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Dr. Alfred Baker Answers Our Questions
Questions & Answers
1.) Some Drs. consider PBC to be a systemic disease and it is not a coincidence that many of us also suffer from other autoimmune diseases/conditions. For e.g. colitis, IBS, arthritis,sojgrens etc. What are your thoughts?
Answer
PBC is generally regarded as a multisystem disorder, or a disease that involves several different organs and systems in the body. The liver is most commonly involved, but patients frequently have Sjogren’s syndrome (dry mouth and dry eyes due to inflammation of the lacrimal and salivary glands that moisten the eyes and mouth along with a disorder such as rheumatoid arthritis), inflammation of the thyroid gland, and difficulty with swallowing because of involvement of the esophagus. Some patients may have pancreatic involvement, but this usually causes no symptoms. Occasionally patients may have scleroderma, a disease that causes thickening of the skin. However, the irritable bowel syndrome and various forms of colitis are not ordinarily thought to be a part of the multisystem involvement that often characterizes PBC.
2.) What can you tell me about crytoglobulins? Some say that crytoglobulin are associated with PBC causing the aches in our legs and ankles. Crytoglobulins cause arthritis like symptoms. What are your thoughts on this subject.
Answer
I believe the questioner is asking about cryoglobulins rather than “crytoglobulins”. Cryoglobulins are large complex proteins that form in the bloodstream in patients with a variety of diseases, and some studies have identified cryoglobulins in some PBC patients. Cryoglobulins are thought to cause tissue injury at least in part, by physically interfering with blood flow in small vessels in the nerves, kidneys, lungs, and joints. There is little evidence that cryoglobulins contribute to the joint and leg pains experienced by many PBC patients.
Certainly, many PBC patients experience the kind of discomfort to which you are referring. The cause is unknown but may relate to the multisystem involvement experienced by PBC patients in a way that we do not yet understand.
3.) Do you think those with PBC need immunizations for Hepatitis B & C? How about flu shots?
Answer
I recommend immunizations against Hepatitis A and B for my PBC patients who are not immune to these diseases. Infection with Hepatitis A or B might have more serious consequences in a patient with underlying liver disease such as PBC. Both vaccines are quite safe. Influenza vaccination also seems appropriate, particularly for older patients with PBC, for those with more severe liver disease, and for those with additional medical disorders such as heart disease or lung disease.
4.) In reading many of the digest notes from other PBCers, I see that often their LFTs go down into the normal range after starting Actigal or Urso. My LFTs, after 10 1/2 years of Actigal have never been in the normal range although my bilirubin continues to be in the normal range and my only symptoms are Sjorgen's and mild itching and arthritis. Is this what is considered normal for those with PBC?
Answer
Ursodeoxycholic acid has been shown to delay the need for transplantation and perhaps to improve survival in several controlled trials. The beneficial effect is probably the greatest in individuals whose liver chemistry tests show the most improvement. However, several studies suggest that a patient with a normal serum bilirubin has a rather good prognosis, although perhaps not so good as an individual whose liver chemistry tests are entirely normal after treatment. For patients who do not have an optimal response to ursodeoxycholic acid, additional treatments may be available, particularly by way of ongoing clinical trials. Patients who have continuing symptoms and abnormal liver chemistry tests related to PBC should consult their physician about the need for additional evaluation and the possibility of further treatment.
5.) Are there specific blood tests we should have to determine certain vitamin deficiencies? Can you explain those that PBCers should be concerned with.
Answer
Patients with PBC may develop deficiencies of fat soluble vitamins, vitamins A, D, E and K. Proper absorption of these vitamins depends on adequate quantities of bile salts, secreted by the liver into the intestine, to aid in the digestion and the absorption of the fat components of the diet. In PBC bile salt secretion may be limited leading to decreased absorption of fat soluble vitamins and inadequate circulating levels.
Vitamins A, D and E can be measured in the serum, and if the levels are inadequate supplements can be administered. Vitamin K is not ordinarily measured directly but is rather assayed by measuring the prothrombin time; if this test is abnormal vitamin K levels can be restored by supplementation. Patients should remember that excessive quantities of fat soluble vitamins may be toxic, so supplements should be taken only to bring levels into the normal range.
6.) Some with PBC are diagnosed merely by a routine lab test. Is there a theory that suggests there are many people with PBC, much more than originally thought years ago? Can those who are asymptomatic, and unaware that the disease exists live a normal life and life span without any problems? Is it the norm in medical practice today to treat the asymptomatic patient?
Answer
Certainly, there are a number of patients with PBC who have no symptoms and for that reason have not come to medical attention. However, the wide availability of screening chemistry tests has resulted in the diagnosis of increasing numbers of patients while they are still asymptomatic. PBC can be a very slowly progessive disease, even in patients with symptoms. Thus, some patients may go for many years without having significant problems with their disease and a few may live their entire lives without difficulty. More patients with asymptomatic disease are likely to live longer without symptoms and perhaps to survive throughout life without difficulty. We have no studies to tell us specifically what the numbers are in any of these categories. Patients with PBC should remember that each individual’s disease progresses uniquely and that a good quality of life is possible for many years.
Question 7. I understand that calcium supplements can bind other meds if taken together. Should calcium be taken at mealtime with actigall or should one wait two hours between the two? Is there a difference between calcium carbonate and calcium citrate as far as the timing is concerned?
Answer 7
Moderate doses of calcium supplements do not interfere sufficiently with the absorption of other nutriuents. Large doses might conceivably inhibit the absorption of some drugs such as Tetracycline. Doses that are ordinarily prescribed range from 1,000 to 2,000 mg per day and do not interfere with absorption. There is no clinical difference between calcium carbonate and calcium citrate in this regard.
Question 8. What is the best method of HRT for PBCers? ESTROGEN by mouth, is it safe to use with PBC?
Answer 8
Estrogen supplementation is generally safe for patients with primary biliary cirrhosis. On occasion, a patient taking estrogen supplements by mouth may have mild worsening of some liver chemistry tests, so I generally recommend repeat liver chemistry tests a few weeks after beginning such medications.
If the change is slight, it is probably reasonable to continue oral estrogen therapy. Estrogens given by patch are almost always well tolerated and represent another alternative for taking this supplement.
Question 8a. Is there a higher incidence of breast cancer in PBCers. Is there data showing this? If so, what could be a cause?
Answer 8a
There is some limited evidence to suggest that the incidents of breast cancer may be somewhat increased in patients with primary biliary cirrhosis. In my judgment this relationship is not clearly established, and the mechanism is unknown. Perhaps the cholestasis which accompanies primary biliary cirrhosis limits the excretion of some toxins by the liver that might contribute to the development of breast cancer.
Question 9. Is it ok to take "Cold Eeeze" Lozenges that contain mostly zinc?
Answer 9
Zinc containing lozenges, when used in moderation according to package directions, are safe for most patients with primary biliary cirrhosis. Excessive use of these lozenges could conceivably result in a build up of zinc in the blood and prolonged administration say beyond a week or so could have similar effects.
Question 10. Can PBCers drink non alcoholic beer? Usually they contain less than 0.5% alcohol in 100cc. If the answer is yes, how often can we drink it? Almost every day, for instance?
Answer 10
Patients with many types of mild liver disease, including primary biliary cirrhosis, can probably safely consume small quantities of alcoholic beverages. Patients with more advanced liver disease, those with esophageal varices or with edema and ascites, should probably not drink alcoholic beverages at all. Patients with Hepatitis B and C should avoid alcohol consumption completely because alcohol can be additive to the harmful effects of the viruses on the liver. Low alcohol content beers are therefore safe for many patients with primary biliary cirrhosis. Because of differences in the severity of liver disease among patients and the details of the medical regimens, I suggest that you consult with your physician about how much alcohol it would be safe for you to consume.
Question 11. Are there any symptoms to be aware of which would indicate pulmonaryor portal hypertension?
Answer 11
The main symptoms of portal hypertension that make patients aware of its presence include esophageal variceal bleeding and the development of ascites and edema. These manifestations of liver disease result in part because of scarring in the liver which prevents blood from flowing normally through the liver from the intestine back to the heart. As a consequence of the increased pressure in the intestine, esophageal varices develop as blood finds an alternate way around the scarred liver. This increase in pressure results in the accumulation of ascites. Physicians often detect evidence of portal hypertension before the patient develops symptoms; an upper GI endoscopy can identify esophageal varices or an ultrasound examination of the abdomen can detect ascites before the patient actually knows about the problem.
Pulmonary hypertension is an uncommon complication of primary biliary cirrhosis, and shortness of breath is the main symptom. In my experience shortness of breath is much more frequently due to the fatigue that accompanies liver disease or to ascites which complicates the course of liver disease than to pulmonary hypertension. An ultrasound examination of the heart and pulmonary vasculature can usually identify pulmonary hypertension in affected patients.
Question 12. I was told Spacial Relations Syndrome is due to a build up in toxins.Can you clarify this as I have never read anything about a balance problem as a side effect of PBC.
Answer 12
I am not aware of any specific relationship between primary biliary cirrhosis and disorders of spacial relationships. Patients who have early hepatic encephalopathy may experience some difficulty in maintaining balance, and this can be treated by appropriate management, which often includes the administration of Lactulose. However, there is no specific brain disorder that accompanies primary biliary cirrhosis.
