Ask the PBC doctor

DISCLAIMER: The following is intended for general information purposes only. It is not intended to be comprehensive. It does not in any way constitute legal or other professional advice, and should not be relied upon as such. The reader is cautioned to consult their own physician and other experts for advice regarding specific health concerns. The Canadian PBC Society is not responsible for the accuracy, completeness, or any action taken on the basis of the information mentioned herein. It is not intended to substitute for and/or supersede one's own physician's advice.

Symptoms and Symptom Management

• I was diagnosed with PBC a year ago. Lately I find I sleep a lot in the day as well as at night. Will this continue? [ANSWER]

Fatigue occurs in up to 80% of patients with PBC. However, on diagnosis up to half of the patients that we see in clinic have no symptoms because they are generally picked up on routine screening with abnormal liver tests. Patients with fatigue often have to sleep during the day or find they have little energy to do much in the evenings. This symptom is usually persistent, although some patients do occasionally respond to medications in drug studies that appear to perk them up. However, this response only occurs in a handful of patients and the new therapies, such as obeticholic acid, appear to have no effect on fatigue. Therefore at this time, there are no specific drugs associated with the treatment of fatigue in patients with PBC.
— Dr. Andrew Mason, 2016

• Is nausea a common symptom of PBC and can anything be done about it? [ANSWER]

Nausea is not a common symptom of PBC. Nausea is usually associated with intestinal problems and a common side effect of taking medications. Fortunately, both Ursodiol and Cholestyramine seldom cause nausea and both drugs are generally well-tolerated. Other medications may cause nausea, especially on an empty stomach. For example, multivitamins can cause a degree of nausea if not taken with food. Common intestinal diseases associated with nausea include peptic ulcer disease, irritable bowel disease as well as other inflammatory bowel conditions. If nausea is a significant problem, it should be discussed further with your doctor.
—Dr. Andy Mason, 2006 (reviewed 2016)

• Do problems with clotting start in the early stages of PBC? [ANSWER]

It is unusual for clotting problems to be present in the early stages of PBC unless it is linked with another disease where clotting is a problem. Clotting problems generally start when patients have cirrhosis, which is associated with low platelets as well as a diminished production of clotting factors from the liver. However, sometimes patients with PBC can have problems with absorbing their fat-soluble vitamins – A, D, E and K. Vitamin K deficiency is associated with lack of production of clotting factors. So if patients have a lack of vitamin K early on in their disease, they could theoretically develop clotting problems. Once again, I recommend a multivitamin for all patients with PBC to avoid these problems.
—Dr. Andy Mason, 2016

• Is hepatic encephalopathy of PBC corrected or improved following successful liver transplant? [ANSWER]

Yes, pretty much in all patients. However, there are other things to consider as well. Patients may have problems with concentrating or thinking due to conditions unrelated to their liver disease and PBC. If this is the case, a degree of neurological disease may continue following transplantation. Also, following liver transplantation some of the drugs used for immunosuppression can make patients concentration a little fuzzy.
—Dr. Andy Mason, 2016

• Why do patients experience aching joints? [ANSWER]

PBC and autoimmune diseases in general, have several features in common. For example, patients with PBC may experience symptoms that patients with other rheumatological diseases experience as well. Patients with PBC and Sjogren’s syndrome experience dry eyes and dry mouth. Patients with PBC also experience aching joints and aching muscles that patients with rheumatological diseases experience. In fact, this is not uncommon in patients with other forms of liver disease such as hepatitis C virus infection, hepatitis B virus infection and hemochromatosis (an iron storage disease) who will also experience aching joints. As long as the pain is not too severe and the joints are not swollen or deformed, we usually recommend taking Tylenol in regular doses to treat aching joints. If the joint problems are severe, we often look for other causes, such as rheumatological disease.
—Dr. Andy Mason, 2016

• What causes brain fog with PBC? [ANSWER]

Dr. Mark Swain at the University of Calgary works on the neurological changes associated with cholestatic disease. There are several complicated mechanisms involved with the brain fog and fatigue associated with PBC. We understand now that, like other liver diseases including hepatitis C virus infection, the problem is not just isolated to the liver. Indeed, some patients with near-normal liver function tests can have quite severe fatigue and brain fog as well. It is currently not known what causes this. Recent studies from the University of Newcastle show that some patients may develop changes on MRI brain scans suggesting structural changes in the brain may be causing the fatigue and other neurological symptoms that may occur in PBC patients.
—Dr. Andy Mason, 2016

• Is unsteadiness on the feet a common occurrence with PBC and, if so, why does it occur? [ANSWER]

The group in Newcastle in the United Kingdom is currently studying this. It appears that some patients with PBC have swings in their blood pressure. Sometimes their blood pressure is high and sometimes their blood pressure is low. This means that when you stand up too quickly, the blood supply to the head can be reduced and patients can become unsteady on their feet and dizzy. It is not known what causes this change in the nervous system controlling the blood pressure; and this is subject to research. Unsteadiness on the feet may occur for several other reasons, and if this is becoming a problem, then patients should see their doctor to be worked up for other causes of unsteadiness that include neurological or potentially cardiac causes or disease.
—Dr. Andy Mason, 2016

• How would the itching related to PBC be described in layman’s terms by a patient? I am experiencing itchy burning and tingling inside my body, particularly on my upper arms, one arm being worse than the other, which has been leaving bruising from scratching in the night. [ANSWER]

Itching in PBC is truly an individual experience. What you describe is not uncommon – a burning, tingling sensation in the skin. The itching associated with PBC is often felt on the soles of the feet and palms of the hands, but may be experienced on any part of the body. Everyone’s experience differs. If you are itching to the point of scratching at night to cause bruising you should contact your treating physician to explore anti-itch therapies which can often really help.
—Dr. Mark G. Swain, 2016

• Is there a relation of rectal bleeding to PBC? [ANSWER]

In general, no, unless there are hemorrhoids which may rarely be related to liver disease. In advanced PBC in people with cirrhosis and portal hypertension, rarely rectal varices can develop. If this happens, and they bleed, the bleeding can be quite dramatic and warrants a visit to the nearest hospital emergency.
—Dr. Mark G. Swain, 2016

• Is there a link between pruritus and restless leg syndrome and PBC? Is there a diet or supplementary to alleviate this? [ANSWER]

Not directly, but certainly pruritus may make it worse. For restless leg syndrome a number of anecdotal reports have suggested that supplementation with vitamin D (most PBC patients should typically be taking vitamin D 2000 IU/day for bone health anyway), vitamin E, calcium and magnesium may be beneficial.
—Dr. Mark G. Swain, 2016

• Are telangiectasias related to PBC? [ANSWER]

Telangiectasias can be related to PBC, but are also common in people without liver disease.
—Dr. Mark G. Swain, 2016

• How can one pace daily activities and preserve stamina and energy when carrying out a full time job and not feel exhausted at the end of ones day? Will it make the disease progress faster working full time and being fatigued all the time? [ANSWER]

Very good question and an important issue for patients with PBC and who experience fatigue. Given that the cause of fatigue in PBC is unknown specific recommendations are not possible. However, typically eating reasonably and keeping ones weight under control, trying to rest when the situation allows and getting a good nights sleep, and keeping as aerobically fit as possible, are mainstays in the approach to fatigue. Try to take a nap when you first get home at the end of the day or you could even try meditation. Make sure that your doctor has ruled out other common causes of fatigue that are specifically treatable (eg. anemia, renal impairment, thyroid disease, diabetes). Sometimes, a full-time job is not tenable and a modification of your work schedule may be needed (ie. part time, working some from home). Fatigue actually does not correlate with severity of disease in PBC patients in general, and there is no evidence that working while being fatigued impacts on the progression of the liver disease.
—Dr. Mark G. Swain, 2016

• What suggestions do you have for a woman with PBC going through severe menopausal symptoms such as: hot flashes, sleeplessness and mood swings. [ANSWER]

HRT is safe but may increase bilirubin, which returns to normal off HRT.
—Dr. Jenny Heathcote, 2006 (Reviewed 2016)

• Is swimming in a chlorinated pool OK? [ANSWER]

Yes.
—Dr. Jenny Heathcote, 2006 (Reviewed 2016)

• I was diagnosed with PBC approximately six years ago, but have had symptoms going back 30 years. I am current 50 years of age. According to my physicians, my blood work liver function tests keep coming back perfect (normal bilirubin, Alk phos, etc.) but the fatigue and energy level that I am experiencing is almost debilitating. I am on Ritalin in order to be alert at work, though the effect seems to be waning as the fatigue increases. I really care about nothing anymore
— I just want to curl up and sleep all of the time. I just want to be left alone, as nobody understands that I am sick. Does the fatigue extent increase with the stage of the disease, and even though the blood work returns normal, is the disease progressing? I don’t remember what stage it was for my biopsy; either Stage 1/2 or Stage 2/3 though my understanding of this is that it is not necessarily an accurate measurement when doing needle core biopsies. [ANSWER]

There is no clear link between the severity of liver disease in PBC and the severity of fatigue (or tiredness) that people experience. This means that even though your liver may be functioning completely normally and your PBC is under good control and not progressing, you may experience severe fatigue. Unfortunately, there are no treatments that have been well studied for fatigue, and so the long-term safety of medications such as Ritalin have not been well-studied in PBC. It is also important to remember that there are a lot of causes of fatigue. Depression, thyroid disease and many other medical diagnoses can cause fatigue. It is important for the doctor to rule out these things too, to make sure you get the right treatment for your fatigue. A liver biopsy in PBC is fairly accurate, though now liver biopsies are rarely done because blood tests and a special ultrasound scan called a “fibroscan” can check the level of scarring in the liver.
—Dr. Angela Cheung, 2016

Related Disorders and Causes

• Are there any Irritable Bowel type symptoms associated with PBC? I have read that one does not get PBC without some other so-called auto-immune disease as well. Has this been found to be true? [ANSWER]

There are no irritable bowel type symptoms associated with PBC. Irritable bowel symptoms such as bloating, abdominal discomfort, mild diarrhea or constipation, can be common in healthy people. PBC can be associated with celiac disease, which is a type of autoimmune disease, which can be without symptoms, or may cause symptoms such as bloating, abdominal discomfort diarrhea or weight loss. PBC is often associated with other autoimmune diseases such as thyroid disease or Sjogren’s, but not always.
—Dr. Angela Cheung, 2016

• Is there a higher incidence of kidney stones among PBC patients? [ANSWER]

I am not aware of any published reports on an increased frequency of kidney stones in PBC patients. However, theoretically, PBC patients do have a higher incidence of something called distal renal tubular acidosis (a kidney acid-base defect) and this has been associated with an increase in renal stone formation.
—Dr. Mark G. Swain, 2016

• I was found to have PBC 18 months ago. With your estimates of cases in North America of 1:50,000, and I’m in the 10% group of PBC (male), I feel really special (1:500,000)! The only things that are in my background is a case of Hepatitis A in 1978 and treatment for ADHD with methlyner since 2000. Both of these things relate to the liver in very small ways
— could either be considered a catalyst? [ANSWER]

It is unlikely that either hepatitis A or the treatment for ADHD with Ritalin are triggers for PBC. Both environmental factors are quite common, whereas PBC is rare. Therefore, it is unlikely that either of these could be considered a catalyst for PBC.
— Dr. Andrew Mason, 2011 (reviewed 2016)

• Has there been any indication that nail polish may either increase your chances of developing PBC or of causing PBC? [ANSWER]

This is a very interesting and controversial question. In a patient interview study from Dr. Eric Gershwin’s group in California (published in Hepatology, 2005), exploring potential lifestyle risks for PBC, they identified frequent use of nail polish as slightly increasing the risk for the development of PBC. Importantly, this increase was very slight.
—Dr. Mark G. Swain, 2016

• Are there any cases of reversal of liver damage that are documented? [ANSWER]

The reversal of liver scarring in PBC by therapy is controversial. Dr. Marshall Kaplan in Boston has described patients in the literature who appear to have had remarkable reversals of liver scarring when treated with Urso plus methotrexate. However, these findings are not broadly accepted, and in general the liver scarring which occurs in PBC should be viewed as irreversible. The goal of current therapy is to stop the liver scarring associated with PBC from progressing.
—Dr. Mark G. Swain, 2016

• How does PBC affect my body in combination with my other auto immune diseases such as Graves, Diabetes, Arthritis and Ulcerative Colitis,? Which specialist could best help me to address the problem of these conditions plus the medications that have to be taken which might impact PBC? [ANSWER]

Each autoimmune disorder described affects the body in different ways. The main problem with having PBC and liver disease is that it affects the absorption of fat-soluble vitamins, and therefore patients with PBC are more likely to develop thinning of the bones as they do not absorb the calcium and vitamin D. PBC may also effect energy levels and cause itching and dry eyes with dry mouth. The other diseases such as Graves’ and diabetes may have some affect on heart function, and patients with diabetes can get blood vessel disease as well as kidney disease if their control of sugar is not good. Patients with ulcerative colitis may get disease of the skin with rashes, as well as arthritis. Generally speaking, if you have a specific disease that is difficult to manage, it is always worth seeing a specialist associated with that disease such as a rheumatologist for arthritis, and endocrinologist for diabetes and Graves’ disease, a liver specialist for PBC, or a gastroenterologist for ulcerative colitis. However, if the diseases are well controlled, it may be simpler and more coordinated to see one physician who is familiar but not an expert in all these diseases.
— Dr. Andrew Mason, 2011 (reviewed 2016)

Medications, Supplements and Treatment

• I was diagnosed with PBC in 1995 and take prescribed URSO 250 mg, 5tablets per day. I also ADEKs vitamin supplements of A,D,E, and K. In the past 6 months, I have started using Women’s One A Day for those aged 50 + in conjunction with ADEKs. I understand from my pharmacist that ADEKs will no longer be manufactured. My question is this: could you please recommend what in your opinion would be an appropriate course of supplements for me to be taking to ensure optimum benefit for someone of my age – almost 56? Does the current research support any particular supplementation for PBC patients? [ANSWER]

Using women’s One-a-Day for those aged greater than 50 should be sufficient for most patients with PBC. There is no proven benefit of using ADEK, and this prescription was made specifically for PBC patients without any evidence that it provided benefit. We usually recommend that patients take an age-appropriate multivitamin, as well as supplements to prevent thinning of bones. This includes vitamin D 2,000-4,000 i.u. a day. Calcium supplements are recommended for treating or preventing thinning of bones and osteoporosis. Discuss taking calcium with your pharmacy as different formulations provide different amounts of elemental calcium. Excessive calcium supplementation should be avoided, however, due to the risk of buildup of calcium in blood vessels and the development of kidney stones.
— Dr. Andrew Mason, 2016

• Is there a safe cholesterol medication to take? [ANSWER]

Most cholesterol medications are safe for people with PBC. The statins, such as Lipitor, rarely cause any changes in liver function tests. As patients with PBC have their liver function tests done regularly anyway, we can usually spot when patients are having a reaction to this treatment. Also, cholesterol medications such as fenofibrates may also be of benefit to reduce cholesterol and triglycerides in patients with PBC. The only drug that reduces cholesterol that may be bad for PBC patients is niacin, which causes a dose-dependant damage to the liver. Patients who have high cholesterol and other risk factors for heart disease such as smoking, hypertension, diabetes, or a family history of heart attacks at a young age could be considered patients for cholesterol treatment.
—Dr. Andy Mason, 2016

• What is the risk for us getting or not getting hepatitis shots? [ANSWER]

Infants in Canada get vaccinated for hepatitis B virus infection. Patients who travel to tropical countries may be exposed to hepatitis A. Patients that have sexual or exposure to blood products from patients with hepatitis B may be at risk of getting hepatitis B. It is thought that patients who already have liver disease may have worse reactions if they get hepatitis A or B. So we would recommend patients getting shots for hepatitis B and hepatitis A if travelling.
—Dr. Andy Mason, 2016

• At what T score level should a PBC patient with a low Bone Density consider a bisphosphonate a necessity? [ANSWER]

There is not an absolute T score. This score represents a variance (standard deviation) of bone mineral density and osteoporosis with severe thinning is defined as -1 to -2.5 reduction. In general, fracture risk increases 2 to 3 fold with each -1 reduction in T score. Therefore, all PBC patients should have their serum 25-OH-vitamin D levels checked as well as bone mineral density. Although a proportion of patients have normal bone mineral density, I recommend all PBC patients should to be on calcium and vitamin D 2,000-4,000 i.u. a day. to prevent bone loss. If people have a low bone density despite taking vitamin D and calcium, then a bisphosphonate treatment can be considered. We could either use the drug such as Didronal to inhibit the breakdown of bone, the stronger bisphosphonates such as Fosamax or pamidronate infusions. Hormone replacement therapy is no longer recommended for patients with progressive liver disease.
—Dr. Andy Mason, 2016

• What are the chances that increasing by 50% to a max Calcium (1000mg and Vit D 500mg) daily supplement will slow further resorption AND increase bone mass without the addition of a bisphosphonate for the average senior PBC patient? [ANSWER]

In the first instance I would recommend taking elemental calcium 500mg (discuss different calcium medications/formulations with your pharmacy) and vitamin D 2000-4,000 i.u. per day. Following this, only monitoring with bone density would be able to determine whether this is effective in building up the amount of bone in a patient. Individuals vary, and therefore this assessment needs to be done on a patient to patient basis. However, if patients remain with low T scores within range for fracture despite increased calcium and vitamin D, I would certainly recommend a bisphosphonate.
—Dr. Andy Mason, 2016

• Do cholesterol drugs affect liver function tests? [ANSWER]

Cholesterol drugs are often required in patients with PBC. This is because when bile ducts are blocked, the cholesterol cannot get out of the liver and it tends to build up in the body. We know that cholesterol can lead to problems with coronary artery disease and therefore we do recommend anti-cholesterol therapy for patients that are at risk for coronary artery disease, heart attacks and strokes. The drugs that are referred to as “Statins”, such as Lipitor, are commonly used in the population to combat cholesterol. These medications are very safe. On occasion, they can cause an increase in liver function tests. However, as patients with PBC are monitored regularly for their liver function tests, it is usually quite safe for them to take these medications. If their liver tests become worse on the Statin, then the dosage can be modified or the drug can be discontinued. There are also some data which suggest that other lipid (fat) lowering agents such as the fenofibrates also positively impact on the progression of PBC and these medictaions are being tested in a clinical trials as they are relatively safe and can potentially improve PBC. The difference between the fenofibrates and the statins is that the fenofibrates can also reduce other fats such as the triglycerides as well as the cholesterol in the body.
—Dr. Andy Mason, 2016

• A friend with liver damage from Lyme’s Disease benefitted greatly from a ‘liver support’ powder called ‘Huan Fat Metaboliser that contained Choline, Methionine and Inositol, which, apparently, work to break down fatty proteins. Do you think that this product would be useful for PBC sufferers? [ANSWER]

It sounds like Huan fat metabolizer that contains choline, methionine and inositol that may be helpful for patients with fatty liver, in as much as choline and methionine deficiency cause fatty liver in animal models. However, there are no studies that I know of that show this in patients. I am unsure how this product would be useful for patients with PBC unless they were overweight and suffer from fatty liver as well.
— Dr. Andrew Mason, 2016

• Can we take vitamin B3? Is Niacin and Niacinamide the same thing or if different, is one safer? [ANSWER]

Niacin is a water soluble vitamin also known vitamin B3. There are different formulations including Nicotinic Acid and Nicotinamide. Nicotinamide is generally better tolerated than Nicotinic Acid and does not generally cause blushing, however nausea, vomiting and signs of liver toxicity may occur when high doses of the supplement are taken. This may be observed with doses of 3 g/day. The current recommendation is to take about 20 mg/day. Patients usually receive enough Niacin in meats, poultry, fish, cereals, vegetables and seeds. Milk also provides Niacin as well. Niacin used to be used to treat high cholesterol but due to liver toxicity, newer medications are preferred such as the “Statins”. Personally, I do not recommend additional vitamin B3 for patients more than can be found in standard multivitamin preparations. Accordingly, I advise most patients with PBC to take a multivitamin with extra vitamin D, 2,000 – 4,000 IU/day as well as calcium to avoid bone disease.
—Dr. Andy Mason, 2016

• Do you recommend vitamin B12 shots for fatigue for PBC if it’s safe. [ANSWER]

Vitamin B12 shots are safe and no toxicity or adverse affects have been associated with large doses. Vitamin B12 deficiency is estimated to affect 10 to 15% of individuals over the age of 60 and is specifically associated with pernicious anemia, which is also an autoimmune condition. Deficiency is also seen with vitamin B12 malabsorption. Both these conditions are distinct from PBC and therefore I do not generally recommend vitamin B12 shots for fatigue for PBC patients. However, up to 5% of patients with PBC can develop the malabsorption syndrome celiac disease that can be associated with low vitamin B12 levels. I would consider evaluating the levels of vitamin 12 in patients with (i) low hemoglobin, (ii) antibodies to intrinsic factor which helps with vitamin B12 absorption, (iii) signs of pernicious anemia and vitamin B12 deficiency with sore tongue, tingling in arms and legs and other neurological symptoms. If the vitamin B12 level is low – I would recommend the use of B12 shots. As part of your work-up for PBC, your doctor may arrange a gastroscopy, look for signs of inflammation in the stomach and may also check other autoantibodies associated with autoimmune conditions that are linked with vitamin B12 loss.
—Dr. Andy Mason, 2016

• Is it safe for a PBC patient to be pregnant? Should she continue to take Urso? [ANSWER]

In general, yes. If they have advanced liver disease then it may be very difficult for them to get pregnant and if they do, there may be increased problems. Stopping Urso because of pregnancy, or a plan to become pregnant, is still somewhat controversial. However, in animal studies Urso does not appear to be teratogenic. Urso is widely used to treat cholestasis of pregnancy safely, and there is a growing (albeit small) body of evidence suggesting that Urso is safe for the baby and mother during and after pregnancy in PBC patients as well. I therefore recommend that my patients do not stop Urso during pregnancy if they are currently taking it. If they are not taking Urso and they have become pregnant, or are thinking of becoming pregnant, then I discuss the potential risks and benefits of starting Urso and then work with them to help them make the right choice for their comfort with the pregnancy and their PBC going forward. Urso can be taken while breast feeding and will not harm the baby.
—Dr. Mark G. Swain, 2016

• Are asthma medications harmful to the liver? Namely, Alvesco and/or Advair. [ANSWER]

No.
—Dr. Mark G. Swain,2016

• Is there any difference between the generic and non-generic forms of Urso (UDCA) ? What about the non-active ingredients? [ANSWER]

In general, most people with PBC can use these two forms of UDCA interchangeably. The non-active ingredients are however different, and this may theoretically lead to altered pharmacokinetics (ie. rate of absorption and plasma levels) of the two Urso compounds. In addition, I have noted that some patients may experience intolerance to some of the non-active ingredients (e.g., rash, diarrhea). However, in general, given the cost differential, I typically support a trial of the generic Urso and if it is effective biochemically (ie. the liver tests fall in newly started patients, or do not rise after switching patients currently taking non-generic Urso to an equivalent dose of generic Urso) and does not cause any adverse side effects or symptoms, I continue on with the generic form.
—Dr. Mark G. Swain, 2016

• Have you heard of Lyrica and Cymbalta. I know some American PBCers are on these drugs for pain. What is your take on them? [ANSWER]

Lyrica was developed originally as an anti-seizure medication and Cymbalta an anti-depressant. However, both of these drugs have been approved in the USA to treat neuropathic pain (ie. pain coming from diseases which affect or damage nerves; such as diabetes). In addition, Lyrica can be used for the treatment of the pain associated with fibromyalgia. In general, these are “relatively safe” drugs and could be used in the setting of PBC (after discussion with your family doctor) if the individual was suffering from one of these conditions associated with pain and they had no other contraindications to their use.
—Dr. Mark G. Swain, 2016

• My wife has had PBC for over 10 years now and in the last 2 years she has had kidney stones. Her urologist first suggested that she stop taking the Urso medication as he thought that this was contributing to the kidney stones. Because she has to take the Urso to slow the progress of the PBC she could not stop taking it and he put her on a lime juice diet. My question is: Do you know of a relation between kidney stones and the Urso medication? [ANSWER]

I am not aware of any association between Urso and kidney stones. I would not recommend that your wife stop the Urso because of kidney stones.
—Dr. Mark G. Swain, 2016

• Can Urso lead to stomach problems – such as irritation, pain, cramps etc. [ANSWER]

Yes. Urso is a bile acid and as such can cause stomach irritation. Taking it with food or at night before bedtime can often improve this.
—Dr. Mark G. Swain, 2016

• Which GERD med is least likely to cause negative interaction with Urso? [ANSWER]

They are all safe.
— Dr. Jenny Heathcote, 2013 (Reviewed 2016)

• Is it safe to take Green Coffee Bean extract if you have PBC? [ANSWER]

There is a growing market of herbal therapies and supplements. It is not generally recommended to take these as none have been shown to have proven benefit in PBC, and some of them can be harmful to the liver, even in healthy people. If you do wish to take a herbal therapy or supplement, please discuss this with your doctor, and also make sure you record the dates you take the supplement and how much you are taking. This way, if the supplements do make your liver tests become worse, your doctor can keep track of what you were taking at the time.
—Dr. Angela Cheung, 2016

• Have you heard or seen adverse reactions to Urso, characterized by widespread thickened skin eruption? [ANSWER]

Very rarely, people can have an allergic reaction to Urso, but this usually causes hives, rather than a rash with thickened skin. People who have an allergic reaction to Urso may be able to undergo a procedure called “desensitization” which can only be done under the guidance of a trained allergist.
—Dr. Angela Cheung, 2016

• If a patient with high cholesterol, given a statin (Lipitor), develops high AST/ALT, is this considered a class drug reaction, or could they possibly change to another statin such as Crestor, with safety? [ANSWER]

Statins, drugs used for high cholesterol, can cause high liver tests in people with PBC as well as those without PBC. It is related to the medication, but it is safe to try another statin if followed closely by a doctor to check for any signs of high liver tests on the new drug.
—Dr. Angela Cheung, 2016

• Is there any slow release or long-acting form of urso being considered for the future? [ANSWER]

No
—Dr. Jenny Heathcote, 2006 (Reviewed 2016)

• For someone with a family history of stroke and/or heart attacks is there a concern with taking 81mg of aspirin daily? [ANSWER]

It is important to weigh the risks and benefits of taking any medications. If your specialist or your family doctor has told you that you need aspirin, it is important that they communicate with your liver specialist if you have cirrhosis or advanced liver disease. People taking aspirin who have esophageal varices (enlarged blood vessels in the esophagus which can only be seen with a special camera) or ascites (fluid in the abdomen due to advanced PBC), may be at risk of internal bleeding from varices, or from kidney problems in those with ascites. If your risk of a stroke or a heart attack is very high, however, it would be important for you to take aspirin to prevent these.
—Dr. Angela Cheung, 2016

• With the threat of osteoporosis, what amount of supplemental calcium (and in what form) do you recommend if at all for patients who are 50+? [ANSWER]

It is now known that it is better to take dietary calcium rather than calcium supplements, as calcium supplements may increase the risk of heart attack and stroke over the long run. Up to 1000 mg of calcium a day (two tablets of 500mg), may be safe, but it is best to take these through diet if possible. Vitamin D 1000 i.u. daily is important to take regularly.
—Dr. Angela Cheung, 2016

• Are there medications that are safe for HBP? [ANSWER]

All medications for high blood pressure are safe in people with PBC without cirrhosis. In those with cirrhosis, a class of medication called angiotensin-converting enzyme inhibitors (ACE inhibitors) and angiotensin receptor blockers (ARBs) should be avoided if possible, as these increase the risk of kidney problems in people with cirrhosis. High blood pressure medicines that are diuretics (that is, water pills), may also need to be avoided if those are already being prescribed to reduce ascites due to cirrhosis.
—Dr. Angela Cheung, 2016

• Should PBC patients be getting the Twinrix vaccination? [ANSWER]

It is recommended that all patients with chronic liver disease get the Twinrix vaccine (that is, the vaccine for hepatitis A and hepatitis B), especially if you have cirrhosis.
—Dr. Angela Cheung, 2016

• Is it safe to take Flexeril with Urso? [ANSWER]

There is no known interaction between Flexeril and Urso.
—Dr. Angela Cheung, 2016

• Is there a need to phase off cholestyramine or can you just stop taking it? [ANSWER]

You can just stop taking cholestyramine, there is no need to phase off or wean off.
—Dr. Angela Cheung, 2016

• Should one get a flu shot? I heard some get the flu after receiving the vaccination- is this true? [ANSWER]

The flu shot is recommended for anyone with cirrhosis. There are many types of influenza (or flu) viruses. Vaccines are created to fight the most common flu viruses every year, but you may get a flu virus that the vaccine does not work for. If this is the case, you will get the flu. This does not mean that the vaccine did not work, it just means you got a type of flu virus that that the vaccine does not fight. The flu vaccine does not make the flu worse, and it does not give you the flu.
—Dr. Angela Cheung, 2016

Diagnosis, Testing and Research

• Should children of PBC patients have their AMA checked? [ANSWER]

We know that family members of patients with PBC have a 10 to 15-fold increased risk of developing the disease. However, we have to take into account that PBC is a rare disease. In North America, PBC occurs 1 in 10,000 to 1 in 50,000 people. This means an increased risk of tenfold translates to family members having a 1:1000 to 1:5000 risk of PBC. I usually tell patients that children do have increased risk and if they have increased liver function tests when they have their general physicals done, they should also have an AMA checked if their liver tests are abnormal. We also recommend having an AMA checked if relatives of patients start to experience signs and symptoms of PBC such as fatigue, pain in the right side of the abdomen, dry eyes, dry mouth or itching. Otherwise we do not recommend routine AMA checking.
—Dr. Andy Mason, 2006 (reviewed 2016)

• How does the AMA affect the liver? And other body structures? [ANSWER]

AMA is a diagnostic marker for PBC. Some patients with PBC, however, have no AMA in their blood. This is a small percentage of approximately 10% of patients with PBC. It is unknown whether AMA does affect the liver or whether this is just a marker for disease. As patients with no AMA may get PBC, I suspect that AMA is not the cause of the liver disease, but a marker of PBC.
—Dr. Andy Mason, 2016

• I was diagnosed with PBC recently. My doctor has suggested a liver biopsy. I am really scared and worried about it. I wonder whether it is necessary and what is the purpose? Thanks a lot. [ANSWER]

PBC can now be diagnosed based on two blood tests: a high alkaline phosphatase (ALP) and a positive antimitochondrial antibody test (AMA). If your AMA is negative, then a doctor should perform a liver biopsy to find out if your high ALP is from PBC. Sometimes a liver biopsy is also done to check if you have any other liver diseases (such as other autoimmune liver diseases). Rarely a liver biopsy needs to be done to check the stage of your disease, as there are now blood tests and a special ultrasound test (a fibroscan) that can be done to check the stage (that is, the amount of scarring and bile duct loss in your liver).
—Dr. Angela Cheung, 2016

• I was diagnosed with PBC approximately four years ago. My liver function tests appear to be good, but recently my creatinine is elevated. Currently I am undergoing blood work and urinalysis for diabetes. Is there a link between diabetes and PBC? [ANSWER]

No. Diabetes affects ten per cent of Canadians and is most common in those who are overweight
— but is not restricted to those who are. Very, very occasionally renal disease is found in subjects with PBC but may not be a true association.
— Dr. Jenny Heathcote, 2013 (Reviewed 2016)

• Is there spontaneous remission and negative AMA serology of prior positive and abnormal LFT patients. If so, how often; and is it seen with Urso treatment? [ANSWER]

AMA positivity may often fluctuate in PBC (that is, go from positive to negative), but that doesn’t mean that the PBC has gone away. PBC is a lifelong disease that needs lifelong treatment with ursodeoxycholic acid.
—Dr. Angela Cheung, 2016

• After a biopsy confirming diagnosis of PBC, what criteria is used for assessing the necessity of repeat biopsy? [ANSWER]

A repeat biopsy is not often done in PBC, unless there is a concern that the person has developed another liver disease in addition to the PBC – this is a concern when a person’s alkaline phosphatase continues to rise despite treatment with ursodeoxycholic acid, or the liver scarring or function gets worse despite a normal or stable alkaline phosphatase. The most common liver disease that occurs with PBC is non-alcoholic fatty liver disease (otherwise known as “fatty liver”). Another common liver disease that can occur with PBC is an autoimmune liver disease called “autoimmune hepatitis”. Lastly, if there is concern that you may have cancer in your liver (which rarely occurs in people with cirrhosis due to PBC), you may need a liver biopsy.
—Dr. Angela Cheung, 2016

• If a patient is on Urso with stable blood results, does this make a difference in the length of time between biopsies? Does it differ with asymptomatic patients? [ANSWER]

Repeat biopsies are not performed on everyone — it really when it is done depends on the indication
—Dr. Jenny Heathcote, 2006 (Reviewed 2016)

• I have heard there is a blood test that is just as accurate as a liver biopsy to determine the stage of PBC. Is this true? [ANSWER]

There are now blood tests and a special ultrasound called a “fibroscan” that can assess the stage of PBC as accurately as liver biopsy. Thus, a liver biopsy is now rarely performed in those with PBC.
—Dr. Angela Cheung, 2016

• What research is currently being done with PBC? [ANSWER]

There are a several studies world wide that are noteworthy. Obeticholic acid, a modified bile salt similar to Ursodiol, has been shown to reduce liver tests by over 25% in PBC patients. Phase III studies are ongoing to see if this medication can help prevent progressive liver disease. Other studies looking at similar agents are underway. My group has an interest in studying a betaretrovirus that we characterized in PBC patients. At the University of Alberta, we have just completed a randomized controlled trial looking at how combination anti-viral treatment with Truvada and Kaletra impact on patients with PBC. We found a significant decrease in liver function tests in patients unresponsive to Ursodiol therapy who received the Truvada and Kaletra treatment rather than the placebo. Patients on antiviral therapy also experienced improvement in liver biopsy associated with a reduction in viral load. However, two thirds of the patients could not tolerate the Kaletra. So we are proposing to study different antiretroviral medications with a better side effect profile that will be both well-tolerated and effective in halting disease.
—Dr. Andy Mason, 2016

PBC Healthy Diet and Exercise

• Have there been any studies on the relationship between nutrition and liver health (in order to maximize liver function in the PBC patient)? [ANSWER]

In general PBC patients should follow a healthy, well balanced diet. If a patient is from European descent then testing for celiac disease would be reasonable (will be present in roughly 6% of these PBC patients). If a patient is overweight, they may have some fat infiltration in the liver which can be associated with liver damage in its’ own right. In this case, losing weight through a well balanced diet and weight bearing exercise, to maintain a normal or near normal BMI, would be reasonable.
—Dr. Mark G. Swain, 2016

• I have heard it is bad for the liver to go for extended periods without food. Could you explain this? [ANSWER]

I guess it depends upon how long of a period one is going without food. In general, extended periods of food deprivation will not hurt your liver.
—Dr. Mark G. Swain, 2016

• I am having a really hard time losing weight. I am almost 44, female, and was diagnosed with PBC a few years ago. I take Urso twice daily and lead a fairly active lifestyle. I am a non-smoker and seldom drink alcohol. I am 5’8” and currently weigh 178 lbs. Can I do a keto diet? Some research on the internet has led me to think maybe a diet without gluten/wheat might help in many ways. [ANSWER]

While it sounds overly simple, a well-balanced diet and exercise are still the best ways to lose weight. For example, to burn off the calories contained in 1 quarter-pound cheeseburger, it takes two hours of brisk walking! It’s all about calories in (diet) and calories out (exercise). For diet, portion size is often more important than composition, unless your regular diet contains food high in fat and simple sugars (like juices and sodas, desserts and high calorie snacks). It is best to eat three regular meals a day. Supper should be the smallest meal of the day, and eaten at least 4-6 hours before bedtime. If you become hungry during the day, it’s better to eat small, healthy snacks like fruits or vegetables, to avoid being very hungry during meals, which then results in over-eating. In order to lose weight, it is important to remember that diet is only part of the equation. Exercise (at least 30 minutes three times a week, involving any activity that makes you a little out of breath, including walking). Fad diets are often hard to maintain in the long-run, and to lose weight, it is important to change your lifestyle (that is, both your diet and your exercise level) to something that you can do regularly.
—Dr. Angela Cheung, 2016

• I was at a liver foundation talk by a dietician and she informed us that we should be on a high protein diet. This went against my understanding. Please advise? [ANSWER]

People with cirrhosis may need a high protein diet to prevent loss of muscle mass, which happens due to cirrhosis. This can be taken through food, or through supplemental protein powders or dietary supplements like Ensure or Boost. People with PBC without cirrhosis can take regular amounts of protein in their diet.
—Dr. Angela Cheung, 2016