Its kind of a big deal

This morning we met Dr. Manning, the director of the Division of of Cardiothoracic Surgery at Cincinnati Children's hospital, regarding Drew and what he has going on. Here's a brief recap of how we ended up here.

Drew has had noisy breathing and a cough for the past several months now. Through a bronchoscopy (camera down his throat) they were able to take a look at his trachea (windpipe) and saw that something wasn't quite right. There were two major issues. The first is the compression they see right where the aorta crosses the trachea. The second is sort of "broken" or misformed cartilage causing the trachea to look funny. I'll touch more on the second issue a little later, but right now I'll try to explain the first. Keep in mind that I'm not a doctor, so if you're reading this and you are a doctor don't criticize.

In your chest behind your breast bone, there is more than just your heart. Your trachea is back there, some arteries, your thymus gland, your esophagus and that’s all I can remember. Anyway, it’s crowded between your breast bone and your spine is my point. Your trachea and my trachea are hard as bone. Babies’ tracheas are soft as butter. Many kids suffer from something called Malacia, which is just some floppiness of the trachea. While they are still little, their already soft cartilage is even softer than normal so when they breathe, rather than the trachea expanding and contracting normally, it doesn’t keep its form and causes rubs against itself a little bit, causing a noisy sound, like you would hear if you had a frog in your throat. Drew has a little bit of this floppiness going on, but additionally, one of the arteries that is back behind the breast bone seems to be pressing on the trachea a little bit. It’s not terribly uncommon, and because of how soft the trachea is at this age still, anything that’s pressing on the trachea will cause some level of compression.

The aorta, one of the main arteries to the heart, crosses over the trachea in all of us. In babies, because of the “crowding” behind the breast bone, sometimes where this crossing takes place there is some level of compression. This is what’s going on with Drew, and here’s why it needs to be fixed. Let me first say that this is not a CF issue. This is an anatomical abnormality that is complicated by the CF, but is not a CF issue. The compression that is being caused by this artery does not allow for proper airway clearance. Drew just coughs and coughs and coughs because this compression is causing sort of a tickle in his throat, and then all of the coughing is creating irritation which is causing more coughing, but when he coughs the trachea just essentially closes for a second and he struggles to breathe. And on and on the cycle goes. So what needs to happen is to move that artery off of the trachea to allow it to remain open the way it’s supposed to be, and the way they do this is with a procedure called an aorta pexi.

On Tuesday, September 7 Drew will have this procedure, the aorta pexi, done. They will make an incision on his left side (the aorta is on the left side of the heart) kind of under his arm and spread his ribs cage to get in there. They will cut out a part of his thalamus so that they can get to his heart. This shouldn’t be a problem because studies have shown that the thalamus’ job is mostly done prior to birth, that it really has no effectiveness once you are born. That said, he will not lose his whole thymus so any unknown purpose of the thymus will be preserved. They will cut a flap in the sack around his heart (his pericardium) and then sew that to the inside of his breast bone. By doing this, they will lift the value off of the trachea and open it up. If you are curious about the order of things in your chest, it goes (front to back) your breast bone, thymus, your heart (arteries and all), your trachea, your esophagus, your spine.

What we know for sure is that by removing part of the thymus and moving the artery closer to the breast bone it will allow a little more space for the trachea and will relieve the pressure of the aorta on the trachea. What we don’t know for sure is how this will help and we won’t know until after the surgery. The reason that we just don’t know is because of problem #2, the broken cartilage. This is the issue that no one has ever seen before and we really don’t know how it’s affecting him, and won’t know until we get problem #1 fixed.

If you take your hands and make a C with each hand and put them together to make a circle, that’s what the cartilage that supports your trachea looks like. Its one nice, big, round piece of cartilage. Now looking at that circle you made with your hands, take one hand and slide the fingers down and in under the other fingers. You make an off shape circle that sort of closes in on itself. This is what’s happening with Drew’s trachea below where the artery is compressing. Its never been seen before and its unclear as to what kind of problem it is. It may not be anything anyones ever seen before, but if its not causing a problem, who cares what it looks like. We will see how this upcoming procedure affects him. With any luck, it will help to open up his trachea so that he can breathe and cough and clear his airways just like you or I, and then our problem will be solved. After this procedure we will just wait. We will wait and see what difference this makes. We will wait months to see if that broken cartilage fixes itself, if he sort of grows out of it, of if its going to be the real problem that needs to be fixed. I hope and pray to God that it doesn’t because the thinking is that he would need tracheal reconstruction which is a major major operation, not done pretty much anywhere else in the country other than Cincinnati, but its not something that we want to have to have done.

So we’ll see what happens on Tuesday. It won’t make things worse. It’s a big deal but its what has to happen so we’re going with it. Keep us all in your prayers for a successful procedure Tuesday and future free of trips to the OR.