Nathan M. Bass, MD, PhD

Professor of Medicine

Medical Director, UCSF Liver Transplantation Service

Division of Gastroenterology

UCSF Medical Center and School of Medicine

San Francisco, CA

Question 19

It seems many with PBC have sleep problems and also depression. Is this common with PBC and what is the cause?

Does it hurt the liver taking medications such as xanax and Ambien ?

Answer 19

Sleep disorders are common in patients with advanced liver disease, but sleep is commonly disturbed in early PBC by itching. Depression is also a common problem in patients with chronic diseases like PBC and should be treated through counseling and appropriate antidepressant medication. Drugs like xanax and ambien will not harm the liver, but xanax, in particular, is habit forming and very sedating. It is not a solution to depression. For someone who is having a really hard time getting sleep, an occasional ambien is fine. If itching is part of the problem, then a sedating mild antidepressant like doxepin is often used, or even an antihistamine.

Question 20

I noticed a large number with PBC have thyroid problems. What other diseases are commonly associated with PBC? Could you briefly explain them?

Answer 20

Thyroid dysfunction is very common in patients with PBC. Other commonly associated conditions include the sicca syndrome with dry eyes and mouth problems (immune damage to tear and saliva glands), reflux problems (burning in the esophagus from acid reflux - a result of smooth muscle dysfunction and scarring), painful fingers in cold weather (Raynaud's syndrome) and tight skin around the fingers (sclerodactyly). A combination of several of these along with calcium deposits on the fingers, and red spots known as telangiectasias is called the CREST syndromes which many patients have heard of but which most never develop. There are many much rarer associated conditions, most of which appear to have an immune basis. These include anemia, and lung fibrosis. To date, the best answer to "how" is we don't know - it appears, though, that immune diseases often associate together. It would be great if we could get some clues from this, but the system involved is still very complex and poorly understood. Bone disease (osteoporosis) is also common in PBC and reflects the age group most affected by PBC, poor dietary absorption of vitamin D and calcium, hormonal effects (or lack thereof) and other poorly understood factors related to cholestasis.

Question 21

I live in a rural area and my doctor knows little about PBC. He says he learns as much from the PBCers group as I do and is always interested in receiving copies of the digest. My question is why isn't there much information out about PBC to help us live healthier with this disease such as diet, vitamins, drinking. What are your suggestions on what we should and shouldn't do?

Answer 21

Actually, there is a lot of information out there, and the PBCer's group sends out a wealth of it. Their annual meetings are a tremendous source of information too. For your own benefit, you might consider a visit to a liver specialist in a major city to get a concise one-on-one education on natural history of PBC and lifestyle counseling. Most lifestyle issues are simple. Eating a "heart healthy" diet with a vitamin diet, exercising, keeping a steady, appropriate weight, keeping interested, informed and doing everything you enjoy is a good start. For advice on treatments, medications, the place of liver transplant and management of symptoms, a consultation with a knowledgeable specialist is probably the best course of action.

Question 22

Is GERD common is PBC? If so why?

How safe are drugs such as Protonix or Prevacide taken on a continuous basis?

Answer 22

GERD is very common in PBC. One study estimated it to be present in up to 50%, although it varied greatly in severity and is often subclinical (asymptomatic). It appears more frequent in patients with Sjogren's syndrome and scleroderma skin symptoms. It is caused by an immunological disorder that leads to scarring in the lower esophagus and loss of normal movement in this region. This results in a loss of the normal ability of the esophagus to sweep acid that rushes upward into the esophagus back down into the stomach and to provide a good seal against acid reflux. Drugs like Protonix and Prevacid (PPIs) are a real blessing, but may be needed in high doses if reflux is severe. The worst cases may benefit from anti-reflux surgery. PPI drugs have been used now for decades and are extremely safe even when taken continuously for years.

Question 23

Palms and fingers tingling/burning, could that have anything to do with PBC or something else?

Answer 23

Could be Raynaud's syndrome, especially if brought on by cold air or water on the hands. Sometime itching can have a burning quality, but not commonly.

Question 24

I was wondering what the current protocol is for people who have PBC and AIH?

Are medication URSO 250 & Predisone common? About how many with PBC have AIH also?

Answer 24

I believe that the combination of PBC and AIH is actually quite uncommon. Current estimates range from 2-5% of all patients with PBC. The problem is that widely accepted criteria for the diagnosis of AIH/PBC have not been developed. The only cases I have seen were diagnosed by other physicians and failed to respond to prednisone or Urso. In the end, I think they simply had PBC. This association does seem to occur, but it is difficult to diagnose with certainty except when the presentation is very unusual for PBC alone, such as a young patient with very high ALT/AST values (very unlike PBC) plus some features of PBC such as a positive AMA. The appropriate treatment in a very florid or severe case would be a trial of prednisone first. Milder "possible" cases should be treated as if they have PBC - with usodiol first. Unfortunately, it seems that patients with this confusing syndrome often do not respond to either treatment.

Question 25

Being that it is summer, I felt this might be helpful to other

PBCer's. I've been told that it is not safe & indeed could be quite

harmful for anyone with chronic liver disease to swim in the ocean.

Is this true? And if so, could you please enlighten us.

Answer 25

No more dangerous than it is for anyone. There is a frequently given

piece of advice that patients with cirrhosis should not eat raw

shellfish because of a bacteria found in shellfish that produces a

rare type of food poisoning. In patients with cirrhosis, this has

been particularly severe, and at times fatal, but it is extremely

rare. I would not extend this already cautious advice to a general

admonition against swimming in the sea. Just don't forget to use

sunblock.

Question 26

I bruise easily especially in areas where I itch. Could you explain

what causes the bruising in liver disease and what we can do about

it. Should this only be a concern in late stage of PBC?

Answer 26

Bruising in liver disease is often attributed to blood coagulation

problems and in advanced liver disease, this is indeed the case. Low

platelets are common in cirrhosis, while a prolonged prothrombin time

can result from more severe liver damage and sometimes from poor

absorbtion of vitamin K. Patients sometimes notice easy bruising in

the absence of advanced liver disease. In some cases this may

indicate poor nutrition and weakening of the skin from inadequate

protein intake. Trauma to the skin like that produced by scratching

may weaken areas of the skin, especially over the arms and legs, and

may also be a factor in producing bruising. What one does about it

very much depends on the cause: Bruising in advanced liver disease

indicates a need for careful monitoring. If the prothrombin time is

abnormal, then a course of vitamin K injections or regular oral

vitamin K might be helpful and is worth a try. Taking extra care not

to traumatize one's limbs is common sense, as are more effective

measures to help with itching.

Question 27

What is the best antidepressant for anyone with PBC to take? Seems

like

there are several out there some of us reading the digest are taking.

Answer 27

There are many that are actually very safe in patients with PBC, but

there are many side effects especially with the SSRI class of

antidepressants (Prozac and related drugs). Prozac has the longest

half life and its effects (including side effects) last the longest

after it is discontinued. So we tend to use this drug less.

However, there are many patients with liver disease who take Prozac

without any problems. The choice of antidepressant usually depends on

severity, associated symptoms (such as anxiety), and overall

tolerability of the drug. Side effects are very individual in

nature - one persons perfectly tolerated drug will be another's

disaster. serious toxicity to the liver is a rare but real

possibility with many of these drugs, but the risk is so low that if

a good indication exists, then they should be used. In terms of

frequency of usage in our practice, determined mainly by drug safety

profile and tolerability, Celexa, Zoloft, Paxil, Prozac and

Wellbutrin are the most commonly taken. Lexapro seems fine too, but

is more expensive. Tricyclic antidepressants are used less these

days, but are often taken for insomnia, or even itching (doxepin).

Many patients suffer from mild to moderate depression and are

prescribed an antidepressant by their primary care physician. This

is quite acceptable, but in the case of patients with serious

depression, I would recommend having a psychiatrist make an

assessment and prescribe the best antidepressant.

Question 28

What is the importance of ductopenia and what does it indicate?

Would it be serious in stage 2 or 3, or no threat at all?

Answer 28

Ductopenia is a term that refers to the loss of microscopic bile

ducts seen on a liver biopsy. This is a typical lesion in PBC, but is

of varying severity in individual patients, and usually most severe

in the more advanced stages of the disease. It is also seen in

several other conditions including certain types of drug-related

liver damage and as a sign of severe rejection of a liver transplant

graft. Damage to bile ducts can be seen as an early feature of PBC,

but significant loss of ducts (ductopenia) is seen most typically in

more advanced stages of the disease associated with a lot of liver

scarring. A really marked loss of bile ducts in PBC is thus usually

associated with cirrhosis. Uncommonly, it is also found in the

absence of much scarring of the liver. In patients with such early,

extensive loss of bile ducts, there may be an early onset of

jaundice, weight loss and severe itching. This appears to be an

uncommon and more rapidly progressive variety of PBC.

Question 29

I am blood type A negative with positive antibodies (my 2 children were rh positive) and I am wondering if I would have possible problems looking for a donor. Besides blood what else has to match when you consider a

possible donor.

Answer 29

The blood type matching rules are simple. In terms of the recipient,

O can only receive from O, A from A or O, B from B or O, and AB from

A, B, Ab or O. The Rh blood group (the "postive/negative") does not

matter at all, so either of your children are potential donors for

you. The other detail important in a living donor match is body size -

usually we will want someone within 20% of your weight. There are

many other factors that go into living donor selection though,

including motivation, liver anatomy, weight, and many health details.

Question 30

What is the importance of the Alk levels?

I would like to know what symptoms exist with PBC and the most

conclusive non evasive test that can either confirm or rule out this

disease.

Answer 30

Alkaline phosphatase (often referred to as the "alk. phos." ) is an

enzyme that is made by bile duct cells. Any damage to the bile ducts

or blockage to the ducts causes the cells to make more of this enzyme

and it appears in the blood stream in increased quantities. An

elevated alk.phos. is quite typical in PBC. There is some

correlation between the progression of PBC and the degree of

elevation of alk. phos. levels, but this is inconsistent. Some

patients may start out at the time of diagnosis with a very high alk.

phos. level which may improve to normal with urso treatment. A lack

of decline in alk. phos. with urso may identify some patients who are

going to progress more rapidly.

As far as symptoms are concerned, the most common typical symptom is

itching. After that, there is a wide spectrum. Patients may

experience none of these or several of them and they include fatigue,

dry eyes, dry mouth, discomfort and/or pain over the liver, and

symptoms of CREST syndrome if this is also present. A liver biopsy

is very useful in confirming the diagnosis of PBC and is considered

an important test by many experts. It is not essential in all

patients, though. A combination of typical symptoms in a woman

between 30-60 years of age, associated with a positive

antimitochondrial antibody and no blood tests or appearances on an

ultrasound examination suggestive of any other diagnosis, usually

provides a conclusive non-invasive diagnosis of PBC.

END............