Im 1 in 100,000

Yesterday was Rare Disease Day. Typical me to be a whole day behind. But for the record, I was so exhausted yesterday I was in bed by 9:30!!

In 2008 I was diagnosed with Budd-Chiari Syndrome. I know what your thinking. Budd Chi…what? That’s the usual reaction I get. Followed by a "what the heck is that?" and a really curious look on peoples faces.

Budd-Chiari Syndrome is a liver disease that affects 1 in 100,000 people. BCS is an obstruction of the blood flow that leads out of the liver to the heart. In my case and in most cases that is a blood clot. The blood accumulates in the liver causing it to become congested and damages the liver cells. Blood has also back up in to my spleen causing it to be enlarged too!

Symptoms of BCS are usually pain in the upper right abdomen, Enlarged liver and spleen and fluid build up in the abdomen (ascites). For me most days the pain isn’t to bad. More like a dull ache. There are days where I feel like I am being stabbed repeatedly though. Mostly when I am not mindful of what I eat. My liver is very enlarged. Last I heard, I spanned from one side all the way to the other side. My GI calls it “impressive” and not in a good way either. And my spleen is enlarged as well. As far as ascites goes, I have it mostly under control. In other words as long as I eat what I am allowed and stay away from the super salty foods, I am ok. I have been warned that eventually I might have to take water pills or if it gets really bad have the fluid drained from my stomach. Doesn’t sound like fun, so I am trying to avoid it all together.

One of the complications of liver disease that has hit the hardest is called Hepatic Encephalopathy. When the liver is damaged it doesn’t filter properly. All the toxins that are usually flushed out are instead able to continue to my brain. This causes me to be confused, easily distracted, exhausted and sometimes I slur. On really bad days I have lost time, forgotten where I was(in the middle of the store) and have had really bad episodes where I felt like I had one to many drinks. Because of this I am unable to drive or work. 

The only treatment for me is to have a liver transplant. And in my situation, I am waiting for a cadaver liver. There is no living transplant for me. I was listed with UNOS on May 25th 2010.