More about Joseph

When last we spoke Joseph had just finished his first dry run coming off of the ECMO machine and was going to do it again the next morning and get an ECHO to help the doctors decide what to do. Well, he did get clamped off again at about 8:oo am, and was able to get the ECHO so that they could look at his heart and make some decisions. This is where the fun begins. You see, Joseph has two groups of doctors making decisions about his treatment, the surgeons who will be fixing the CDH, and the Cardiologists who are watching his heart condition (more on that later). After successfully coming off of the ECMO for the second time in 24 hours there were two different opinions on what to do, and Joseph wasn't helping to make it easy. It turns out that they were kind of hoping that he would react in a way that told them what was having a greater effect, the CDH, or the heart. Well, he did so well both times it was not 100% clear what the next course of action should be, do we take him off and do the surgery, or leave him on? Well the cardiologists were worried that the root of his problems was the heart and he should stay on ECMO for the surgery and watch the heart after, the surgeons wanted to take him off and do the surgery without the Heperin in his system


After much discussion, it was decided that it would be best to do the surgery while still on ECMO for a couple of reasons:
1. After coming off twice, he has shown that he can tolerate it pretty well.

2. We still aren't sure if the CDH or the Heart is the bigger problem.

3. Once you come all the way off ECMO you can NEVER go back on.

4. If the real problem is the heart, and you come off for the surgery, things could go south.

5. To find out what the real problem is you need to eliminate one of the variables, ie the CDH.

So, the surgeon will be doing the surgery tomorrow, December 26. We needed to wait until Joseph got rid of some of the excess fluid he has been carrying since going on ECMO. He has been on a constant Lasix drip to encourage him to dry out. The surgeon said she would wait until he was a raisin, the nurses all said that she likes her patients to be "blowing dust" before she'll operate while on ECMO. So since Friday he has been dumping fluid like crazy to get ready to have the surgery.

All of that being said, I think it is time for a little more information on the problems Joseph is facing. With the help of one of our letterboxing friends, Wisconsin Hiker, I finally found a good site to describe a Congenital Diaphragmatic Hernia (CDH).

http://www.pedisurg.com/PtEduc/Congenital_Diaphragmatic_Hernia.htm

Here are the pictures from that site:

This is what Joseph's insides look like.

This is what they should look like.

Tomorrow the doctor will make an incision on his left side, just below the rib cage and begin to pull the organs back down to where they belong. After everything is back in place, she will repair the hole in one of three ways. If the hole is small enough, she'll just sew it shut. If the hole is larger, she will use a material (whose name I forget) to patch the hole. If this material, which is kind of floppy, is too difficult to work with in Joseph's situation, she will use Gortex, that's right, Gortex. (And you thought it was just for jackets.) After the operation, Joseph will still be on ECMO for about 3 more days. We do have to watch for bleeding because we are doing the surgery while loaded up on Heparin. Our surgeon is very confident that things will go well.

You have heard me talk about Joseph's heart condition. The presence of this heart condition is what is complicating the issue. The name of this condition is Pink Tetralogy of Fallot. The cardiologists describe this as kind of "eh" and say that if you're going to have a heart condition, this is the one to have. You might be asking if this is no big deal, why is it an issue? This is best answered with this description, if you have one small problem it's no big deal, but if you have two small problems, it becomes a greater issue. With the "Tet" and CDH we have two small problems that are a greater issue. The heart condition is difficult to describe, except to say that it involves two holes in the heart, an obstructed flow out of the heart, and a strangely positioned aorta. A much more in depth description can be found here:

http://www.nhlbi.nih.gov/health/dci/Diseases/tof/tof_what.html

Here are some pictures illustrating this problem.

I hope that this helps describe the issues that Joseph is facing. He has fantastic doctors who are VERY good at what they do, not to mentioned some very dedicated nurses & ECMO techs. We are going to spend the night trying to rest up for tomorrow, provided that Emily will let us. We hope to report good news tomorrow afternoon.