Sunday, September 26, 2010

A Whirlwind Day

I'm not even sure where to begin with today's post. I know people are expecting some updates, and I know that it's not required of me to post every day, or multiple times a day, but something just compels me to expunge some of this into writing to help me sleep or relax or whatever.

Aurelia is doing well today. The funny thing is I can't even remember what I've passed on and what I haven't to everyone that wants to know about her story. It's absolutely exhausting when you're working to keep tabs on what feels like a million different departments, specialists, lab results, doctors, and changes in your baby's status. I'll try to give a comprehensive update of where Aurelia is with all her issues, hoping that I don't dull your senses with things I've said already, but give you the new information that hasn't made it out of my head yet.

Aurelia's blood clot has disappeared for now and this is a wonderful wonderful thing. It will allow her to receive blood thinners and be put on bypass for her heart surgery and it means one less course of action we have to take to correct her cardiac function. The neurologists will consult with us again tomorrow about the status of her clot (as well as any impact her enlarged #2 ventricle will have on her development, if any) and I wouldn't be surprised if they schedule her for another MRI before she goes into surgery for her heart. The neurologists will also be working with the ophthalmologists and the geneticists to determine what, if any, genetic abnormality or syndrome is at the root cause of all these troubles.

To that end, the ophthalmologists and the geneticists will likely consult tomorrow as well. They've done some preliminary work, but I wouldn't be surprised if they do more work tomorrow, or even schedule Aurelia for tests to try and get at what might be causing all these troubles. It's quite plausible that Aurelia has several defects among her chromosomes and that combined they each result in the many problems she faces. This 'syndrome' would have no name per say, but would explain why so many things are going wrong in her physical development.

The urologists consulted on Aurelia this morning and between them and the kidney specific teams they have decided to leave well enough alone and let her kidney stay inside her at the moment. It's quite possible that her kidney will die and shrivel up inside her. Either way, those kidneys are prone to infection and as a result she will be on antibiotics for her entire life (or as long as the surgical team decides to leave the kidney in place, I suppose. There is a test they will perform at about one month of age, and that will clarify the course of action  they choose to take, but that is still weeks off, of course.

Aurelia continues to recover well from her surgery the other day. She has shown no signs of internal bleeding and she has already had several bowel movements. This is key, as anytime your intestines are operated upon they often shut down for a period of time (up to a week!), but in Aurelia's case they are up and running again. They are still running suction into her stomach to ease the burden on her newly reorganized and re-routed digestive track and they will keep her off food for one week as they prepare her body for surgery. Thankfully, she has not yet been fed any food (other than nutrients through her umbilical lines) so she does not yet know what it means to be full or hungry.

Her heart surgery is scheduled for October 4 (her original due date) and it will be a modified Norwood procedure. In children with HLHS the first procedure, called a Norwood, widens the pulmonary artery, separating the main pulmonary artery from the left and right portions and joining it with the upper portion of the (typically smaller) aorta. This allows a mixture of oxygenated and non-oxygenated blood to move through the body, pumped by the one remaining good ventricle.

A man-made shunt (think of a canal connecting two existing rivers) is then installed on the subclavian artery to connect it to the pulmonary artery. This allows the single ventricle to move the non-oxygenated blood into and out of the lungs and into the body's circulatory system. I've included an image below to help make some sense of the medical jargon.

In Aurelia's case the left ventricle is still present (a victory, as in most HLHS kids the ventricle is not functioning) and her mitral valve and aortic valve are smaller, but still considered adequate for life. In most HLHS kids, these valves are not functioning, or will not function normally.

This means that Aurelia will have a more hybrid procedure, which will place a stent in the aortic bridge and a band around the enlarged pulmonary artery to prevent over-circulation to her lungs. It's also hoped that this band will help trick her heart into repairing and closing the existing hole in her heart between her right and left ventricles.

If all goes according to plan, Aurelia will do well and hopefully begin to eat and put on weight during her recovery.

To that end, I should mention that Aurelia's cleft palette is not a concern for her eating at this point, and it is so far down the list of issues that we are facing that we haven't even talked about getting a consult on what to do to fix the problem. I suppose that will simply be a bridge we cross when we get there.

Jillian and I also agreed to be part of a study at CHOP for parents. It is called, C.O.P.E. and it is designed to scaffold parents into care for their congenital heart defect baby. It's a new program designed to show parents the many similarities (and of course the differences) between a baby born prematurely or a long-term NICU baby and those babies with cardiac difficulties similar to Aurelia's. For instance, both share some very different sensory experiences and both may experience developmental delays as they age. We'll probably write more about this program soon, but we're very excited to be a part of our first study at CHOP - as parents. To us, this is one of the things that makes CHOP different; it isn't just about the patients, but also their families as they struggle to adapt to life with a congenital heart defect child.

Similarly we also have arranged for Madison to meet with a life counselor who will work with her in the coming weeks to handle the transition to having a younger sister. While often difficult enough for older siblings who have a newly arrived younger brother and sister, having a new arrival in the house that has very specific needs and requires additional attention can be even more challenging. Above all we want to be sure that Madison's emotions and experiences are validated, that no unfair expectations are placed on her, and that she is permitted to create a relationship with her little sister that is genuine, and not hurried or pushed by us or medical concerns.

It's going to be a tough road, but we'll get there!


  1. Thanks for sharing! They say journaling is a good thing! Just want you to know you are in my thoughts and prayers!
    Peggy Carper CCPL

  2. well done charley, prayers and many many hugs coming your way! love u all lots.

  3. Thank you so much for sharing. We are keeping you in our thoughts and prayers at NHS! Thinking positive thoughts!
    - Donna