Ruby Hope McKee Starling

Well...
Ruby is now 20 days old and has been on ECMO for 17 days.

Ruby is having a hard time. She is still shunting and very labile. Today is one of the days where it is very hard to have hope. She was not supposed to be this sick. She has plenty of lung tissue. She has severe pulmonary hypertension. We are slowly but surely ruling things out. She is on antibiotics to rule out infection. They are increasing her sedation to see if that helps. The other cause could be that her hypertension is so bad that ECMO isn't helping her. If that's the case the prognosis isn't good. No one is at the point of giving up, yet.

We've all had rough day today.

We had an uneventful night. Chris and I slept in a sleep room here in the hospital. We were right down the hall from Ruby. It was nice to be so close to her. We only stay the night when something major happens. We will hopefully go back to our apartment this evening. The hospital offers these sleep rooms to parents of the sickest babies. So the fact that we qualify for one is kind of distressing in and of it self. I am however grateful that they have them.

Ruby is quite swollen again, which we expected. Her doctors are continuing the antibiotics for a total of 7 days just to be safe. This makes us very happy. In the past week they would stop the antibiotics after a couple days when they couldn't identify an infection. This time even though they feel strongly that the positive test was a contaminate, she was clinically showing signs of infection. So they are giving her a full course of antibiotics. We are hoping that a full course of antibiotics and the ECMO circuit change will be what it takes to get over this latest road block. Her doctor has said she can stay on ECMO as long as she needs. We, however, would like her to get off as soon as possible. We just need Ruby to tell us that she's ready.

The plan for today and tomorrow is to just let her rest. They are going to cut back on the ECMO support since they had to up it with the change yesterday. She's going to continue receiving the antibiotics for another 2 days (for a total of 7 days). Then on Friday will start pushing her kidneys again to try to get the fluid off.

Mom and Dad need a couple rest days after the last few, too. I'm looking forward to a couple days of just watching her sleep and holding her hand. I have a feeling the roller coaster ride will start up again this weekend.

Chris and I are managing to get out a bit. Yesterday we walked to a little shop down the street and I got a new pair of flip flops (nothing like a little retail therapy). Hopefully we can go out to dinner today or tomorrow, too. I hate to go to far and stay gone for long, but I also understand that we need to get out. Plus my doctors and midwives come down and check on me periodically. I want to be able to report them that I have gotten out and that I am taking care of myself. I guess that's a perk of the Special Delivery Unit. It is really nice that they really do care about how the babies they deliver are doing.

We have some pictures of Grandma and Ruby that we need to post. We also have proof from Governor Rick Perry that Ruby is an official Texan! CHOP's network won't let me upload pictures. Hopefully this evening when we get to the apartment we can post those things for the internets to see.

Ruby's doctor was in favor of a new circuit, so we went ahead and swapped it out about noon today.  The actual switch took about a minute and she did fine during the moments she was off ECMO.  It took a lot of sedatives to get her to calm down, but she's finally asleep now.  Her stats are currently good, but she has more support from the ECMO and medication than she previously did.  Once she's adjusted to the new circuit, they'll ween her down to her previous levels of medication and ECMO flow.    We expect her to get worse before she gets better, since she'll undergo another inflamatory response to the new tubing and equipment that her blood is coming in contact with.  But then she should be able to get better.  We're going to sleep at the hospital tonight in case she has more instability, but I'm hoping it's for nothing. 

We had rounds this morning and the prevailing thought is that she could have an infection that's not being picked up by the blood cultures, possibly because she's receiving so many transfusions of blood products (usually 1 platelets and 1 FFP or Cryo every day -- I'm not sure of the quantities, but they usually look like about 60ml).  So the positive culture was definitely a red herring, but there could still be a bug lurking in there.  Another possibility is that her body is just getting irritated by the ECMO circuit and is having another inflamatory response. 

Either way, we're going to stick with the antibiotics and there is serious discussion of switching out her ECMO circuit.  This means her body will have to adjust to all the new tubing and lung membrane and will likely increase her edema in the short term.  But if there is an infection in her blood, it will literally remove half of it.  And if she's irritated by the circuit, it'll give her a fresh one.  Additionally, with her circuit being 14 days old, the tubing and connections and membrane and bladder are nearing the end of their expected lifespan.   Even with our modern technology, we can't build better blood vessels than the ones we're born with.  Nothing is definite yet, though.

She seems a little more alert and active today, but she's more swollen.  However, she gained less weight in the past 24 hours than the previous 24 -- around 160-something grams.  She's rather fussy right now and she just got a dose of fentanyl.  They also went up on her morphine/versed infusion.  Being so swollen is not comfortable, I'm sure. 

Ruby had a rough day today.  She gained 200g since yesterday -- nearly half a pound.  This weight gain is due to fluid retention.  Her urine output dropped significantly and she is very visibly swollen again.  She slept most of the day and it was obvious that she is not feeling well.  This morning during rounds, the doctor said that she could only assume at that point that this was all due to an infection, given the positive blood culture.  However, later in the day the Infectious Disease department got back with results that seem to indicate otherwise.  All subsequent cultures that have been taken have come back negative -- no bacteria.  The one culture that was positive grew a bacteria called corynebacterium, but not the diptheria or "JK" group, which are more serious.  The type of corynebacterium in the positive culture is commonly present on most people's skin and typically doesn't infect a person or cause sepsis.  So the Infectious Disease doctors believe it was a contaminated specimen, and Ruby does NOT have an infection.

So we're basically back to the drawing board as to why Ruby is swelling up again.  This is very frustrating.  We were making such great progress and on Saturday she looked really great.  But now we're heading in the wrong direction quickly.  We're on day 14 of ECMO and every day, the tubes that make up the ECMO circuit are aging and wearing more.  If any parts of it need to be replaced, Ruby will have to go through the inflamatory response again and likely will swell up even more.  Also, every day she's on ECMO is another roll of the dice for complications, so we really want to get her off as soon as possible. 

In a way, we were kind of hoping for an infection, since it has a very clear solution.  But it would have meant having to stay on ECMO until we were 100% sure it was gone, which could have meant another 7-14 days.  If we can just get the edema to go away, then we can start moving toward going off ECMO and having her repair surgery sooner than that..  So I'm hoping that not having an infection is a good thing.  Maybe the extra days on ECMO will be exactly what she needs for her lungs to heal and be free from pulmonary hypertension. 

Even though she was asleep most of the day, she would wake up enough to grab and hold onto our fingers.  Holly read her Dr. Seuss's "Oh The Places You'll Go", which she does every day.  Hearing our voices seems to make her content and sometimes even calms her down from a shunting fit.  So the Ruby that we know and love so dearly is still there.  She's just not feeling very well.

Now, for a few hours of sleep and then back to the NICU in time for early morning rounds again. 

Ruby had a busy weekend!  My cousin Malissa came down to visit from NYC:

Ruby is doing OK, but still hasn't peed off all the fluid she needs to before they can take her off ECMO.  She's getting close, and they started a new diuretic (bumex) today, after deciding that the other new one didn't work.  She's nowhere near as swollen as she used to be, but they'd like her to be as good as possible before taking the next big steps.  But those steps may be delayed...

The big bad news of the day is that one of her blood cultures came back positive for an infection.  They routinely take cultures from the umbilical and ECMO lines in her to make sure they're not getting infected.  The ECMO culture from 48 hours ago grew some gram positive rod bacteria.  Ruby's Grandma, who works in the lab at a hospital, said that it is most likely that the specimin got contaminated due to mishandling and that most of the scary things like staph are not rod bacteria.  More cultures were taken today and we'll see if they grow anything overnight.  Sepsis is the last thing that Ruby needs right now.  They've been worried about it for a few days, since her retaining this last bit of fluid could indicate an infection.  So she's been on super-strong antibiotics for about 5 days now, so it seems unlikely.  And if the latest cultures do come back positive again, she's already on the medication to cure it.  We're just afraid that if it isn't a contaminated specimin and she really does have sepsis, it would push back the timeline to get her off ECMO.  She is NOT running a fever -- I personally checked it today and it was 36.9C (98.4F).  So I'm inclined to think it's just a false alarm, but we're still very, very worried. 

Basically, though, we'll know more in the morning.  They should know if the 2nd round of cultures have grown anything overnight and they should be able to identify exactly which bacteria that is in the positive culture from 2 days ago.  Based on this and how much she's able to pee tonight (they re-installed her catheter), and the results of the echocardiogram that's scheduled for tomorrow, they will hopefully be able to put together more of a game plan for getting Ruby off ECMO and having her surgery to repair her diaphragm.  We'll certainly update when we know more.

Over the weekend, Ruby has become Little Miss Grabby.  She grabs at everything around her and gets in trouble for grabbing the ECMO cannulae ( tubes on the right side of her neck that carry blood in and out of her body to the ECMO machine) and for grabbing the NeoBar (the blue bar taped to her face that anchors tubes that go into her lungs and stomach).  We usually keep the canulae hidden with a blanket, but today she was quite interested in attacking her NeoBar.  At one point, I caught her with her fingernails underneath the tape and she was literally picking at it, trying to tear it off.  So instead I offered her my finger, which she was content to squeeze while kicking her feet in the air for about an hour straight.  Here's the little trouble maker in action:

Today was Ruby's 2-week birthday!

Here's a picture of the happy family:

Ruby got moved to a new room today (note the Yellow walls).  We were pretty worried about this, but the move went very smoothly, especially considering how much machinery had to be rolled down the hallway while still connected to her.  The new room is a bit more cozy, quieter, and is next to the other ECMO baby in the NICU.  The nifty blue armband I'm sporting on my left wrist isn't my admission pass to ACL (Austin City Limits Music Festival).  It's my NICU armband.  Each parent gets one, and when Holly was a patient, I got one with her name on it too.  We decided to take it as a good sign that they felt Ruby was stable enough to be moved, since CDH babies are generally so unstable that they will do their surgery right there in the NICU.

I believe someone had asked about Ruby's personality.  She's probably best described as stubborn and rambunctious.  She's beginning to get a reputation among the nurses for being active and difficult to sedate.  For her move today, they lined up extra-large doses of morphine, Versed (midazolam) and Pavulon (pancuronium), so she could be moved without getting upset.  Pavulon is a powerful paralytic agent, which they use so babies won't squirm while they're working on them.  They assembled an army of nurses, doctors, and specialists to move Ruby, injected the drugs to knock her out and waited for it to take effect.  And waited.  And waited.  After a while they decided it should start to take effect soon so they might as well move her.  But they said she was awake and kicking her feet, looking around and wiggling her fingers for the entire trip, and seemed to enjoy the change of scenery.  She didn't act the least bit upset.  She then proceeded to stay away for the next 4 hours, blowing bubbles, kicking her feet and grabbing things with her hands.  I dare say all the drugs just made her more active. 

Otherwise, she's very interested in her surroundings and enjoys being touched and talked to.  She realizes that the tubes and junk on her aren't supposed to be there and she'll grab at them sometimes.  She likes us to rest our hand on her head and to let her hold one of our fingers.  She also likes it when you hold your hand up for her to kick and push her feet against.  I need to get some more pictures and video of her when she's awake.  It's always so entertaining that I forget to take pictures.

Here's a new photo from today:

We never in our worst fears thought Ruby would be on ECMO this long.  She is peeing and loosing weight, but she still has more weight to loose.  She's been started on a new diuretic and we're going to see how it helps over the weekend.  She is still shunting and on Monday she will have another Echocardiogram to determine if it's due to the excess fluid.  Hopefully after that we will know more.  It was very hard to not get frustrated and discouraged after our talk with the Dr. this morning.  It seems like we are stuck.  She can't be on ECMO for ever.  It's getting harder and harder to not worry about the worst.

CHOP's network is very inconsistant.  Some days it allows us to update the web page and other days it doesn't.  Couple that with extreme exhaustion, it's been hard to update the past couple days.  We are trying and we appreciate all the support.

We got surprise when we got home this evening!  I'm not sure if we ever mentioned what happened with the "Texas Dirt".  We did put it under the delivery bed.  All of the staff knew about it and thought it was hilarious.  When we got to our apartment this evening we found the following waiting for us.

If you notice he stops short of declaring she's a Texan, but it does have the oficiall seal of Texas.  I'm betting it's as close as she's gonna get.  We're not sure which of our friends pulled this one off.  Whom ever did this please know you made our day.

Now for  the Ruby picture of the day.  I spend most of my day right next to her bedside reading, talking, or just simply holding her hand.  It's hard to campture her personality in pictures.  It's there under all the tubes and sedation. 

Ruby had another stable day today.  She's continuing to pee and "get skinny" as her surgeon says.  She's starting to look a lot more like Ruby again.  We can see her facial features.  She is also able to interact with us a bit more now that she's more comfortable.  It's really cute to see see her feet move around.  The Occupational Therapist came by today and gave us some suggestions on things that would calm her.  She said that holding her feet and letting her push them against our hands is very comforting for a baby.  Couple that with putting your hand lightly on her head and speaking softly to her can sometimes calm her down.  I did this a lot today, when the nurse was doing something to her that she hates.  Ruby hates to have her mouth suctioned.  I would try to hold feet and head when the nurse was suctioning her.  I'd like to think it helped.  She didn't need any "rescues" today.  Maybe it did work or maybe Ruby decided she didn't need any help today.

Being parents in the NICU is hard.  There is constant beeping and crying  babies.  Plus we don't ever get any alone time with Ruby.  Due to the severity of her illness she has two nurses around the clock just for her.  One is the ECMO Specialist and the other is for her nursing care.  Neither of these nurses can leave Ruby's room without someone to relieve them.  We spend most of our days in Ruby's 10x10 cubicle with these two nurses.  Various doctors and specialists come by throughout the day to check on all the aspects of her care.  The NICU is a very busy place.  It's very tiring to sit in there all day long.  We, however, want to be with Ruby as much as possible.  I hate the fact that she might open eyes and one of us not be there.  The long days are starting to wear on us.  We left a bit earlier than normal this evening with hopes of getting a bit more rest.  Ruby will hopefully have surgery at the end of this week and when that happens I'm sure we won't have the luxury of getting some extra sleep.

As for her surgery.  We mostly likely won't know when it will be done until the day of.  Her doctors evaluate her several times a day and when they see the timing is right they will jump on it.  Please don't ask us when she will have surgery.  When we know we will let you know.  Until then we are waiting.  We also don't know if she will be taken off ECMO before or after her surgery.  Again the doctors haven't decided yet.

This evening before we left I put another pair of booties on Ruby.  These tie on.  Hopefully she won't kick them off as easily.

Updates will probably come from me during the week.  Chris is taking advantage of the rest days and working. 

Ruby's lungs have opened up again!  Her latest x-ray looked better.  She has been peeing  A LOT.  Her catheter bag is getting really full.  This is all good news.  We talked to her surgeon today.  She said that Ruby isn't ready for surgery yet.  She still needs the ECMO support.  We are just taking it day by day.  As soon as she gets a window to do the surgery she will.  We trust Dr.HH decisions and hopefully Ruby will give her the signs that she needs soon.  While we understand we are on Ruby time, it would make her parents feel better to have her off of ECMO and her surgery done.

Keeping her sedated is one the biggest issues the past couple days.  Ruby wants to stay awake and join the party.  We need her to rest and not move much.  As a parent I love interacting with her.  I do understand the need for her to be still.  The canulas for the ECMO go directly into her heart.  So squirming around is not a good thing.

Yesterday was a tough day for all of us.  Some days it's harder than others to stay positive.  It's especially hard when Ruby throws a curve ball at us and seems to take a step in the wrong direction.  It's hard to remember that this is a roller coaster and will have ups and downs.

Ruby's nurse today changed the tape and repositioned the tubes in a different way.  We can now see more of her face!

When she is calm enough I've been able to help do parts of her care.  I've gotten to change her diaper, take her tempurature, and wipe drool off of her face.  Her finger nails are getting kind of long, but we can't trim them.  ECMO requires her to be on heprin.  They don't want to risk any bleeding.  No manicures for Ruby for a while.  Helping with her care has really made me happy.  it's hard to sit and watch someone else care for your baby.  I understand the need, but it is hard.  I'm sure in a few months I'll be thinking how crazy i was to be so excited to change her diaper.