Tuesday, August 06, 2013

Adam's Heart - Part 3


After delivering the big news, Dr. Morris and one of the fellows spent some time explaining what they saw during the echo, including some helpful freehand drawings they made of the heart. Adam had three issues which I’ll detail here. They are independent abnormalities and a name has not been given to them collectively, but according to his doctors, they do occur together as a group often. In the general population they are rare, but to the expert doctors and surgeons at Texas Children’s they are fairly routine.

1.       A bicuspid aortic valve. The aorta is the large artery leading out of the heart that delivers oxygenated blood to the rest of the body. There is a one way valve at the entrance to the aorta which, on most of us, has three “leaves” that make up the valve. Adam’s only has two. Despite this, it appeared to be functioning well. He may need additional surgery for this at a later date, but hopefully not until he is a teenager or adult, as the valve needs to be sized appropriately, and an implanted one would not grow with him. There is also a good chance this bicuspid valve will serve him just fine throughout his life.

2.       A ventricular septal defect (VSD). This is the classic “hole in the heart.” It will probably help to look at the diagram below. The ventricles are the chambers of the heart that pump blood out – the right ventricle goes to the lungs, the left ventricle goes to the rest of the body. These two chambers are separated by a wall of muscle called a septum. A VSD is a whole in the septum. This is a surprisingly common problem, occurring in .1-.4% of the population. A small hole is not a big deal and often closes over time.

3.       An aortic coarctation. This is a constriction of the aorta somewhere along the arch made by the aorta as it leaves the heart. Along this arch, arteries depart the aorta to serve the brain and arms, and fortunately his constriction was after these departures. The result was that his lower body, including the vital organs, were not getting sufficient blood flow, and this was magnified by the VSD. This was the imminent threat to Adam’s life and the reason he was showing symptoms of very heavy breathing and elevated heartrate. There is a small blood vessel called the Patent Ductus Arteriosis (PDA) that directs blood from the pulmonary artery to the aorta in fetuses. (They get oxygen from mom instead of from their own lungs.) This blood vessel closes in the first few days of life. While Adam’s was open, it bypassed his constriction, but as it closed, problems started to present themselves.

 
 
We are counting many blessing through this process. One of them was the fact that Adam’s PDA closed a bit later than some, and did so slowly. This allowed us a few days at home to enjoy those first precious days of life, and got breastfeeding off to a good start. The fact that it closed slowly meant he showed symptoms the way he did, rather than having organs start failing without much notice. In other babies with his conditions, they do not tolerate the surgery as well when their other organs have been compromised.

The cardiology fellow explained they would consider doing one surgery to repair both major defects, or do one surgery to repair the coarctation, then another in a couple months to repair the VSD. They would do much more imaging to determine how substantial the VSD was, and whether it was necessary to fix it right away. Adam was started on an IV and given a medicine called prostaglandins to keep his PDA open. Pretty soon, a bunch of folks showed up to help move Adam to the NICU. He was transported up there in his heated bassinet, and we got our first visit to a level 3 NICU, the rating for those capable of handling the most severe patients.

While we registered at the reception desk outside the NICU, we unexpectedly ran into an acquaintance from our church named Jeremy. He was there with his wife and one-month old son, doing routine cautionary care for a newborn with fever. We didn’t know Jeremy well at all. He is an elder, kind of a public figure in the congregation, but I think I’d only met him once. It was surely bizarre to see someone you know at 11pm at the NICU in a city of 4 million, but I couldn’t believe how comforting it was to have a familiar face with us. We spoke for a few minutes, sharing Adam’s diagnosis. I met with him again the next day – sharing my fears, praying for Adam and Katherine and me, and shedding some tears. As I mentioned earlier, we received so many blessings in this process. I really believe that Jeremy and his family were placed there by God intentionally. We had a six day old baby facing heart surgery, and we were overcome with fear, shock, and sadness. Having Jeremy beside us and sharing that moment was such a magnificent comfort and, I think, a reminder that an almighty God would be guiding us through the coming ordeal.

The NICU was not what we expected - dimly lit, quiet, carpeted, lots of fun colors. It was actually a pretty inviting place. We met some of the staff and answered the standard questionnaire again while the cardiologists continued with an extensive echocardiogram. Adam was an absolute champ through this whole process. He remained calm with the help of a paci, and a few drops of “baby crack.” That’s what the doctors call a simple solution of sugar water that can be dropped in his mouth. After a couple hours of echo, they recommended Adam be moved to the cardiovascular intensive care unit (CVICU). That is where he would be following his surgery, and they were concerned that he may need emergency surgery if the prostaglandins didn’t work to keep his PDA open. The CVICU is right next to the cardiac operating room. Pretty soon, a whole bunch of staff showed up to execute the move. They are clearly prepared for emergencies along the way – his transport bassinet had all kinds of devices for oxygen and other stuff. We all took the elevator to the 18th floor, Adam’s home for ten very important days.  

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