Monday, August 16, 2010

What Is Primary Biliary Cirrhosis (PBC)?

Thank you Craig... I always know where to go to find good information. I met a man today (who is doing GREAT post-transplant) that needed a new liver due to PBC, which of course I had to find out more information about as soon as possible... so here it is!

Primary Biliary Cirrhosis (PBC) is a chronic liver disease that slowly destroys the bile ducts within the liver (intrahepatic bile ducts). Liver inflammation over a period of years may cause scarring which leads to cirrhosis. PBC is NOT alcohol or drug related, and it is NOT contagious.

The name "Primary Biliary Cirrhosis" is somewhat deceiving since cirrhosis only occurs in the last stage of the disease (stage 4) after many years of inflammation. With early diagnosis and proper medications, most with PBC will never reach the cirrhosis stage of PBC.

PBC is also called "Chronic Nonsuppurative Destructive Cholangitis" and "Primary Autoimmune Cholangitis." However, these alternative terms are not widely accepted and therefore not in use.

Definition from New American Medical Dictionary:

Primary: First in order of development, most important, arising spontaneously.
Biliary: Relating to or affecting the bile duct system or bile.
Cirrhosis: An inflammatory disease of the liver associated with the replacement of liver cells by fibrous tissue. Passage of blood through the liver may eventually be obstructed by the cirrhosis.
The cause of PBC is still unknown, but it is not alcohol or drug induced. Current studies suggest it may involve autoimmunity, infection, or genetic predisposition, and does seem to appear more often in certain families. Women are affected 10 times more than men, and PBC is usually diagnosed in patients between the ages of 35 to 60 years.

Those with PBC usually look extremely healthy, and many are 10 to 30 pounds overweight. The slight bronze pigmentation of the skin is often present in the advanced stage of the disease, and makes the individual look tanned. The outward appearances doesn't tell the story of what is going on inside their bodies. Even on the transplant list stage, many with PBC look healthy. A person with PBC commonly hears comments such as "you look so healthy or you don't look sick."

Upon diagnosis, some doctors may suggest their patient start:

Start a reduced sodium diet and or low fat diet. Nutrition
In severely damaged livers, proteins may be restricted.
Drink plenty of water and other fluids such as juice.
Calcuim and Vitamin D. Calcium is the most common mineral in the body and is required for proper functioning of most organs. It is particularly needed in the normal development of the bones and teeth. Osteoporosis is a bone disease where calcium leaves the bones, causing them to weaken, and is commonly associated with PBC.
Avoid or lower intake of alcohol
Lower caffeine intake
Avoid undue stress
Exercise, if possible. Walking is the most common recommendation for exercise.
Stop smoking
The above suggestions have been found to be very helpful in liver disease, but are common sense considered part of healthy living. PBC Menus and meal plans by Norma J. Thiel, RD, Clinical Nutrition Manager Mountainview Hospital.

The number of patients being diagnosed at the asymptomatic stage has risen dramatically over the past few years due to widespread laboratory screening. Typically, the blood lab pattern reveals an elevated alkaline phosphatase level with a normal bilirubin. Bilirubin does not increase until final disease stage. There are four disease stages of PBC.



Medical tests used to confirm PBC:

A liver biopsy helps confirms the diagnosis, but is not a requirement.
Ultrasound exam may be performed to visualize the bile ducts to exclude an obstruction.
Blood lab tests that show liver dysfunction:
Liver function tests
Antimitochondrial antibodies. Positive AMA is found in about 95% of PBC patients.
Serum cholesterol and lipoproteins may be increased.
Haptoglobin & ACE levels may be altered
Diagnostic tests
PBC advances slowly over a period of years. Most patients lead normal lives for years without symptoms, depending on how early diagnosis is made. There is no cure for PBC, but patients are showing good results in slowing the disease progress with URSO 250 , Actigall and methotrexate. With the current medications, it is becoming more common for the PBC patient to live a long life without any complications from PBC, and in some cases actually lowering the disease stage from original diagnosis stage.

PBC is considered an autoimmune disease. Some diagnosed with PBC, may also be diagnosed with one or more other autoimmune diseases. Click for diseases and conditions associated with PBC.

Most patients remain without symptoms for many years, and some may never notice any symptoms. The initial symptoms vary among PBC patients, and the varying symptoms can sometimes make it difficult for doctors to actually diagnose PBC. The varying symptoms may be do to the individual, autoimmune nature or other diseases associated with PBC. Symptoms may be present in any combination and include any of the following:

Fatigue may be the first symptom the patient notices, causing him or her to visit their doctor. The fatigue associated with PBC appears to be totally different from any other sort of fatigue. In early stages, many patients have commented they could sleep for hours. While some in the later stages say sleeping becomes more difficult. At this time, there is little research into the cause and treatment of the liver disease fatigue. It is usually not due to depression, and some researchers believe it is an abnormality of the axis between the pituitary and the adrenal glands. Support and understanding from family members, friends and the doctor is very important, making it somewhat bearable.
Intense and unrelenting itching of the skin.
Gradual darkening (hyperpigmentation) or changes in skin texture, and various skin rashes.
Small yellow or white bumps (xanthomas) under the skin, or around the eyes.
Dry Eye Syndrome
Dry mouth, sometimes referred to as cotton mouth.
Thyroid problems

Arthritic aches and pains in bones, muscles and joints are common. In some, the pains can be severe and debilitating. Some even report severe pain just touching leg, feet and hip bones, but this is NOT common.

Over the years, as the PBC progresses, other symptoms may appear. These symptoms may include any of the following:

Osteoporosis or other metabolic bone disease. See osteoporosis exercises.
Enlarged abdomen from fluid accumulation.

Easy bruising or bleeding

Jaundice (yellowing of the skin and eyes)

Increased bilirubin

Internal bleeding in upper stomach and esophagus, that may be caused by varcies.

Hepatic encephalopathy causing personality changes: dulling of mental functions, neglect of personal appearance, forgetfulness and trouble concentrating, changes in sleeping habits, confusion, breath odor and muscle stiffness. Encephalopathy occurs in final stage of PBC.
Hypersplenism, enlarged spleen
Fever, nausea and vomiting
Reflux and stomach ulcers
Weight increase or decrease
Swelling of the hands, legs and ankles. See edema and ascites
Sexual problems (impotence in men, absence of periods in women, lack of desire.)
Trembling hands
Difficulty in sleeping and changes in sleeping habits. Some PBC patients have noted that itching intensifies when they lay down to sleep, and those who have liver pain say it is more severe in a sleeping position.
Hepatorenal syndrome, progressive deterioration of kidney function leading to kidney failure in a person with liver failure.
Hepatopulmonary syndrome, associated with difficulty with breathing.
Liver cancer (hepatocellular carcinoma)
Abdominal pain or pressure in the liver area. (Yes it's real)
As the PBC progresses, some patients require vitamin A, vitamin D, vitamin E and vitamin K replacement therapy to add back fat-soluble vitamins which are lost in fatty stools. A calcium supplement may be prescribed to help prevent osteomalacia and osteoporosis.

When medical treatments, such as URSO 250 & URSO Forte, Actigall and methotrexate, no longer control the disease, the patient should be evaluated for a liver transplant. The end stage of PBC is liver failure. Many signs indicate liver failure: increased bilirubin, jaundice, fluid accumulation or ascites, malnutrition, gastrointestinal bleeding, intractable itching, bone fractures and hepatic coma. Transplant is recommended before most of these symptoms occur. Recent studies suggest that about 30% of those diagnosed with PBC will require a transplant. The transplant outcome for PBC patients is excellent.

As with any other chronic illness, support and understanding is very important in helping the PBC patient cope with day to day living.
Author: CNL Created: 4/25/2010  Ref #:46546
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16 comments:

  1. i have been keeping up with your blog, and i think you are doing a great thing, informing everyone of how important the liver really is. i am 23 years old and have AIH/PBC and am currently waiting on a liver transplant. and have been waiting for 6 years now.

    erin

    ReplyDelete
    Replies
    1. Good sharing, yes, liver dysfunction adversely affects the digestive, immune, endocrine, circulatory and nervous systems in a variety of ways. I would recommend Lifestream Chlorella supplement. It contains a high concentration of chlorophyII, Chlorella Growth Factor (CGF), nucleic aids, amino acids, enzymes, antioxidants, vitamins and minerals all beneficial to overall liver health maintenance and restoration. Read more at:
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      Delete
  2. Erin, It's hard to think your only 23 years old and have AIH/PBC. When did you start to get sick?

    ReplyDelete
  3. Your site has been very helpful, i am 25, male, and have been recently diagnosed. Keep up the good work.

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  4. this is the first site i have read which makes me feel hopeful that i can hope for a normal lifespan without a transplant. i am 51 and about to have a biopsy to confirm the stage of the disease. i do not suffer from fatigue, my only symptom is very severe itching and my bilirubin is normal (obviously other blood tests are not).

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  5. Hi All,
    I am newly diagnosed with PBC.
    Still testing because of the fevers I run on andoff.
    Also, they want to re look at a cyst I have for years on my adrenal gland and see if that is adding to the problem.
    I would like to know about diet for PBC, Any ideas?
    Websites with menus?
    Thanks
    Anna in NJ

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  6. Thanks for giving data concerning Primary Biliary liver {disease} (PBC) Its specialized to grasp concerning the disease , it's useful for others conjointly
    cirrhosis treatment

    ReplyDelete
  7. This is really a pleasant journal giving knowledge regarding the info Procedure To Treat Liver disease of the liver of the liver ,as it clears all the doubts related to disease of the liver of the liver and additionally the because of cure it through communication ,thanks for putt this on cirrhosis treatment

    cirrhosis treatment

    ReplyDelete
  8. I must say that this kind of illness if one have the proper knowledge of liver problem symptoms, for with it there many people can be save.
    Anyway, thanks for sharing some important information here regarding liver disease.

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  9. Thank you for this info. I am in the process of confirming the PBC disease. Liver biopsy on Tuesday. I have been being treated for systemic sclerosis and was hospitilized without any diagnosis of what was going on except the autoimmune disease. Yhe pain undet my right rib cage was dismissed for years until one of my awesome doctors found high positive AMA. I have hiatal hernia and severe stomach inflammation waiting to see what the biopsies indicate. Could my stomach issues be related to the PBC?
    Thanks again
    Kim

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  10. A brilliant article. I have only just been diagnosed, but it would appear that I have had pbc for a much longer period of time and now have the cirrhosis and severe joint pains. In a funny way, it is comforting to have read this reassuring article and know that "it's not all in the mind"! At the age of 77 I sincerely hope I will retain my 'marbles' for as long as possible - for my family's sake, for mine too and for the sake of all those to whom I teach art - a super bunch. Thank you CNL.

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  11. This disease not only found in elders but also found in children's. One of the major complications that arise is variceal hemorrhage. Thanks for sharing. childhood cirrhosis

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  12. I have high AMA'S, horrible pain in the area around my liver and extreme fatigue. Also painful joints, particularly my wrists. My doctor is reluctant to give me a diagnosis of PBC even though everything points to this. Apparently one enzyme which would normally raised with PBC is St normal level. But I have all the other symptoms. I had a liver biopsy 9 days ago and haven't had results yet. It made my pain worse, so bad that I've almost gone to the hospital a few times. I just wish I could get a diagnosis so I can get on whatever treatment I need to go on!

    ReplyDelete
  13. Herbs have great approach in treatment of liver cirrhosis. These herbs include Phyllanthus niruri, Milk thistle, Bhringraj, Kalmegh, Guducchi and Dandelio. These herbs along with other herbs have been used for hundreds of years to treat liver diseases including liver cirrhosis, fatty liver, Hepatitis, Hepatomegaly, etc.

    ReplyDelete
  14. Am so happy, i never believe i will be this happy again in life, I was working as an air-hoster ( cabby crew ) for 5 years but last year i loose my job because of this deadly disease called Hepatitis B (HbsAg), I never felt sick or have any symptom, till all workers were ask to bring their doctor report, that was how i got tested and i found out that am HbsAg positive that make me loose my job, because it was consider as an STD and is incurable disease, i was so depress was thinking of committing suicide, till i explain to a friend of mine, who always said to me a problem share is a problem solved, that was how she directed me to this clinic called HEALTH MED LAB CLINIC, that was how i contacted them and i get medication from them and i got cured for real, I just went back to my work and they also carry out the test to be real sure and i was negative. Please contact this clinic if you are hepatitis B positive their email is ( healthmedlab@gmail.com ) they are life saver.

    ReplyDelete