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Dr. Hugo E. Vargas Answers Our Questions
Questions and Answers
November 1999
1.) What is your opinion on the view that urso causes weight gain in patients who take it. Also is a fat stomach related to fluid retention, or bad eating and little activity. I find it strange that most of us with PBC report that they have large stomachs.
Answer
I have not seen much weight gain in patients with PBC, although a cirrhosis becomes more significant the weight gain may have to do with water retention and ascites.
2.) Fatigue plagues most everyone with PBC. It is debilitating -- really interfering with life and daily plans. Please explain what causes the fatigue. Is any research being done to solve this issue? Should one push to continue exercising during a fatigue episode?
Answer
Fatigue is not unique to PBC and is one of the more common problems in the setting of chronic liver disease. Despite efforts to figure out why it develops, it has been hard. It is particularly difficult to study because it is a complaint that although real is difficult to measure. I recommend to my patients to exercise as tolerated.
3.) What is your opinion on using Milk Thistle as an adjunct to Urso as pharmacotherapy for PBC?
Answer
Milk thistle probably will not hurt PBC patients but the information is very scanty. The problem is compounded by the fact that one product in the market cannot be compared with the next and thus it is difficult to endorse any one in particular.
4.) There is much conflicting advise about pbcers following low fat diets and avoiding additives etc. As the liver filters everything that is put in our bodies I would be interested to know your thoughts on this. Also since I'm now on low fat diet I am craving salt....do you have any ideas on this?
Answers
Low fat diets are recommended based on the fact that PBC causes increases in cholesterol. I make no specific recommendations on fat unless the levels of cholesterol are extremely high. I do not know what you mean by additives, if you are referring to vitamins, I recommend a multivitamin tablet, avoid mega doses of anything, but discuss any specifics with your hepatologist.
5.) Are there different stages of varicies? What is the proper care and feeding of varicies? What is the difference betwen varicies in the esophagus and those in the stomach?
Answer
Varices are engorged veins found in several parts of the body due to cirrhosis. Those in the esophagus and the stomach can bleed and make the severity of cirrhosis worse. Your hepatologist has probably already suggested endoscopy to stage the size of the varices and based on this procedure the varices can be graded (the larger, the higher the grade and the higher the risk for bleeding) There are medications to decrease the likelihood of bleeding (beta blockers, nitrates) and if you have already bled, then endoscopic banding should decrease the risk of re-bleeding.
6.) If there is no correlation between liver enzyme levels and extent of liver damage, why are these tests done periodically and consistently?
Answer
The liver function tests are being done to follow the trends in your liver function. They do change over time and can alert us of problems that may complicate PBC.
February 2000
7. For those of us with PBC that suffer from the severe itching, most of us have been told that the itching is a result of bile acids building up in the blood stream. Please explain.
Answer
The answer that pruritus (itching) of PBC is due to increased bile acids is probably too simplistic. Not all patients with high bile acids itch and the converse is true. The honest truth is that the answer is more complicated than that and that there may be mediators that the cholestatic liver (liver with diseases such as PBC) overproduces or under-metabolizes that play a role.
8. There's been some information that the itching may actually be caused by the body's creation of an overabundance of Opiates, rather than Bile Acid, in the bloodstream. Do you have knowledge of this?
Answer
Opiates have been implicated and an antagonist of the opiates naltrexone can be used, however, success is mixed.
9. Is there any specific liver function test an indicator as to who should have the itching, and to what extent (eg. AST, GGTP, etc.)? And, some of us have been on the Questran/Cholestyramine powder, with no relief. Why would it help some and not others?
Unfortunately the best marker for itching at this time is the itching itself. Because the itching in PBC is probably multi-factorial, different agents work in different patients with varied rates of success. I even find that for any one patient what may have worked once does not work later in the disease.
10. Is any research being conducted in the specific area of itching in order to provide the badly needed relief? Many of us have no symptoms other than the relentless itching, and could deal with the illness much better if there was relief other than cold showers, antihistamines, Questran, Sarna lotion, etc. that only bring very short term relief, if any.
Answer
The answer is yes. One researcher that has notably specialized in this area and whose work I like to follow is Dr N. Bergasa in Beth Israel Medical Center in New York. In her work she gives very interesting insights to the problem.
(For our PBCers, Dr. Bergasa recently joined NY Columbia University and Dr. Worman wrote us about her. She is the leading expert on itching in PBC and will be conducting trials in the NY area (as well as other experimental trials in PBC) The PBCers will be contacted prior to beginning the project. Thanks, Linie)
11. I seem to itch worse when in the sun or exercise, while others complain their itching is worse when they try to sleep. Why would any of these make the itching worse?
Answer
Your observations are quite on the mark. High ambient temperature makesthe symptoms worse. I usually recommend cool baths and cooler sleeping quarters for my patients who itch with poor symptom control. The itching is probably more noticeable at night (you may lay in bed focusing on the itching).
12. What would cause the itching to improve, but the PBC to progress in stage? It would seem the itching would get worse as the stage progresses.
Answer
It has also been observed that as the liver fails the itching improves. Some have postulated that the PBC liver functions abnormally causing the pruritus, but as less function is available, the evil humors that cause the itching are also manufactured to a lesser degree.
June 2000
13.) What is the criteria for determining the dosage of Actigall? Is the dosage based on the person's weight? Does the dosage increase as the disease progresses? Is Actigall or Urso recommended for those in the final disease stage, or is it stopped after a certain stage of te disease?
Answer
Dose levels are usually 12-15 mg/kg, adjusted to make the dosage easy and to toleration of the drug. Obviously, the earlier the treatment starts the better. I generally do not start ursodeoxycholic acid treatment at the end-stage. I know that some investigators are looking at higher doses and at combinations with methotrexate.
14.) Is a lack of iron in the blood common with PBC ? Is low count of hemoglobine and hematocrite related to the PBC? Can iron injections (Fercayl) cause a problem? Would it be better to have them intravenous?
Answer
Iron deficit may be due to many problems in the course of PBC but generally not directly from PBC. I would follow your doctor's advice regarding how to replace it
15.) Is "benign essential tremor" (shakiness of hands & voice) connected in any way to PBC. Medication for this condition is Topiramate (topamax). I understand the medication is metabolized mainly by the kidney and usually the person is on it indefinitely. Could this medication cause problems in the liver?
Answer
Topamax can be poorly metabolized in liver disease. However, this is not an area in which I am an expert and your treating clinician should be consulted about this issue. Benign essential tremor can coexist with PBC but I am not aware of any links between the disorders.
16.) I have heard some people taking Carnitor to help with the fatigue. Can you give me some information about what this drug does and what the indications are for its use? Who should take it? Do you have suggestions for the PBC fatigue?
Answer
Carnitine metabolism has been found to be abnormal in patients with PBC. However, I know of no study that links supplementation of Carnitine to improvement in fatigue. The leading theories about the fatigue of PBC include endocrine impairment, serotonin neurotransmitter abnormalities and possible auto-immune effects.
17.) How long would you estimate a woman age 45 diagnosed with PBC stage 4 (by biopsy) stay healthy enough before requiring a transplant? Which would more likely increase the chances of being listed for transplant, symptoms or increased labs?
Answer
I unfortunately do not have enough information to answer that. Mathematical scores for PBC usually require knowledge of PT, Bilirubin, age and use of diuretics. The rule of thumb you should use is if you already have cirrhosis (stage 4), jaundice and decreasing albumin you will need a transplant sooner than if you don't have any of these, but please ask your doctor with all the specifics on hand.
18.) How important are elevated Alk Phosphate levels in predicting the progression of PBC? Can the disease still be progressing even if LFT's are back down to normal ranges? If taking Actigall or Urso could the labs drop down to normal range, yet the disease still progress and symotoms increase?
Answer
Alkaline phosphatase is almost always elevated in PBC (it is related to the bile ducts, which are the injured part of the liver in PBC) Ursodeoxycholic acid prolongs survival without transplant but does not arrest progression. Unfortunately, the hallmark of the disease is a period of time with relatively normal numbers (this varies) followed by relatively rapid decline in liver function, so no, normal numbers do not guarantee a good long term outcome.
