A functioning liver

For the first 19 years and 6 months, we didn't know that Angela's liver wasn't able to function properly. We spent 11 years treating crazy wild mood swings, *severe aggression*, seizures, grogginess, a stroke and eventually two comas before figuring out that she had an Abernethy type II malformation.

A normal ammonia level is 15-45 mcg/dl. When we first discovered the hyperammonemia, and they thought she had a Urea Cycle Disorder, our goal was to keep her ammonia levels under 50. With heavy scavenger drugs it was a struggle, but we could do it. Then suddenly in January she started spiking again, and not responding to the rescue drug Ammunol. A very intense and dangerous drug, with one dose being a 24hr IV drip in the intensive care unit, only she needed two back to back doses then spiked again. That's when they finally moved to additional testing and found she had the portal liver shunt.  The old goal was tossed out the window. Now it didn't matter how high her ammonia was, they told us not to bring her back unless she was having seizures or fell into a coma again. Really, not very comforting, but they needed time to discuss with other doctors around the country an come up with a fix for this. She was discharged from the hospital with her ammonia at 175. 

When we set a date for surgery, her medical team had come up with a plan, something that had never been attempted before. Over the course of 3-4 surgeries the would use a graft vein from her groin and gradually close the shunt, a little more with each surgery, allowing her liver time to adjust to this new blood flow.

This morning her doctor came in and explained they had change of plans, and yesterday met to discuss this new idea. It is what they did today. Without going into the long explanation, they used the graft vein to make a smaller shunt, small enough that with her next surgery it can be occluded in the cath lab without having to make another huge incision in her belly. They also found a bunch of tiny shunts that went "every which way" into and out of her liver, and were able to just clamp them without problems. 

What does all this mean? It means that Angela's liver now has adequate blood flow and should be able to cleans her blood without ANY of the nasty drugs she's been taking for the last few months. It means she gets her independence back. It **MAY** mean she will no longer need her feeding tube because her swallow is so greatly affected by the ammonia and that will no longer be an issue. So this afternoon, three hours out of surgery, her ammonia levels were checked. Her liver ROCKS!!!! Her ammonia is at 28!!!!!! That is without ANY SCAVENGER DRUGS!!!

Just the thought of all this, of the potential for her life, of this new young lady we have seen emerge from the ammonia fog....all of it...has me sitting here sobbing tears as I type. This is a true miracle for Angela. For us. 

Pain management has been an issue this afternoon. Now that I've updated everyone here, I'm going to take a nap while she has a few minutes resting quietly in the Intensive Care Unit.  I cannot thank you all enough for your love, support and prayers!!!!!