The "What's Wrong" post

This is the part where I try to explain everything that we've been learning over the last three days, in a condensed enough version to fit into one blog post. Wish me luck.

 The picture above is your basic heart. Essentially, it has four chambers (which you probably learned in basic middle school biology); uppers are atriums, lowers are ventricles. "Blue" (un-oxygenated) blood is pumped into the atrium on one side, then pumped out of the ventricle on the same side into the Y shaped pulmonary artery. That artery takes blood to the lungs to get oxygen. On the way back, it's then called "red" blood, and is pumped into the atrium on the other side, and transferred to the corresponding ventricle. From there, it goes up the candy-cane shaped aortic artery, where it's distributed to the rest of the body.

This is the most recent illustration we have been given of the issues presented, and, although not the most artistic (I have one that was done in pink and blue hi-lighter, and another that looks like a doodle on someone's homework), it certainly makes the most sense.

To start, you'll probably notice that the pulmonary artery looks a little different. Originally, they thought that it connected but was blocked off, doing essentially nothing. Turns out, it is functional, but not nearly the size that it needs to be. This is obviously an issue when it comes to getting blood to the lungs for oxygen, and is the reason for the low pulse oximeter reading that lead us to the hospital in the first place.

The red circle marks something called the Patent Ductus Arteriosis, or PDA. It's something that everybody has had at some point, but is generally only used in the womb. Anywhere from hours to days after birth, it closes off and is no longer necessary. Before birth, babies don't need to oxygenate their own blood...that's what Mom is for. The PDA causes most (and most is a key word here) of the blood to bypass the lungs to save any unnecessary effort. However, because of the insufficient size of the main pulmonary artery, that duct is actually the only thing allowing blood to get to the lungs at all. The medication Brandt has been on (prostaglandin, if you care...although the spelling on that may be atrocious) is keeping that duct open, and allows his heart to function at a pretty basic level. It isn't perfect, but it's functional! It was when this started closing that the murmur developed, alerting us to the bigger issues.

Finally, the purple circle points out a hole in the ventricular wall, or a VSD. This isn't exactly what Brandt's looks like; his is actually much more extreme. The opinions have jumped from "there's just no wall there at all" to "there's a whole ventricle there somewhere, but it's too tiny to work", and now, they've concluded that there's a "nubbin" of ventricular wall there, toward the base of the heart. It's essentially a really big hole. They aren't sure if it's fix-able or not, and I'll go into that during the surgery part of this post.

What all of this means is that the blood that enters the heart all gets mixed the second it is pumped into the ventricle. Then, it's pumped into the two arteries with differing amounts of success. A tiny bit makes it through the pulmonary artery, and the rest goes up the aorta. Once there, some makes it down the PDA, into the pulmonary arteries and to the lungs, and the rest goes to the rest of the body. The stuff going to the lungs is already partially "red", and the stuff going to the body is not as "red" as it should be. The heart pumps, but not efficiently.

On top of all of this, his heart is on the wrong side! They call it dextrocardia. This doesn't further complicate things, aside from the fact that surgery is like working through a mirror. Just a little more difficult, but like Dr. Ruggerie said, "That's what we do!"

So the full list of words to describe his "condition" is: Dextrocardia, pulmonary atresia, and a large ventricular septal defect.

The Surgery

So far, we don't have a totally defined, 100% sure plan. Tuesday, all the doctors will meet together to discuss their game plan, and we should hear back from that by Wednesday morning sometime. However, we have run through the most likely option with a couple of different cardiologists.

If this is the plan they go with, we're looking at 3 surgeries over the course of about two or three years. The first, which will be some time in the next couple of weeks, will be to insert a shunt to perform the job that the PDA is currently doing--allowing blood flow into the pulmonary arteries. With that, we can take him off his medication and allow the PDA to close like it was originally intended to. His heart will function like that for the next few months.

Then, between 4-6 months of age, he'll have his second surgery. As they don't think that putting a patch into the ventricle will work (too big of a hole), bypassing it with the blue blood is the next best option...one I didn't even realize existed! On the far left side of the picture above, you'll see that the right atrium has two direct feeds of blue blood; one coming from above (the head and neck), one from below (fed by the rest of the body). The second surgery will place a shunt that redirects the blood flow from the upper body directly to the pulmonary artery, straight to the lungs, which takes the heart totally out of the equation for that round. They call this a Glenn shunt, if you'd like to Google for a diagram.

The third surgery is to put in a Fontan shunt, which is essentially the same thing, but its purpose is to redirect the blood from the lower half of the body away from the heart and directly to the lungs, instead. This surgery doesn't really have a time frame aside from "in the next couple years", as it's based more on the needs of the child, and how well his heart does after the first surgery.

When these three surgeries are done, the heart will essentially be performing half of its normal duties. The majority of the blue blood will run past the heart, but then get pumped through the left atrium and the one ventricle he has, up through the aorta, and out to the rest of the body. This takes most of the strain off the heart, and leaves him with a body that can function nearly up to par!

The doctors tell us that he should grow up to be a totally normal kid. He may never get to olympic-stage athleticism, but I'm sure he'll get over that part. After the next couple years, it looks like life will go essentially back to normal! It's a short little gauntlet we run, after all!

A quick daily update, since I haven't posted one from yesterday: He's on a little bit of caffeine because of a little bit of apnea he's had, caused by the medication that is keeping his PDA open. It's to be expected, and they aren't concerned about it at all. We've gotten to snuggle him almost more than he's been laid down, during the day, and he's been able too nurse!! I've been pumping and pumping and pumping, so he's been getting bottle feeds at night, and then I nurse after pumping during the day, so he gets to get full feedings that way, as well. He's a champ nurser, which will makes things a lot easier after the surgery...one less thing to have to worry about.

Next post will probably be Wednesday, after we've heard back from the doctors.