PRIMARY BILIARY CIRRHOSIS

WHAT IS PRIMARY BILIARY CIRRHOSIS ?

P.B.C is a slow, chronic liver disease that can gradually destroy the bile ducts within the liver. The ducts carry bile, which acts as a detergent, breaking up fat from the food we eat into small droplets. It also enables the body to absorb vitamins A. D. E and K from our diet. Approximately 90% of people with P.B.C are women. The cause and why it occurs mainly in women are as yet unknown. P.B.C is really an inappropriate name for this condition, since many people do not actually have cirrhosis when the diagnosis is made, nor indeed for many years after. P.B.C is thought to be an autoimmune disease in which the body's defence system attacks the cells lining the bile ducts as if they were foreign. Bile ducts are channels that carry bile, a yellow fluid made in the liver, to the upper part of the bowel. Bile acids, normally drained through the bile ducts, build up and damage the liver cells causing inflamation and scarring. Inflamation and scarring may, after many years, become extensive. A damaged liver can regrow without scarring. But when the damage is severe and continuous, the regrowth goes wrong and the healing process is incomplete. Scar tissue forms and the combination of this and irregular regrowth is known as corrhosis.There is conflicting evidence to suggest P.B.C is an infectious disease. It is not related to cancer or malignancy in any way, nor to excessive alcohol consumption, though people living with P.B.C are more susceptible to the effects of alcohol. Cirrhosis is frequently assumed to mean damage to the liver caused by drinking too much alcohol, but this is often not the case. Though, as previousely stated, people living with P.B.C are more susceptible to the effects of alcohol. Cirrhosis is advanced scarring that changes the structure of the liver and is caused by a number of liver diseases eg, long term infection with hepatitis B and C viruses, inhereted liver diseases, as well as P.B.C.

       

SIGNS AND SYMPTOMS OF P.B.C.

Despite its name, not everyone with primary billiary cirrhosis will go on to develop cirrhosis. In some people P.B.C does not produce any symptoms. Most people have few or no symptoms for several years. If symptoms develop you may notice one or more of the following depending on the stage of the disease.

  • Permanent fatigue - Mild or Severe 
  • Intense itching of any part of the body.
  • Dry eyes and/or dry mouth.
  • Persistent ache or discomfort in the upper right hand side below the ribs.
  • Indigestion or nausea, poor appetite.
  • Arthritis.
  • Pain in the bones.
  • Mottled palms with red or pink blotches.
  • Weakness in wrists and/or hands.
  • Diarrhoea.
  • Dark urine and/or pale stools.
  • Jaundice - Yellowing of the skin and whites of the eyes.

Thinning of the bones occurs with increasing frequency as people get older and is more common in women, especially after the menopause. P.B.C can often accelerate the degree of bone loss and some people may require additional treatment to reduce this and lower the risk of bone fractures in later life. Plenty of weight - bearing excersise and a good calcium intake in the diet are the two most effective ways, but sometimes medication is important. Osteoperosis can be detected in many ways such as the"Dexa Scan", which is a special type of x-ray. Anyone concerned about this should discuss this with their doctor.

WHAT ARE THE CAUSES OF P.B.C.

It is not known exactly what causes P.B.C, although knowledge of the condition is increasing all the time. Usually, but not always, P.B.C affects women of middle age or above. It is more common in families with a history of the disease and about 50% of people with P.B.C have gallstones too. Occasionally the condition becomes apparent during or just after pregnancy, but it is not clear whether this is because of close medical supervision, or is in some way related to the pregnancy. Also, research has shown abnormalities of the immune system in people with P.B.C. Some doctors think the trigger could be an infection, a toxin or some hereditary factor.

TESTS FOR P.B.C. ( Primary Billiary Cirrhosis ).

P.B.C . Is diagnosed on the basis of symptoms and blood tests. A liver biopsy may be needed not only to confirm the diagnosis but also to assess how early or advanced the disease is.If your doctor suspects that you may have the condition, you will be referred to a hospital specialist for tests, expert advice and treatment. The specialist you may see could be a gastroenterologist (digestive disease specialist) or a hepatologist (liver specialist).

ANTIBODY TEST. ..... Most people with P.B.C. have an antibody called anti-mitochondrial antibody (A.M.A) that can be detected by a blood test.

LIVER FUNCTION TESTS. (L.F.Ts) .....Liver function tests are blood tests that are done to check how well the liver is functioning.

ULTRASOUND SCAN. Often the bile ducts are examined by ultrasound to exclude the possibility of other diseases. Ultrasound is a quick examination and completely painless. A specila jelly, which may feel cold, is smeared on the skin over the liver and a small probe like a microphone is passed over the area. Echoes from sound waves are sent into the body and are used to build up a picture of the liver (for more information see section under scans).

LIVER BIOPSY. ..... A liver biopsy is the procedure by which a tiny piece of the liver is taken for examination under a microscope. It is usually performed under a local anaesthetic and may require an overnight stay in hospital, although some patients may go home later on the same day. A fine hollow needle is passed through the skin into the liver and a small sample is withdrawn with the needle. (for more information see section under scans).

TREATMENT.

Although there is presently no known cure, there is a range of treatments to ease symptoms and slow down the progression of the disease. Drugs that dampen down the body's immune system may be prescribed. A drug called URSODEOXYCHOLIC ACID (urso for short) has been found to be a help in a few patients, possibly in delaying the progress of the disease, but its use remains contraversial. CHOLESTYRAMINE (trade name questran or questran light) can be prescribed to help relieve itching. It works better when taken with meals, especially breakfast, but it may take some days before the treatment is effective. Some people taking questran experience side effects such as altered bowel habits or bloating, if this is a problem tell your doctor, there are other treatments that may suit you better. Many people suffer with a dry mouth and dry eyes, but this can be helped by taking lozanges and artificial tears prescribed by your doctor. For a few people who eventually go on to get advanced cirrhosis, a liver transplant may be recomended when their quality of life has deteriorated and medical treatment can no longer control their symptoms. Because P.B.C. usually develops slowly, transplantation can generally be carefully planned well ahead.

DIET.

                                

 When someone has a long term liver problem such as P.B.C., the livers ability to carry out its many functions may not be as good as in a healthy person. In particular, the livers ability to break down alcohol and medicines may be impared, however this is often not a problem. If in doubt, check with your doctor. Many people with P.B.C. can eat a normal diet, while others may need more detailed advice. If you are well with few symptoms you may not need to make any changes. Although it is important to eat as healthily as possible. If you have cirrhosis or other complications such as fluid retention (ascites and oedema) or mental slowness or confusion (encephalopathy), you may need specialist advice from a registered dietician. A few people have problems digesting fat and can develop a type of diarrhoea called steatorrhoea, in which stools are bulky, pale and difficult to flush away. There may also be nausea. If this occurs, it may help to reduce the amount of fat in the diet under the supervision of a dietician. A low fat diet should be followed only if steatorrhoea is causing problems.The aim of a low fat diet is to improve the diarrhoea, abdominal pain and the discomfort associated with steatorrhoea. As fat is an important source of energy, anyone following a low fat diet should eat extra carbohydrate, such as starch and sugar. Some people may need energy supplements and injections of fat-soluble vitamins. Others are prescribed medium chain triglycerides (M.C.T) which are fats that are easier to digest. A few people experience heartburn and an unpleasant taste in the mouth, usually caused by acid from the stomach going back up into the gullet. Eating small amounts often helps. A good idea is always to carry something to eat, preferably food which contains carbohydrate. It is always important to talk to your doctor or dietician before making any changes in your diet.

ALCOHOL


                          ALCOHOL ..  Many people with P.B.C find they can no longer tolerate alcohol. Some may be advised to drink only a little on special occasions, while others should not drink at all. Sensible drinking advice varies from person to person and will depend on many factors, such as the severity and stage of the disease, as well as your general health. Your specialist is the best to advise.

LOOKING AFTER YOURSELF.  ......Tiredness is the commonist symptom of P.B.C. Some people may need to consider making changes to their lifestyle, such as giving up work or a particular activity. Some people find that pacing their daly activities helps to preserve stamina and energy. Gentle exercise such as walking and swimming can be beneficial. It is important to tell your dentist that you have P.B.C as there may be an increased risk of bleeding. Also there are a few medicines that are best avoided, and the dose of others may need to be reduced. For exampl, it is better to take paracetomol rather than aspirin to combat aches and pains. However, you should check this with your doctor first. They are the best equipped to advise you. IMPORTANT ! ... Talk to your doctor before taking any medicine not prescribed by him or her, including paracetomol, herbal remedies and chinese medicine. If you are female and middle aged, it is important to discuss with your doctor the best way of reducing your risk of developing osteoporosis. Your doctor may advise various measures which might include increasing the intake of calcium in your diet, taking calcium supplements and H.R.T (hormone replacement therapy) if appropriate. Most doctors will advise not smoking.