The aim of treatment for PBC is to slow down the liver damage and reduce your symptoms.
All types of liver disease, including PBC, can be helped by following some general health advice.
- stop smoking
- maintain a healthy weight
- not regularly drink more than 14 units of alcohol a week – read more about alcohol misuse
Following this advice could:
- help prevent PBC getting worse
- mean all the treatments for PBC are available to you if you need them – for example, a liver transplant
Avoid certain medicines
The damage to your liver can affect its ability to process certain medicines.
This means you may need to avoid certain medicines – for example, non-steroidal anti-inflammatory drugs (NSAIDs) like aspirin and ibuprofen.
Ask your doctor about any medicines you should avoid, and tell any healthcare professional treating you that you have PBC.
Ursodeoxycholic acid (UDCA) is the main treatment for PBC.
It can help prevent or delay liver damage in most people, particularly if you start taking it in the early stages of the condition. But it does not improve symptoms such as itchy skin or fatigue.
Once you start taking UDCA, it's likely you'll need to take it for the rest of your life.
You may need regular blood tests to monitor your liver function, and you'll be assessed after a year to see if you've responded to UDCA. If not, you may need additional treatment.
Weight gain is a possible side effect of UDCA. People gain an average of 2.3kg (5lbs) during the first year of taking the medicine, although there's not usually any more weight gain after this.
Other side effects can include diarrhoea, feeling and being sick, and thinning hair.
Obeticholic acid (OCA) is a new treatment that works by improving bile flow and reducing inflammation.
It may be offered as an option for treating PBC, either:
- in combination with UDCA – if UDCA is not working well enough
- on its own – for people who cannot take UDCA
People who may need OCA will first need to be assessed by a specialist team with experience in treating PBC.
A possible side effect of OCA is itching. If itching is a problem, your dose may be lowered or you may be offered medicine to treat the itching.
If you have very advanced liver disease, you may be prescribed a lower dose of OCA, or it may not be recommended.
Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC.
It usually comes in sachets as a powder that can be dissolved in water or fruit juice. It's a good idea to take the powder with fruit juice because it has an unpleasant taste.
You may need to take colestyramine for a few weeks before your symptoms begin to improve.
If you're taking UDCA as well as colestyramine, you should avoid taking them at the same time because colestyramine will affect how your body absorbs UDCA.
You should take UDCA at least 1 hour before colestyramine, or 4 to 6 hours afterwards.
This also applies to any other medicines you're taking. A GP can 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're taking colestyramine long term, it may affect your body's ability to absorb vitamins A, D and K from food. Taking additional vitamin supplements may be beneficial.
A GP can advise you about whether you would benefit from taking vitamin supplements.
Alternative treatments for itching
See a GP if your itchiness does not improve after taking colestyramine.
Alternative medicines are available, such as an antibiotic called rifampicin and a medicine called naltrexone.
These are usually only prescribed by the specialist that looks after your PBC – for example, a gastroenterologist or hepatologist (liver specialist).
Regularly using moisturisers to stop your skin becoming dry may also help reduce itchiness.
No medicines are currently available to specifically treat fatigue associated with PBC.
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 include avoiding caffeine, nicotine and alcohol late at night, and ensuring you have a comfortable sleeping environment.
Treating dry mouth and eyes
If you have a dry mouth and eyes, a GP may recommend eyedrops containing "artificial tears" or saliva substitute products.
A liver transplant may be recommended if it's thought the liver damage may eventually put your life at risk.
Planning for a liver transplant often begins before significant damage to the liver has taken place. This is because:
- the national average waiting time for a liver transplant is 65 days for adults, so it's important your specialist doctor considers assessment and listing for transplantation 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're still relatively healthy
Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue.
Like all organ transplants, liver transplants carry a risk of complications. Your immune system may reject a donated liver, which could be life threatening.
Medicines that suppress the immune system are very effective at preventing this, but they need to be taken for life to reduce this risk.
Most people live more than 10 years after having a liver transplant and many live for up to 20 years or more.
But having a liver transplant does not always completely cure PBC. Around 1 in 5 people who have a liver transplant for PBC develop the condition in their new liver. This is known as recurrent PBC.