Blogging about life in Minnesota, raising our six kids with Down syndrome while battling Breast Cancer.

Be the kind of woman that when your feet hit the floor in the morning the devil says, "Oh shit! She's up!"

Sunday, January 24, 2016

More but not enough information

Words for today:

Portal Hypertension - Increase in blood pressure in the veins within the liver, usually caused by a blockage.

Portal Thrombosis - Blockage of the portal vein, usually by blood clot.

Revascularization- Where the blood supply inside Angela's liver is not working correctly, she has revascularized into a "multitude" of new vessels. Some of you have known her long enough to remember she also has a revascularized area in her brain that for many years they classified as MoyaMoya Disease. After many more studies it was found the circle of Willis on the right side of her brain was not fully formed, and the left side had crossed over the mid-brain and had taken over the job. When a person has an area of revasularization anywhere in the body its possible they can have other areas. 

Focal Nodular Hyperplasia - benign tumors of the liver. They do not usually grown and it is unknown if they are related to all the other things wrong with her liver, or if they are just an incidental finding. Angela has "multiple" area of FNH, ranging in size from 1 - 1 1/2 cm.

Now take all those words, put them into Angela's liver, add a little "We don't know enough yet" and "You may need to go down to Mayo instead" and you have a picture of Angela's liver.

So Angela has portal thrombosis, with a multitude of revascularized areas where the body is creating new pathways for the blood. Because of all this narrowing and new, tiny vessels, she has developed Portal Hypertension. The interesting, and good part, is that her Vitamin K (which is needed by the liver to produce clotting factors) is low. If it had been at a good level all this time she likely would have thrown a major clot and suffered a catastrophic stroke. The other interesting thing is that Angela's liver should not be functioning well, but her liver function is actually just fine. Its just that she has blood bypassing the cleaning that needs to be done by the liver.

Still, we don't yet know if this is fixable. The radiologist who read the scans today felt they should be read by a specialist with more experience. That will happen tomorrow. The new radiologist will take all three scans (ultrasound, MRI and CT) compare image to image to come up with information we can use to make treatment decisions.

Unfortunately we may have surpassed the skills of our excellent doctors. The liver specialist at Childrens says this is likely beyond their range of expertise. This is a very rare, complicated problem Angela has, and we may need to move to a special liver center: either the University of MN or Mayo. The UofM is 20 minutes away, Mayo is 90 miles away. Tomorrow's report will give us a better idea where we're headed.

There were a few other scary terms tossed around today that I'm not even willing to discuss yet. We're not there. We need more information.

Angela is now on all the drugs she could possibly be on, at the max doses, and her ammonia is staying down to normal levels. However, she's not getting protein yet. Tonight and tomorrow we'll see if all these drugs can keep her system stable even with a protein load. Unfortunately none of the doctors seem very hopeful.

Today Angela was very chatty and perky. She's not walking around much but I'm sure tomorrow she will be. We're stopping the TPN tonight and adding in her formula with protein. That means disconnecting all but her gtube line. Please pray her system can keep up to the protein load. She is HUNGRY and is having a hard time with that. 


eliz said...

praying (((HUGS)))

Heather said...

Praying for Angela and you all during this scary time.