Progression

It's been a few weeks since I've given an update, so here's how the past few weeks have been going:

-More bloodwork, bilirubin up to 15 and clotting factor looking worse
-Increase Ursodiol dosage (liver medication), increase ADEKs dosage (vitamins) and start on an additional vitamin K supplement
-Lincoln goes on a hunger strike, not sure if it's related to the increase in medication dosage, but we suspect it is
-Kidney surgery moved up to Feb. 17
-Another liver ultrasound, blood flow to the liver is good
-Somehow Lincoln's growth is still on track, he is so amazing!

That gets us up to this past week.  On Friday we had an echocardiogram, EKG and consultation with the cardiologist. They had originally wanted to sedate Lincoln for the tests, which we were against, so they agreed to let us try it without sedation. And it worked, phew!  He wasn't exactly cooperative the entire time, but he wasn't terrible.

The original plan had been for me to head back to work after the echo and have John and the cardiologist call me so I could hear the update.  I was getting ready to head out when the cardiology fellow came in, told us they were looking at Lincoln's echo, and asked me to stay.  Never a good sign.

Lincoln has a piece of tissue growing beneath his aortic valve. They call it a subaortic membrane. We knew it was there and were hoping for no progression. Unfortunately, it progessed from mild/moderate to severe over the past two months. The doctor is concerned at the quick progression and wants to schedule surgery to remove the membrane, plus remove a little extra in hopes of getting rid of any faulty cells that like to grow there. While they're doing that, they'd also look at his valves and correct or replace any that have been damaged. The heart team has a weekly meeting on Tuesdays to discuss all the cases and we'll know for sure after that meeting, but the cardiologist told us to expect the kidney surgery to be postponed and heart surgery scheduled in the next month.

While we were at the hospital we also stopped into the GI clinic to have Lincoln's doctor take a look at his belly. The night before it had looked a little bigger than it normally does. The doctor told us to head down to get bloodwork and come back in a few hours for another liver ultrasound (Lincoln can't eat for four hours before the ultrasound so we had to wait). The bloodwork showed Lincoln's direct bilirubin up to 26 and that his clotting had worsened a little. So he got a shot of vitamin K to help with the clotting, still nothing we can do about the worsening liver function.

The liver ultrasound also showed that there is decreased blood flow to the liver. This isn't good, but there is a bright side. The liver folks think it's possible that the heart is putting stress on the liver and that could be the reason for the sudden worsening of liver function. It makes sense - both the heart and liver declined quickly over the past couple of months. It stands to reason that the two are related. The hope is that once the heart is corrected that stress will be taken off the liver and we'll be back to something reasonable. It wouldn't fix the liver, Lincoln still doesn't have enough bile ducts, but it could help.

John, Lincoln and myself are staying strong and positive. It's not easy, and we have our moments,  but being sad and feeling sorry for ourselves doesn't get us anywhere. We ask that you continue to send Lincoln your prayers and/or good thoughts. We also ask that when you think of Lincoln please keep a positive outlook on your end as well. Feeling sad for Lincoln doesn't help him, but positive thoughts and confidence in him and his team of doctors does.