I'm burning up in here!

Today has been a relatively quiet day. Aurelia went through the night with only a few hiccups, trying to turn over a few times, needing a pain management rescue now and then, and otherwise attempting to wiggle free of her intubation. Truly, though, she's doing relatively well. She's a wonderful fighter determined to beat all that comes her way. She inspires Jillian and me to keep faith and strong together.

Plastics came by this morning and moved her jaw two millimeters. Aurelia took it on the chin like a champ (da da dun, ching!) even after only 30 minutes lead in time with a pain med treatment. She did so well the plastics folks are intending to come back tonight for another 2 millimeter extension.

The day itself has been rather uneventful which oddly keeps me on edge more than a crisis would. Aurelia had some mild alterations in her pain medications to keep her sedated and from turning over (which she successfully completed early this a.m.) and subsequently aggravating her jaw. The IV she lost yesterday in her foot has yet to be replaced because they taped down and isolated the remaining IV in her left arm with a brace. We hope her veins are repairing themselves in case we need to drop a second line in for medicine administration should this last IV infiltrate.

Aside from that small adjustment to her pain medication, our daughter continues to gain strength. Her oxygen saturation levels are good, no longer suffering from momentary desaturation and she appears to be bouncing back well. We have a few concerns, such as a stubbornly resistant slight temperature, a fairly good amount of phlegm coming up from her lungs via suction, and a resulting junky sound over the stethoscope. The attending isn't terribly concerned; fevers are common after surgery and broken bones, and Aurelia's labs yesterday came back great. Similarly, if there is an infection there have been no substantial changes to Aurelia's vitals, meaning she is resisting the bacteria/virus well on her own. If there is a change in any vitals then we'll culture some of the phlegm from her lungs and draw more labs.

We are hoping for some more good news soon, however, particularly regarding her tracheomalacia. ENT did not mark malacia as present during the scope, so we don't know for sure, but we can hope that our little warrior has outgrown this airway debilitating problem. On a scale of 1 - 4, with 4 being, "HOLY CRAP, CALL IN THE REINFORCEMENTS," Aurelia's throat is listed as a 1, or a non-critical airway. That thrills us, since six months ago she'd have airway collapses from getting too worked up! We look forward to final confirmation from ENT, but we think the news will be good. We hope, anyway.

So that's all we have for now from CHOP. Maybe more later, if not, definitely more tomorrow.

Here's our girl this morning, resting after having her jaw advanced. Please no comments about her thighs. She's very sensitive about them. ;)

What a whirlwind

So. Here we are. I wonder why it is this blog only ever sees attention when I have difficult things to post? We came here Sunday night from Connecticut (after visiting parents on both sides) to participate in a sleep study. We wanted to see if Aurelia had sleep apnea. The test revealed severe apnea, to the point of her oxygen saturation dropping thirty to forty points into the 50s. The results astounded us, making me question my ability to care for Aurelia at home; how could I have overlooked something this dangerous? We gave her oxygen, measured her saturation rates and even monitored her O2 levels daily, so how did we miss sleep apnea this bad?

Little did we know, but babies with terrible apnea often learn to position their bodies optimally for improved breathing. Aurelia's preferred sleeping position was on her side, 3/4 turned, back arched, head back. She subconsciously moved her tongue forward in her palette and minimized the impact of enlarged adenoids every night. During the sleep test, wrapped as she was and forced to lay flat on her back, she sounded like a freight train rumbling on decrepit tracks, squealing under heavy load.

Since 3:00 a.m. that morning we've been reacting to constantly changing circumstances, expectations, assistance, and fears. Monday came and went without any surgery and only the beginnings of a plan to treat Aurelia and Tuesday brought more frustration as plastics, ENT, and cardiology attempted to develop a care plan for our little girl. We did our best as a family to stick together though we all felt completely helpless and unable to control any part of the situation. Jillian and I tried to get organized for what could be a multi-week stay at CHOP, and Madison persevered valiantly, trying to have fun and understand why her sister was so sick and we couldn't go home to see our cats, Ulysses and Shiloh.

Frantically, we canceled therapy and doctor's appointments for this week, made arrangements for friends to care for our cats, secured lodging at the Ronald McDonald House, and convinced Jillian's parents to come down for a few days during and after Aurelia's surgery. We completed these tasks amid ever changing plans for Aurelia. Doctors informed us that Aurelia's care could require weeks at the hospital, that we'd be having one surgery, or two surgeries, maybe repairing her cleft palette, maybe not, maybe looking at a tracheotomy, maybe removing her tonsils, maybe doing nothing, maybe moving her jaw. What registered clearly for us was that until these breathing treatments were resolved, Aurelia would not have her next heart surgery in July.

We trust the care teams at CHOP with our daughter's life, so really we knew that our preparations had to be for the most severe scenario - a two to three week recovery from multiple surgeries leading straight into her next open heart surgery in mid-July, with us getting home sometime right around the beginning of August. I cried, cursing how a stupid sleep test could turn into more than a month away from home. I fumed, infuriated that my family was thrown into tumult with little I could do to restore balance and order.

To make Aurelia's (and Jillian's) time in the cardiac intensive care unit somewhat easier - remember that these rooms are four pods a piece, with limited family members allowed bedside by each patient, I left Philadelphia with Madison Tuesday. To her credit, Madison rallied her spirits here wonderfully, but she is just a bit too loud for promoting appropriate care in an intensive care unit. At home I could both occupy Madison and take care of some loose ends at the house. Jillian remained with Aurelia, focusing her care entirely on our little one as surgery approached.

We went into surgery this morning with a plan in place. Aurelia would have tubes inserted into her ears, her throat and bronchial tubes scoped, her vocal chord paresis examined, her enlarged adenoids trimmed down, and her mandible advanced. The surgery would involve both ENT and Plastics, and last for several hours. Aurelia has come through that surgery wonderfully and now recovers. In future posts, I will talk more about the procedures and some of the people working on Aurelia during this most recent stay here at CHOP.

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.