Cirrhosis

Located in the right upper part of the abdomen, the liver is the largest organ in the body.  It plays a role in approximately 500 different functions and is unique, in that the liver can ‘regenerate’ (make its own healthy new tissue), but only under certain conditions.  To explain, the liver:

  • Is a Producer (e.g., produces bile, glucose and cholesterol)

  • Is a Cleaner (e.g., detoxifies blood)

  • Is a Storehouse (e.g., stores vitamins, minerals and energy)

What is cirrhosis?

When there is repeated injury to the liver, for example untreated viral hepatitis or alcohol use, this injury causes inflammation.  Over time, this inflammation will lead to irreversible scarring of the liver.  This scarring is known as cirrhosis.

What causes Cirrhosis?

There are many causes of cirrhosis.  In Ireland, the most common causes include:

• Alcohol

• Viral Hepatitis (B & C) 

• Metabolism related Fatty Liver Disease (NAFLD/MAFLD) and Non-alcohol related Steato-Hepatitis (NASH)

• Auto Immune Hepatitis (AIH)

• Bile duct disorders such as Primary Biliary Cholangitis (PBC) and Primary Sclerosing Cholangitis (PSC)

• Also, genetic conditions including:

o Haemochromatosis

o Wilsons Disease

o Cystic Fibrosis and Alpha-1 Antitrypsin Deficiency

How is cirrhosis diagnosed?

The ‘gold standard’ test for cirrhosis is a liver biopsy, however this is invasive and is now less commonly used to diagnose cirrhosis.  There are also non-invasive tests available, including:

Fibroscan: A fibroscan is a test to determine the elasticity (stiffness) of the liver.  It is usually carried out by nurse specialists in Hepatology units around Ireland.  It is a quick and painless test with instantaneous results.

X-ray/radiology imaging: Ultrasound, CT and MRI scans are all useful tests to diagnose cirrhosis.

Physical exam and blood tests also aid in the diagnosis of cirrhosis

What are the symptoms of Cirrhosis?

In the early stages of cirrhosis there are little or no symptoms. People with stable cirrhosis feel completely normal. However, over time and with continued liver injury, symptoms can include:

• Fatigue

• Itching

• Bleeding gums or frequent nosebleeds 

• Bruising easily

• Loss of appetite, nausea

• Weight loss/muscle loss

• Confusion or changes in alertness

• Fluid build-up in legs (oedema) or tummy/abdomen (ascites)

• Yellow discolouration of the skin or whites of the eyes (jaundice)

🚩Warning Symptoms

If you experience any of the following symptoms, you must see a doctor immediately.  This is particularly important if you have recently been diagnosed with cirrhosis.

  • Difficulty breathing

  • Vomiting blood

  • Bleeding in bowel motions, particularly black, tarry stools

  • Confusion or drowsiness

  • Fever

  • Jaundice (yellowing of eyes and/or skin)

  • Abdominal swelling

What are the stages of cirrhosis?

Cirrhosis may be stable (compensated cirrhosis) or complicated (decompensated cirrhosis). 

Cirrhosis is also graded using what is called a MELD score and a Child Pugh Score.  You may hear some of these words being used when you attend your specialist service. The MELD score stands for Model of End-stage Liver Disease and can help determine the degree of sickness and therefore how much you need a transplant.  A MELD score ranges from 6 (most stable) to 40 (sickest). The Child Pugh score grades cirrhosis into A, B and C.  Childs Pugh A means compensated, whereas B & C are decompensated.

Patients with compensated cirrhosis may feel completely normal and look fine. Their eyes will be normal colour, they will not have any fluid on their tummy/abdomen, they will not have symptoms of confusion, or gastro-intestinal bleeding. Patients with compensated cirrhosis are often followed up routinely in out-patient clinics and/or by their GP. Such patients require active treatment wherever possible, and regular screening for complications related to liver disease.

Decompensated cirrhosis describes someone who has an active complication as a direct result of their liver not functioning properly.  These complications typically require specialist intervention and, in some cases, hospitalisation.

Specific Complications

It is important to stress that an individual with cirrhosis may never experience any of these complications.  However, it is important to be aware of these signs and symptoms.

Portal Hypertension

Portal hypertension is a complication caused by increased pressure in the portal vein of the liver (‘high blood pressure around the liver’). The portal vein is responsible for feeding blood to the liver from the spleen and bowel. Due to scarring and stiffness within the liver, blood finds it difficult to pass through, leading to a build up of blood pressure around it. As the pressure affects the portal vein, this is known as portal hypertension. Having portal hypertension means you are more at risk of developing other complications including ascites and varices.

Ascites

Ascites is a term used to describe a build up of fluid on the tummy (abdomen). The excess fluid may or may not be present in the legs. The fluid build-up may happen over a short space of time; you may notice a gradual, or sharp, increase in your weight. This fluid build-up is caused by the liver being unable to filter blood as it usually does (the liver is a cleaner). The clogged filter causes a build-up of pressure (portal hypertension). A combination of portal hypertension and low levels of a protein called albumin, lead to the build-up of fluid in the abdominal cavity.

Large amounts of ascites can be very uncomfortable.  It can induce nausea, constipation, decreased appetite, and abdominal/back pain. It can make it difficult to breathe comfortably when lying down.

Ascites can be controlled by sticking to a ‘no added salt’ diet and using medications called diuretics (water tablets).  When ascites is severe, it may be referred to as ‘tense’. In this scenario, the individual is brought into hospital for a procedure called a Large Volume Paracentesis (LVP).  This involves a small tube being inserted into the abdomen to drain the excess fluid.  This is usually organised by your Hepatology or Gastroenterology service.

Recording your weight weekly can be helpful to your hepatology team, and yourself, as it aids in early detection of fluid build-up.  A general rule of thumb is weight gain of ≥5kg in one week, needs further evaluation by your medical team.  

Varices

The presence of portal hypertension can cause increased pressure in other vessels surrounding the liver, which become enlarged, similar to varicose veins in the legs.  In particular, varices form in the vessels lining the oesophagus (food pipe) and/or stomach. 

All patients with a diagnosis of cirrhosis are offered an endoscopy exam, called an OGD (Oesophago-Gastro-Duodenoscopy). This is a screening exam to look for varices, and if any are found, to treat them accordingly.  All patients with cirrhosis are usually offered this screening OGD every 2 years.  Please consult your Hepatology/Gastroenterology service if you are unsure if you are due an OGD. 

Varices are not painful and do not impact on eating and drinking. However, they can be life threatening if they bleed.  That is why it is so important to attend your endoscopy (OGD) appointment.  

Treatment for varices involves medications and/or treatment at endoscopy. You will be prescribed a specific blood pressure medication called a betablocker (carvedilol or propranolol). Even if your blood pressure is within normal limits, this is to reduce the high blood pressure around the liver (portal hypertension). Endoscopic treatment involves ‘banding’ or ‘ligation’ as is performed by a specialist. Both of these approaches reduce the risk of bleeding from varices.

The signs of bleeding varices are vomiting bright red blood or passing black ‘tarry’ stool. If this happens, you must go to your local emergency department immediately by ambulance.  

Hepatic Encephalopathy (HE)

When the liver is unable to remove toxins from the blood (the liver is a cleaner), these toxins can build-up within the body. A particular toxin called ammonia, builds-up which can affect the brain causing confusion. The level of confusion varies and can present itself in different ways.  If left untreated, hepatic encephalopathy (HE) can lead to coma and death.

Symptoms of HE include:

• Fatigue

• Forgetfulness

• Mood/personality changes

• Trouble sleeping at night/sleeping more during the day

• Shortened attention span/poor judgement

• Slurred speech

• Slow reaction time- it is unsafe to drive

• Tremulous hands on closing eyes (known as a hepatic flap)

HE can have a very powerful effect on an individual’s quality of life.  It is important that family members know what HE is, understand why their loved one is behaving they way they are, and most importantly, know what to do in the event of an acute episode.

Good bowel management is VITAL to preventing, controlling and treating HE.  General management involves:

• Aim to pass 2-3 stools/bowel motions per day

• Use a laxative/stool softener (such as lactulose) to aid this

• Take prescribed medication (e.g., Rifaximin)

• Avoid medications that might make you drowsy/sleepy including sleeping tablets, medications containing codeine and/or benzodiazepines

• Avoid dehydration

In most circumstances, it is family members who notice subtle changes that could be related to HE.  If you, or your loved one is concerned that you are developing HE, you should seek advice from your GP or Hepatology service. If you are not acting like yourself, are acutely confused or very sleepy you must attend your local emergency department. It is extremely important that you do not drive or operate any machinery if you suffer from HE.

Liver Cancer

Patients with cirrhosis are at an increased risk of liver cancer, known as hepatocellular carcinoma (HCC). HCC is a leading cause of death from cancer worldwide, and is curable if picked up at an early stage.

Screening for HCC involves a cancer marker blood test (alpha fetoprotein, AFP) and liver ultrasound which are done 6 monthly in all patients with cirrhosis. This is coordinated through your Hepatology/Gastroenterology service.

Useful links:

https://britishlivertrust.org.uk/information-and-support/liver-conditions/cirrhosis/