Primary Biliary
Cirrhosis Education & Research
- Educate
PBC patients, family members and friends.
- Make
the medical community aware of the PBCers organization and how we can help
one another.
- Overcome
some of the myths about liver diseases in the general public.
- Since
PBC is considered a rare disease, there isn’t much interest in PBC
research. As a group, we need to
get more doctors interested in PBC research and making funds available for
new research.
To help in our PBC education
campaign, it is important for each of us to educate our family and
friends. This can be difficult because
family members do not want to hear their loved one has been diagnosed with a
chronic illness such as PBC. But they
need to be educated and we need their help in the fight against
PBC. Give family members and friends a
copy of “What is Primary Biliary Cirrhosis?” and ask them to read it. By reading the correct information they will
be able to understand PBC, and not be frightened by the unknown. They will understand PBC is not alcohol or
drug related, and that looks in a chronic illness can be deceiving.
We started as a group of 3, and
have grown to over 2,400 members worldwide and accomplished more in the past 9
years than we thought possible. Imagine
what we can accomplish if we get our family and friends involved in the fight
against PBC.
Ways you can help:
· Wear PBCers logo shirts, hats or carry a PBCers tote. Logo items are a great way to start conversations about PBC. Please visit our PBCers Organization online store.
· Wear your PBC Awareness Lapel Pin and Bracelet.
· Post in online newsgroups/message boards about PBC regional meetings in your area and invite PBCers, family members, neighbors and friends.
· Talk to anyone and everyone about PBC....in grocery lines, at church, doctor’s office, clinics, buses. Anywhere you have a chance to educate someone about PBC.
· Ask family members, friends, neighbors and coworkers to spread the PBC word.
· Educate the medical community. There are so many medical professionals who know very little about PBC and nothing about our organization.
· Work with your area newspaper about having an article published on PBC.
· Pass out PBCers Organization/Axcan Pharma flyers in your area doctor offices, clinics & hospitals.
· Write to your government officials about PBC research and funding.
·
Anything
you can think of to help with PBC education and research.
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. 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.
Upon
diagnosis, some doctors may suggest their patient
- Start a reduced sodium diet and or low fat diet.
- Lower
red meat intake and avoid raw meats, fish & shellfish.
- Drink plenty of water and other fluids such as juice.
- Take Calcium
and Vitamin D supplements. 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.
- Eat smaller meals four or five times a
day.
- Stop smoking
The
above suggestions have been found to be very helpful in liver disease, but are
common sense in healthy living.
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 confirm the diagnosis and
stage of the disease.
- Blood lab tests that show liver
dysfunction:
- Liver function tests
- Antimitochondrial
antibodies. Positive AMA is found in about 95% of PBC patients.
(Biliary enzymes
& Pathology-PBC
disease progression slides)
Serum cholesterol and lipoproteins may
be increased. - Haptoglobin
& ACE levels may be altered
- Diagnostic tests
- Other
exams and 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. When diagnosed, it may be associated with one or more other
autoimmune diseases such as
rheumatoid arthritis, sjogrens syndrome, raynauds phenomenon, celiac sprue, systemic
lupus erythematosis ( SLE or lupus) or scleroderma.
Patients
remain without symptoms for many years, and some may never notice symptoms
other than itching and fatigue. The initial symptoms vary among PBC patients,
and because of the varying symptoms can sometimes make it difficult for doctors
to actually diagnose PBC. The varying symptoms may be due to the individual,
autoimmune nature or other diseases associated with PBC.
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 Kreplacement 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.
PBCers Organization
Copyright 2004