Waiting....

God I'm so nervous. Aurelia has been in surgery for two hours now, the first being spent with anesthesia and this last having her chest open, tubes and lines placed, and getting through her scar tissue. Reports from the team are positive so far, but I cannot fathom how Aurelia endures this. I cry thinking about how strong and determined she has to be, without choice, in order to live. I would do everything and anything to take this burden from her, to let her live without this pain and trauma.

I know some people believe that young children do not remember events such as these, but I believe they do. I believe that any person, no matter how young, remembers these traumatic events and that they are imprinted, that their development as people is changed forever. I don't fear these difficulties impeding Aurelia's development and personality, but rather I think the courage and strength and happiness she shows now will only be magnified, increasing as she ages, as these traits have served her best today and so far.

When she comes back from surgery Aurelia will have a number of lines placed in her body. She will have a chest drain, an arterial line, pacing lines, foley catheter, and an IV. Her chest will hopefully be closed, packed, and covered. Our primary concern then will be two fold (if all else goes well): will Aurelia's smaller left ventricle be capable of pumping blood for her entire body without assistance and will her heart's electrical tissue be damaged, requiring a pacemaker? Unfortunately, one of her VSDs is located in and around a place where her heart's electrical tissue exists. It is possible that Dr. Spray will have to stitch in that area, and if so, it may disrupt her heart's normal rhythm.

So for now, all we do is sit and wait.

Will these scares always be with us?

Yesterday was very long for Jillian and I, but even longer for Aurelia. We're stressed about so many things. Jillian is simply done with being away. I don't blame her. She hasn't said much at all on the blog about her ordeal, but this has been one of the hardest paths she's ever walked, if not the hardest. She's finished with being at the hospital, being away from home, living out of a suitcase, keeping Madison away from home, trying to balance her needs, Aurelia's needs, and the feelings and emotions of family and friends, and putting up with a sometimes petulant husband.

For my part, I've shared some posts laced with some of the emotional challenges I face and did so again yesterday as we moved throughout the day's surgery. The partial nissen went well, as did the hernia repair and her g-tube installation. She spent the evening in the CICU still intubated and under heavy sedation. One bonus of being with this team of amazing professionals for so long comes from working one on one with nearly every attending, fellow, and resident that cycles through the sixth floor heart units. For example, her surgeon yesterday was the same surgeon that did Aurelia's intestinal operation on day 1 of life; her anesthesiologist yesterday was also the one that repaired her first PICC line after it went bad a few weeks ago (a month ago already, maybe?), and the attending doctors last night and today/this evening, Drs. Naim and Kirnch, both cared for Aurelia for weeks at a time.

This mattered to us because the team knew to keep Aurelia sedated while still intubated or else risk her becoming fiercely worked up and potentially at greater risks for stress-generated troubles. It took longer than we hoped for her to be extubated today, but given her respiratory history their caution is understandable. As such she's still on high flow room air through a gargantuan nasal canula, but other than the discomfort that's causing her, her breathing has been good.

Her g-tube looks good and if she maintains positive numbers through night rounds they will start her up on feeds, probably at 1/4 her caloric needs. We're excited about this for obvious reasons, but also because Aurelia had been on fluids only so much this last week that her electrolytes have been all over the map. So much so that tonight, while Jillian and I were in a class on how to administer feeds and medication through a g-tube, her heart rate began to stutter with a few early beats before crashing to the 70s. Naturally this caused more than some panic. Thankfully our girl showed her grit and brought her rate back up on her own and showed no real issues on her EKG, meaning they didn't have to administer any real kind of resuscitation.

So, now with her hernia repaired, her nissen hopefully helping with her reflux, and barring any more cardiac scares this evening, we hope to be on the path to getting fed, stable, and out of here in a couple of weeks.

And yes, we're crossing our fingers. We're not really getting our hopes up because we've been there too many times before, but we are starting to finalize our action plan for all the things that have to be in place for departure so that whenever it happens we will be able to focus on our girls and not administrative poppycock.

Hope you're all doing well, and as always, thank you for your prayers and love, and support.

A whirlwind of thoughts, feelings, and happenings

We know it has been some time since our last post, but things in the Cardiac Care Unit have kept us on our toes. In this post we hope to give you an update on all of Aurelia's conditions, the plans we have to help her, and some of the other goings on involving our little family as we continue our pilgrimage here at CHOP.

This week has been a tale of competing ideas. Our primary attending prior to yesterday's shift change, Dr. O'Connor, kept a very close eye on Aurelia's breathing, hoping to take her off of room air flow (2 liters) by the end of this weekend. There has been a good deal of concern with Aurelia's breathing. Typically, cardiac kids her age are operating their respiratory system unassisted, but Aurelia is a bit different and so we are trying to figure out why she needs flow assistance when it comes to breathing.

Similarly, Aurelia's progress with bottle feeding is also a concern. While cardiac infants often struggle to get started on the bottle, there is a good chance of success to not only resume bottle feeding, but eventually transition back to breast feeds. Aurelia had been taking about 5 ml on the bottle during her feeds, but she wasn't making much progress otherwise and was having some trouble with acid reflux. As a precaution Dr. O'Connor called in the specialists from Ear/Nose/Throat to examine her esophagus. It turns out that Aurelia's streak as a great gambler continues. She once again came up triple 7's when betting on her vocal cords being paralyzed during heart surgery. E/N/T found that her left vocal cord came out paralyzed and this most definitely would impact any attempts to bottle feed (as would her cleft palette). This can also make breathing a bit more difficult as well.

Even with these small changes the team decided not to proceed with the heart catheter that had been scheduled for either Friday or Monday. Dr. O'Connor felt that the data on heart pressure wouldn't really give us any more information that would be useful regarding her breathing, particularly since her echo-cardiograms came back so excellent both last Wednesday and Friday.

The plan would be to use this weekend to wean Aurelia off the room air flow and begin to talk about discharge sometime early next week, getting us home by the middle of the week. Jillian started talking to our case manager to arrange for home care and equipment use for Aurelia's journey home. She arranged at least two to three home nurse visits and set up our rental of a feeding pump. She also will make sure we have a family meetings sometime early next week to talk with all of the members of Aurelia's team to be sure that we have plans in place for all of Aurelia's problems.

A family meeting to coordinate care is a real concern for us as we move closer and closer to coming home. The details are getting so numerous that it is hard to track them and the big picture at the same time. For instance, Aurelia's feeds here are supplemented with 22 calories of enfamil formula, which is something that we have to demonstrate proficiency in before leaving. We also had to take an NG tube class and a CPR class yesterday and get briefed on how to use the pump. Details like these are important but begin to block out how it all fits into the big picture, particularly when you add in the seven medicines she gets, the different timetables, and cleaning procedures for all the equipment.

All of this became a bit more complicated when Aurelia exhibited respiratory distress Friday night into Saturday. The result was bumping her back to 2 liters of air flow (she had worked herself down to 1 liter!) and avoid weaning her off before discharge The plan to discharge now included her going home with oxygen and us leaving on Monday (according to our nurse). Leaving Monday?!?!?!  When we heard this yesterday, we were stunned.

Needless to say, our nurse giving us this information greatly distressed everyone. We had no training on using oxygen tanks at home, or as portable devices, we didn't even know if our case manager had or could make arrangements for all the needed equipment before discharge on Monday! And what about our family meeting? And was there a class we needed to take? And we hadn't even inserted her feeding tube on our own, OR done 24 hour care! And what about the urology test that was to come up in a week or two? And what was the plan with plastics? Or with Occupational Therapy and Speech Therapy?

Complicating issues was a shift change in attending doctors. Dr. O'Connor went off as our primary and Dr. Vetter came on; we did not know what to think about this change because it seemed that once again when the weekend came with its new doctors and nurses the pace to have us discharged quickened. We felt genuinely overwhelmed and under-prepared and upset that Aurelia would be leaving here on Monday. We felt a bit frantic, I think, yesterday afternoon when Aurelia's acid reflux took a hard toll on her. She had several spit up incidents, needed a lot of suction, and cried her small vocal cord paralyzed cry for most of the afternoon. So sad, that cry.

Thankfully, when Dr. Vetter came to us she put all our fears at ease. She introduced herself and prompted me to ask all the questions I had about Aurelia's care. I started by saying how concerned I was that we didn't know why she was breathing so hard and needing room air flow to stay oxygenated, and how that might impact the plan for us to discharge on Monday.

Dr. Vetter's reaction was fabulous. She held up her hand politely, indicating for me to stop, and said, "Well let me put that rumor to rest, because I don't know where you heard such a thing. There is no way this little girl is going home on oxygen when I don't know why she's breathing so hard. I've called pulmonary for a consult and they'll be stopping by and taking some x-rays tomorrow. She's not going home anytime soon."

I about cheered and kissed the woman. She told us that Aurelia was an excellent candidate for a program that kept her as an outpatient here so that moving forward we could coordinate all her care under one umbrella and be sure that we need not have many multiple visits that complicated communication between doctors and the quality of Aurelia's care. She said that she really felt the need for a family meeting and would be pushing the case manager to set one up when she came in on Monday. Additionally, we stopped bottle feeds for the foreseeable future until we know why Aurelia's having such trouble breathing, and what the progress is with her vocal cord recovering from its paralysis.

So, in brief, that's where we are - a few other things in list form to keep you all in the loop.

• If pulmonary's consult doesn't yield anything, then we'll likely have the heart catheter sometime in the upcoming week. Pulmonary did a cursory examination this morning and will present to the attending there after the x-ray is taken. We have not yet heard the result of Aurelia's consult.Dr. Svwast, our lead cardiologist is now contributing to that discussion.
• Our case manager will be contacting 'complex scheduling' and arranging the family meeting starting tomorrow.
• Aurelia's medications include a calcium supplement, a chloride supplement (now and again), prilosec and zantac, amoxicillin, and lasix. She receives a number of them throughout the day, some multiple times a day, some with feeds, some without. They are hoping to increase her reflux medication as one possible solution to her reflux problem. Another is extending the feeding tube beyond the stomach sphincter. 
• We haven't heard anything in a bit from urology or plastics. Expect new updates later this week. 
• While we are upset about the lack of bottle feeding, we are glad that we at least have a plan in place to help Aurelia's vocal cord issues as much as we can.
• They are drawing labs on Aurelia daily, looking for low calcium and chloride. She's been tested twice for a rhinovirus (cold) that has been going around the hospital. So far, she's come up clean each time. 
• She has the cutest little baby butt ever. EVER!

That's all we have for now, and as this post is wicked long we'll call it a day. More pictures and maybe some video coming soon.

Bye-Bye Arterial Line....

Hi everyone,
Just a quick mid-day update from CHOP to give you a summary of what we know today.

Aurelia has been seen by general surgery (the folks that did her work on her intestines) and they are pleased with her progress. All seems well with her recovery on that front. Similarly, the kidney folks swung by to look over the latest labs and fluid counts, etc., and they are pleased with the way her one functioning kidney is progressing and working.

They will be making a few changes to Aurelia's medication and ordering an x-ray to look at her lungs today. It's common for newborns in a NICU, and particularly among HLHS children, to experience increased breathing difficulty after a few days of life. As the body begins to grow and produce fluid that needs to be processed and passed through the body it can be hard for an HLHS baby to keep pace with the body's needs.

This is particularly true of fluid that can build up in the lungs. Aurelia has had some trouble breathing, and the increased air flow into her lungs has not really 'lightened the load' she's had to bear when it comes to her respiratory rate. She's working awfully hard to keep the fluid from her lungs and while she's not losing ground to the fluid, they really want to try and ease her struggles to breathe.

To that end they will begin administering a diuretic twice a day to help her get those fluids out. Additionally, they will increase the levels of nutrients she gets daily via her feeding lines to make sure she stays hydrated. All this will be monitored via x-ray to be sure her lungs don't begin to lose ground to the fluids.

Otherwise the only real news is that they are removing her arterial line today. This line is used to constantly monitor her blood pressure. She's been doing so well that they feel no need to keep that line present. They'll take her vitals via little baby blood pressure cuffs and if they need fluids for lab tests they can take it from the venal line.

So, today is a good day so far - as the other consults come and go we'll try to keep you posted. If we don't, it's because we really need a nap!