Ah piss

Note: I wrote this yesterday in my diary, only now am I getting it posted.
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Ah piss. Aurelia continues to improve and I continue to worry. Our girl begins to sit on her own, even in a hospital bed, and I freak out over her needing a 1/4 liter of oxygen at night to sleep. Everything, all my fears, are based off of past experience. It seems every time we approach discharge something happens that may delay our leaving.

This morning we arrived at the hospital to find Aurelia with bandages over her ears. It appears that some sort of infection has settled in over her distractor sites and we are waiting to see what, if anything, will come of it. We have, too, begun the usual dance around Aurelia and her oxygen. As such a difficult ween, it seems we may convince the doctors to send us home on oxygen - only if her ears look O.K.

Still, I do believe they will let us go home tomorrow or Tuesday. Even a little break would be so very welcome.

Hi everyone! I hope I get to go home today!

Can you spot the differences?

Before

After

Can you spot the differences? The top picture was taken yesterday and the bottom one just a while ago. What do you see as differences? :)

Get this thing outta me!!

We had another interesting evening here in Philadelphia. Unfortunately Aurelia's last IV infiltrated and we had already left for the day. Jillian, Madison, and her mother spent the night in Gettysburg, much like I did last week, caring for the cats, cleaning up from lack of use, and doing some shopping. Dr. Davies, the fellow on duty, called at midnight to tell me that unless a viable vein could be found we would have to place a central line.

Those lines enter an artery at the groin and, while more permanent than an IV, are still less i9nvasive than a PICC line. I gave my consent, knowing that the procedure itself is fairly low risk, and today she looks far more comfortable.

Medicinally, Aurelia is down to 1.5 mcg/hr of Dexmedetomine and 2mcg/hr of Fentanyl, both steps in the right direction as we move toward extubation. Our plan is still to extubate today, and her nurse, Jessica, is weening Aurelia off the ventilator. Aurelia's temperature is fine, or low grade, as she wrestles with whatever it is she has in her lungs.

I'm hopeful today. Maybe we will have this tube out of our girl's lungs and we can get down to the business of accurate and adequate pain management for her distractors. Ideally I want plastics confident enough in Aurelia's status to train Jillian and me to move her jaw and thereby release us before we have Aurelia's open heart surgery. There is a chance they will want to keep us here until then, anyway, and that frightens me terribly. One day at a time, though, so we'll see.

Telling time

You can tell time with hospital monitors. Sudden beeps, chirping machines, and intermittent cries mark milestones, victories, achievements, and setbacks alike. Nurses come and go with the changing of the guard and every so often doctors appear to mark the noon and midnight of hospital life, rounds. Parents greet the day like so many commoners swept away in tidal events beyond their control, pulling with or pushing against, the rushing daily currents.

So much is beyond your control as a common cardiac parent. Every day is toil; exhausting, unyielding trudging toward whatever end. Aurelia is a warrior amid this struggle and her care team the aristocracy, grand and minor nobles alike, but we parents are commoners. We search for whatever ways we can to better our lot and the lot of our daughter in a world largely beyond our control. Maybe it's through an observation we share with the care team, or some piece of knowledge gleaned from experience as her day to day caretakers, or even questions we ask to keep ourselves and everyone else on their toes.

We scramble around like this out of love, of course, but also to fight off fear and its potential powerlessness. This is a good and worthy struggle, but it can make you so tired. Everything slips behind an omnipresent haze, clouding conversations, thoughts, even feelings. Some days little can clear away that haze except for the passing of time, one nurse, doctor, beep, or cry, at a time.

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Today Aurelia made some progress, baby steps size, but progress nonetheless. Her labs came back mostly clean, so she's off contact separation and whatever she does have brewing in her lungs is so not concerning that folks are going to let her fight it off on her own. Her fever is not sticking - if it shoes up, it goes away again with some tylenol (and even that is not always needed) - and she continues to tolerate her jaw movements reasonably well.

The tough part about that is avoiding any jiggling or jostling of the screws while attaching the screw driver. Her jaw is still mighty sore, so attaching that to her can be the hardest part of the job. We are slowly weening her off the respiratory machine and we hope to extubate her sometime tomorrow. I'm very excited about that as Aurelia will no longer need to be restrained. Watching her flail about because her arms are tied down to the crib is heartbreaking. Still, she is so brave, and so strong, and so determined. When she's awake now it isn't to have those silent screams every time, it's to make eye contact and to furrow her brow and to blink, as if to express her will and effort to overcome this struggle. Or, more likely, she's trying to fart. She's been really gassy lately.

Speaking of which, she's only had two bowel movements in over a week. We're not worried, but man, you ever seen a 17 lb. baby unleash a breast milk diaper? I swear to you that my time in Purgatory is greatly reduced because of what we've seen, smelled, and cleaned. To that end, in an effort to be sure all of Aurelia's insides are working right we did a hypoglycemia test today, hoping to see if her retching at the end of meals is related to a drop in blood sugar. We'll keep you posted. We'll also be doing a hearing test to dot some 'i's" and cross some "t's" with our doctor. I'm inserting a picture below of her jaw apparatus, via x-ray, so you can get a clear understanding of how this all works.

Here you can see a number of things on, around, and in Aurelia. From the bottom up: You can see the wires wrapped around her sternum from her previous open heart. Just above that you will see some of the sensor wires on the outside of her skin, wrapping around her torso. Above that are two separate marks on the x-ray; to the right is the band around her pulmonary artery, restricting the blood flow to her heart. On the left, moving up her throat from that location, is the breathing tube intubated through her mouth. That brings me to her jaw. You can clearly see the mandible device attached to her jaw in two places on each side. Between those two anchor points is the incision that splits her lower jaw. As you follow the metal bracings up behind her ears, you see the two screws that emerge from her skin. As we turn those with a special screw driver, the jaw splits along the seam between the anchors, moving the lower portion further forward and bringing the tongue along with it at the same time. This will help her breathe by enlarging her airway.

And she looks like a bad-ass combination of Skeletor, Megatron, and the Terminator. My daughter is a ass kicking cybernetically enhanced warrior. Bring it on, open heart surgery! I HAVE STEEL JAWS!

Ugh

It was not a good night. After attempting to lower Aurelia's pain medication to give her a little more consciousness as we move toward removing her intubation, we had a serious setback at about 3:00 this morning. During a diaper change Aurelia used ninja reflexes to yank her sutured breathing tube out of her nose. As the team scrambled Aurelia held her own for a few minutes before crashing. Her heart rate and oxygenation plummeted and the team had to do chest compression for 3.5 minutes as they attempted to circulate a variety of medicines through her body.

Aurelia did not lose her pulse or stop breathing, but in order for the paralytic and stimulants for her heart and lung to reach the important locations the team needed to be sure her body kept its blood and oxygen moving. Once paralyzed they picked her up to a standing position and intubated her through her mouth. Things became more complicated when she did not come out of her medicated paralysis the way everyone hoped, so we stopped all pain medication, using the pain to stimulate her body into moving again. As of now, we are back at 1/2 the dose of pain medications we were using yesterday.

Of course this morning Aurelia lost her remaining IV and needed to get a new one in her right leg. She also has a fever again and we drew labs and sent off some of her phlegm to be cultured. There is some thought that an infection could be causing Aurelia's difficulty breathing without assistance. I personally believe this is a reflection of her typical difficulty coming off oxygen. When she had pneumonia this spring we needed to keep her on 1/32 a L of oxygen for a week just to keep her airway open. That's nothing - it's like a whisp of air that barely does anything. Aurelia has always taken a tougher track when it comes to breathing on her own. Another thought could be swelling from her adenoidectomy. We'll see. Either way, it doesn't look like we will be removing her breathing tube tomorrow.  I hope only for a good response from the labs and culture so that we can just worry about getting this tube out of her throat.

She's got a bath now, new linens, and she finally had a bowel movement, so here is to a day of no problems or troubles.

Here's our girl after her rough night. You can see the tube now taped down into her mouth as opposed to her nose, you can see her right arm restraint, keeping her from yanking at the tube, her new IV line behind the brace on her right leg, and a fresh diaper. 

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.

Simmer down now!

It's been an interesting weekend. After Jillian's father arrived in the wee hours of Saturday morning, unexpectedly, Jillian and I hoped for a nice quiet weekend. For the most part we got what we wanted, but there are a number of unanswered questions as our sixth week begins.

Aurelia's heart continues to do well. We smile some at the irony of our stay here as her heart is doing so wonderfully that our discharge could have been weeks ago! To date there are no problems with her urinary tract and the VCUG she underwent showed no back-flush into her dead kidney at all. We are still too far out to do anything about her cleft palette and the latest brain ultrasound showed that her clot is still resolving itself and shrinking every day. These are very good things!

Her genetic evaluations have come back with nothing significant that requires any immediate therapy (if any at all)  and Occupational Therapy liked her status as of last Thursday (we won't see them for some time). The Peanut's weight continues to increase and even though feeding has been a disaster she's still holding her own with only a potassium supplement to her daily nutritional fluids.

The PICC line is stable and uninfected, her peripheral line is still functioning and not painful, and the latest cardiac data on the pressures in each ventricle is good, as is the ability of her mitral valve to hold its own. The pulmonary band continues to be effective and the aortic arch reconstruction has not been rejected in any way. In fact, all of that surgery has been a success.

The treatment for her NEC has been bumped to ten days from seven, but that isn't necessarily a bad thing; her daily films show no advance of the infection, but they also do not show it lessening. Even if we *do* get the OK to start feeds again in ten days we probably won't until this airway business is completely sorted out.

The current issue on the table is Aurelia's upper and lower airway. She has had three airway collapses since she's made the move back to the CICU last Friday. She generally has one every twelve hours and only when she becomes particularly worked up. This usually follows a diaper change or a bout of hunger pain. Only one of these episodes, other than the very first which got us back into the CICU, required any kind of sedative to calm her and restore breathing function. The typical pattern for these collapses is a progressive desaturation of oxygen in her blood when she's having a fit. Then we'll see a sudden drop in her saturation levels as her airway (described as 'floppy') is unable to remain open. At this point they will slap the oxygen mask on her and pump her with anywhere between 2 to 4 liters of room air to push the airway open. They will then slowly ween her from the room air over the next few hours and she will be fine.

In the intervening time the staff suctions her nose and mouth for secretions every one to two hours. Because of her severe acid reflux (which is still being medically treated) and because they have a sump running into her stomach to keep everything out of her intestines, and because of her cleft palette, our daughter produces far more secretions in her mouth and esophagus than normal. This goop is usually withdrawn via a tube inserted through the nose into the back of the throat, or directly into the mouth and down the throat into the upper airway.

Dr. Naim is our attending again (yay!) and she'll be setting up a time for Ear/Nose/Throat to examine Aurelia's airway sometime this week. They will (or maybe not) intubate her -  something that is not good for her vocal cord paresis or future eating ability - depending on how far down they have to examine her airway. Treatment can be anything from C-Pap, respirators, to temporary or lifetime tracheotomy. If we do have to go with either of the last two issues we will be here for another two months or so, give or take. Feeding at that point will be done via g-tube. This test coming up is a bit more invasive so it counts as yet another surgical procedure down in the operating room.

So that's where we are. My superintendent let me be absent from a professional development day today so that I could be here for rounds and the E/N/T consult/evaluation. We'll do our best to keep people updated.

Rake an eel Malaysia

It has been a very difficult two days.
Yesterday Aurelia became symptomatic of NEC, or necrotizing enderocolitis (contains graphic pictures).  The culture is still growing in the lab but our doctors are calling it and treating it as NEC until otherwise proven. A particularly nasty infection of the bowels (with uncertain causes), NEC (pronounced 'neck') attacks and kills the intestines, releasing gases in the process. These gases build up in the intestinal wall and result in perforation, eruption, and tissue death.

Treatment and the infection itself is difficult because often diagnosis is made only when physical symptoms, like hardened stomach, blood in the stool, and abdominal discoloration, are noticed during routine examination. Luckily Aurelia's case was caught particularly early by complete accident. Little A's NJ tube had been moved back into her stomach the day before and she was quite irritable yesterday morning. Our nurse, Laura, (who was an angel these last few days) noticed and worked with the doctors to get an x-ray taken to be sure the tube was still in the right place in her stomach.

That x-ray revealed the NEC. Doctors are hesitant to use the word, I've discovered. I'm not sure if they want to avoid scaring us as parents or if they treat it like teammates treat a pitcher tossing a perfect game in the seventh; as if nothing special is going on because you don't want to jinx any good luck. Aurelia began broad spectrum antibiotics, taking three of the most powerful drugs available to attack this invasion. Her feeds were immediately stopped and the NG tube removed and a sump put in place to drain her stomach. The IV process was disastrous as Jillian mentioned in a previous post, but this is when today's troubles actually first manifested. During the tortuous procedure Aurelia became so agitated her lungs refused to take air. She wasn't holding her breath; on the contrary she was pulling and tugging for all her worth, but air could not get into her lungs.

This caused concern. She settled however, was taken off the oxygen she was temporarily put on, and began responding well to treatment. Other than being cranky due to her lack of food you wouldn't have noticed much out of the ordinary. They doctors scheduled a surgical procedure to insert a PICC line (essentially an IV on crack) for today. This line centrally delivers Aurelia's medicines and fluids directly to her circulatory system at a place near the heart. While prone to infection, these lines are necessary for long treatments since IV lines tend to become useless after several days (not to mention painful!).

After a quiet afternoon and night spent with Jillian while I manned the RMH with Madison and Cassandra, Aurelia treated us to a nice and relaxed morning. We went down to the Cardiac Prep and Recovery Unit (CPRU) and met with the anesthesiologist working on Aurelia today. He failed to impress us in his level of concern and approach to Aurelia. He possessed decent understanding of her previous surgeries and recognized that a different approach with ketamine would help Aurelia avoid general anesthesia. Jillian and I supported that practical course of action since it would avoid yet another intubation and damage to Aurelia's already abused and battered esophagus.

The procedure itself went well. The PICC line went up through her femoral artery and sat nicely near her heart and we successfully avoided general anesthesia. The penalty for this was increasing the amount of ketamine given to Aurelia over time in measured and small doses. Ketamine, for the uninitiated, is a sedative with a number of interesting challenges that includes a particularly nasty streak in infants as they awake. Aurelia, from 2:00 p.m. on (minus a ten minute transfer from CPRU to CCU) wailed for all she was worth. Her sad and strained cries came with increasing pain and agitation for over two hours.

It felt helpless to watch her in such a state. I still think I am reeling from the experience. Everything happened relatively quickly upon our return and even though each individual decision made in the intervening hours happened so fast it felt like I had some sort of time lapse mechanism working to heighten my senses. My ears strained to hear every word from each doctor and my heart raced. I could feel the blood pounding through my head in my ears, almost whooshing with every heartbeat.

I had just sat down to pay some bills online when Dr.Laurence, our resident, came in for a routine check. She expressed alarm at Aurelia's effort to breathe. She smiled at us, mentioned that she'd be back in about an hour to check on her and left.  Within two minutes she returned with the fellow alongside. He examined Aurelia and his normally chipper demeanor vanished. He murmured something to Dr. Laurence and left. Jillian and I knew something had gone awry when moments later he returned with our attending, Dr. Cohen.

The time mechanism I mentioned above tripped on seconds later when it seemed like our room became part of some set on ER or any other medical drama. Doctors rushed in to her bedside, her nurse scooped her up and the examinations continued. Jillian and I somehow got shuffled to the periphery and we watched this movie unfold. At the center we saw Aurelia screaming and working herself to such a frenzy she could not breathe. The attending, resident, and fellow surrounded her talking about collapsing airways and Malaysia (what the hell that had to do with anything I would find out later) and her crying being good. Other nurses arrived and the anesthesiologist came to consult on Aurelia's condition. Clearly our team in the CCU found his contributions unimpressive. Our group dismissed what he had to say because his greatest observation consisted of, "She was like this when they brought her over to us" and "She looked pale coming off the anesthesia and worked herself up, but she did calm down."

He shuffled in and out of the room for the next half hour, unwanted, not needed, and fueling rising anger and irritation in both Jillian and me. With his hunched shoulders, heavy jowls, slovenly gait, and saggy paunch he would wander bedside and make comments like, "what, is she STILL crying?" and "Is this one still worked up?" and "could this be related to her NEC?" While the venomous stare from the senior doctor on our team silenced him after that last comment, I had to leave the room previously, furious and ready to lash out at the callous and arrogant ass for his behavior.

It was like every horrible comment made by that fool punctuated the horror we saw surrounding Aurelia. First the team called for a consult from the attending in the CICU. We blanched at the request, frightened by the sudden need for critical care. The doctors kept maneuvering Aurelia around, searching for positions to ease her breathing and trying to find some way to help her get air into her lungs. They kept looking at one another and noting that she'd somehow get a few breaths in and then her body would clamp down and she'd fail to pull any air.  The CICU attending arrived and they talked in serious tones, gesticulating and touching their necks. Each kept talking about Malaysia. Some small voice in my head kept saying, "what the f*ck?!?" each time I heard it mentioned. They ran over all the particulars of Aurelia and Jillian and I chipped in multiple times when something went overlooked in the rush to get an opinion. I felt particularly proud of Jillian in that moment knowing that when she needed to be sharp and focused for our girl she shone brilliantly.

Our attending and fellow came over to us while the rest buzzed around Aurelia talking and monitoring and trying to calm her and help her breathe. They told us that while the obvious concern was still NEC it had become more and more apparent to them the last three or so days that some sort of airway problem continued to impact Aurelia's improvement. She said that cardiac babies with a paralyzed vocal cord often suffer similar issues, but that this seemed somewhat different as the problem went beyond the previously suspected lung disease. They suspected tracheal malasia. Rake an eel Malaysia??!?!

Apparently tracheal malasia is a weakening condition of the trachea in which malformed or poorly formed cartilage collapses during exhalation which prevents any air from reaching the lungs during inhalation. Dr. Cohen just finished explaining this to us when she told us that we'd be transfered back to the intensive care unit. Tears flowed in our eyes at the step back. We knew that something like this might happen but I still hate that it has. Truthfully, even typing about it now makes me cry some knowing that our little girl needs such extensive care still. She is such a fighter, but it is so hard watching her struggle and hurt so much for so long now.

It is the best place for her. Until we get this airway issue nailed down Aurelia needs the closely monitored 24 hour care. The real blow to our emotions and endurance came in two parts during this discussion with Dr. Cohen. First she talked with us about the big picture, making it quite clear that in all likelihood we will go home with a g-tube of some kind and that if this is tracheal malasia that we may be facing some very scary decisions and treatments - the kind that might forever change how our daughter looks and talks and breathes. Second, and even more psychologically damaging I think, she described Aurelia as first and foremost 'fragile.'

What a word. I know it's true and I know it has been true all along. She is such a determined girl, though that I cling to her strength. It sounds silly, doesn't it, that a 33 year old man and father of a CHD baby clings to that same child's strength? She has been the fuel that keeps me going back and forth between here and Gettysburg, or between Gettysburg and work, and above all in my own mind where I have to fight the loneliness of living alone away from my family and working long days in a thick emotional fog.

She is such an amazing girl. She gives me all the strength I need that I think hearing Dr. Cohen use the word fragile described not just Aurelia's physical condition, but also how this emotional and spiritual marathon has left Jillian, Madison and me. Of course this is all reflection after the fact, because moments after Dr. Cohen said, 'fragile' I left to store our personal affects in a locker on site and the team called her away to examine what appeared to be a hardening stomach.

I returned to see Jillian looking even more distraught. Aurelia's distress revealed a hardened stomach, a classic sign of progressing NEC. Immediately the pace and intensity in the room ratcheted up and an all hospital page went out for the x-ray unit to immediately come directly to our room in the CCU. Aurelia so discolored herself and was pushing her lungs and diaphragm so hard that she actually revealed what appeared to be a umbilical hernia. Our room was full of nurses from the CICU, our nurses, attending physicians, fellows, and residents. Even the social worker from the CICU showed up (and thank God she did) to help talk us through this disaster even as Aurelia screamed so loud we could hear her in the hallway. (For the record, that was such a feat given her one paralyzed vocal cord that we couldn't even focus and had to go back in to her room for a  moment to be sure she was OK).

I swear I felt a mixture of awe and fear and pride at the same time during all this. Fear, of course, for Aurelia and for Jillian and me, but awe at this unbelievable mobilization of medical talent and material, and pride at Aurelia's pure determination and will to fight and not give up during any of these challenges. She continues to inspire.

We made our way to the CICU and whisked our way into pod 5, bed 4, where a nurse we had previously in the CICU, Michelle, stood ready. The teams there transferred Aurelia's care with precision and focus and I thanked myself again for the CCU nurse, Laura. She stayed longer than anyone else to work closely with the CICU team to inform them about Aurelia's care and preferences, and even promised to look in on us tomorrow. Not ten minutes later the level of care seen in the CICU versus the CCU became apparent. The fellow informed us of a way to discover malasia (though Aurelia was so worked up we couldn't try it), they called for a mild sedative to simply allow Aurelia to rest, took a babygram (full body x-ray), and got labs drawn to get immediately analyzed.

Aurelia settled finally and we left her in good hands knowing that we needed to see Madison and that we could think clearer with a little distance and space. We'll keep you all posted as to how things develop and please know that your love and prayers and positive thoughts are so very much appreciated.

An NG or a NJ or an ABC to your XYZ

Aurelia goes in for a procedure in just a little bit. the folks down in IR (Interventionary Radiology) will be putting a feeding tube into Aurelia that runs not just into her stomach but beyond it and her duodenum and leaving it smack dab in her intestines.

Currently, Aurelia's tube ends in her stomach and she receives bolus feeds, or feeds every three hours. With this new NJ tube she will be fed 24 hours a day at a very small rate. The hope is that the NJ will lessen her acid reflux and thereby improve her ability to breathe.

It sounds silly, but I worry more about this than I do for her potentially coming heart catheter (done at the end of the week most likely. Maybe it is the immediacy of the procedure or maybe it's my own paranoia that something so small and 'routine' could end up disastrous. Either way your thoughts, prayers, and well wishes are welcome!

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.

So where are we now?

After the long journey of the past couple days there are so many things to write about and so many improvements and challenges that time has slipped away from us and this update is coming essentially a day late.
Because of all that's gone on, I think I'm going to take a less elegant approach to this post and provide a snapshot via list of Aurelia's journey and current situation.

Yesterday, day one of Aurelia's recovery, featured some great strides and a few setbacks, but all in all it was a very good day.
- Aurelia's incision looked good and the amount of blood coming from her chest cavity fell so much that the doctor's removed her drainage tube.
- Similarly, her blood-gas readings progressed to such a point that the doctors removed both her breathing tube (taking her off the respirator) and her sump (leaving her intestines to process all the fluid/nutrients in her stomach.
- In preparation for feedings later in the day the team installed a feeding tube through her left nostril. As a side note, when they removed the breathing tube and suctioned out her nose in advance of inserting her feeding tube the doctor's pulled out more mucus and snot and dried blood than I knew possible. It makes me more than a little sketched out to think about just what is sitting in my sinuses.
- Our first scare came when Aurelia had a wet diaper. As silly as that sounds, Aurelia so hates wet diapers that she worked herself into a fierce frenzy that resulted in a back flow of non-oxygenated blood above her pulmonary artery. This, literally, turned Aurelia blue. As the blood Aurelia needed to oxygenate got clogged above her pulmonary artery her oxygen saturation rate fell precipitously and the team installed a nasal line and forced room air into her lungs at 70% capacity. After about fifteen minutes of actually suffocating her body Aurelia calmed down and over the course of the evening the team dialed back her room oxygen intake to 50% (where it remained until earlier today when it dropped to 40%).
- Our second setback came when Aurelia's fluid intake level became too high. While she did pee (thank God for the pee!), she hadn't had any bowel movements and she wasn't peeing nearly enough. This kept us from starting scheduled feeds and prevented us from doing anything than a mouth treatment, or a coating of the lips and gums with breast milk.

Evening rounds passed without event and when we arrived this morning our current situation two days after the surgery included:
- Aurelia still having too much fluid in her, an x-ray showing hazy lungs, and lasix being ordered for her to compensate her decreased kidney output.
- Two bowel movements (yippee!) putting us back on schedule for feedings to start today.
- Bleeding from her incision (yikes!) that was clearly more than post-operation oozing, but not enough bleeding to cause a change in her vital signs. Still, it was worrisome enough to generate two ultrasounds and an x-ray
- a slight increase in the amount of fluid her kidney processed resulting in only a +100 or so intake from her second day.

Thankfully, we had a good day after those few concerns. Genetic screening (a genome array test) came back and showed no anomalies, meaning that as of now Aurelia and all her problems is simply our beautiful and perfect freak of nature and not the victim of a genetic disorder. Our geneticists are ordering one last test that is the best insurance can buy to see if there are any abnormalities at all that could account for all these problems. This test will take some time to come back, but we are thrilled to know that there is no genetic syndrome at play in our new daughter's struggles.

Additionally, Aurelia had only one real temper tantrum today and she recovered well, meaning that we didn't need to increase the amount of oxygen she received. Similarly, the ultrasound of her head and chest (as well as the x-ray) came back negative, proving that her heart was not bleeding and that there was no fluid build up around her heart or in her heart cavity, or around her lungs. The best guess of the team is that her chest muscles acquired increased blood levels during and after surgery and this is the subsequent squeezing of a sponge; her muscles releasing this blood intake and her incision becoming the natural outlet for the blood.

Dr. Ince also heard bowel sounds during her rounds and because of the two bowel movements we started Aurelia on feeds through her nasal tube. She has had two 5 ml feedings and one 10 ml feeding as of my writing. She will get feeds every three hours or so, barring any complications.

Lastly, Aurelia has had two ENORMOUS diapers, and when we say enormous we mean GARGANTUAN! These things weighed a ton, by all accounts. Or maybe a couple of ounces, but still, it means that she's now really passing that fluid.

Other than that, the medical news is stable and good. She's progressing well and it seems as if the bleeding at her incision has slowed. I'll keep you posted as I learn more and perhaps post a more 'human' reaction to the last several days.

With love,
Charley

Her next heartbeat

Jillian and I are the sort of tired happy that comes after a long car trip - happy to be at your destination, unsure of exactly how you got there, and worried about what else could possibly go wrong before the day is over.

Aurelia is amazing. I am astounded at her resiliency and fortitude. This girl was born with so many complications, so many problems, endured invasive bowel surgery during her first day and followed up that performance with open heart surgery at age eleven days. Just yesterday her sternum was cracked, her ribs spread wide, and her body frozen into submission. Today she is resting, with a half-smile on her face and covered in residual orange dye; a silly sort of October pumpkin.

The surgery yesterday took far longer than we had hoped or even considered. Aurelia was out of our hands and actually in surgery from seven-thirty in the morning to three in the afternoon. In surgery the doctors changed their game plan after they saw the situation in her heart. The initial plan included putting Aurelia on bypass and then banding her pulmonary artery before making any incisions into the actual heart (excluding the sack that exists around the muscle). When they got in to her chest, however, they recognized that the aortic arch was far more narrowed than anticipated and required their skilled attention first. Thankfully, Dr. Gruber insisted on going in her chest and his decision made sure that they widened all of the narrowed parts of`Aurelia's arch; had they done the previously mentioned side incision they would have missed part of her narrowed aorta and would have likely needed to do a second surgery.

While I am on the topic of Dr. Gruber, I want to make sure I record just how amazing it was to see him in action yesterday morning. On Sunday all of the cardiac surgeons & fellows, some fifteen in all, met and discussed Aurelia's case. Dr. Gruber chaired that meeting from Cleveland (he was at a speaking engagement) and apparently a number of the team disagreed with his assessment that we enter Aurelia's chest cavity from the front.

When we met with Dr. Pillai that Sunday afternoon the message we received was that of the dissenting team members - that Aurelia's surgery would be from the side, most likely - and we were happy with the decision to go with a less invasive procedure. At crib-side Monday morning Dr. Gruber gave us one final debriefing on the procedure and mentioned going in from the front. Jillian noticed the discrepancy immediately and made mention of the plans we heard the day before to go in through her ribcage.

Dr. Gruber underwent some sort of Fellow Transformation, reserved for only the experts in his field. First he ordered one nurse (or doctor, for that matter - nobody outranked him during those next few moments, no matter their title) to retrieve Aurelia's paperwork. He concisely, but not rudely, discredited the fellow who conferenced with us Sunday by indicating his utter displeasure with our receiving incorrect information. His words to us included, "I'm not sure what that Fellow heard or said, but there clearly was a miscommunication. The plan was never to go in through the side. There was some questioning of what our final decision would be, but I clearly ruled out going in from the side."

After he directed the many staff to clean and re-stamp Aurelia on the front of her chest he called surgery and seemed pleased that everyone in the operating room had been planning to go in through Aurelia's chest. Thankfully the repair to Aurelia's arch, facilitated by that decision to open her chest, went without incident. I have such a hard time trying to imagine my daughter laying on an operating table with her chest open wide and blue from the cold that has shut down all the major systems of her body. I just can't fathom what it looked like to watch her heart slow and slow one beat at a time until it sat silent and still. I cry thinking about her there, her body dead and a machine carrying precious oxygen and blood to her brain and limbs.

The surgical team moved quickly after that, repairing the band and slowly moving Aurelia off bypass. At this point the first of the challenges facing Aurelia (as if all that came before was easy!) manifested itself in the form of excessive bleeding. Throughout any complicated surgery like this many nicks and cuts are made in the body, along veins and organs and muscle and tissue, but very rarely is bleeding seen by the surgical team. A body in Aurelia's state is so cold that you can't see the amount of bleeding until they begin to take her off bypass and warm her up, waking her heart from its slumber.

Aurelia underwent a series of cooling procedures over two and a half hours to slow the excessive bleeding inside her chest cavity. These procedures amounted to literally filling Aurelia with ice, waiting fifteen minutes, warming her up again, checking for bleeding and re-applying ice if needed. Eventually Aurelia's leaks sealed themselves and the doctors made sure that all the vents and lines into her firmly lay in place. At that point Aurelia had a drainage line installed, two pacemaker wires, an IV directly to her chest cavity and heart, another IV in her foot, and an arterial line into her belly button.

We met with Dr. Gruber after the surgery for his recounting of the surgery and he showed enthusiasm and pleasure with Aurelia's progress. Other than her bleeding he felt confident that she would emerge from the procedure with much improved breathing and heart function. Dr. Gruber decided to set the pulmonary artery function at 90% its original and he avoided looking for other potential problems with Aurelia's mitral valve and her existing muscular VSD (the large hole between her left and right ventricle). Jillian and I were happy with the outcome and Dr. Gruber's decision to avoid looking for further problems with Aurelia's valve and hole, because both may improve themselves over time and because the valve may actually not have much of a problem once her heart regains strength.

When we first saw Aurelia we were thrilled. As scary and difficult as it was to look at her I will never forget the joy in my heart at that moment. All that afternoon and evening I felt like running and hugging and kissing everyone I saw, from Dr. Ince to the admitting orderly out by the entrance to the CICU. Jillian felt more than mild embarrassment at my excitement and expression. I don't blame her. It's quite possible that I mortified not only Jillian, but Aurelia, too.

After about one hour the bleeding from Aurelia's chest cavity slowed to barely a trickle and we started to face the second and far worse concern with her surgery, this time revolving around urine. When put on bypass and our temperatures lowered our systems shut down one by one. As we come back to life our body slowly regains heat (Aurelia's temperature still hadn't reached normal two hours after her surgery finished) in controlled increments and the body's systems begin to reboot one by one. We skipped over any trouble with Aurelia's respiratory system by having her on the machine that took care of that process for her, her bleeding in her circulatory system had slowly come under control, her nervous system seemed to be intact, she hadn't suffered any strokes, and brain function appeared normal.

Unfortunately, prior to, during, and after surgery Aurelia hadn't passed any urine through her catheter. In any normal person this wouldn't raise much of a concern, but with only one kidney the chance of system shock and failure meant life or death for our baby girl. Dr. Ince, whose praises I sang before, kept a measured response and reassured Jillian and I that this sort of delay did not mean anything out of the ordinary. Stressed nonetheless we felt increasing tension as the minutes went by and as the staff began discussing their response to a kidney failure. The sheer mention of 'what if' scenarios had me in near panic. All I wanted for her was to pee. I have never, ever, wanted anyone to pee so badly in my life.

She kept us waiting nearly two hours, the cut off point for 'no concern' we later found out, until we saw a few drops in her catheter. I about jumped for joy at the sight of a yellow droplet in her tube. This seems silly, I suppose, but looking at her bruised chest, swollen face and arms, and knowing that all that fluid she had been infused with during surgery had to escape, I think I actually felt more fear than I did when they wheeled her away for the operation earlier that morning.

We stayed late last evening with her and left her happy that she once again faced down tough odds and beat them with courage and tenacity. Though a machine was breathing for her and her chest still bled some, and despite the low urine output, Aurelia inspired strength and hope in us that we needed to fight back the worry and concern we felt all day.

I confess that at points during the day I let my mind drift away to places dark and sad, wondering what it would be like to lose her. We agreed to participate in a genetic study  before Aurelia's surgery, permitting the CHOP staff to keep tissue and blood samples from Aurelia's heart permanently. Dr. Gruber and his team will use the sample (amongst many others) to try and identify the genetic cause behind HLHS. It is suspected that the disease is genetic, but much more work needs to be done before a specific cause is identified. I found a minuscule measure of comfort knowing that even if she died, Aurelia's heart and blood could still help someone else at risk of HLHS. I even felt good knowing that if Aurelia's DNA showed anything even remotely useful that Jillian and I could then give our own DNA and blood to support scholarship and research into the defect.

As for today, other than one minor scare, Aurelia continues to do well. I'll give a more detailed post a little later, but I know folks have been waiting for details so I wanted to write this before my memory  lost pieces of Aurelia's journey.

Thank you everyone, for all your positive thoughts and prayers and love. Your support has resulted in immeasurable strength needed and used by Jillian and me. We love you.

Busy busy busy

It has been a bit busy over at what I'm now calling the Fall Cottage, or Children's Hospital of Philadelphia. Aurelia was moved from one pod to another (not a very stressful or difficult thing, but the staff needed to shuffle around some room for beds. Overall, she's doing well and stable for the most part, but there have been numerous small changes and alterations to her treatment as they prepare her as best they can for Monday's surgery.

In general treatment over the last few days has revolved around Aurelia's digestion and breathing. Initially our plan was to have feedings begin on Friday, but general surgery wants to wait until some time after Monday since Aurelia's stomach still isn't processing enough fluid. This isn't really a problem, and it's certainly not something that has anyone too concerned, just a readjustment of an already fluid timetable.

The only real ailment that our team is watching very closely during these last few days is Aurelia's breathing, or respiratory rate. She had been on a diuretic two times a day earlier in the week, but seemed not to need so much help ridding herself of fluid so the team dropped the dosage to one. They also stopped her daily chest x-ray and adjusted her electrolytes as she began to have some changes in her nutrition intake.

This morning, however, Aurelia's respiratory rate climbed and hovered in the 100's. Normally somewhere in the sixties or seventies, this constantly elevated rate caused her too much duress so they administered some additional lasix and moved her diuretic up to the original twice a day dosage. The doctors anticipated ordering an afternoon chest x-ray but our girl bounced back, filled up her diapers, and has a negative fluid level for the day so that precaution has been called off.

This makes me very glad because her quick response to the medication and her rather exuberant urination means her kidney is working great. Add to that the still decreasing amount of fluid coming from her stomach and she's still doing well. Her weight loss is well within normal limits as well, and the only thing we'll see in the future that is new to us today is the arrival of a central line after surgery. We expected this, but in the commotion and details of Aurelia's case we both forgot that she'd have this procedure done to supplement her  feedings after Monday's surgery.

The nursing staff is still great and Jillian and I were excited to see our favorite, Pam, come over to our pod and visit with us for a good ten minutes. We just chit-chatted and got caught up, but it really made us feel welcome and a part of the care team here in the CICU. Every nurse has her own personality of course, but Pam really makes this place feel like home for us, and in a scary place where children in warmers or cribs come and go, nurses and doctors scurry from one patient to the next, and monitors beep ominously over the heads of innocent babies, that says something.

There was even a moment where six or seven nurses gathered in our pod (only one baby beside Aurelia is here) and just talked with one another, laughing and chuckling, sharing stories and smiles, and it made Jillian and I feel so relaxed and relieved, and really comfortable among everyone working. We didn't feel at all in the way or unwelcome or awkward, and that is great.

I'll post an additional reflection later today, but it had been some time since we updated and I wanted to share this news. A few other things to share include my 4th period students chipping in together and convincing the mother of one of the students to create and gift us with this amazing flower arrangement brimming with care and love and concern. I cried in class (and over lunch when they couldn't see me) and Jillian cried as well when she got the voice-mail. Madison was hysterical when she saw me today, running toward me in the parking garage and then being so excited she ran in circles around me with a huge smile. I think most special of all (other than those moments when Madison was snuggled on my shoulder napping) was holding Aurelia for a good hour straight. She kept her body temperature up, she cooed, smiled, pursed her lips, made little faces, pooped (yay!), and twitched away in her dreamland. It was heavenly, even though I was exhausted and could barely keep my eyes open!

For now Jillian and I are going to head back to the Ronald McDonald House and get some dinner and then call it a night. As much as I'd love to be here for hours this evening, I need to rest up and prepare for what will be a very stressful few days and evenings. Love to you all!