Thursday, September 4, 2008

2008 - February

February 4th
We had our follow up appointment with Dr. K. He had reviewed the liver biopsy with another specialist at Long Beach Memorial Hospital. They came to the conclusion of the diagnosis of Primary Sclerosing Cholangitis (PSC). PSC is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts that channel bile from the liver into the intestines. Here is the description from the Mayo Clinic.

Primary sclerosing cholangitis is a disease in which the ducts inside and outside the liver are narrowed due to inflammation. In the disease's early stages, the bile ducts (bile is a liquid that helps break down fat in food) and the tissues around them become inflamed. With the spread of inflammation, the bile ducts are destroyed and scar tissue is created. Continued inflammation and scar tissue buildup can lead to cirrhosis, where scar tissue distorts the liver, disrupts blood flow through the liver and interferes with its normal function.

The cause of PSC is unknown, but it is thought to be an immune response in which the body attacks the cells lining the bile ducts of the liver. This response may be due to exposure to something in the environment, a virus or bacteria, or a person's genetic makeup.

The course of PSC is unpredictable, but in most people it progresses slowly. Although PSC can be life-threatening, many people with the disease live active, productive lives.

Dr. K prescribed Ursodiol (Urso) which should help to slow the progression. A liver transplant may be in the future, but hopefully not for 10-15 years (at least). Dr. K orders an MRI (or MRCP) and a bone density test. I also have more blood work done to check my vitamin levels as this disease can cause deficits in A, D, and K. I will see him every three months.

February 7th
MRCP (Magnetic Resonance CholangioPancreatography). It’s basically the same thing as an MRI except no dye is necessary and it focuses on the biliary tract. I’ve never had anything like this done before and it makes me think of eighties movies that were futuristic where everyone and everything is in white, with bright lights. But I lay on a table, get strapped in, wrapped in sheets and the table moves into the tube.

Now, when the technician took me back to change my clothes in preparation, she kept talking to Jim thinking he was “Shawn” (even though I was wearing the medical bracelet). They told him to remove everything from the waste up and put the gown on. I asked, “What about my bra?” (wondering about the metal clasps). Response, “No, that’s okay.” She also made no mention about jewelry at all. But I had heard that you shouldn’t have any metal, so I took all my jewelry off. When I went into the room, they kept checking me and my name (still expecting a boy).

So as the table is moving into the big magnetic tube, Jim pipes up and says “She still has her bra on…?” The two technicians freak out, pull me back out and have me take it off. I have horrible images of little pieces of metal ripping through my body. AAAHHHH!!! J So what did that first tech think she was answering when I asked the question?

The test itself went fine. I didn’t get claustrophobic, but I can see why people do. Jim and I were worried that I would have an itching fit if I had to lie still for too long of a time, but no itchies bothered me. It was pretty loud in the tube and I had earphones on that the techs could talk to me through. The whole thing was maybe 10 minutes.

A few weeks later I received a survey asking how my experience was. I made sure they knew that at no time did anyone inform me to remove all metal. It was only luck that Jim and I knew about it.

Dr. K called a week or so later with the MRCP results which just were a confirmation of the PSC diagnosis. There are signs of scarring in the bile ducts, but doesn’t sound like it is too bad or serious at this point. Definitely in Stage 1 (of 4).

Adjusting to medication was a bit tough. I was pretty tired, lethargic, and blah. But about a month later I was feeling better.

No comments: