Scimitar Syndrome

I finally got a call today with the results of Caden's MRI. We have been waiting for these for about 2 ½ weeks now. It turns out that Caden has “Scimitar Syndrome.” This is a well-known but very rare condition where the right pulmonary vein drains into the right atrium instead of the left. It is also commonly associated with hypoplastic (small) right lung which Caden has. In addition, his right inferior pulmonary vein drains into the inferior vena cava. And from what the surgeon tells me, this combination of defects makes things very complicated.

Normal pulmonary vein circulation carries oxygenated blood from the right lung to the left side of the heart. But in case of the Scimitar Syndrome, an anomalous vein connects between the pulmonary venous circulation and systemic venous circulation which creates a left-to-right shunt. Scimitar syndrome is a rare congenital anomaly in which a part of the right pulmonary venous blood return to the inferior vena cava. When seen in an X-ray the anomalous vein resembles the shape of a “scimitar” sword and hence the name scimitar syndrome.

I am unsure as to the statistical occurrence of this syndrome, but I have heard 1 in 10,000, and 1 in 100,000. Either way it is not very common.

At this point, the surgeons feel it is necessary for Caden to have some more studies done prior to surgery. At this point, that means a perfusion study and a heart catheterization. The perfusion study is non-invasive but the heart cath is not. Right now the thought is to do these studies in the 2-3 days immediately preceding surgery.

I asked the surgeon how the repair would be done and he said they can either detach both of the pulmonary veins and re-attach them in the correct places. Or, there is another option of creating a baffle (tunnel) through the atrium wall by making a hole (creating an ASD) thereby directing blood into the left atrium. He said the decision of which option they choose is made in the operating room.

We were talking about having surgery at the end of March at Sutter Hospital in Sacramento. However, based on the new level of complexity, we aren't sure that is the right fit for Caden anymore. We may go back to Stanford or possibly somewhere else depending on what is best for him. We have full confidence in the surgeon at Sutter, but if we have surgery at Stanford, we are hoping he will participate in surgery there as well. We are just thinking since this is so rare, we should consider being at a facility that is used to dealing with more complex cases.

As I write this blog entry, I am taken back to the feelings of uncertainty and being overwhelmed that we felt before Caden was born. Even the first two weeks after he was born while we waited for him to have his heart/diaphragm surgery. It seemed that every day, or multiple times/day, the information we received changed. Every doctor we spoke to had a different opinion and it seemed impossible to get everyone together to talk about it at one time. It was very stressful as we were just trying to keep our heads above water as we struggled to hold it together emotionally.

Now that we are considering having surgery at Stanford, this brings back another rush of emotions. It brought back a lot of difficult memories just in the short time we were there in December for Caden to have an echo and MRI. And now to be discussing having surgery there brings back so many other bad memories. Caden coded and had to be brought back to life when he was just 2 ½ weeks old. That alone makes it hard for me to want to spend any time in the hospital. Thinking about living in the Ronald McDonald House for three months when Caden was born reminds me how much he struggled to survive.

It is so discouraging to come from a place of feeling like we were over the worst of things. That Caden was doing so well and had come so far. Only to feel like we are going backwards. In many ways this surgery will be much more difficult for us to deal with than when he was a baby. He is older and has more opinions about everything. He will be much more likely to remember this one and the pain he experienced. For these reasons alone (and there are many more reasons), this surgery is going to be very hard on our whole family.

I have been working on some ideas of how to prepare him for surgery. But for right now, we are choosing not to tell him or Kyler about because we don't want either one of them stressing about it. We want to wait until things are more concrete in terms of surgery date. And we want to get much closer to the actual date. So please don't mention it to either of our boys if you talk to them. And if you ask us questions about it when the kids are with us, don't be surprised if we don't answer you or change the subject. We just don't want them to worry about things before we have more details in place and before it's absolutely necessary.

Our “Diary of Hope” continues. We continue to put our hope and trust in the Lord Jesus Christ. He has sustained us throughout our journey so far and we know he will continue to provide for us no matter what happens. Thank you so much for your love and prayers! You have no idea how much it means to us to know that we have people around the world lifting us up in prayer. Thank you!