Primary Schlerosing Cholangitis

[theresajoyce]

I am a 44 year old Black female. On Mar 1, 2002 I went into the hospital due to bile in the urine;After two ERCP attempts,and then a big colangiogram thing thru my liver, a liver biopsy, etc. they were still unsuccessful in getting the bile ducts open. There was an obstruction in the center of the right/left/center bile ducts.  After not being able to get to the bile ducts, the doctors decided to remove my gall bladder and put a line to guide to the bile ducts. After this was done they were able to get to the bile ducts and open them up by ballooning. After 15 days I was sent home with a diagnosis of Primary Schlerosing Cholangitis (PSC), a terminal liver disease, unless you have a liver transplant, well within a week the bile ducts shut down again and I was back in the hosp. This time they put stents in the bile ducts and I was sent to the Mayo Clinic. They did not agree with the conclusion of PSC. I was not happy with the Mayo Clinic as they concluded scar tissue due to the gall bladder removal, however, we thought that is impossible as all my symptoms were there prior to the removal of the gall bladder.  So my real doctor, whom I greatly respect, decided to do what is called stent therapy.  After having an ERCP to remove and replace stents every month for six months he said I should be okay. However, since October, date of last surgery, I have been having constant liver pain as well as swelling and nausea.  My urine isnt indicating bile in my system so I am not sure what is continuing to go on here.  The doctor says I shouldnt be having pain and because he cant see the swelling probably thinks I am crazy.  However, I can feel the swelling, as it pushes the liver up against my rib cage and causes great discomfort.  My blood test appear to be normal. My weight however is increasing even if I dont eat much.  I do realize my metabolism has slowed down since the gall bladder was removed but this is ridiculous.  I also have moments of extreme weakness.  Because my liver doesnt function properly I have high cholesterol and intermittent high blood pressure. If I take the medication for high blood pressure then my blood pressure gets too low.  And at this point they arent sure I have PSC.  Sometimes the pain in my liver is if this were the heart side, I would swear I was having a heart attack.  I do not have hepatitis, I have never been drunk in my life and I have never done drugs. I do notice an increase in the swelling during my menstrual cycle.  If there is any advice you can give me I would appreciate it. I do have medicine for nausea Phenerghan and that is the extent of it. Just looking for answers, any advice would be appreciated.

Theresa

[Kevin Pho, MD]

Hello - thanks for asking your question.  

You are having constant liver pain, weight gain, swelling and nausea.  There is a questionable diagnosis of PSC.  A liver biopsy and ERCP with stent placement was performed.  You have had a cholecystectomy without improvement of symptoms.  A referral to the Mayo Clinic was not revealing.  

I will give some possibilities, but if your personal physician and the Mayo Clinic are not able to come up with definite answers you can surely appreciate that it would be difficult to come up with ideas that have not already been thought of.  

The most obvious possibility would be PSC.  Right upper quadrant pain occurs in 10 to 15 percent of patients.  The most useful test would be cholangiography (which you have already have).  Magnetic resonance cholangiography may also be an option.  

Unfortunately there is no treatment that slows the progression of the disease.  Liver transplatation is the definitive treatment.

To treat the biliary strictures, stenting is performed (as in your case).  Studies show up to 77 percent of patients have improvement in symptoms.  A rarely used option would be surgical dilation or choledochojejunostomy.  Surgery carries an increased risk of postoperative infection and increases scarring.  

A variety of immunosuppressive and antiinflammatory agents have been studied in patients with PSC, including:
D-penicillamine
Steroids
Cyclosporine
Methotrexate
Azathioprine and 6-mercaptopurine
Ursodeoxycholic acid
Tacrolimus

The results vary for each agent - you may want to discuss these options with your gastroenterologist.

Unfortunately liver transplantation is the only therapy that is shown to improve survival in PSC.  

One other consideration would be Sphincter of Oddi dysfunction (SOD).  It typically causes biliary pain and may be present after cholecystectomy.  It may be related to unmasking of preexisting SOD due to removal of the gallbladder, which may have served as a reservoir to accommodate increased pressure in the biliary system occurring during sphincter spasm.  This condition can be diagnosed using Sphincer of Oddi manometry during an ERCP - you may want to discuss this with your personal physician.

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Thanks,
Kevin, M.D.

[theresajoyce]

Additional info: I have had every scan you can think of..I had several cct scans, hida scan, some pancreatic cholangiogram, liver biopsy, my bilirubin count stayed above 6. for over two weeks....it went down to two during my initial release from the hospital, but back up to six within 4 days. At this point I would like release from the liver swelling, nausea and pain...

[Wools]

theresajoyce: It sometimes requires alot of testing before a diagnosis can be made...My son(age 22) was told he had PSC after bouts of itching, dark urine, pale stool, 8.5 billirubin, pain in the side above the liver.  MRI and a CT scan showed a growth at the base of the liver and scaring in the ducts throughout the liver. After recovering 3 months of pancreatitis (due to the ERCP) he had a stint placed in the main duct and put on ursodeoxycholoric acid (Actigall). This was just a temporary fix but he felt better. All of the initial systems got better except the itching. It was a constant battle keeping the stint open. He was also told to eat a low fat diet. His
liver numbers were all high during this time, especially the
Al Phos.  He was fortunate to receive a new liver within a year of getting sick. He now works part time attends college and his health is wonderful.  We have learned alot about PSC over the past few years. If we can be of any help, please let us know.
There is a wonderful hepatologist at Shands Hospital in Gainesville, Fl if you need one.

[Patrick J.]

I thought I had PSC (I still don't know). I was told I have Fatty Liver, diagnosed by CT scan. I have a past history of UC that was cured 30 yrs ago, & gallbladder removal 15 yrs ago. I get intermittent itching all over & RUQ tightness, like a finger is pushing on the area (adhesions maybe?) But my labs are all good. Alk Phos - 54, ALT - 41, AST - 26, T Bil - 1.0, ALB - 5.0, GGT - 22, PT - 10.6. Sometimes I get a RUQ ache. I researched my labs back 10 yrs & they were always good. Any ideas from anyone? Does Fatty Liver cause itching??

[seven7]

I am researching Pancreas Cancer on this site, but most of the questions concern the liver.   During my reading about Pancreas cancer in physician's books,  I read that  presistant itching can be present in some types of pancreas cancer.

[theresajoyce]

Dear C Wools

I appreciate your comments and it is always good to have insight from other people who have experienced the same thing.  My doctor has put in stents to keep my bile ducts open and I was given the same medicine as your son, Actigall, which they will most likely put me back on.  They did the stents every month or so for six months to remold the bile ducts.  My last surgery was Oct 2002, however now I believe my ducts are closed again as my symptoms are consistently back.  They have ordered retesting again and I was wondering if you would mind sending me your email so I may ask qestions from time to time.  I am not sure why I am not yet on the liver transplant list.

Theresajoyce
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[reader99]

There is useful diet and lifestyle information about all types of liver trouble at www.liverdoctor.com.  She talks a lot about weight loss, because that's what catches peoples' attention, but her real interest is good health.  Dr. Sandra Cabot's book "The Liver Cleansing Diet" has helped me with Fatty Live and Gall Bladder pain.