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Dr. Marshall Kaplan Answers Our Questions
September 9, 1999
Questions & Answers
1. There has been some discussion in the digest of PBC patients avoiding foods containing copper. Is there research to suggest or support that PBC livers retain more copper than those not infected with PBC?
Answer
Patients with PBC do retain more copper in their livers than others. However, there is no information that this causes harm. This is in contrast to patients with Wilson's disease where copper is harmful. Copper seems to be stored differently in patients with Wilson's disease.
2. Does it happen often that those with PBC will have positive AMA, and later negetive AMA? Many in the digest have had this happen and I thought once positive it wouldn't change.
Answer
AMA TITERS tend not to change very much with time.Changes are more often due to problems with the assay. At least, this has been my expeience in every such instance. Some of the commercial labs have had problems with quality control.
3. What is your opinion of Naproxin for joint pain? Are these type drugs safe for the liver?
Answer
It's ok. NSAID's are usually safe if used in moderation.
4. Could you please discuss the dizziness many of us are experiencing - some call it dizziness while others say ligh theadedness. Is it a PBC symotom or other liver diseases? Cause and treatment - and what effect taking urso might/will have one it.
Answer
Dizziness is not usually part of PBC. However, it is common in hyperventilation syndrome, a kind of anxiety that some of my patients have had. Your doctor should check this out.
5. Can intravenous chelation with the main ingredient being EDTA be harmful to persons with PBC?
Answer
I'm not aware of chelation therapy being used to treat PBC. To the best of my knowledge, chelation is only useful to remove excess amounts of certain heavy metals. This not a problem in PBC.
6. What are your thoughts on the vitamin preparation ADEK, which is manufactured by Axcan. Is this a good vitamin for PBCers or do you suggest another one or does the vitamin depend on the individual needs?
Answer
VItamin replacemnt therapy has to be individualized. Vitamins are only useful if a patient is deficient in a certain vitamin. This can be easily measured. I have not used the preparation you mention so I can not really comment.
October 1999
7. Many in the group have discussed edema or ascites. I thought once you blow up with water either in the stomach or ankles that you have to see a Doctor to get rid of it. Are there other alternatives?
Answer
It is best to see a doctor. Fluid retention may be a serious problem and should always be evaluated by a physician.
8. There is a newly available supplement called SAM-e (S-adenosylmethionine). The insert says that it's been studied extensively and clinically found to promote and support liver health among other things. Physicians are encouraged to contact the company for scientific information. The company is Nature Made. Ph # is 1-800-276-2878. The company is Nature Made. Do you know anything about this?
Answer
Yes. I know a lot about it and used to use it in biochemical research 35 years ago. It is one of many products that has no established role in the treatment of liver disease, certainly no role in the treatment of PBC. I can only assume that some entrepreneur or company hopes to make money by promnoting its sale. In contrast ot "ethical drugs" e.g., URSO and Actigall, our government does not regulate this industry, one which many of us feel should be more carefully monitored.
9. Any thoughts on BETA-CAROTENE. (1 A DAY EQUIVALENT TO 25,000 I.U. VITAMIN A)
Answer
Most PBC patients do not need it. If there is doubt, blood levels can be measured and it cna be taken by those who are lacking it.
10. What are the side effects of low dose methatrexate when used in conjunction with Actigall to treat PBC. What are the risks? Has it been clinically proven to slow down progression of the disease?
Answer
It's well tolerated by most pts. Less than 5% note nausea or loss of appetite the day they take it. Some complain of hair loss, but hair loss is very common in PBC and pts on ursodiol also complain about this. When I began to use methotrexate, I was worried that it might damage the liver but this does not happen. Likewise, in low dose it has no bad effect on the bone marrow. I reported a 15% incidence of methotrexate induced pneumonia 6 years ago in a group of PBC patients who were in a resarch study with MTX.
I have not seen a case since. Dr. Munoz who is conducting a multicenter study of methotrexate in PBC presented a paper last year at the AASLD meeting entitled "Absence of pulmonary toxicity in primary biliary cirrhosis treated with methotrexate and ursodiol". It was based on 266 patients followed for 4 years. (Hepatology 1998;28:392A.) The use of methotrexate is controversial and not accepted by many liver specialists. I introduced MTX to the treatment of liver disease 20 years ago, wrote the first paper about it 13 years ago and probably have more experience with it than all other physicians, even if their experiences are combined. In my experience it has not only slowed down the rate of progression of disease, but has reversed it.(Kaplan et al, Ann Int Med 1997;126:682).I am quite comfortable that my positive experience with methotrexate will eventually be reproduced by others.
11. What about the levels of liver enzymes reported after a liver panel (ALP ALT etc), are they directly related to the amount of damage to the liver?
Answer
There is no correlation between the serum levels of ALP, ALT or AST and the extent of liver damage. This was recognized more than 40 years ago when these tests were extensively studied. I fear that many younger doctors may not know this older medical literature.
12. Is pbc related to sensory neuropaty?
Answer
I know of no relationship between PBC and sensory neuropathy.
December 1999
13.) Can PBC reoccur after a transplant and if so, how common is it?
Answer
It is very rare for PBC to recur after OLT if adequate immunosuppression is used. I have not seen it in any of the PBC patients who have had transplants at NEMC since 1983. There is some controversy because researchers at UC Davis and Mayo have considered recurrence to have occurred in some of their patients who are well, have normal blood tests and normal liver biopsies but who have a finding on liver biopsy that is only demonstrable using a research technique, immunohistochemistry. In my opinion, no one knows what this finding really means. I would not consider these patients to have recurrent PBC.
14.) Among our PBCers we have greatly varying experiences with the frequency of liver biopsies. What purpose(s) do they serve, and how often should they be performed?
Answer
I do them at baseline and then at one to five year intervals, depending upon how serious the inital biopsy findings were and how well the subsequent biopsies look. I use biopsies to determine if the PBC is improving or worsening because the blood test results do not always parallel what is going on in the liver. I read the biopsies myself and find them very important in managing patients. They would not be as helpful if I relied only on the pathologist's interpretation because I can correlate symptoms, physical findings and blood test results with the biopsy findings.
15.) Many of our PBCers complain of "fuzzy brain". Some members have hepatic encephalopathy and some do not. Could you explain hepatic encephalopathy, including pre-hepatic encephalopathy.
Also, in the absence of hepatic encephalopathy, could our cognitive problems be related to PBC?
Answer
I am not aware of cognitive problems in my PBC patients except for those who develop hepatic encephalopathy, a sign of advanced PBC, liver failure and the need for liver transplantation. Many PBC patients are very fatigued and may confuse this with cognitive problems, particularly if they fail to get enough sleep. This is probably no different from any one else who is sleep deprived.
16.) If a person's ammonia levels are too high, is it possible they could actually give off a body odor? If so what type of smell would it give off?
Answer
I am not aware that a high blood ammonia level by itself gives off any smell, except perhaps for that of ammonia if the levels are very, very high; rare in PBC. Patients with advanced liver disease of any sort may have an odor called fetor hepaticus. This is due to sulfur containing chemicals in breath, compounds that would normally be metabolized by the healthy liver.
17.) Is methotrexate indicated for all PBC patients, or only those who don't respond to ursodiol?
Answer
I now use methotrexate in patients who have failed to respond to uursodiol alone or ursodiol plus colchicine. As you are probably aware, this is a controversial area in PBC. I have been using methotrexate in PBC since 1986 and find that it is very effective in many patients, particularly those who fail to respond to ursodiol and colchicine. What i have found is that patients respond quite differently to medical treatment. I don't know whether this means that PBC is more than one disease, what we call a syndrome, or whether it is the same disease but with different responses to treatment.
18.) I have always expressed to my family that I want to be an organ donor when I die. What affect will PBC have in regards to any of my organs being able to be used?
Also, suppose a person received a transplanted organ and died, can that organ be harvested and used for someone else?
Answer
I believe that other organs can be used in transplants. As far as I know, an organ can only be transplanted once.
