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Dr. Nathan Bass Answers Our Questions
Question2 & Answers
1.) Is it possible to have stable LFT's indicating you are doing ok with PBC but the liver disease is actually silently progressing to end stage?
Answer
Yes, although current evidence suggests that if your numbers are normal or near normal and stable, progression is slower. This is commonly the situation in patients on ursodeoxycholic acid treatment. Important lab values to watch that indicate the true stage of disease are bilirubin, albumin and pro-time.
2.A) Why is there so much variation in biopsy experiences among PBCers? Some people say it was a breeze and others have so much pain. Is it the patient or is it the practitioner?
Answer
Both, but I believe it is mainly the experience of the practitioner. Very apprehensive patients often suffer more, but with some sedation, can have a completely uneventful experience (they may not even remember it!). The practitioners technique is most important, and with good technique and experience, a biopsy can be less disturbing than having a dental filling. However, even the most experienced and accoplished docs have bad days or encounter an aspect of patient anatomy that just foils the optimal result. Even with the best approach, some patients do experience more pain after the procedure than others. Pain experience and threshold are known to be extremely variable, but even so, the pain that follows a biopsy should not last more than a day or two.
2B.) Many people with Hep C say that they've been given Versed, a sedative. Others have been given nothing except a local. What or who determines whether you get anesthesia, sedative, or nothing]?
Answer
Most of the time a biopsy can be performed without sedation. Versed is usually safe, but adds risk to the procedure. Reassurance and explanation beforehand help a lot to calm nerves, but in the case of a patient who remains very apprehensive and scared, sedation is appropriate. If you feel you cannot undergo the procedure without considerable anxiety, discuss the option of mild sedation with your physician.
3.) Can those with PBC have an occassional glass of wine?
Answer
Alcohol is hepatotoxic and its use in certain liver diseases has been shown to be detrimental. Similar data are not available for PBC. If you want to play it absolutely safe, no alcohol. If you want to take advantage of the lack of data, its your decision.
I personally advise abstinence as the most prudent practice to maintaining liver health in PBC. Also, everyone seems to have a different idea about the meaning of occasional.
4.) Most of us have itching....some say their itching is localized (Arms, face, hands, buttocks) while others say they itch all over. Does the itching depend on the stage? It seems I itched more in the earlier stage than I do now in later stage.
Answer
There is little work on itching patterns and we have recently become quite interested in this. Itching is commonly worst on the extremities (arms, hands, legs, feet) and inaccesible places like the back. It is often worse at night. Yes, itching may abate as the disease progresses. The reason is unknown, but may relate to a failure of the liver to manufacture the "pruritogen" that causes the itch in the first place, as the overall function of the liver fails.
5.) What are the five most important indicators for liver transplant? Are they the same indicators for prognosis? How long is the average wait on a transplant list or does it depend on the facitlity?
Answer
The indicators that are officially in use contitute the variables that are used to calculate a child Pugh score. Three are laboratory variables, two are clinical. They are: serum albumin, serium bilirubin, prothrombin time, ascites and encephalopathy. The average wait for transplantation depends on many factors including stage of listing, blood type and facility location.
6A.) Can PBC BE A CAUSE of an elevated sedimentation rate, if tests for other diseases are ruled out?
Answer
Yes, but not usually very high.
6B.) PBC is an inflammation of the bile ducts so could IT CAUSE an elevated sed rate? It is not a routine blood test for pbcers, should it be checked regularly?
Answer
It has no established value, in PBC for monitoring or prognosis, and I do not use it routinely.
7.) What are the qualifications for getting a live donor transplant? I know about blood type, but what about the rest. Is it ethnic background, age, weight, height etc.
Answer
Adult-to-adult living liver donation is a very exciting, new, and so far remarkably successful procedure. It is also a big procedure for the donor- more so than donating a kidney. Any patient who is considered a candidate for a liver transplant could be a recipient from a live donor. Some problems may hamper this option, such as having a portal vein blockage, but this is potentially manageable too. Age is not an issue. We have evaluated one patient who was 70 as a living donor recipient. The donor, on the other hand is generally going to have to be youngish (less than 55), in excellent health (including no liver disease), a non-smoker, light to non-drinker, not very overweight, no previous major abdominal surgery, and motivated by deep concern and care for the potential recipient. The donor need not be related, but this may help the outcome in terms of less rejection. The donor must have a matching ABO blood type (O to O, A to A, etc.) or be a blood type O (universal donor - can donate to any blood type). Finally, the donor should match the recipient in size or be a bit larger. Potential donors need to undergo a very careful health evaluation as well as evaluation of the size and blood vessel anatomy of their livers.
8.) Can you describe "all" the symptoms of end stage PBC?
Answer
End-stage (implying very advanced) PBC is attended by many symptoms of PBC including itching, and fatigue, which may be present in the early stages as well. True end-stage symptoms result from serious complications of the disease and include leg edema, abdominal bloating from fluid, altered mental functioning, including poor concentration, forgetfulness, or confusion from encephalopathy, and internal (intestinal tract, variceal) bleeding. The latter may manifest as vomiting blood or passage of blackish stool. Easy bruising and nose bleeds may signal the deficiency of blood clotting factors that occurs in end-stage liver disease. Patients with end-stage PBC are usually markedly jaundiced as well, and the depth of jaundice will often worsen progressively. Severe bone disease and fractures of vertebrae or other bones may occur before really advanced liver failure, but is also considered a serious complication, usually occurring in patients with longstanding disease. Rarely, patients may develop a form of nerve damage from cholesterol deposits called xanthomatous neuropathy. There are many other symptoms that can occur in PBC, but these do not signify end-stage disease: These include CREST, and sicca symptoms
9.) What causes the AMA to increase in a PBC patient? Does it increase as the PBC progresses? Is it possible for AMA to almost double over a period of a couple of years?
Answer
The AMA will often increase, but I have known it to decrease as well (even to undetectable), and to fluctuate. The condition known as "autoimmune cholangitis is considered by some experts to be PBC without any appearance of the AMA. Yes, it may certainly double or increase ten-fold over a period of a few years. However, since we have no confident idea as to the meaning of the AMA or its role in the disease process in PBC, why any of these things happen is essentially unknown.
10.) Why is it that so many with PBC have terrible bone aches and pains? Is it just PBC or all liver diseases? What do you recommend we take for the pain?
Answer
Many types of liver disease may be complicated by bone thinning, but this problem is clearly worst in PBC. The loss of mineral (calcium) from bone in PBC is the main cause of bone pain, and is most incapacitating when this leads to fractures or collapse of vertebra from the weakening of bone structure. Rarely, an inflammation of the fibrous cover of the bone may also occur (periostitis). The factors causing bone loss in PBC include postmenopausal hormone changes in women, poor absorption of calcium and vitamin D from the digestive tract, and factors that accumulate in the blood stream in cholestasis that inhibit the cells that form the bone, while there is increased activity of the cells that remodel or remove bone tissue. Other genetic factors that determine the way an individual responds to vitamin D may also play a role. The bone pain in PBC can be tremendously disabling, and fortunately, can be prevented by well-timed liver transplantation. Further bone loss should be prevented by appropriate treatment with calcium, vitamin D, hormone replacement and bone-building medications such as alendronate.
The pain should be treated with painkillers as strong as needed, but not non-steroidal anti-inflammatory drugs (e.g., Ibuprofen), as these can cause gastrointestinal bleeding in patients with portal hypertension. Some of the newer so called COX-2 selective drugs like Celebrex or Vioxx may be effective and safer, but are untested in this situation and are considered contraindicated in patients with liver disease. They, occasionally have caused serious internal bleeding. Opiate pain killers will help, but are habit forming and sedating and worsen encephalopathy. This is a difficult problem to treat satisfactorily once it has progressed to an advanced stage.
11.) PBCers & articles I've read state there's a higher incidence of breast cancer in PBCers. Is there data showing this? If so, what could be a cause? Or could it be the autoimmune process causing the higher rate?
Answer
It is unclear whether the risk for breast cancer is really increased in PBC. Some early studies found this to be the case (Wolke, Am J Med 1984;76:1075; Goudie, BMJ 1985; 291:1597) but this was not confirmed in later studies (Witt-Sullivan, Hepatology 1990;12:98; Loot, Hepatology 1994; 20:101). The theory was that a carcinogen that is normally excreted in the bile accumulated in the body in PBC because of cholestasis. Of course, that was a theory based on unconfirmed results. Cancer of the liver may occur in PBC, but compared with many other causes of liver disease, is quite rare.
12.) Are there any symptoms to be aware of which would indicate pulmonary or portal hypertension?
Answer
Portal and pulmonary hypertension are two very different problems. Portal hypertension (high blood pressure in the blood vessels draining the intestines and spleen) results from increased resistance to the easy flow of blood in the portal vein through the liver. Invariably complicates advanced PBC and is usually asymptomatic until it is complicated by a gastrointestinal bleed (see above) or build up of fluid (ascites) in the abdomen which causes it to swell. Enlargement of the spleen also occurs, and rarely is a cause of abdominal discomfort. Pulmonary hypertension (high blood pressure in the pulmonary artery) is a rare complication of PBC (and may occur without portal hypertension) or other liver disease. It is also commonly without symptoms until at an advanced stage when severe shortness of breath, chest pain and fatigue may indicate its presence.
