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Dr. Nancy Bach Answers Our Questions
October 1999
Question & Answers
1.) Several of our members have made comments about Tylenol being very harmful to the liver. In your oppinion, is Tylenol considered dangerous to our livers? Is there an other pain relievers that would be better in your oppinion, & what kind?
Answer
While there is no question that excess doses of Tylenol can be deadly, in its recommended dose Tylenol is probably one of the safest medications available. In therapeutic doses, tylenol does not cause liver injury. People who consume alcohol on a daily basis (several drinks/night) have a lower threshold for what is considered therapeutic, so people who are chronic alcohol users should probably avoid Tylenol. This does not apply to most PBC patients (or other non alcoholic liver diseases). I generally recommend limiting Tylenol to <6 regular strength tablets per day (less than 2 grams/day). In terms of safety, for pain management, I prefer Tylenol over drugs such as aspirin or Motrin, Advil, Aleve. Tylenol however is not always the most effective pain medication.
2.) I tend to get nasal and chest congestion that is allergy related. It doesn't happen often, but when it does I take Allegra and Entex, as well as using a Vanceril inhaler. Are these meds safe for PBCers? If not, what is acceptable?
Answer
Allegra (an antihistamine), Entex (a decongestant) and Vanceril are relatively safe drugs for patients with PBC. Drugs similar to Allegra can cause irregularities in the heart rhythms of patients with liver disease. This is because of the way the drug is metabolized or broken down. The medications that you are referring to are unlikely to be a problem in patients with liver disease, however, any medication has the potential to cause toxicity to the liver. You should therefore always discuss medication use with your doctor.
3.) If blood type is A negative, does that mean when, and if, a person needs a transplant, the donor's blood type needs to be A negative, or is type A sufficient for a match. Also, would Liver Transplant still be considered when the patient has a congenital absence of the right kidney, provided the serum creatinine is within normal limits?
Answer
Several factors are important in matching a recipient and donor for transplant. One is liver size. The other is the recipients blood group. Matching a recipient with Blood type A to a donor with type A is usually sufficient. It is possible to cross match certain blood types, although results have not been as good as those with a perfect blood group match, and it is generally not done.
Different centers have different guidelines about accepting patients as potential liver transplant candidates. It depends on the centers experiences and their expertise in general. I cannot speak for other centers, but congenital absence of a kidney would not necessarily be an exclusion for liver transplant at our center (Mount Sinai Medical Center, New York).
Questions 4 & 5 are conbined answer
4.) Is there a direct impact on one's fatigue level if the thyroid as well as LFT's are in the high ranges? Does any one blood test indicate fatigue? Do symptoms of fatigue and general malaise correlate with stages of the disease?
5.) Can you explain why some sufferers of pbc have chronic fatigue and others are totally unaffected and can carry on as normal, and yet they have or have had extreme itching?
Answer to 4 & 5
I thought these two questions could be answered together.
Thyroid disease, independent of liver disease, may cause fatigue, and thyroid disease is more common in PBC than the general population. So, the answer to the first part of the question is a definite yes.
However, no blood test can predict fatigue in patients with liver disease. In fact, the level of elevation of the liver blood tests has no bearing on fatigue.
As well, fatigue can occur during any stage of the disease.
Fatigue is reported in 60 to 90 percent of patients with PBC and the cause is unknown. The fatigue may be intermittent,or it may occur for prolonged periods of time.
The subjective nature of the complaint has made it difficult to scientifically study this symptom. The same thing goes for itching. In late stages, the itch may persist, although like fatigue, itching can also be one of the earliest manifestations of the disease and it may or may not persist with or without treatment.
6.) I would like the Drs comments on the connection (if any) between PBC and thrombocytopenia.
Answer
Any liver disease, including PBC, may cause thrombocytopenia (a low platelet count). Platelets are blood cells that help you clot. When blood flow to the liver is impeded by damage in the liver, platelets may start accumulating in the spleen. This causes a lower than normal count of the platelets in the blood. Usually because the remaining circulating platelets function normally, and you do not need a normal platelet count to clot, clotting is not a problem. In rare cases, the low platelet count is due to an autoimmune disease called autoimmune idiopathic thrombocytopenia. It has been reported in PBC patients, but is pretty uncommon.
February 2000
Question
7.) What suggestions do you have for maintaining weight? My mother has PBC and has steadily been losing weight since her diagnosis (Stage 4) 2 years ago.
Answer
Weight loss is not typical of PBC. However, PBC may be associated with other conditions that cause weight loss. For example, depression, which sometimes occurs in patients with liver disease, can lead to weight loss. Another less common cause of weight loss is celiac disease. This autoimmune disease is infrequently associated with PBC and can result in malabsorption and weight loss. Your mother’s weight loss should be investigated further to see if there is another underlying cause.
8.) Are there guidelines for prescribing thyroid medication for PBC patients with low thyroid that are any different from such patients without PBC?
Answer
Thyroid disease is often associated with PBC, and is treated the same as it would be for a non- PBC patient. Though the two diseases are associated with each other, they are treated independently.
9.) What might explain a Direct Bilirubin of zero in a PBC patient and an elevated Indirect Bilirubin?
Answer
Most bilirubin is produced by the breakdown of hemoglobin (red blood cells). Total bilirubin can be divided into direct and indirect bilirubin. Put simply, the direct bilirubin is the bilirubin that has been processed by the liver (conjugated). An elevated indirect bilirubin usually reflects either an increase in the breakdown of red blood cells with increased release of bilirubin or defective processing of the bilirubin before it reaches the liver. Slight elevations in the indirect bilirubin are generally not of great significance.
In patients with PBC, red blood cells may break down as they pass through an enlarged spleen. This may result in the increased production of indirect bilirubin.
A less common cause is an autoimmune disease resulting in the breakdown of red blood cells, and subsequent elevation of the indirect bilirubin. In the general population, the most common cause of an elevated indirect bilirubin is Gilberts syndrome. This is a benign condition resulting from the defective processing of bilirubin.
10.) Is it possible to have an occasional glass of white (or red) wine after being diagnosed with pbc or should one avoid alcohol in any form, including any that may be used in cooking recipes? Thank you for your input.
Answer
There is little data regarding alcohol use by people with PBC. Because alcohol does not play a role in the development of liver disease in PBC, it is probably reasonable to have an occasional glass of wine. Women (the population most commonly affected by PBC) are more susceptible to alcohol related injury and therefore daily or heavy consumption of alcohol is not wise. In chronic hepatitis C, alcohol seems to play an additive role in the development of scarring in the liver. It is unclear whether this information is at all applicable to patients with PBC. Since alcohol generally evaporates when used in cooking, it shouldn’t be a problem. Patients taking methotrexate need to be particularly careful because alcohol increases the likelihood of scarring in the liver.
11.) The insert for Actigall says that if there is coughing you should notify your doctor immediately. Is coughing a side effect of Actigall or an association with PBC? If so for either, why?
Answer
In rare cases, patients with PBC may develop a lung problem that can cause coughing and shortness of breath. I know of nothing to suggest that actigall causes or is associated with the development of a cough.
12.) Should aspirin and aspirin-related products be avoided? I usually take Ibuprofen.
Answer
Aspirin, aspirin related products and non-steroidal drugs (including ibuprofen) can be used in PBC, but should be monitored and used judiciously. These medications may cause ulcers, and patients with liver disease are more prone to develop ulcers that are more difficult to treat. These drugs may also interfere with the function of platelets; platelets are important to the clotting process. Lastly, those with advanced liver disease may develop kidney problems and these drugs may have adverse effects on the kidneys.
April 2000
13) I attempted to give blood thru the Red Cross. I was denied. They could not find PBC in their reference books and their medical experts had never heard of PBC. They said that they deny people with auto-immune diseases. Do you know of any reason why we can't give? I am also registered as a bone marrow donar, do you know of any reason why I couldn't donate? Would either of these procedures be bad for my PBC?
Answer
Blood and bone marrow donations are generally not accepted from individuals with liver disease or other medical illnesses, for that matter. I can't speak to your situation specifically. I would suggest you contact the medical expert associated with your local Red Cross center. My guess is that they denied your blood because while there is no evidence for danger in transfusing blood from a patient with PBC, there is no evidence or experience to support its safety. You are correct that PBC is not infectious and that there is no evidence that autoimmune diseases are transmitted by blood. Your blood should therefore be safe. In general donating blood should not be deleterious to your liver disease. Those with medical conditions or more advanced disease, however, might not do well having a unit of blood removed. Also many patients with autoimmune diseases are on medications that would exclude them as a donor. Those accepting the blood can not discern the extent of your liver disease and thus their refusal is, in part, to protect you. Thus the practice guidelines have been formulated to decline anyone with liver disease or certain chronic medical conditions.
14) Actigall does help my itching;does it do a better job of this if my pills are spaced throughout the day instead of taking them all at once or just twice a day?
Answer
Most recommend dividing the dose of Actigall (ursodeoxycholic acid) based on the dosing schedules that were used in the large studies proving benefit from the drug. In the only study I know that specifically looked at dosing schedules, it appeared that taking the drug once daily was as effective as dividing the drug up (of course assuming the total daily dosage was the same). The benefit of once a day dosing is perhaps increasing compliance with the medical regime.
15) Since I'v been taking prevalite I have been experiencing musle aches and pains in my back, hips and knees. Could this be a side effect of the drug? I take two packets a day in the morning for the itching. If this is a side effect of the drug, would you advise your patient to stop taking it? Is there something you would prescribe?
Answer
Prevalite (cholestyramine) should not cause the symptoms you describe. However, any drug is capable of causing any side effect in a given individual. Under your doctor's supervision, you might want to discontinue the therapy for a week or so and see if the symptoms resolve. If they do, I would recommend testing the medication again in a few weeks to see if the symptoms are truly due to the Prevalite.
16) Could you please tell me why some of the PBCers have a huge hard stomach when there are no ascites present?
Answer
Having seen several hundred patients with PBC, it has not been my experience that PBC patients are any more likely to have a "huge hard stomach", more so than patients of similar age and sex. I know of no study that would suggest that characteristic either. About 50% of those with PBC have an enlarged liver. This should not cause what you describe.
17) If you have diseases such as gerd, sojgrens, arthritis etc., that seem to be triggered with the onset of PBC, do they go away or diminish after transplant?
Answer
To the extent that certain diseases associated with PBC would respond to the immunosuppression used after a transplant, some symptoms might resolve. For example, if one had rheumatoid arthritis, prednisone used after the transplant might ameliorate some symptoms. Gastroesophageal reflux disease (GERD) is not linked to PBC and would probably not resolve after transplant. I don't think anyone has looked specifically at Sjogrens syndrome; perhaps we should. I would guess the symptoms would not change since diseases associated with PBC are just that, associated but not caused by PBC, and they tend to run their own courses.
18) What do you generally prescribre to your patients for itching? Do you advise them to avoid the sun and hot baths?
Answer
I usually try antihistamines to start, though if the itching is truly liver related, they are not particularly helpful. Sometimes Benadryl or Atarax taken before bedtime is enough to take the edge off and allow one to get a good nights rest. The standard therapy for the itch is cholestyramine. Though the pathway that makes this medication work isn't clear, it is successful in most cases. While hot baths may exacerbate the itch, I have found UV light and sun to be helpful. Occasionally the itch is not related to the liver disease and moisturizers may be helpful. There are several other second line medications that can also be helpful to varying degrees.
