Treating primary biliary cirrhosis

The aim of treatment for primary biliary cirrhosis (PBC) is to prevent liver damage as much as possible and reduce your symptoms.

Self-help

All types of Steatosis , including PBC, can be helped by following some general health advice. You should:

• stop smoking
• maintain a healthy weight
• not regularly drink more than 14 units of alcohol a week - read more about alcohol misuse

Not following this advice could speed up the progression of your condition. It may also affect whether you can have a liver transplant, if you need one. For example, you may not be offered a liver transplant if you drink more than the recommended daily limits of alcohol.

Avoid certain medications

Besides the medications below, you should also be careful about takingsome medicines if you have PBC because the damage to your liver can affect its ability to processcertainmedications.

For example, you may need to avoid non-steroidal anti-inflammatory drugs (NSAIDs) such as aspirin, although paracetamol can usually be taken safely.

Ask your doctor about any medications you should avoid andinform any health professional who is treating you that you have PBC, particularlyif they suggest takingmedication.

Ursodeoxycholic acid

Ursodeoxycholic acid (UDCA)isthe maintreatment for people with PBC.Itcanhelp prevent or delay liver damage in most people particularly ifyou start taking it in the early stages of the condition although itdoesn't improve symptoms such as itchy skin or fatigue (extreme tiredness).

Once you start taking UDCA, it is likely you will need to take it for the rest of your life. Regular blood tests may be carried out to monitor your liver function and you will be assessed after a year to see if you have responded sufficiently. If not, you may need additional treatment.

Side effects

One possible side effect of UDCA is weight gain. People gain an average of 2.3kg (5lbs) during the first year of taking the medication,although most people don't gain any more weight after this.

Other side effects can include diarrhoea ,nausea, vomiting and thinninghair.

Treating itchiness

Colestyramine(previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC.

It usually comes in sachets of a powder that can be dissolved in water or fruit juice. It is a good idea to take the powder with fruit juice, because it has an unpleasant taste.

You may need to take colestyramine for several days before your symptoms begin to improve.

If you are taking UDCA as well as colestyramine, you should avoid taking them at the same time because colestyramine will affect how your body absorbs UDCA.Youshouldtake UDCA at least one hour before colestyramine, or four to six hours afterwards.

This also applies to any other medicines you are taking. Your GPcan give you further advice about this.

Constipation is a common side effect of colestyramine, although this usually improves once your body gets used to the medicine. Other side effects can include bloating and diarrhoea.

If you are taking colestyramine on a long-term basis, the medicine may affect your body's ability to absorb vitamins A, D and K from food. In such cases, taking additional vitamin supplements maybebeneficial. Your GP can advise youon whether you would benefit from taking vitamin supplements.

Alternativetreatments for itching

See your GP if your itchiness does not improve after taking colestyramine. Alternative medicines are available, such as an antibiotic calledrifampicin and amedication called naltrexone.

These are usuallyonly prescribed by a specialist such as a hepatologist (liver specialist), but your GP can refer you if necessary.

It's thought that the female hormone oestrogen may make itching worse. Oestrogen is found in some types of hormonal contraceptives, such as the combined contraceptive pill . Therefore, you may want to consider using a different type of contraceptive, such as condoms .

Regularly using moisturisers to stop your skin becoming dry may also help reduce itchiness.

Managing fatigue

At the moment, no medications are available to specifically treat the fatigue caused by PBC. Your GP may want to rule out and treat any other possible causes for your tiredness, such as anaemia (a lack of red blood cells) or depression , as fatigue is a difficult symptom to treat.

You should try to exercise whenever possible, but you may need to pace yourself and limit your daily activities to a manageable level. Adjusting your daily routine around fatigue (which is often worse later in the day) can make a big difference to your quality of life.

If you have difficulty sleeping, sleep hygiene measures may help. These includeavoiding caffeine, nicotine and alcohol late at night, andmaintaining a comfortable sleeping environment.

Treating dry mouth and eyes

If you have a dry mouth and eyes, your GP may recommend eye drops containing "artificial tears" or saliva substitute products.

You should also make sure you maintain a good standard of oral hygiene and visit your dentist regularly because having a dry mouth can increase your risk of tooth decay .

Liver transplant

A liver transplant may be recommended if it's thought that the liver damage may eventually reach the stage where your life is at risk.

Planning for a liver transplant often begins before significant damage to the liver has taken place because:

• the average waiting time for a liver transplant is 145 days, so it's important to start searching for a suitable donor as soon as possible
• the better your general state of health, the greater the chance of a successful transplant, so a transplant should ideally be carried out while you are still relatively healthy

Having a liver transplant resolves a number of symptoms, most typically itchiness.Fatigue, however, is usually persistent.

Like all organ transplants, liver transplants carrya risk of complications. One of the most serious complications is that your immune systemmay reject the donated liver, which could be life-threatening. You will usually need to take medication to suppress your immune system for the rest of your life to reduce this risk.

The success rates of liver transplants for people with PBC are generally better than most other liver diseases. Studies have found that more than 90% of people who have a liver transplant for PBC are still alive after a year and more than 80% will live at least another five years.

There is a risk of PBC developing in your new liver, but this isn't usually a major concern because it often takes a long time to develop.