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Dr. David Bernstein Answers Our Questions
November 1999
Question & Answers
1.) It seems we pbcers all have some of the same symptoms some have one thing and someone else has something different but it seems there is one thing that we all share and it the extreme fatigue. Is there nothing that we could take to improve this condition? It is so debilitating to have to sleep when you should be doing something else and seems to cause depression and weight gain among other things.
Answer
Fatigue is considered the most common presenting symptom of PBC. Despite its frequency, there are no well accepted therapies to improve fatigue. Many PBC patients have fatigue on the basis of underlying thyroid disease or depression. Therefore, the thyroid should be checked and treated in necessary. I often recommend anti-depressent medications to PBC patients as depression may cause part or all of their symptoms of fatigue.
Ursodeoxycholic acid (URSO or Actigall) has been shown to improve the symptoms of fatigue in many patients. Other modalities which may improve fatigue in PBC include exercise, implementation of a well balance diet and exposure to sunlight.
2.) I've had itching from the beginning, usually on the soles of my feet and palms of my hands. I've also had an itchy throat, ears, and eyes for the same length of time and always considered them some kind of allergic reaction. When we speak of itching as a symptom of PBC, is the itching confined to a specific area of the body or is it possible to have itching throat, eyes, etc., as a result of liver disease, especially when allergy tests are negative?
Answer
Itching in PBC is generally not confined to a specific area of the body. It is usually worse at night and may effect any part of the body. The itching is usually worse when coarse garments are worn and may be exacerbated by dry skin or hot, humid weather.
PBC is commonly associated with a condition known as the Sicca syndrome. The sicca syndrome is characterized by dry eyes and dry mouth. Studies have shown the prevalence of sicca syndrome in PBC to range between 72-100%. Therefore, it is common for PBC’ers to develop itching in the throat, eyes and ears.
3.) If PBC has progressed rather quickly up until now, will it continue to progress quickly? Does the progression rate vary in each individual? Is rapid early progression (after diagnosis) indicative of prognosis?
Answer
Patients who have a rapid progression of PBC are likely to continue to have more progressive course than those who are asymptomatic. The rate of progression in highly variable from patient to patient but the long-term course of all patients is progression. Rapid progression after diagnosis is indicative of prognosis with these patients unfortunately progressing more rapidly to cirrhosis.
4.) I know that not much is known about this disease, but I wonder if pbc can be inherited. Also, it is my understanding that you can have this disease for 15 or 20 years before it shows up in some way (I developed clubbing in my fingers which led to extensive testing). Do you think this is an inherited disease, and can we have it that many years without any type of symptoms?
Answer
The etiology of PBC remains unknown. Although genetic factors appear to play a role in PBC, the disease is not inherited in any simple recessive or dominant pattern. However, there are reports of family occurrences involving sisters, grandmothers and granddaughters, mothers and daughters, brothers, and sisters and twin sisters.
Healthy relatives of PBC’ers are more likely to have a positive anti-mitochiondrial antibody. Patients with PBC probably have the disease for many years before it is diagnosed. Most patients are diagnosed if they have symptoms or if they are noted to have abnormal blood tests.
Most people with the PBC prior to diagnosis are either asymptomatic or do not regularly visit a physician. Therefore, most patients probably have had the disease for many years prior to diagnosis.
5.) As a PBC patient should we have flu shots? What about pneumonia shot?
Answer
I recommend flu shots yearly to my patients with PBC. I also recommend the pneumonia vaccine once in their lifetime.
6.) How does PBC affect your eyes? I hear so much about dry eyes and mouth. Is this actually a part of the PBC disease?
Answer
PBC may affect the eyes. Keratoconjunctivitis sicca is common in PBC and leads to decreased lacrimation (tearing) and this causes dry eyes. The sicca syndrome is characterized by dry eyes and dry mouth. Studies have shown the prevalence of sicca syndrome in PBC to range between 72-100%. Therefore, it is common for PBC’ers to develop itching in the throat, eyes and ears. This condition is part of the spectrum of PBC.
Hypothyroidism can also cause dry eyes and this must be looked for in PBC patients with complaints of dry eyes.
July 2000
7.
What are the signs and definitions for the stages of PBC. How many stages are there in PBC?
Answer
There are 4 stages to PBC. They are as follows:
Stage 1: Asymmetric destruction of septal and interlobular bile ducts
Stage 2: More widespread destruction of bile ducts, portal fibrosis
Stage 3; Development of bridging fibrosis
Stage 4: Cirrhosis
These stages are not characterized by any specific symptomatology
8. Please explain the difference between Sicca and Sjogrens syndrome. Thank you.
Answer
The word sicca means dry in latin. We sometimes refer to a person as having sicca syndrome when they have dry eyes associated with their arthritic condition. This term is not an accepted medical term, however, and when used, is usually synonymous with Sjogren's syndrome. Sjogren's syndrome is a slowly progressive autoimmune disease characterized by inflammation of the exocrine glands leading to dry eyes and dry mouth.
9. I've read that taking thyroid meds for hypothyroidism could affect bone loss. Is this true? If so, would taking Fosomax have any additional affect on the bone loss?
Answer
There is no literature to support the statement that taking thyroid medication for hypothyroidism affects bone loss. People may develop bone loss and osteoporosis as they age but this in unrelated to thyroid medications. Fosomax is effective in slowing down the process of bone loss and can be used in the appropriate clinical setting. Since each patient is different, I suggest contacting your individual physician to determine if Fosomax is right for you.
10. Premarin versus the patch....Would the patch be better to use instead of taking Premarin in pill form? Does the patch bypass the liver?
Answer
All estrogen preparations are metabolized through the liver. Premarin versus the patch? There is no real medical difference. The choice of one or the other is dependent upon patient tolerance and comfort.
11. Can you tell us what over-the-counter antacids are safe for those with PBC to use? I've read that aluminum-based antacids bind with actigall/urso. Would this be true of all antacids?
Answer
Aluminum based antacids have been shown to adsorb bile acids in experimental models but not in human subjects. However, aluminum-based antacids may reduce the absorption of URSO/Actigall and therefore decrease its effectiveness. On-aluminum based antacids, such as Rolaids, Tums, or Milk of Magnesia should not effect the absorption of URSO/Actigall.
12. PBC can cause severe Portal hypertension and varices. I read an article about Portal hypertension causing pulmonary hypertension. What's the link between them and what are the effects of that complication? How does one know this complication is occurring?
Answer
Any condition that can cause cirrhosis may lead to the development of pulmonary hypertension. However, this is an uncommon occurrence. The reason why pulmonary hypertension occurs in cirrhosis is not known. Most patients with pulmonary hypertension are without symptoms. The most common symptom of pulmonary hypertension is shortness of breath with exertion. Other symptoms include syncope, chest pain and coughing up blood or hemoptysis.
