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.