Cholangiocarcinoma 101 & Our Screwed Up Medical System

It has been a while since I have posted about the "basics" of cholangiocarcinoma (lets call it CC for short) and I thought that today would be a good day to review just exactly what CC is all about.

Cholangiocarcinoma (or bile duct cancer) is a cancerous (malignant) growth that originates in one of the ducts that carries bile from your liver to your small intestine.  CC is a relatively rare disease that only one out of every 100,000 people will ever get diagnosed with.  It can occur anywhere along the bile ducts and since the bile ducts are located within the liver it often spreads (matastasizes) into the liver and other organs in the body.  CC can be further categorized into three categories, extrahepatic (or perihilar or hilar), intrahepatic, and distal extrahepatic.  The most common form of CC is extrahepatic or Perihilar/hilar and originate in the hilum, or the place where the hepatic ducts have joined and are leaving the liver.  Another form of CC is distal bile duct cancer which actually forms outside the liver closer to the small intestine and is included in the "extrahepatic" category.  The least common form of CC is "intrahepatic" which develops in the smaller duct branches inside the liver.  Only 1 out of every 10 bile duct cancers are intrahepatic making it the most rare form of cholangiocarcinoma.  Imagine my surprise to find out that I have intrahepatic CC!  Not only do I have a rare disease, but I have the rarest of forms of this rare disease as well, lucky me.

The symptoms of CC can vary depending upon where the tumor is located within in the bile ducts.  If the tumor is hilar the patient usually has symptoms of jaundice, itching, abdominal pain, progressive weakness and weight loss.  If the tumor is distal there is often no abdominal pain but there is jaundice and itching (pruritus).  And finally if the tumor is intrahepatic there is often no jaundice present but there is itching (pruritus), slight to severe abdominal pain, unexplained weight loss, progressive weakness, fatigue and loss of appetite.

Before I was diagnosed with intrahepatic cholangiocarcinoma I had all of these symptoms except for the jaundice.  The back of my head itched like crazy! I remember thinking that "I don't have evidence of dandruff so why does my head itch like hell?"  I used apple cider vinegar every day to try to get the itching to stop but it wasn't working.  I had severe abdominal pain and extremely poor bathroom habits.  My right shoulder hurt all the time ( I found out later that this is where the liver often expresses its pain since there are no nerves to express pain in the liver) and I was losing weight (although at the time I thought this was a good thing).  I definitely lost my appetite, although at the time I didn't attribute that to anything other than I felt crappy whenever I ate (gassy, bloated, nauseous, etc.)  I often vomited in the mornings prior to my diagnosis and it was usually bright yellowish green and slimy (I'm guessing this was bile) and tasted horrible.  I had a HUGE hard mass right in the middle of my abdomen at the place where the ribs meet just below the sternum.  It felt like hard lumpy cottage cheese and protruded out from my abdomen.

The real problem with all of this is that I went to the emergency room at Swedish Hospital in Ballard (a neighborhood in Seattle) with these complaints and severe abdominal pain.  They ordered an ultrasound thinking I had gallstones.  After spending nearly an hour looking at my insides they determined that I was just fine and sent me home with orders to see my primary suggesting that I get yet another (i had already had three) endoscopy.  Knowing that another endoscopy was pointless, and that yet another doctors visit with the same set of problems was going to get me nothing except for more prilosec, I chose to ignore making an appointment with my primary.  Fast forward 3 months...I was in my primary's office for another reason and asked her to feel this giant lump in my abdomen, telling her that it hurt like hell and that it made it hard to breathe (like a knife in my stomach if I took a deep breath).  She felt it, or in medical terms, "palpitated" it and told me these exact words, "I see nothing remarkable, just take this prilosec for a month and let me know if it gets worse."  She was positive it was a hernia even though I explained that I had a lower back injury and that there was no way I could have gotten a hernia because I NEVER lift anything heavy.  This was at the 45th Street Clinic in Seattle.  My suggestion...DON'T GO THERE! or to Swedish either for that matter.

This is the problem with Cholangiocarcinoma, it is hard to diagnose.  Many of the symptoms fall into the "digestive disorder" category, especially if you are unfortunate enough to have the "intrahepatic" kind which doesn't usually present with jaundice (an indicator that something is wrong in the liver).  By the time one is diagnosed with CC it has already done extensive damage to the liver and has grown aggressively.

I'M NOT BUYING IT!

Why?  Because if I can look up these symptoms on the internet and find that "giant lump in abdomen", "itching or pruritus", "abdominal pain", "weakness and fatigue" "vomiting smelly slimy yellow stuff" and "weight loss and appetite loss" are all symptoms of cholangiocarcinoma SHOULDN'T A TRAINED MEDICAL PHYSICIAN KNOW THE DIFFERENCE  between a rare incurable cancer and a hernia!!!???

If this disease is rare and incurable then why aren't doctors trying harder to connect the dots with unexplained symptoms such as those above and CC?  Why?

Why is is that the medical professionals cannot even tell the difference between IBS (irritable bowel syndrome, which is what they said I had for more than 10 years) and Cholangiocarcinoma?!  Is it that they don't care?  Is it because insurance companies are willing to pay for endoscopies and colonoscopies but are not willing to fork over $800 for a CT scan with contrast?  Why do doctors just run the same old tests on you with the same results for years on end, wasting money and time and getting nowhere when they could just LISTEN to what you are telling them and think outside of the box for just one second to help their patients find the cause of their symptoms?

Cholangiocarcinoma is a relatively slow growing cancer in comparison to other cancers out there.  I am certain that all those years I complained about digestive disorders and abdominal pain I had CC growing inside of my liver.  Instead of ever ordering a simple liver panel or CT scan I got more pills, I got told that it "was all in my head", and that I must have a hernia.  I got endoscopies and colonoscopies and countless prescriptions to cover up my symptoms, but never a simple blood test to check my liver functions.

What do I take from all of this?  Let me give you some very important advice my friends.  This advice can quite possibly save your life.

BE AN ADVOCATE FOR YOUR OWN HEALTH


No one knows your body better than you do.  If you feel that something is not right, be persistent and question your doctor if they are not helping you find the reason you feel bad.  If they don't help you FIRE THEM and find another doctor who will help you.  The doctors do not always know what is best for you, some of them don't even care.  You pay good money (and a lot of it) for healthcare and you deserve better than a lame excuse for whatever you feel is wrong and the "brush off" of yet another prescription to mask your symptoms.

Those of us diagnosed with Cholangiocarcinoma know just how screwed up this all is.  Many of us have gone through similar experiences with doctors overlooking our symptoms and writing it off as other disorders.  The survival rate of those with CC isn't entirely uplifting...those diagnosed with extrahepatic CC have a 5 year survival rate twice that of those diagnosed with intrahepatic CC, but even then the rate of survival past 5 years is at best 30%.  Of course this is just all based on statistics.  I am of the opinion that I make my own statistics, just as you make your own statistics.  No one can tell us how long we have on this earth.  My point in bringing up survival rates is that CC is more often than not a certain death sentence.  It just seems to me that with the odds of survival being so slim and the increasing diagnosis of CC in young adults being on the rise, the medical community and society in general should be spending a little more time and attention on this killer of a disease for which there is no cure.  That is just my opinion.

Sites referenced:

http://www.cancer.org/acs/groups/cid/documents/webcontent/003084-pdf.pdf

http://www.hopkins-gi.org/GDL_Disease.aspx?CurrentUDV=31&GDL_Disease_ID=A6D10E80-887D-49A7-B3BB-0517D38CE757&GDL_DC_ID=320F4EDD-0021-4952-83D7-8B0C67B47BFF