively.  She's down to 120 from her maximum dose of 300.  Today they are switching her from IV to oral doses of both.  We've been warned that since she is still on such a high dose, this transition can be rough.  The whole weaning process can be rough.  She will be going through withdrawal.  While they will do everything to make sure she is comfortable and not in pain, we will hit some bumps.  We've also been warned that weaning could take awhile, as in weeks.  If necessary she could go home on methadone, but again we don't know if this will be necessary or not.  As usual Ruby will tell us what she needs.

Then finally we have all the feeding issues to deal with. Right now she is being feed through an NG tube.  This tube puts the food directly in her stomach.  She doesn't have to swallow or suck to get it.  Meaning she doesn't have to expend any energy to eat.  Once she is on low flow oxygen we can (and will)  start to work on feeding her with a bottle and/or breast feeding.  Most likely she will be fed through a combination of all  three things for some time.  She will need to be consistently gaining weight feeding by mouth before we can go home.  Also feeding by mouth is when the reflux issues usually surface in CDH babies.  Almost all CDH babies have issues with acid reflux.  We are not expecting the feeding to be an easy road.

All in all we feel very relieved that Ruby is doing so well.  These last hurdles, while time consuming and frustrating, are nothing compared to what she has already overcome.  We have gone from daily conversations preparing us for the worst to being on the slow road to home.  We couldn't be happier.  We are being cautious.  The weaning of the meds can cause some respiratory distress.  While we would hate for Ruby to be put back on CPAP, it might happen if she needs more support.  For now though she's doing well on the high flow and tolerating the weaning.  As we all know this can all change in a second. 

We are guessing that we are at least 4 weeks from being discharged.  We wouldn't be surprised if it was more like 8.  We'll just have to wait and see.  For now, here are some really cute pictures of Ruby from earlier this week when she still had the CPAP.

Ruby shed her CPAP today and is back on the high-flow oxygen canula!

They took out her broviac central line today, but an arm IV replaced it, so the tube count remains at two.  You can see the yellow NG tube in her nose, and the white board on her right forearm is to make it harder to pull the IV out.  Unfortunately, the board and IV line also prevent putting  too many cute clothes on her at the moment.

They weaned her morphine and versed down to 120 today and are planning to HALVE it tonight, while replacing the other half with oral doses.  She should be fine with that change tonight, but tomorrow night might be hard if she starts having withdawal symptions.  But until then, she's resting peacefully and we plant let her take it easy tomorrow.

Ruby has been quietly making progress and doing great.  Today they weaned her morphine down to 140, and her Versed is still at 160.  Yesterday they switched her from the Drager CPAP that uses nasal prongs to one that uses a nasal mask -- no more swim cap.  They periodically switch babies between the two to minimize irritation and soreness in and around their nose.  Her CPAP pressure setting went from 5 cmH₂O to 4 cmH₂O, which is basically the lowest setting either machine will function at.  In real-world units, that's about 0.08 PSI.  In other words, she's needing very minimal support.  They've been stepping down the oxygen she's breathing as well, and she got down to 25% today (normal "room air" is 21%).  However, we bumped it up to 28% tonight for a babygirl makeover. 

Tuesday night, the IV in Ruby's arm stopped working, and it was unnecessary anyway so the nurse took it out.  The night nurses usually bathe and change the babies clothes and beds, and with the IV out she was able to wear clohes again -- no more blue CHOP gowns!  And you guessed it... her tube count is down to TWO!

1. broviac central line
2. nasogastric feeding tube

She'll probably keep these two for a while.  The central line will stay in until she's weaned completely off the morphine & Versed.  And the NG feeding tube will be there until she's able to get 100% of her nutrition from bottle or breastfeeding.  We can't try direct oral feeds like this until she is on the low-flow oxygen canula and her breathing rate is consistently below 70.  She's getting there. 

So this morning we came in and the nurse had put her in her purple Daddy's Girl pajamas:

The nurse had been promising us a chance to give her a bath, and we finally were able to take the opportunity.   We disconnected all the beeping monitors and removed all the sensors and peeled off (almost) all the tape and removed the CPAP mask , and Ruby pitched in by personally yanking out her own NG tube.  This is the startled Ruby we discovered underneath:

We wiped her down with some warm, mildly soapy water and rinsed off with a cloth soaked in clean water.  We even sat her up and washed her back and the back of her neck and head.  She then got lotion all over and a little mineral oil on her scalp, since she has a touch of cradle cap (dry flakey skin). Afterward, the nurse demonstrated how to replaced her NG tube. 

We then tried putting her in a new outfit that was going to include some pants that were optimistacally labled as for "0 - 3 months", but they looked like MC Hammer pants on her:

We'll save those for later. 

Instead, we put on her Rock & Roll kimono:

She handled her day at the spa very, very well and only fussed a couple times.  Her voice is getting stronger, but she still doesn't cry full-volume.  But she's usually so easy-going and happy that she doesn't get much practice.

We had a good Memorial Day.  No big changes, just hanging out and resting.  We took her Versed down to 160 again and her feeds went up to 50ml.  We scooched down her oxygen to about 34%.  Incidentally, she's having to wear hospital gowns because the IV in her arm won't allow "normal" clothes to go on.  Unfortunately, the only color they come in is baby-boy blue.  I think she looks nice in it.  Ruby spent most of the day out of her crib with mom and dad:

Afterwards, it was time for bed, so we rolled her up into a little baby burrito. 

Yesterday Ruby said, "Whoa, slow down!  I'm just a little baby and you're changing all these things on me!"  Or at least that's what she would have said if she could talk.

Instead, we got a call at about 5am from Ruby's night nurse to tell us that her doctors and nurses had decided she was working too hard to breathe and was wearing herself out.  So they were either going to re-intubate her or put her on a CPAP machine.  So we got dressed and drove up there early and by the time we arrived, she was on the CPAP, much to our relief.  CPAP stands for Continuous Positive Airway Pressure.  This is the same type of machine that adults with sleep apnea use, only in mineature form.   It provides a continuous flow of air at a low pressure to help keep her airway open and to assist with breathing.  The biggest difference from the ventilator is that it uses just a nasal cannula that just straps onto her face instead of a more invasive endotracheal tube down her throat.  So her mouth is free and available for great new things we've never been able to do before such as: crying, spitting up, sucking on her fingers, sucking on a pacifier, sucking on her entire hand, drooling on mom, and any combination of all of the above.

Here's Ruby in her new scuba gear and swimcap on.  It's even blue so she's ready to join the Balcones Woods Blue Wave, our neighborhood's swim team.  The moisture in the line is because the air is humidified to make it easier to breathe. 

They think that Ruby just needs a little big of a rest on the CPAP for a few days before we try her on just the regular oxygen canula again.  Some kids go bezerk and can't deal with the CPAP, but she seems to tolerate it well as long as the straps stay adjusted so they're out of her eyes (we adjusted them right after the photo above).  The "swim cap" slips off easily and she's squirmy now that she's feeling better and is less sedated, so it's an exercise in constant re-adjustment.  We're really not looking at this as too much of a setback, since she just needed a little extra help and we've made progress in other areas.  They started her breathing 50% oxygen through the CPAP, and she's already down to 38%.  The pressure settings on the CPAP are already as low as they can go, so she's really only getting minimal assistance. 

One of the accomplishments yesterday was getting her LEFT chest tube removed.  That's right, all the chest tubes are GONE.  That took our tube count down to 4!

Then, they increased her feeding to 45ml yesterday and 50ml today, so she no longer needs the intravenous lipds.  Without that, she didn't need as many IV lines, so the scap IV was removed Saturday night.  That brings the tube count down to....  3!  Here's what's left:

1. broviac central line
2. arm IV
3. "NG" feeding tube

We're kind of not counting the CPAP since it doesn't go INSIDE her, but maybe we should?  Ok, so it's four tubes.  Or maybe three and a half.

At any rate, with less tubes to tangle, Ruby has more freedom to move about and do things, including Tummy Time.  Since she's resting on her back all the time, as is recomended for all babies these days, it's important for her to get time laying on her tummy to help develop neck and abdominal muscles and to help keep her head from getting flat on the backside.  Here's Ruby demonstrating her new skill of sucking on two fingers, drooling all over mom while resting on her tummy.  Mom loves it:

Here's Ruby from this morning demonstrating her underwater pacifier technique:

We were very excited that she took it for the first time today.  Babies that have been intubated and on a feeding tube for a long time develop an oral aversion and will have difficulty feeding and sucking and swallowing.  We're hoping that this is an indication that she won't be too orally averse.  For her safety, we can't begin feeding her directly with a bottle or breast until she's on a low-flow oxygen cannula and her breathing rate is consistently below 70. 

On the pharmacological front, they increased her morphine and versed back up to 180 and 180 yesterday.  They think that part of her breathing problems and distress could have been related to withdrawal symptoms.  This morning, they snuck the versed back down to 160 (sshhh! don't tell her!).  They're planning to discontinue her HAL today, since she's getting 50ml feedings now and won't require additional calories or nutrients.  This means that the IV in her arm will be unnecessary and they'll let it "expire" and then remove it in a day or two.  This will make Ruby very, very happy because it currently has a "no-no" board on it to immobilize it and prevent it from getting ripped out.  She completely and utterly hates that.  She can bend her elbow, but not move her wrist or fingers, so she waves this big white bundle around and bops herself in the head periodically.  It's rather amusing, but you can't help but feel sorry for her.  We've caught her sucking on the IV port, since she can't get to her fingers.

She's currently sleeping soundly, so we're going to go grab some lunch and hopefully enjoy a quiet and leasurely Memorial Day afternoon in the NICU, hanging out with Ruby.  It doesn't sound like fun, but there isn't anywhere else in the world we'd rather be right now.  We hope everyone enjoys their holiday too!

I appologize for not updating sooner today, but Holly, Ruby and I all kind of took it easy today.  I took at least two naps today in the big comfy chair next to Ruby and Holly had a few as well  Needless to say, Ruby did her share fo snoozing today as well.  All of this was well-deserved after the big changes yesterday.  To recap, we had:

1) Extubated - this means they removed the endotracheal tube through which the ventilator had been blowing into her lungs.  She has a high-flow nasal canula now that delivers some oxygen directly into her nostrils, but her own lungs and diaphragm do all the work of moving air in and out now.  The concentration of oxygen being given to her has gone up and down a little in the last 24 hours, but she's currently at 45%.  They started the airflow through her canula at 2 liters of air per minute but moved her up to 4 liters.  She seems to have stabilized at these settings, but she is still a little "tachypneic" -- that is, she breathes rapidly.  Her heartrate has been a little elevated as well.  The doctors and nurses and respiratory therapists all assured us that this is expected and that as she gets stronger, her breathing will settle back down into the normal range.  However, the oxygen saturization has been good and her blood gas tests have been awesome.  So even though she's working a little harder, her oxygen and carbon dioxide and whatnot are all in the normal range for a normal baby.

2) Chest tube removed - Her right chest tube was taken out.  This one hadn't drained any fluid in about 2 weeks, so it was well past due.  I wasn't aware they don't use any sedation for this procedure and it didn't look comfortable at all.  Ruby did well, but she's still very hoarse from having had a breathing tube down her throat for the first 6 weeks of her life.  So all she could do about it really was croak and whimper a bit.  She's still not able to full-on cry yet, but she'll make the face and wave her arms and legs in the air, as if she's pantomiming a crying baby. 

3) Moved to a crib - That's right, Ruby graduated to a big girl bed!  As our nurse put it, "Ruby's no longer a critically sick baby, so why keep her in a bed for critically sick babies?"  The funny thing, though, is that CHOP is standardizing one size crib for all ages up to about age 4.  So Ruby's crib, although it's brand new, is big enough for octuplets.  The label on it says that the weight limit is 250lbs, so we've been joking about spending the night with her in it.  We're very excited about it, though.  It means she's no longer on a baby warmer and she is able to wear her own clothes.  She's only wearing shirts for now, and only those that button down the front.  But in a few days she'll be in proper "onesies" that a baby her age would wear.  This also means that she's no longer sleeping on a scale all day, so we have to pick her up and put her on a scale to weigh her.  We didn't want to hassle her any more than necessary today, but her last weigh-in was 3105g, w hich is about 6lbs 12oz.  This is "real" weight, not water weight like before.  

4) Less bad stuff, more good stuff -  They've weaned her Morphine down to 160mcg and her versed is 160mcg as well.  They've also increased her feedings to 40ml of breastmilk every 3 hours.  She's getting less intravenous HAL & Lipids for nutrition now, so once she's at "full" feeds of 50-60ml, they can remove the HAL & lipids and the broviac central line in her chest.  She's currently being fed through the yellow nasogastric tube that goes through her nose, down her throat in to her stomach.  Holly and I have both learned how to feed her through this and the nurses allow us to do it when we're there -- typically 9am, noon, 3pm, 6pm and 9pm.  We also change her diapers most of the time, take her temperature and do other miscelaneous care items for her now.  Back in the dark ages, the NICU was very much a place only for babies, doctors and nurses.  But CHOP (and others) have seen the importance of involving the parents in the babies care and they are happy to let us do as much as we are willing to try.

Current tube count is 5:

1. left chest tube
2. broviac central line
3. scalp IV
4. arm IV (replaced foot IV)
5. nasogastric feeding tube

We've added the nasal canula, but it hardly counts since it doesn't prevent us from picking her up or eventually taking her home.

Holly holding Ruby yesterday:

Here's Ruby's new crib.  Note how little equipment there is there beside her.  No vent, no nitric oxide tanks -- just the IV poles and a few hoses for her oxygen canula. 

And here's Ruby trying to figure out what to make of all the changes she's been through in the past 24 hours.:

It's late and we've all had a long day.  Ruby included.  She was extubated, a chest tube was removed, and she was moved to a crib.  More of an update tomorrow we promise.

The doctors increased her feedings to 35ml today and reduced her morphine from 200mcg to 160mcg.  Her versed was reduced to 180mcg yesterday.  Ruby did well with her MRI yesterday, although they had to sedate her very heavily for it and as a result, had to increase her vetilator support.  But this morning they reduced the vent settings back to their original state and she's doing great with very minimal support.  So good, in fact, that they will be extubating her this afternoon.  That means that the huge tube in her mouth and much the tape on her face will go away.  She'll still have the feeding tube in her nose and the tape for it.  And she will most likely have an nasal cannula for some additional oxygen.  But it means that there will be less equipment (or maybe different equipment) in the room and one less tube on our tube count! 

Current tube count is seven:

1. endotracheal tube for breathing
2. nasogastric tube for feeding
3. right chest tube for pleural effusion
4. left chest tube for chylothorax
5. scalp IV for morphine & versed
6. foot IV for misc meds
7. broviac central line in her chest for hal & lipids

Here's Ruby doing her best to get the ET tube out a little early:

First off on Tuesday morning we had one of our favorite nurses.  I love her becasue she likes to clean and oraganize, my kinda girl.  We reorganized Ruby's room.  She also helped me give Ruby a "bath".  Since Ruby is on the vent and has a central line, 2 IV's, and 2 chest tubes a proper bath isn't possible.  We did however get some soapy water and scrub her down as best we could.  I got to touch and see Ruby's back for the first time! 

We have had a lot of visitors in the past couple weeks.  Becca from Austin stopped buy on Saturday to visit her cousin's baby who strangely enough is Ruby's neighbor here in the NICU.  It really is a small world.  Then Amy P came for a visit.  Not only did Amy visit she whipped up some Pousson magic in the kitchen. Grandpa is coming back tomorrow , too.  Ruby is one popular girl.  Once she well enough and cold and flu season is over we might have to take Ruby on a world tour to meet everyone. 

Ruby is also making great strides as far as her recovery goes.  Her chest tubes haven't drained anything in 24 hours.  So, this morning we started her on breast milk through her ng tube for the first time!  If she continues to have no drainage from her tubes they will take them out.  If there is some drainage we'll switch her back to the monogen and leave the tubes in for a while longer.  I'm excited for to her to finally be getting the breast milk.  I have been diligently pumping for almost 6 weeks now.  It's been hard mentally and physically, and I'm happy to see it all might pay off.   The result of her ECHO yesterday was good. The NO has been turned off!  They don't even think she needs the syldenifyl!  Which is good news. Maybe just maybe the PPHN won't be as big of an issue as we all thought. 

Ruby has an MRI scheduled for 3:30 this afternoon.  All CDH babies get an MRI, to rule out any other problems.  I'm glad she's having one if for no other reason than the fact that she spent 25 days on ECMO.  That's long time to be hepranized.  We have no reason to be concerned,  but the chances of them finding something are higher due to ECMO.  She will be heavily sedated for the MRI.  Mostly so she doesn't freak out and to make sure she stays still.  This is why they want to do the MRI before they extubate her.

Now for the big news.  If she looks this good tomorrow morning she'll be extubated!  We've been warned that she might not do well and they will need to put her back on the vent.  Which isn't a big deal, if she's not ready she's not ready.  We'll never know unless we give her a chance. We're on Ruby's schedule and she's setting the pace.  Her docs wouldn't be doing it unless they felt she was ready.  This is a really big deal.  She's been on the vent for 6 weeks.  The only time she had to breathe on her own was the few minutes right after delivery before she was intubated.  She will most likely be on high flow oxygen cannulae to give her some support.  The good news is that she can go home on these if she can't be weaned. She'll still have some tape on her face due to the feeding tube and oxygen, but not near as much. I can't wait to see her with out the huge tubes in the way.

Hopefully Ruby won't have any problems this afternoon or over night.  Tomorrow is another big day for our little girl.  It's amazing how fast things can turn around.  Two weeks ago we were being warned that she might not handle coming off of ECMO or even make it to her repair surgery.  We've still got a long road a head of us and things can change quickly.  For  now though Ruby is a "Rock Star".