Needed a break

Hi folks. Sorry for the delay in posting these last few days, but I needed a break from writing. It's been a stressful day or two and I just didn't have the wherewithal to sit down at the computer and type something out. Aurelia is stable and in a similar place to where she was a few days ago. She still is terribly gassy, has needed medicine to help move her bowels, and is still watched closely for the amount she urinates. Her fevers spiked a few nights in a row, but last night it was not quite so dramatic a rise.

In terms of pain Aurelia is on oxycodone and tylenol, if she needs the additional relief. Sleep has been difficult for her, in part because of the gas, but also because she is so nosy. Our girl has to inspect everything going on in and everyone entering the pod. We hope to be out of intensive care soon so we have a shot at a private room. Her recovery should go well there.

Aurelia's breathing is still assisted by 1/4 liter of oxygen, but that is what she came in on, and she is a terribly difficult ween. We hope to start weening her off morphine and diazepam again soon, and maybe her oxygen, too. She has to relax, more, though, and breathe deeply for that to happen.

Not much to report other than that. Jillian went home for a night and returned with my in-laws and Madison as we approach this next phase in the step-down unit. I'll be returning to Gettysburg for a few days to be with the cats tomorrow morning sometime, I think. Well, thanks as always for all your support and prayers. Hope you are all well!

PS - I fixed the comments - everyone should be able to comment now.

Just a little fluid

Good news continues unabated this morning as Aurelia's improvement continues. All of her tubes and wires, other than the IV, are out and gone, her normal feeds have resumed, other than morphine and diazepam she is off pain medication, and her breathing has not gotten any worse. Our only concern thus far is a stubborn bit of fluid in her lungs. We have started chest PT and increased her diuretics in an attempt to drain her lungs more so we can ween her off oxygen.

A liberal estimate has us leaving the intensive care unit today and maybe moving into the step-down unit tomorrow. I cannot believe Aurelia is doing so well! I don't want to jinx all this progress, but I confess to feeling a bit of disbelief regarding her progress. Never has anything like this been so smooth for us, so I keep looking up for the proverbial shoe, or anvil, or whatever, to drop. I sincerely hope it doesn't, but have a hard time believing it won't!

We have a few questions for doctors on rounds today, when they make their way to Aurelia:
1. How long do we have to do wound care behind her ears?
2. Now that her tracheomalacia resolved itself, do we still need her inhalers?
3. Can we get a swallow study while we are here?
4. Can audiology follow up with Aurelia while she is here, too?

We also want to try and contact our home care company contact here and get a letter of medical necessity to help our application for medical assistance. We want to try and obtain a full time home care nurse for Aurelia and see if we qualify for medical assistance insurance, in the event our insurance starts to play hardball with CHOP and us. We received our first rejection for care a few weeks ago, so we are thinking the fights are beginning as she vaults way past the multi-million dollar baby level. We estimate her care to be at or beyond $2,000,000 since her birth in September. Combine that with 119 days of leave without pay for Jillian and we're thinking medical assistance is something we definitely need to have! We'll keep you all posted!

A quiet afternoon

All good news from CHOP this evening, even as chaos reigns in the pods around us. With critical emergencies proliferating in the CICU Aurelia bucked the trend, enjoying a quiet afternoon of rest and healing. Her O2 saturation is wonderful at 2 liters of oxygen, she is not maxed out on her pain medication in case she needs more, her color is good and her kidney is operating wonderfully.

That last bit, about her kidney, had us particularly concerned. When you have your heart stopped and you go on heart-lung bypass there is a risk that your other organs will shut down and have a tough time starting back up again when your heart begins beating again on its own. Given that Aurelia only has one working kidney, if that doesn't fire up and work well from the get-go then she runs a risk of fluid build up and that can be dangerous.

Regardless, Aurelia is having great fluid discharge through her foley catheter and the only fluid she's needed (besides nutrients) is some potassium, to replenish what's being lost due to her lasix. Her blood gasses have been great all afternoon, drainage from her chest tube is within acceptable limits, and even her wound care went well around her distractors. All in all, it has been a super afternoon. Thank you to everyone for all your support! Jillian and I feel tremendously blessed to have such a strong and dedicated support network of friends, family, and colleagues.

She's through!

We just received word that Aurelia has successfully underwent surgery. Dr. Spray repaired both her holes and augmented her pulmonary artery. He says that although her mitral valve appears slightly misshapen there is no leaking or backflush of blood across the valve, which is good, and it appears that whatever muscle bundles might have been in the way of her VSD repair have been cut away. Apparently, Aurelia's heart has become a little larger to compensate for the resistance placed against blood flow due to the PA band she received in October. As a result, the increase muscle tissue in her heart might have obstructed those areas the surgeons needed to access when repairing the VSDs.

The echo cardiogram taken immediately after surgery shows no leaking across the two chambers of the heart and no leaking across the valve, as I mentioned above. It also appears that her heart is successfully handling the full blood flow into and out of the lungs, and through her body. Whether or not her heart could handle pumping by itself is a concern, primarily due to the fact that her left ventricle sits right on the border line of a functioning/non-functioning ventricle (which is based on its capacity and ability to maintain adequate pressures and flow).

Currently, Aurelia is off the heart lung machine, being extubated, and undergoing an x-ray. We will be watching her carefully over the next day or two for effusion, or increased fluid around the heart, and any change in her heart's ability to handle blood flow to the lungs and body. Again, due to her heart's increased musculature the trick is going to be getting her heart to relax substantially, so that it can fill to complete capacity, and subsequently pump blood to and from the lungs to complete capacity. In the past her heart built up its strength and did not pump to capacity because of the restrictive PA band. Hopefully, though, given the medicine she is on we will see improvements over the next few days as her heart begins to fully manage the appropriate blood flow of her body.

Thank you to everyone for their prayers and well-wishes. These next 24 hours will be crucial in Aurelia's recovery, so we will keep you informed. For now Aurelia is out of the dark heart of the forest and onto the path, but the way is steep and overgrown, and fraught with briers and other dangers, so keep those prayers and positive thoughts coming.

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.

Phew!

I'm sure I've used that title before, but man am I tired. It has been a whirlwind these last three days. Aurelia and Madison are adopting to life at home again, and for the most part that's going smoothly. Aurelia does her best to be good natured even as we turn screws in her jaw every day, disinfect and slather up her ears in ointment. Madison is coming around to once again be the girl we knew in April and May, but this is the first time I've seen her adaptation slowed somewhat by jealousy. Her needs have visibly intensified for the first time since this odyssey began.

Our poor cat Ulysses had a serious infection on his hindquarters. Due to stress, we hope, he started rubbing his ass along the rug while we were gone. He rubbed his skin raw. He was a bloody, swollen, infected mess. Now he's still swollen, but he's shaved, bathed three times a day (which he loves), and instead of bloody he's all covered in a sheen of ointment. Yes, that's right, I get to rub ointment all over my cat's privates, including his butt. Be jealous. In the pity category, he is a cone head kitty for a few weeks and has to stomach the indignity of being given two different medicines throughout the day. That's a fun time for everyone, let me assure you.

It has been an interesting time for me and Jillian as caretakers during this week long hospital respite. Aurelia's care schedule is so complicated and covers so many medicines with changing dosages each day that we had to make up medicine and care charts, different ones for each day, so that we can be sure she comes off her pain medication comfortably. It's been challenging to balance those needs (which are frequent), Uly's needs (less frequent), and Madison's increasing needs (less frequent still, but more than ever before), with our needs to catch up with and take care of everything here at the house from our recent stay away.

Now that we are on the second half of the week and moving toward our next stay at CHOP things are beginning to shift into planning mode for our time gone. We think we'll have Madison stay here at the house with my in-laws to reduce the burden of travel on her. We are also thinking about how we'll handle Ulysses and the other household issues that my in-laws would have to take care of while we're gone; we are appreciative of their help, but we certainly don't want to abuse their good will!

As the pace quickens I hope we don't forget anything. We'll see!

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!

A fast few days

We have made our way to the step-down unit in CHOP. In brief, Aurelia chugs along, improving with each passing day. Her pain medication woes lessen, her strength increases, and her oxygen doses decrease over time. Today she's needed no additional tylenol or ibuprofen, she is down to 1/2 a liter of oxygen, and she managed to sit upright on her own with limited support for nearly a minute. All these are wonderful milestones.

We are pushing the doctors to let us go home for even a bit before her next surgery on the 13th. We have to return here on the 12th as it is, so any time at all would be welcome. Some sense of normalcy brings with it amazing relief, even for a few days. I imagine we will be back here for two weeks more starting on the 12th, so I pray for a break soon. Our family was together at home for only a week after school ended for me before we traveled to Connecticut and found ourselves here two weeks ago.

Let me say thank you to so many of you who have supported us; your facebook messages, blog comments, phone calls, caring for our cats and one helluva hardy fish at our house in Gettysburg, and the countless prayers, positive thoughts, and well-wishes have sustained us mightily. One day last week more than 100 people read our blog, sending their good thoughts and prayers Aurelia's way, and for that we say 'thank you.'

It has been brought home to us time and again, this most recent stay no exception, that our employers, the Northeastern School District and Carroll County Public Library System, are amazingly compassionate. Colleagues have sent cards and gifts, offered help and support, and have made themselves available to us in this, and all our other, times of need. Thank you. You have helped raise our daughter and have made sure that Aurelia not only lives, but lives well and strong. You are a part of her family and we look forward to the time she meets you all.

I want to add, too, that so many of you have asked after our other daughter, Madison. Although only two, in the last year and a half she's had live-in house guests for nearly twelve weeks when my wife was on bed-rest with Aurelia, she has had to live away from home in a hotel room for nearly four of the last ten months, and she spent nearly three months with her dad two hours away in Gettysburg, as I had to work immediately after Aurelia's birth. Madison endures with great poise, and determination, and joy. Such pure, happy joy as only a two year old can have. She has been a gift to all of us, helping us laugh and smile, and encouraging us to always remember that family, no matter the challenge it endures, is most important. You have all helped raise her as well as Aurelia, in your own way. Thank you.

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? :)

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. 

It's been a winding road

It's been several days since we posted and Aurelia endured ups and downs during that time. She got past her cold, and the flu, with relatively little fanfare. In the days since Jillian's last post her parents both stayed behind and watched over Aurelia's recovery with us until Wednesday. Jillian and I made a good transition once they left, balancing all these changes well and moving into the Ronald McDonald House here in Hershey.

What a lovely facility. The people there are very nice, the house is much less restrictive, and it is right across the street from the hospital. I feel very welcome there, and the house is in terrific shape. Just being this close to Aurelia has been a boon for our family, allowing one of us to sleep with Madison close by (on tempurpedic mattresses, no less), while the other keeps vigil with Aurelia. My work is only thirty to forty minutes away, allowing me to take only one or two days off here or there, so I don't have to burn sick days needed for Aurelia's anticipated second surgery, and being so close has made us much more comfortable confronting any deficiencies in Aurelia's care.

The hospital itself, with the exception of a few days in the middle of the week, has done a nice job caring for Aurelia. We've only encountered one or two bumbling/disengaged nurses, and after a particularly bad night a frank conversation with the attending increased communication between her care team and us as parents. The night in question saw Aurelia (who had been moved out to the general pediatric floor from their intermediate care unit) have her feeds not only delayed by hours, but wasted, spilled onto the floor by a nurse who had more concern for the online course she was taking than getting a feeding tube connected correctly. Add to this an increasingly difficult couple of hours where her need for deep suctioning (where they stick a tube down your nose into your lungs) increased so much that every thirty minutes nurses had to intervene to improve oxygen saturation rates, and what we had was a girl who needed 1 liter of oxygen just to maintain baseline oxygen levels.

We found ourselves in the intermediate care wing again, waiting in a little pod for a room, at 3:30 a.m., with our possessions (and a cot) scattered around us. With a fever spiking, Aurelia retching on phlegm, and my temper fuming, we couldn't even find an attending, let alone a resident. Needless to say, Jillian and I were more than slightly distressed. Jillian took over for me the next morning and did a great job straightening out the staff here, and ever since Aurelia's care has improved, even though the diagnosis worsened.

Aurelia has been diagnosed with pneumonia. The first twenty four hours did not go well. Antibiotics started late, the chest x-ray took hours to schedule, and we had serious concerns that the course of action to heal our baby girl had been derailed by poor attention to detail. After their conversation with Jillian, however, things turned around sharply. Aurelia has been on the mend since, her fever holding steady, the garbage in her lungs coming up with increasingly less invasive suctioning, and her mood improving drastically.

She's sleeping well, smiling, laughing, and doing great for a girl that has had a serious cold, flu, and now pneumonia over the last three to four weeks. We expect to be here several days yet, barring any set backs, and we'll keep you posted.

Thank you all for your prayers and good wishes - they mean the world to us!

Time to settle in

And a good morning to everyone from Chocolatetown, USA. Here in Hershey we had an uneventful night punctuated with some PT and suction, and just a bit of tylenol. Aurelia did well last night, her SATs dropping nowhere near to the level they had the night before, and other than a return of her fever (which was very borderline) she made steady improvements.

This morning she is in fine spirits, playing with her toys in her crib, and making friends with today's nursing and doctor teams. We are still in the step down unit and we will likely be here for two more days at least. At rounds this morning we discovered that the rhinovirus she has is going around and giving kids a lot of trouble, setting some up for other infections like pneumonia and RSV if not treated quickly. This made us feel good, obviously, and somewhat vindicated as well. It's hard for people to grasp that even the common cold (what the rhinovirus usually causes) can end up creating situations terribly troubling for CHD kids.

So Aurelia continues to fight, and with a smile while she's at it. The discharge plan for Aurelia will come only after her lungs are fully clear and her secretions drop, and there is no fever. So, at minimum today and tomorrow, maybe longer. Jillian and I will be working to make plans for the week, so we can be sure that we are here to take care of our Sweet Pea.

The briefest of updates

Jillian and I realize we've not posted in far too long. Truthfully we've spent these last few weeks re-learning what it means to be a family at home again; so much is familiar but everything is different. We anticipate regular updates again soon, but for now please know that we are all well, and short of a few colds Aurelia's transition could not be better.

We'll write again soon.

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.

Lucky Seven...

We're still waiting for surgery number seven to start. We had thought 12:30 pm would be our go time but we've only just now arrived in the cardiac prep and recovery unit. it could be anywhere between 15 minutes to an hour before anesthesia begins......

I spoke too soon....anesthesia is here.....wish us luck. Another update to come soon.

Back a little closer to home...

So we're back in the CCU! Since we've been gone they've upgraded the rooms with new all-in-one computers and televisions. We are back in the same room we were after Aurelia came back from her first PICC line, when her airway first collapsed and the NEC watch sent us back to the CICU. I don't mind this case of deja vu if it means we get to come back to the CCU instead of leaving for some place a little more urgent.

In sadder news one of our housemates, a wonderfully nice family from New Jersey, lost their CHD baby, Luawn, last night. She had a defect very similar to Aurelia's and unfortunately developed a secondary problem that resulted in her heart's ventricles never fully relaxing. As a result her heart couldn't fill itself with enough oxygenated blood between beats. Her folks got the call around two o'clock a.m. and when they arrived they removed all the tubes from Luawn's body and she rested peacefully in her mother's arms until she passed away.

It's a tough reminder about the dangers of a CHD when you see so many of these babies that fight just as hard as Aurelia lose their battles. We can only hope that what afflicts Aurelia cannot match her own determination and strength. When combined with the skill of this amazing medical team we have faith that our girl will give this fight all the will she can in order to live a full and healthy life.

Still, things over here are much better for our little A. She's off oxygen and the high flow and is only on a room air canula. She had a little gas this evening and so was a bit fussy, but our awesome nurse Laura (who has been nothing short of magical) takes such good care of her that with only a burp and some anti-gas medication our little girl is sleeping soundly.

we hope that the news about the NEC watch continues to be good and that after the treatment course ends Tuesday we'll get good news about a surgery schedule for Aurelia's hernia, nissen, and g-tube.

One last note - Grandma Dittrich left today for Connecticut after a two week stay and her help was invaluable. She took great care of Madison and Jillian, and poured out love on Aurelia. Madison is voicing all the words Grandma taught her and loved taking walks around the city with her Grandma. They read so many wonderful books together down in the play room and it was the first time in some time that all the wash was up to date and the room here at the house was clean.

We will all miss her much! Grandma and Grandpa DeAlessio arrive some time today barring any misfortune on the roadways, and we hope they make it here safe, sound, and in a timely fashion!

The world shifted and someone forgot to tell us

As many of you know from our facebook updates, text messages, and phone calls, we are back in the intensive care unit. Here's today's tale as best I can remember it:

I got up at 4:30 this morning and went into the bathroom. I planned on taking a shower and driving to work from Philly this morning. I wanted to spend as much time with the girls as I could and Madison has been having a good number of Daddy-days this week and I planned to take advantage of that all I could. I stepped out of the shower, toweled off, dressed, etc., and stepped out of the bathroom to hear Jillian on the phone.

Dr. Jansen had called from the CCU; Aurelia was on her way back to the CICU with the approval of our current attending, Dr. O'Connor. Jillian absorbed all Dr. Jansen told her with magnificent clarity. She told me that after we left Aurelia last evening she never really settled down to sleep. Problems escalated through the evening and combined they led to the decision to intervene and change the level of her care.

Yesterday afternoon Aurelia had a procedure to drop her feeding tube from her duodenum (which had been operated on during her first day of life) to a spot further in her intestine. We hoped to increase her comfort over the next week as we prepared for a g-tube by lessening her reflux, gagging, and spit-up. The procedure took over an hour and reportedly gave the people in Interventional Radiology quite a challenge. As a side note, Dr. O'Connor is disturbed by this and is investigating why a relatively simple procedure became so difficult.

Aurelia took to her feeds after the procedure well and returned to full feeds by the time Jillian and I returned to the Ronald McDonald House. A constantly elevated heart rate of 180 gave the team some concern, but Aurelia had a very long afternoon and we thought with rest she would eventually calm down. Between when we left at 5:00 p.m. and when we got the call at 4:00 a.m. Aurelia's condition worsened instead of improved. Her heart rate never dropped and as the evening wore on she became increasingly uncomfortable. They stopped her feeds and ordered an x-ray.

Around 1:00 a.m. they got the first film back and it showed possible evidence of necrotizing enterocolitis. That's right, NEC had returned; or at least it might have. To be sure, the team ordered another film and restarted Aurelia's feeds until they could be positive that a NEC watch would be appropriate. As the feeds began again Aurelia became more distressed. Her heart rate remained elevated and her respiratory rate climbed.

The second film, taken around 3 a.m., revealed evidence of pneumatosis, or gas cysts in the intestinal wall. At that point the wheels began to turn and they started the process of another transfer to the CICU. Aurelia became increasingly troubled as phlebotomy struggled to find a vein for an IV. They found one, she went NPO (pre-operative fasting, or removal of all food and supplement with fluids only) and the transfer was completed about fifteen minutes before our arrival here at 5:10 a.m.

At this point today's tale becomes more disconcerting to Jillian and me. Her nurse here from 5 to 7 a.m. seemed competent and worked with us as Aurelia was stuck for labs, lined up for another x-ray, and scheduled for a second IV. Apparently her heart's hard work during the previous twelve to fourteen hours had 'chewed up' some of her platelets and she needed a transfusion to maintain optimum blood count. This transfusion would require a second IV. Rightfully so, Jillian and I hesitated over this course of action and expressed concerns at the duress IV attempts always caused Aurelia. While a simple procedure, IV placement takes so long and requires so much stress for her that we felt this a bad idea. The need for her to maintain an optimal blood count won out, however, and the IV plan went forward.

We didn't express these concerns lightly; Aurelia's tracheal malasia flares up when under physical stress and she had weakened herself with 12+ hours of 180 bpm of heart racing exhaustion and crying. Add to that her vocal cord paresis and we felt that additional trauma at this point in the morning would not serve her best. We left to get something to eat and when we returned we found Aurelia with a blood soaked right foot, blood on her bedding, and her face strapped to a CPAP machine.

It felt like someone had rotated the earth a half turn and left us standing stock still. Our girl had been fine the day before; we even got permission to start taking her on stroller trips around the hospital! We had rigged her crib with visual stimulation, her OT exercises were going wonderfully, and for the first time we really felt as if the end was near. All we had to do was get that g-tube in, get trained, and one week later we would be home. Now our daughter's hypersensitivity is in overload thanks to the ridiculous CPAP, with sedation and anti-anxiety medication the only things keeping her from lashing out over hunger and the harness, she's on 6L of 30% oxygen, two IV's in her, a blood transfusion completed, NEC invading her bowels, getting x-rays every six hours, and belly measurements every four. To top it off, the bungled second IV and transfusion fiasco so upset Aurelia that her airway completely collapsed, her oxygen saturation dropped to the 40s from the low 90s, and her heart rate plummeted from 180 bpm + to 80. They had to manually pump her oxygen to keep her heart beating and her blood saturated until the CPAP could be hooked up.

I still don't know how we got here. What a vicious reminder of how precarious Aurelia's health is, even as we prepare to leave here in the coming months. I'm scared of what the future will hold for her, as much as we love her, as we struggle to keep her well and alive. Our immediate next steps include scheduling a PICC line for Friday in a sterile environment, monitoring the pnematosis on the regular x-rays, pump her full of a broad spectrum antibiotic cannonade against the NEC over the next seven days, ween her off the CPAP machine, and get her strong enough to endure a three-fold surgery scheduled for late next week.

General surgery came in to see her and their assessment is another open body surgery to repair Aurelia's hernia, insert the g-tube, and conduct a nissen fundoplication. I'll post more on the other issues and concerns in upcoming posts, but that's where we stand now. We're being booted from the CICU while they bring another baby back from surgery, so we're going to dine with Madison for dinner and then visit with her for the pet-therapy dogs that come to the Ronald McDonald House every Wednesday. We'll be back here tonight I'm sure.

We'll keep you posted as best we can and thank you to everyone for your positive thoughts, well wishes, offers of help, and prayers. You are the strength that keeps us going.

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!