Worth a Thousand Words

Josephine is still in CICU and still attached to the ventilator...but she continues to make progress.  Her kidneys have finally decided to start working (YAY!), her lungs are mostly clear, and I'm pleased to report she's a little over one pound up from her birth weight. :)  Our next big hurdle will be helping her gain enough strength and endurance to be extubated.

On Saturday the RT changed out the tape holding Josephine's ET tube in place.  Michael and I stood ready with the camera so that we could capture a few photos of her face, minus all (or most) of the tape.  She's a little red (from having the tape pulled off) and a little unhappy (which you can see from the slightly perturbed, lip-tucked expression on her face), but she's still about the most beautiful baby I've ever seen.  I'm only a tad biased. :)

Baby, I'm Amazed by You

Two tense days intermingled with two calm days...have I mentioned how very up and down things have been as of late?

Josephine is on the mend from a bout with flip flop lung collapse (obviously, not the official medical term. Although I probably heard it half a dozen times, the actual terminology for the condition never really embedded itself in my memory. So for now, we'll go with flip flop.)   After the excitement with the chest tubes over the weekend, we arrived on Monday to discover that Josephine's morning x-ray had revealed a section of haze hovering in her right upper lobe - a partially collapsed lung.  Much to what I'm sure was Michael's utter dismay, I was very vocal in voicing my displeasure to the attending physician on duty, an extremely nice woman who we had not previously met but who was quite kind in accepting momma bear's angry rant.  Despite what seemed to me to be a concerning issue, Josephine managed quite well over the course of the day.  Her stats remained good, and her breathing seemed fine aside from the normal difficulties associated with having a ventilator tube.  The next day brought with it a good deal of respiratory therapy to address the lung issue.  Josephine was positioned on her left side to give the right lung a chance to re-inflate, and the therapist spent some time using the ventilator bag to condition Josephine's lungs and help the right side snap back into position. 

Wednesday morning's x-ray showed a huge improvement on the right side...but the left side was now covered in a grayish snow just as the right side had been earlier in the week.  Another collapse, only this time on the side that had previously been healthy.  The RT team was again deployed to help remedy the issue using a protocol that continued the techniques of the day prior and added a rather curious new method that employed a vibrating massager to help loosen secretions.  (Not surprisingly, little diva rather enjoyed her new massage therapy.)

While her lungs are still not perfect, today's x-ray looked significantly improved.  The problem in the left lung has been corrected and only a small section of the right lung has failed to return to normal levels.

As I sit here watching Josephine sleep and listening to the squawk of her ventilator, I'm amazed at how resilient she is.  The past week has been a challenge for all of us, but through it all Josephine has remained calm and maintained her sweet demeanor (with the exception of about fifteen minutes of utter panic while she was being bathed yesterday.  I think baths may rank just below suctioning in Josephine's world.)  Most days, you'd never have known she was dealing with anything outside of the norm.  She's beautiful and tolerant and strong.  She's our amazing little girl.

Riding a Roller Coaster in the Dark

Someone once told me that the emotional impact of losing a loved one is like riding a roller coaster in the dark: you know the lows are coming, but you're never quite certain when they'll hit.  While our current circumstances are very different, I'd say the past few days have brought on a similar sort of stress.  One minute we're calm and happy and feeling positive.  Within mere moments, however, we find ourselves blindsided by an emergent situation that we never could have anticipated...riding the roller coaster in the dark.

Each of the last three days have included some amazing moments with our sweet girl.  Josephine has been periodically awake and very alert.  Although she's still not moving a great deal (primarily because of the ventilator), she has taken to making the most adorable faces.  My favorite among these has been the "oh-my-goodness-there's-something-in-my-mouth-oh-wait-that's-my-tongue" look, which is typically followed by a sweet bubbly-mouthed smirk and a cute eyebrow raise.  (We'll soon start teaching her the "are you kidding me" single brow raise, a trademark look that I know will serve her well in the future.) :)

The past three days have also included some fairly discouraging setbacks.  On Friday during Rounds (which I'll grant her, was a pretty darn good time...I mean, if you're gonna get yourself into an emergency situation it seems wise to do it when a group of doctors is standing outside your door), Josephine began to desaturate.  (Essentially this means the oxygen level in her blood began to drop...and fairly rapidly.  A person with a "normal" cardiovascular system generally stays at right around 100% saturation.  Josephine's range is slightly lower - between 75% and 90% most days.  She dropped to 63% in a matter of mere minutes.)  After ordering and evaluating a chest x-ray, the doctors determined that she had developed pleural effusions on both the right and left sides.  To remedy this, they decided to re-insert a chest tube on her right side in the hope that the fluid - as well as the fluid from her left side - would drain out and relieve the pressure being placed on her lungs.  The procedure, while emotionally traumatic for Michael and I, went well.  Josephine rested and relaxed for the remainder of the afternoon and evening.

Saturday started with renewed positivity.  We were optimistic that things would now head in the right direction, and based on the morning's events (some quality eyeball time with Josephine), we felt certain that the right decision had been made.  Around 4 p.m. Josephine began to spontaneously desaturate again.  The nurses and respiratory therapists jumped into action and, after around an extremely tense hour of repositioning, suctioning and tweaking ventilator settings, they managed to get Josephine stabilized.  The effusions were once again determined to be the cause of the drama, yet rather than place a second chest tube, the doctors opted to try pharmaceutical therapy - lasix and albumen.

In cardiovascular terms, Josephine stayed relatively stable overnight from Saturday to Sunday.  Her heart rate, blood pressure and oxygen saturation remained within reasonable limits.  During her bath early Sunday morning, however, the nurse noticed that her chest tube (the one placed little more than a day prior) had come loose...the only option at that point was to simply remove it all together.  A few hours later Josephine's daily x-ray showed that the loose chest tube had allowed air into the pleural space - a pneumothorax - as well as moderate to severe effusions still growing on the right and left sides.  So, at around 9:45 a.m., Josephine underwent another procedure (the second in less than 48 hours) to place two additional chest tubes.

Currently, Josephine is stable.  (I'd even say she seems remarkably well in spite of the drama that has taken place over the past several days.  She's still clutching her pink pony, and sporting her gigantic hair accessories and matching socks...I'm not sure she even realizes anything is different.)  We're a little disappointed at the idea that she has taken what seems to be a step backwards, yet we're hopeful that the intervention will succeed in mobilizing the fluid so Josephine can move toward extubation. She's a fighter, no doubt about it.

Looking forward to a better day tomorrow, followed by a miraculous week! Thanks for your prayers to that end.

Patience is a Virtue

Josephine has been making small baby steps of progress over the past few days.  The foley catheter, which has been a very on and off thing, is currently out.  Her belly drain has also been removed.  The fentanyl drip has been stopped in lieu of methadone and morphine, both of which will prevent the withdrawal symptoms Josephine is expected to experience after two full weeks on narcotics.  While the ventilator tube is still in, the respiratory therapists have started the weaning protocol.  (Essentially, this involves tests twice daily to insure that Josephine can remember how to breathe after two weeks of having it done for her.)  The most exciting thing I can share is that she has become noticeably more aware.  When she opens her eyes and looks at us, we see a sweet little spirit instead of just blank, glazed-over glances.  These glimpses into our little Josephine are still farther between than I would like, but the fact is they're happening...and I wouldn't trade that for much of anything at the moment.

This afternoon the doctors started Josephine on a new diuretic to help get rid of some of the chest edema that has plagued her since the first surgery.  Michael and I are optimistic, but somewhat nervous about this development.  The last diuretic, lasix, was too harsh for Josephine even at very small doses and may have contributed to her kidney problems.  Dr. Nugent, the attending physician this week, has assured us that this new medication - fenoldopam - was selected because it is gentle on the renal system.  Once Josephine can shed some of the extra fluids she'll be able to breathe better, and hopefully, will finally be able to come off of the ventilator.  As with everything else, it's a very delicate balance that we're praying will be successful.
 
On a lighter note, our Pheeny has become quite the little diva.  This afternoon, she was treated to a personal training session (a.k.a. Physical and Occupational Therapy), a private concert (a.k.a. Music Therapy) and a luxury baby massage (a.k.a. BABY MASSAGE!)  Perhaps tomorrow will bring a blowout (not of the diaper variety) and a relaxing mani-pedi.  Here's hoping. :)

No News is Good News

They say no news is good news.  I think in our situation - at least this week - that applies.

The past couple days have been fairly quiet.  Slowly but surely, Josephine's kidney function is improving.  She is now off of the epinephrine, and the sedative/pain medication has been decreased to only a very small dose.  Her heart rate, blood pressure and oxygen saturation levels have remained stable.  The doctors have been optimistic, but very non-committal.  Josephine is getting things accomplished on her time...and despite the fact that we're anxious to get her off of the ventilator and moved into the step down unit, Michael and I are working really hard at the patience thing.  (Not always an easy task, and especially so when we've not been able to hold our sweet girl for nearly two weeks now.)

During our time at CMC, we've managed to go through no less than 20 nurses.  Although we definitely count some as being better than others, we've noticed that they all tend to observe a few key things about Josephine and our current state.  From these comments, we've compiled a rather telling list of the top five things heard in Josephine's room over the past three weeks:

5. Have you been getting sleep?  Are you eating?  (The sleep one is usually directed at me, so I can only guess I've been looking a bit rough as of late. To be honest, I'm not totally sure how to answer this question any more...does it really count as sleep if you dream about being at the hospital? :p )

4. Josephine.  Is that a family name?  (This is usually followed by "What are you going to call her?")

3.Wow!  She's got really good pulses. :)

2. Ding, ding, ding! (I'm sure I mentioned the equipment alarms in a prior post.  They are absolutely relentless and incredibly annoying.)

1. Look at her long fingers/big feet/cute nose!

Hopefully sometime very soon we''ll be able to add one more key statement to the list: "You're going home."  :)

Living on Hospital Time

I have officially transitioned to hospital time.  Similar in form to summer break when you're a teacher (only without the intrinsically carefree component), living in hospital time involves completely losing all notion of day, date and hour.  It is simultaneously agonizingly slow and faster than can be rationally comprehended.

It's hard to believe Josephine is already just over two weeks old.  While we're pleased the doctors have decided to move at more of a conservative speed this time around, both Michael and I are anxious to get Josephine out of the hospital and settled at home.  She's absolutely making progress toward that end.  Over the past several days, Josephine has been weaned from her dopamine and epinephrine (although the epinephrine continues to make an appearance at low doses from time to time.)  The hard chest tube - which was reinserted during the third surgery - has now disappeared, and the soft chest tube was removed yesterday because the stitch holding it in place had loosened and the tube had started to slip out.  The pacer was turned off today and Josephine has continued to breathe over and above the ventilator.  In addition to the standard bounty of monitoring lines (which includes stickers on her head and back to monitor oxygen distribution, wires strapped to her feet to measure temperature, and the red glowy "ET" device - which is now attached to her toe - to measure oxygen saturation), she still has the oral ventilator tube, an ng tube, intercardiac wires, and a belly drain, plus a number of ports from which they can draw blood and administer various medications.  The status of the tubes and wires is in constant flux.  Something is eliminated most every day...so, most every day we're closer to getting out of CICU and into the step down unit.  We're definitely thankful for that.

The biggest struggle over the past few days (aside from my emotional struggle when Josephine breaks out the "Momma, help me" eyes - usually in response to being suctioned, having a diaper changed or passing gas) has been her kidneys.  Apparently, they didn't much like the three surgeries or the high doses of antibiotics, so have decided to rebel a bit.  The doctors have adjusted her nutrition formula in an attempt to lighten the load on her kidneys a little and give them time to recover.  Since extubation (and finally seeing Josephine's face without huge masses of tape stuck everywhere) depends on kidney function improving, we're hoping the change in formula will do the trick.  

As always, thanks so much to all who have supported us over the past couple weeks. Your prayers and thoughts are so appreciated! 

Gratitude

We're grateful for the times between the chaos that aren't filled with dings and beeps and nurses rushing in and out of the room.  We're grateful for the calm that grants solace and allows us to take in the sweetness of our precious little girl. We're grateful for days like today.

Our weekend began in a wholly unexpected and harsh way.  After Josephine's emergency surgery  on Thursday we were told one of Dr. Forbess' partners, Dr. Guleserian, would be closing Josephine's chest early Saturday morning.  Our plan for the evening prior was easy enough: we'd come home to get a good night's sleep (which typically ends up being about 4 - 5 hours after trying to manage all of the things we can't do from the hospital during the day), then get up at 4:30 a.m. to make the drive back to CMC and love on Josephine before surgery.  Our evening was productive.  A load of laundry was done, bills were paid and a few basic housekeeping tasks were completed.  When we crawled into bed at about 11:30 p.m., everything with Josephine was fine (which is to say, stable.)  And then, as I was exiting the shower at about 4:30 a.m., another of the dreaded phone calls.

Sometime during the wee hours of Saturday morning, Josephine's nurse noticed that her chest tube had begun to drain large amounts of bright red blood (this, after draining only serous fluid for the entire day before.)  Wisely, the nurse called the attending physician who got in touch with Dr. Guleserian.  By the time they contacted us, the doctor and her entire surgical team were on their way to the hospital to once again perform emergency exploratory surgery on Josephine - the second time in barely as many days.  Dr. Guleserian was able to quickly find the source of the bleed and get it sutured, and felt confident enough to go ahead and close Josephine's chest.

It's hard to come to terms with the fact that our sweet girl - who is now just 12 days old - has already had three surgical procedures (which is more than most adults I know.)  It's hard to think she might be in pain or scared, and it's really hard to think about the possibility we could lose her before we even really get to know her beautiful spirit.  Thankfully, days like yesterday only come so often...and hopefully, they'll start to come much less frequently (or disappear all together) as we progress through this process.

We're praying for more days like today...quiet, stable and calm, yet productive.  We're praying to soon see Josephine open her eyes and interact with the world again.  We're praying to have the chance to hold her and cuddle her and love her.

Scrunchy-face is due for another appearance...and soon.  Here's hoping tomorrow's the day.

Back to Square One

Josephine had a very rough morning.  When Michael and I arrived back at the hospital at 7:30 a.m., three to four nurses were in the middle of intervening for another drop in blood pressure.  Josephine looked a bit pale and was unmoving because of the paralytic (which they explained was given so that she could completely relax and they could do all of the work for her.)  A few of the stitches in the middle of her chest had come loose and were covered by a piece of gauze spotted with blood.  The right side of her abdomen where the chest tube had previously been removed was bruised and swollen. Needless to say, to see her this way was upsetting...particularly when she had been moving and responsive less than 12 hours prior.

As we waited for them to stabilize her, it became evident that things were not improving.  Pretty soon more nurses entered the room, followed by doctors.  Josephine was now surrounded on all sides, waiting for blood to be delivered from the hospital pharmacy to compensate for her low hemoglobin.  (The acceptable level, we later learned, is 14 - 15.  Josephine was at an 8.)  For the first time since Josephine's treatment began, the Attending Fellow turned to us and said she was "worried", her thoughts amended only a few minutes later to "very worried".  Soon after, Dr. Forbess entered the room.  He took a quick look at Josephine, glanced at her stats and decisively announced, "It's not what you think.  I'm going to fix this."  Michael and I were then escorted from the room as they prepared it and Josephine for emergency surgery.

So where do we stand now?  When Michael and I left the hospital this evening, Josephine was stable.  As it turns out, during one of the early morning blood pressure episodes the nurses began to suspect that Josephine's shunt had clotted - a catastrophic event.   To remedy this, a bolus of heparin had been administered.  The heparin thinned out Josephine's blood and caused some small undetected bleeds in her chest to bleed out of control.  As the blood filled her chest cavity, it compressed the shunt and surrounding arteries and veins, including those that supply blood to Josephine's lower body. Dr. Forbess absolutely made the right call.  By acting when he did, he was able to relieve the pressure in Josephine's chest, clear out the excess blood and suture the bleeds before supply to Josephine's head or gut were compromised.

While I'm so thankful that our sweet girl has been given a second chance, I'll admit that I am also disappointed - we're now back to square one.  Due to the high risk of further bleeding and swelling, Josephine's chest was left open after today's surgery.  She now has a 1 -1 1/2" tape-covered window that extends from just below clavicle level to just below sternum level.  She is back on the paralytic, totally dependent on the ventilator and all of the medications from which she had been weaned are back at full strength.  They've told us that her chest will likely be closed this weekend, after which they'll begin to deescalate treatments just as they did before: first by eliminating the paralytic and gradually lowering the dosage on the dopamine and epinephrine, and eventually, by removing the breathing tube.  But instead of moving to the step-down unit tomorrow, it will be at least another week (if not more) in the CICU.  All of this, of course, depends on how well Josephine's body reacts. 

Today was rough.  Tomorrow will certainly be better.  Please keep our Josephine in your thoughts and prayers.

The 3 a.m. Phone Call

The 3 a.m. phone call from the hospital where your child is being treated...not something I'd wish on anyone.

Wednesday was an exceptional day for Josephine.  By early morning, she had been weaned to only a small dose of epinephrine.  The paralytic had worn off  completely, so she was able to initiate breaths over and above the ventilator and was doing so consistently.  The doctors O.K.'d removal of her chest tube and had decided to add an ng tube to begin feedings.  The respiratory therapist was called in to begin ventilatory tests.  Josephine passed each one with flying colors, so she was scheduled to have her breathing tube removed today.  She was doing better than anyone expected, and there was talk of moving to the step down unit by Friday. 

And then the phone call...

Sometime around 3 a.m. this morning, Josephine's blood pressure began falling which caused her blood oxygen levels to decrease significantly.  To counteract, they increased her epinephrine and put her back on the paralytic. :(  The breathing tube will definitely not come out today.

I'm absolutely heartbroken that this world isn't treating our sweet little girl better. 

Please send your prayers and positive thoughts our way.  We all really need them right now.

Things That Go Ding in the Night

I've heard more times than I can count that you don't get any real rest while in the hospital.  Monday evening's campout in Josephine's room definitely proved that statement to be accurate.

Josephine's surgery on Monday morning went exceptionally well.  They got started on time, were able to get Josephine prepped and to sleep without issue, and performed the procedure almost exactly as expected:  Dr. Forbess ligated the PDA and placed a 3.5 mm shunt between the carotid artery and the pulmonary artery.  (This was a slight deviation from the original plan, but it turned out it worked a little better with Josephine's anatomy.)  They had her cleaned up and back in her room by around 12:30, and although it was extremely difficult for us to see her laid out in the bed with all manner of tubes and wires connected to her, we felt fortunate in that they were able to close her chest.  (Sometimes the surgery requires the chest be left open for a few days. Yikes.)  Her vitals, even immediately after surgery, were all really good.  She didn't appear to be too swollen, was a fairly nice rosy color due to better-than-average oxygen saturation levels, and had a decent heart rate that didn't even require pacing.  Our one disappointment was that she had to be placed on a breathing tube which went through her mouth instead of her nose.  The next several hours were entirely uneventful.  We celebrated that Josephine had made it through the procedure, blissfully unaware of what was to come in the night ahead.

Around 5:00 p.m., the dinging began.  Out of basically nowhere, Josephine's blood pressure started falling and a cascade of medical interventions were set into motion: calcium was administered, epinephrine and dopamine drips were started, and three rounds of packed red blood cells were infused.  Because many of the treatments were designed to counteract each other, it was a delicate balancing act which danced back and forth over and over again. With every added intervention, a new series of alarms dinged and beeped, letting us know something was out of balance or needed attention.  This went on well into the night.  Needless to say, very little sleep was had by either of us.

Almost as suddenly as it all started, everything evened out and the alarms finally stopped.  While I can never know for sure, I feel almost certain I know the reason for all of the drama.  During the quiet time early Monday afternoon, a nurse made the mistake of calling Josephine "boring" - compared to most other heart patients, she had needed little care and required few interventions up to that point.  I quickly corrected the nurse and said that Josephine was just an A+ baby, but Josephine had heard the slight and didn't take kindly to it.  Our feisty little one then proceeded to show that nurse just how entertaining she could be. In the future, I imagine the medical staff will think twice before offering any blatant challenges like that to our Josephine. :p

Today went mostly well, in spite of another rather frustrating round of repeat interventions that took place in the afternoon.  Josephine was briefly taken off of epinephrine, dopamine and the pacer, but was placed back on due to falling blood pressure.  On some very positive notes, her foley catheter was removed, the paralytic was stopped, and she began to move and initiate breathing on her own.  She even opened her eyes and wiggled her toes a little.

As we head to bed tonight, we know without a doubt we've got a long road ahead...but Josephine is definitely moving in the right direction.  We're so proud of our little fighter!

Josephine's Big Day

The past couple days have gone well.  In addition to taking her first meal - of which she ate about 45 ml (Momma was so proud :) ) - and learning some truly adorable new expressions, Josephine has remained medically stable.  The doctors started her on Billiblanket therapy due in part to rising Bilirubin levels (indicating slight jaundice) and to inhibit excess bleeding during surgery.  I'm not sure Josephine has enjoyed the glowing blue light of the Billiblanket, but as with all things so far, she has endured it like a champ and complained very little.

Based on the results of the CT scan done on Thursday, Dr. Forbess and the heart team have decided shunting is the best option for Josephine's anatomy.  Basically, this will involve closing the Patent Ductus Arteriosis (held open since birth by IV prostaglandin therapy) and placing a 3 mm Gor-Tex tube between the aortic arch and the pulmonary artery.  Doing this will insure that, for now, Josephine's blood will be able to travel from her heart to her lungs to be oxygenated.  Because Josephine will continue
to grow and the shunt obviously won't, the solution is only temporary and will be followed by another procedure in 4 - 6 months when she is larger and has gained some strength.

At this point, I think we're equally excited and terrified.  Excited because Josephine has managed to make it this far and is stable enough to undergo the surgery.  Terrified because...well, she's our baby.  It's hard to turn her over to someone else and trust that everything will be O.K.  I've said from the very beginning of the pregnancy that it has been a lesson in surrendering control (which is SO hard to do.)  But really, there's no choice.  Tomorrow we surrender our precious Josephine to the hands of the surgeons and the will of God.

Officially, surgery should begin at around 8:15 tomorrow morning and continue through lunchtime if all goes well.  After surgery, the most critical period will be the following 8 - 12 hours.  Prayers and positive thoughts on Josephine's behalf would be greatly appreciated during that time.  We know she has made it this far because she feels the love shown by our wonderful family members, friends, coworkers, church groups, prayer circles, friends of friends and acquaintances.  Amazing things can be accomplished through love...Josephine is the proof.