Gettysburg Medical News
The Clinical View
by P.E. Hoffsten, M.D.
6 February 2008

WHAT IS BILE?

             A lady recently came to the clinic to establish new physician relationship and renew her medications.  She said that she been diagnosed with a condition called “primary biliary cirrhosis” ten years ago and had been using a medication called ursodiol to treat the condition all this time.  She said she had been miserable prior to starting the medication and had been told by her previous physicians that her symptoms occurred because she had accumulated “bile” in her system.  The medication was designed to help her get rid of the bile.  She said that even though she had had this condition ten years, no one had ever told her what bile was.

            In the basic economy of the body, everything that goes into the body must come out some place. The two major systems for elimination of waste products from our bodies involve urine and stool.  The urine takes care of waste products that are water soluble such as salt, urea, potassium, calcium, etc., etc.  But there are many waste products that are only oil soluble and these also need to be eliminated from our body.  As everyone knows, oil and water don’t mix so there needs to be a way to eliminate oil soluble waste products.  This is what bile is for.  In addition, bile helps digest our food.

            Bile is made in the liver.  The liver takes up waste products from the blood and converts them into tiny oil droplets that are initially stored in the gallbladder and eventually emptied into the intestine to be excreted in the stool.  As long as this process is carried out in an orderly fashion, the person has no symptoms. 

            But what happens when bile can’t be excreted into the stool and the waste products accumulate in the body?  This results in a condition called “jaundice” and the person turns a pumpkin yellow color.  The symptom that most bothers most people with jaundice is itch.  Exactly how or why bile accumulation causes itch is unknown but the symptom can be horrible. 

            The itch is what brought this lady originally to medical attention.  She was 51 years old at the time and noted that she was having increasing problems with itch virtually every place on her body.  When she finally got to see a doctor in California where she lived, there were scratch marks any place that her fingernails could reach.  The doctor quickly made the right diagnosis of jaundice and then began to look for the primary cause of the jaundice.  Perhaps the most common cause is gallstones that block the major duct that empties bile into the intestine.  When this duct is blocked, bile backs up in the body and symptoms such as the patient had occur.  But usually gallstones hurt and this lady had no pain at that time.  Other things that can result in this kind of problem include alcohol excess, but she was not a drinker.  Some patients develop a condition called viral hepatitis that can result in liver disease and jaundice but when the tests were done, she didn’t have this kind of problem.  To make a long story short, eventually, a liver biopsy was done in which a needle is directed into the liver and a piece of liver tissue is taken out to examine.  When that was done, the diagnosis of primary biliary cirrhosis was made.

            The lady said she wasn’t real sure just what primary biliary cirrhosis was or why she had it.  It turns out that this is a condition with strong hereditary factors in which the person becomes “allergic” to their own bile ducts.  These are very tiny ducts that carry the bile away from the liver cells to bigger and bigger ducts and eventually in the “common bile” that empties into the intestine.  It is often said the truth is stranger than fiction; in the case of primary biliary cirrhosis that is certainly true.  For some reason, patient’s with this condition, become allergic to these tiny bile ducts that carry bile away from the liver cells toward the intestine.  Why this occurs, no one really knows.  But one can think of it as having a reaction very much like poison ivy on the inside.  The bile ducts become swollen and inflamed and can’t pass the bile out of the body.  The condition is slow and progressive and can result in end stage liver failure in a few short years.  Most commonly, the first symptom that a person has is what occurred in this lady.  The person begins to itch and then may notice they are slightly jaundiced with a yellow color to their eyes.  Diagnostic procedures as described above eventually lead to a diagnosis.

            Very fortunately, there is an effective treatment for this condition.  Ursodiol slows the destructive process and in some people arrests it all together.  A few people with this condition will develop end stage failure within 3-5 years.  Others such as this lady have no symptoms and no apparent progression of their condition for years if they are treated with the medication called ursodiol.  Somehow, this medication taken twice a day blocks the allergic process in the bile ducts, clears their inflammation and the bile ducts continue to function normally.  This doesn’t work for everybody but fortunately for the majority of patients with primary biliary cirrhosis this is a very effective treatment.

            Sometimes patients with this condition are treated with cortisone to decrease the inflammation in their bile ducts until the ursodiol can have an effect.  Sometimes, the cortisone needs to be taken for a long period of time and can result in all the complications that come from long-term cortisone treatment.  Most prominent among these is bone loss, osteoporosis, and broken hips, or broken backs, or broken wrists.  Guarding against this complication is an important part of long term care of those with primary biliary cirrhosis.

            The healthcare professionals at your local clinics can’t make the diagnosis of primary biliary cirrhosis but they can make the diagnosis of jaundice very easily and get started on the diagnostic steps that will eventually lead to this diagnosis if it is correct.  Primary biliary cirrhosis is a very rare condition and most patients with jaundice will simply have gallstones.  But even in our small central South Dakota clinics, rare diseases as a group happen commonly.

This and other articles available at www.macpierre.com.