Stephanie Aged 2


What is Primary Biliary Cirrhosis?  


Primary Biliary Cirrhosis (PBC) is a chronic liver disease that slowly
destroys the bile ducts within the liver (intrahepatic bile ducts). The name is
somewhat deceptive since cirrhosis only occurs in the last stage of the
disease after many years of inflammation. With early diagnosis and proper
medications, most 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.

The cause of PBC is still unknown, but it is not alcohol or drug induced or
contagious. 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.  
A person with PBC commonly hears comments such as “you look so
healthy or you don't look sick." 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 appearance doesn’t tell the story of what is going
on inside their bodies.  Even on the transplant list stage, many with PBC
look healthy.

Symptoms may be present in any combination and include any of the
following:

· Chronic 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. Red color to the palms and bottom of feet.

· 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.  

· 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, which 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.  

· Sexual problems (impotence in men, absence of periods in women, vaginal
dryness and 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.  Also, some
who experience pain in the right quadrant liver area, 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 the pain is real – It is
believed the tissue and lining around the liver become inflamed and the
enlarged liver cause the discomforting pain.

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, 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. The transplant outcome for PBC patients is excellent.

As with any other chronic illness, support and understanding are very
important in helping the PBC patient cope with the disease symptoms and
day to day living.

PBC Fatigue is the number one symptom

Fatigue is an early PBC symptom.  Over time it may become severe and
even debilitating. The difference in PBC is that fatigue is not situational nor
is it generally relieved by rest and sleep. Most people are tired from either
lack of sleep, stress or situational or true depression. Once people are well
rested, stress is relieved or depression resolved, fatigue improves. This is
not true in PBC as the source of fatigue is different. The fatigue is a result of
the liver disease and is progressive. It tends not to get better.  Only
treatment of the disease will improve fatigue and currently, there are no
good therapies that improve the fatigue associated with PBC.

Please help in the fight against PBC by making a tax
deductible donation to PBC Research

PBCers Organization, 1430 Garden Road, Pearland, Texas
77581

(281) 997-1516


Get on-line support by joining: PBCers@yahoogroups.
com

Find out more about living with PBC at: PBCers.org