DOCTORS PANEL QUESTIONS
& ANSWERS
We wish to thank all the members on our Doctors Panel, for giving us their
time answering PBCers questions. We greatly appreciate all they do
for us.
The following questions were answered by:
Melissa Palmer, M.D.
Specialty: Gastroenterology and Hepatology
Private Practice Long Island, NY
Author of "Guide to Hepatitis & Liver Disease" revised edition which
was
published in June 2004. http://www.liverdisease.com/
Dr. Palmer will be one of our guest speakers at the 2005 conference in
Vegas
2004
QUESTION 1
Is arthritis one of the effects of the PBC or is
it a natural occurrence
because of the ages of most patients?
I take Vioxx for arthritis. Is this safe or at-risk
to liver
ANSWER 1
Rheumatoid arthritis, which is characterized by joint aches and joint
deformities, can be seen with an increased incidence in people with PBC.
Since many
rheumatologic disorders - such as arthritis, have an autoimmune origin, it
is
not surprising that there is a high degree of association between all
rheumatologic disorders -including arthritis, and PBC.
Women in general, with or without PBC, have a higher
incidience of arthritis
than men. Since PBC is primarily a disease that affects women, arthritis
often occurs. Systemic lupus erythematosus
(SLE) may also occur in people with
PBC and is characterized by fever, skin rash, and arthritis.
Acetylsalicylic acid (aspirin) and other NSAIDs
are drugs that are widely
used for their anti-inflammatory and analgesic effects. They also have the
potential to cause drug-induced liver disease. In fact, many
NSAIDs have been
withdrawn from the market due to their
hepatotoxicity. All
NSAIDs have the potential
to cause liver injury. However, some NSAIDs are
more hepatotoxic than others.
NSAIDs presently on the market that have been
frequently associated with
liver injury are aspirin (ASA), diclofenac
(Voltaren), and sulindac
(Clinoril).
Ibuprofen (Motrin) has been reported to cause severe liver injury in people
with
hepatitis C. A new generation of NSAIDs, known
as the cyclooxygenase-2
(COX-2) inhibitors, has recently been approved by the FDA. This group
of NSAIDs has
the advantage of having fewer gastrointestinal side effects - less abdominal
discomfort and less risk of gastrointestinal bleeding, than conventional
NSAIDs. There are three different COX-2 inhibitors
currently available to the
public- Vioxx,
Celebrex and
Bextra. COX-2 inhibitors have been associated
with
some liver dysfunction, although not as commonly as other
NSAIDs.
It is recommended that people with liver disease, especially those with stage
3 or 4 disease, avoid using all NSAIDs. If
NSAIDs are medically required for
the treatment of another medical disorder, a reduced dose should be used
for a
limited period of time and only by people with stable liver disease. Older
women with liver disease seem to be particularly susceptible to the
hepatotoxicity of NSAIDs
and are advised to avoid NSAIDs altogether.
Since NSAIDs may
cause salt and water retention people with fluid retention problems such
as
ascites or leg swelling may suffer worsening of
these conditions. People with
decompensated cirrhosis are at increased risk kidney
damage stemming from the use
of NSAIDs. Since this may lead to
hepatorenal syndrome, people with advanced
liver disease are advised to totally avoid all
NSAIDs. Furthermore, people
with ascites (fluid accumulation) may not respond
to treatment with water pills
(diuretics), while on NSAIDs, as they counteract
their actions. People with
liver disease who have had internal bleeding, - from an ulcer or esophageal
varices, for example, may be at risk for recurrent
bleeding induced by NSAIDs, and
should totally avoid this class of medications. People who are also taking
corticosteroids (such as prednisone), or anticoagulants (such as
coumadin) may
have and increased risk of complications from
NSAIDs. Finally, people with
liver disease who smoke cigarettes or drink alcohol should avoid
NSAIDs as they
are also at increased risk for its complications.
QUESTION 2
What concerns and recommendations are there for a female PBC patient's use
Premarin (Oral and Vaginal
Creme) ?
Are PBC patients more likely to have breast cancer from the hormones?
ANSWER 2
The use of estrogen supplementation in people with
liver disease is a
controversial issue that needs to be studied in more detail.
While
estrogen-hormonal therapy has been demonstrated to increase bone mass, oral
estrogen
replacement should generally be avoided in people with liver disease - especially
those with PBC, as it may cause additional liver problems, such as worsening
of
cholestasis. Furthermore, estrogen supplementation
may cause certain benign
liver tumors, such as hemangiomas and/or hepatic
adenomas to enlarge. Estrogen
patches and cremes are generally a safer choice.
Implantable estrogen, a recent
development, is probably safe, but long-term studies as to its effect on
liver
disease have not been done. Soy estrogen, originally thought to be a safe
alternative, should be avoided, as recent reports have suggested that it
may
cause drug-induced hepatitis. Finally, the use of estrogen has been cited
as even
potentially triggering the autoimmune response that leads to the development
of PBC.
Some studies, although not all, have found an increased
incidence of breast
cancer in people with PBC. However, since the likelihood that a person with
PBC
will develop breast cancer is quite low, routine breast cancer
surveillance-more diligent than that recommended for the general populations-
is not
mandatory.
QUESTION 3
Shortness of breath & fatigue
Can shortness of breath due to liver disease occur in earlier stages of PBC
or just stage 4?
I've talked to other PBCers and many of us get shortness of breath shopping,
walking, etc.
Can you explain the reason and give us recommendations?
As the PBC progresses does the fatigue and need of sleep increase, or could
this be due to something else?
ANSWER 3
Sarcoidosis is a disease characterized by the formation
of granulomas
(nodules filled with a variety of inflammatory cells) in many organs including
the
lungs, skin, liver, lymph nodes, and bones. This disease resembles primary
biliary cirrhosis (PBC) in that both diseases are
characterized by the formation of
granulomas. PBC and
sarcoidosis occasionally coexist together.
When
sarcoidosis affects the lungs, it may cause shortness
of breath. However, the
shortness of breath that occurs in people with PBC is usually due to fatigue.
Fatigue is the most common symptom in people with PBC, occurring in
approximately 65 percent. The cause of fatigue in people with PBC is not
known,
however, depression and sleep disorders may be contributing factors. The
degree of
fatigue does not correlate with the severity of the disease and may be just
as
debilitating for a person in an early stage of PBC as it is for a person
in an
advanced stage of PBC. Question 4 below addresses the treatment of fatigue.
QUESTION 4
I know PBC is causing fatigue. I read that taking Coenzyme Q10 with
L-carnitine fumarate
can help getting our energy back. What about
Provigil?
Is there any danger to the liver in taking these?
ANSWER 4
Successfully treating fatigue can be a challenge.
In attempting to combat
fatigue, all of the possible causes of a person's fatigue should be looked
into.
Some causes are easily managed. Some occur commonly in people with liver
disease. The potential causes of, and/or contributors to, fatigue include
the
following:
o Thyroid disease (may be primary cause of, or contribute to,
fatigue).
o Anemia (may be primary cause of,
or contribute to, fatigue).
o Disorders of other organs, including
the heart (for example, congestive
heart failure) or the brain (for example, brain tumors).
o Nutritional deficiencies (for example,
a lack of iron or a lack of
protein).
o Disturbances in fluid and electrolyte
balance (for example, a low sodium
level).
o Depression
o Some medications and drugs (a doctor should review all
prescription and
over-the-counter medications that a patient is taking. If possible,
those
that cause fatigue should be discontinued).
o Excessive use of caffeine.
o Excessive use of alcohol.
o Emotional stress.
o Lack of exercise.
o Lack of sleep.
o Overwork.
If fatigue continues to persist after ruling out or correcting any of the
above factors, there are some lifestyle changes
that may be helpful. For example
- eating a healthy, low-fat, well-balanced
diet; quitting smoking;
refraining from alcohol consumption; and exercising daily, are all lifestyle
changes
that can ameliorate fatigue. Also it is recommended to drink plenty of water
and
limit caffeine-containing beverages to one or two servings per day.
Never consume large heavy meals. Four to six small meals throughout
the day
will keep energy levels high, and will minimize fatigue. It goes without
saying, but bears repeating anyway: people with liver disease should avoid
drinking alcohol. Alcohol can worsen any liver disease and is a cause of
fatigue as
well. It's also a good idea when necessary, to begin a healthy weight-reducing
diet to eliminate any excess weight. (Never lose more than one to two pounds
per week).
The demands of a hectic job or harried home life may need to be
reduced-overwhelming stress may cause fatigue even in a person who is not
suffering from
liver disease. Excessive stress, including emotional stress, is often the
cause
of fatigue in people with liver disease. Therefore, friends and family should
attempt to reduce the normal load of obligations and expectations that they
may normally have had of the person.
If possible, a thirty to forty-five-minute daytime nap should be taken daily.
This can help rejuvenate a person with liver disease. In some cases, it may
be necessary for a person with liver disease to incorporate naps into her
daily
schedule. Often, a doctor's letter to the patient's employer or supervisor
may be needed. A person with fatigue should feel free to ask her doctor
for
such a note. If a daytime nap is impossible, try to do some mid-day
exercises.
When sitting at a desk job all day, for example, blood tends to move away
from
the head and muscles. This results in both mental and physical
fatigue. It
is important to get your blood circulating throughout your entire body. Try
to
get up and walk around or simply do some stretching exercises.
Even a
little exercise will help get you concentrating and motivated again.
Remember, fatigue cannot be cured by a pill! Be especially wary of any
products that boast "improved energy levels" on their label. Because the
liver is in
charge of breaking down all supplements and medications, more harm than good
may result from taking such products. Also,
be aware that taking excessive
amounts of vitamins and minerals in an attempt to combat fatigue-especially
vitamin A, niacin, and iron-can lead to a worsening of liver disease. Coenzyme
Q
10 is vitamin-like antioxidant found in all parts of the body. This
substance
aids in the production of energy and stimulates the immune system.
It has
been used to reduce the side effects of cancer chemotherapy. Thus, it may
be
helpful in reducing interferon-related side effects, although no studies
have been
performed to assess its efficacy in this area. COQ 10 has been used to treat
allergic disorders, such as asthma, mental disease such as Alzheimer's and
heart disease. The amount of COQ 10 in one's body decreases with age. Therefore,
it is recommended that people over the age of 50
supplement their diets with
this substance. Deficiency of this substance may lead to tooth decay and
diabetes. COQ 10 has been found to be effective at dosages ranging
from 50 to 200
mg daily. Foods which are good sources of CoQ 10
include mackerel, salmon,
sardines, beef, peanuts and spinach. I am not familiar with the use
of
l-carnitine for people with liver disease. It is
essential that a person with liver
disease consult a liver specialist prior to taking any supplements or products
that promise to cure fatigue.
Ondansetron (Zofran)
is a medication used in the treatment of nausea and
vomiting associated with cancer chemotherapy. There have been anecdotal
reports
on the use of Zofran ( 4
mg twice a day) in the management of fatigue
associated with liver disease. Other medications have been found to be helpful
in
treating fatigue associated with other diseases
such as HIV, melanoma and chronic
fatigue syndrome. These medication include
bupropion (Wellbutrin),
methylphenidate (Ritalin), and modafinil
(Provigil). Provigil
is a medication approved
for the treatment of narcolepsy - a neurological disorder marked by
uncontrollable attacks of daytime sleepiness. Anecdotal evidence suggests
that Provigil at
a dose of 200 mg/day may be a useful adjunct in the treatment of fatigue
associated with liver disease. More studies need to be conducted on patients
with
liver disease before any of the above-mentioned medications can
be routinely
recommended for use in the management of fatigue.
QUESTION 5
What substances should PBCers avoid such as cosmetics,
perfumes, colors for
the hair, nails polish, home cleaning products?
ANSWER 5
There is no specific recommendations concerning the above products in people
with PBC. However, it is important to have good ventilation available
when
using home cleaning products or any products creating excessive fumes.
QUESTION 6
Is it possible that itching is caused by
Urso treatment? I had
very little
itching prior to starting the urso.
I wonder if you've heard anything about a supplement
SAMe being used for
treatment of PBC?
ANSWER 6
Pruritus occurs in approximately 80 percent of
people with PBC, and its cause
is not entirely known. Any medication may cause itching, but most studies
have indicated that less than 3 percent of people develop adverse side effects
from Urso. When side effects from
Urso are experienced, they include diarrhea,
decreased white blood cell count, elevated glucose levels, elevated
creatinine
levels, peptic ulcers, and skin rashes (which also may cause itching).
SAMe is a derivative of the amino acid
L-methionine. It is made in the body
when methionine combines with energy (adenosine
triphosphate [ATP]). In
SAMe is considered a drug to treat liver disease,
fibromyalgia and
depression. SAMe may improve liver enzyme elevations
and may reverse, or even prevent
liver toxicity which is caused by various drugs as well as alcohol and some
chemicals. It may increase natural levels of glutathione - an antioxidant
enzyme
produced by the liver that protects the liver from free-radical damage.
And it
has also been postulated that
SAMe may prevent liver
tumors. Further study
needs to be conducted with
SAMe before it can be
routinely recommended for
people with liver disease. In fact, studies using
SAMe in people with PBC
are
ongoing. If used, dosages should not exceed
800 mg twice per day and should be
taken on an empty stomach.