Black Clouds

Tanya and I are burnt, spun out, and frazzled. Overwhelmed. For four months, we've been learning and preparing for what was to come with Eli. We've had a chance to ease into this. Now he's here and the future has been realized. I don't know if it's a matter of everything settling in because we're looking the battle in the face, or an inability to hide behind denial any longer. To be fair, I really don't think we ever employed denial. However, as we receive more information today, the pot has begun to spill over.

Tomorrow at 8:00am is Eli's first open heart surgery. Here are some details, for those who want them. This being the first of at least three of these kinds of surgery, there are three specific goals for tomorrow's surgery:

One: Rebuild the main aorta, which is too small. This amazes me. Part of the main aorta is too small. They're going to cut out the small section, and cut the entire aorta free from the muscle that surrounds it, so that the aorta can slide freely within that muscle. They will stretch the aorta and sew the two cut ends back together. It will re-fuse with the surrounding muscle on its own.

Two: The pulmonary artery is the one that moves "blue" blood from the heart to the lungs. Eli's is completely normal, 100% sized. However, because the mitral valve is so small (4.5mm diameter versus 12-15mm, like it should be), the blood can't get back to the heart from the lungs as quickly. In addition, the hole between the right and left ventricles is very large. So... they are going to put a restricting band around the pulmonary artery to restrict flow to the lungs, but just a little. The restriction will force blood from the right ventricle to go through the hole in the heart, and into the left chamber. This will effectively create a bypass of the lungs, for some of the blood. Without this, there would be hypertension of the lungs, and they'd fill with blood.

3: They're also going to remove the wall between the left and right atrium, so the blue and red blood have a better chance to mix and relieve pressure off the areas of the cardiovascular system that can't handle it.

On another surgery, they will combine the two major pumping valves (one to the lungs, one to the body) into a single larger valve, and will finally turn the heart into a 2-chamber, one artery system. Blood flow to the lungs will be passive rather than directed. I'll know more about this process as we get closer.

Then, we learn today that Eli has thrown another significant curve ball into the equation. Eli has genetic mutations. It's extremely likely (not yet verified) that he has Holt-Oram syndrome, or "Hand-Heart Syndrome." This is a mutation in the TRX5 chromosome (that's just what the geneticist told us, I'm not that nerdy), which is the chromosome that controls the development of the heart and upper limbs at the extreme early stages of fetus development. We haven't verified yet, but Eli might have an extra joint in his thumbs. They're still opposed, but possibly longer. We won't know until the X-rays come back. These mutations are unusually common with congenital heart defects. There's even the possibility that they could be tied to underdevelopment of the brain, leading to significant cognitive issues. Whatever the truth eventually turns out to be, this only adds complication to an already extremely difficult situation. I'll share more as I learn more. We're told that any genetic mutation leads to more difficulty through this process. Great.

So the part that's tearing me to pieces:

We can rejoice that this technology even exists. The surgeon told us that 20 years ago, babies like this were sent home to enjoy the short time they had. I asked him if Eli would live 6 months without surgery, and he told me "Not a chance."

Each of these surgeries carries a 90% chance of survival. Dropping a body's core temperature by more than 30 degrees, intentionally stopping a heart, and trusting in a man-made machine to temporarily take over for the heart and lungs... is wrought with potential complications, it turns out.

We're told that 60% of kids who go through this make it to the age of 5. We've also come to understand that should everything go well, and without further significant change in technology, we should recognize the grim reality that we should expect to out live Eli, anyway.

It's been a tough day. Thanks for sharing in our pain.

I don't even know what to ask you to pray for. I'll trust the Holy Spirit to come through on his promise there. Just pray.

Bring the rain.