Liver disease is the fifth major cause of mortality in England and Wales, and one of the few causes of premature death that is increasing (Fig 1), yet the most common causes - obesity, undiagnosed viral infection with hepatitis B and C, and harmful drinking - are avoidable. Rates of chronic liver disease (CLD) and cirrhosis in people aged under 65 years have increased by around 20% over the last 10 years in the UK, but many European countries have seen cases of CLD fall in the same period (Davies, 2012).
As there is no national strategy to improve the detection and treatment of liver disease, the All-Party Parliamentary Hepatology Group (2014) has recommended 20 actions to do this. The National Confidential Enquiry into Patient Outcome and Death’s (2013) report on hospital deaths from alcohol-related liver disease revealed a wide range of missed opportunities when caring for these patients:
Death was considered avoidable in 10% of the cases reviewed;
Care was rated as less than good in more than half of patients admitted.
Nurses from all areas of practice must be able to offer health information and education to patients to raise awareness of liver disease and promote healthy-living strategies. As the mortality and morbidity associated with CLD and cirrhosis increase, nurses need to develop their knowledge and skills in caring for people who have advanced liver disease. The Royal College of Nursing has developed a competency framework for caring for people with liver disease (RCN, 2013).
Table 1 lists common causes of liver disease, and some of the tests and investigations used to confirm its presence (British Liver Trust, 2007). Ultrasound imaging may be used to assess the size and shape of the liver and surrounding organs, detect the presence of ascites, observe the direction of blood flow through the portal vein and detect focal abnormalities indicative of hepatocellular carcinoma. More imaging with computerised tomography or magnetic resonance imaging may be required.
Non-alcohol related steatohepatitis (NASH)
NASH is the accumulation of fat - mainly triglyceride, cholesterol and phospholipids - over 5-10% of liver weight. The term “non-alcohol related fatty liver disease” was introduced to explain the varying degree of severity of liver fibrosis in patients not exposed to alcohol. NASH is the more severe form and can progress over years to cirrhosis; it now accounts for 10-20% of all cases of cirrhosis (Dooley et al, 2011). NASH is part of a metabolic syndrome that includes obesity (although not all patients are obese), diabetes mellitus, hypertension and cardiovascular disease; these comorbidities may complicate care.
Treatment of NASH is aimed at weight loss and dietary intake, tight control of blood glucose in patients with diabetes, and management of hyperlipidaemia and hypertension. A multiprofessional team approach, with dietitians, diabetes specialist nurses and NASH pathways between primary and secondary care, optimises treatment outcomes.
End-stage liver disease (ELD)
Liver cirrhosis occurs as a result of any CLD. Repeated damage to the hepatocytes results in the development of fibrosis and nodular tissue. This alters the liver’s cellular structure, impedes function and affects the blood flow in and around the liver, leading to portal hypertension. Once cirrhosis has developed, it is usually irreversible and can lead to liver failure (Sargent, 2006).
The liver is a vital organ with many functions including: metabolising carbohydrates, fats and bilirubin; storing glycogen; and cleansing blood. The cirrhotic liver may be able to function adequately - termed a “compensated” liver - but once the functions start to deteriorate and complications of portal hypertension arise, it is “decompensated” and the patient has ELD.
Common presentations of decompensated cirrhosis are:
Jaundice (icterus);
Ascites;
Hepatic encephalopathy;
Variceal bleeding;
Sepsis, including spontaneous bacterial peritonitis, septicaemia, chest infection, urinary tract infection;
Lethargy or weakness;
Anaemia and chronic gastrointestinal blood loss;
Nausea and vomiting;
Pruritis (itching);
Malnutrition; and
Peripheral muscle loss.
Complications of ELD
Common complications of ELD include those experienced by Ms Brown (Box 1), such as ascites, hepatic encephalopathy, variceal bleeding and malnutrition.
Ascites
Ascites is fluid that collects in the peritoneal cavity. Many factors are involved in its development including increased pressure in the portal venous system resulting from cellular and blood-vessel changes in the cirrhotic liver (European Association for the Study of the Liver, 2010). Ascites occurs in 60% of patients with compensated cirrhosis within a 10-year period (EASL, 2010); it can cause abdominal discomfort, breathlessness and restricted mobility due to the large volume of fluid collected.
Initial treatment of ascites in patients with normal renal function is to use potassium-sparing diuretics such as spironolactone. Patients are encouraged to cut their sodium intake (not adding salt at the table or in cooking, and avoiding high-salt foods), and may need support with this. Spironolactone may be used with frusemide (Dooley et al, 2011). Renal and weight monitoring is vital to ensure symptom relief and early detection of diuretic side-effects such as electrolyte imbalance and dehydration.
In 10-15% of patients, ascites does not respond to diuretic therapy; these patients may need paracentesis (a drainage catheter is inserted into the peritoneal cavity). This may be required regularly and carries risks of infection, pain and bleeding. Patients who need regular paracentesis may be considered for transjugular intrahepatic portosystemic shunt and stent (TIPSS), in which a stent is placed into the cirrhotic liver to facilitate portal blood returning to systemic circulation. However, TIPSS is contraindicated in patients who have developed hepatic encephalopathy (EASL, 2010).
Hepatic encephalopathy (HE)
HE is a complex and potentially reversible neuropsychiatric syndrome seen in patients with ELD. It may be episodic and is a result of the cirrhotic liver’s inability to break down nitrogen-based substances that arise from the bacteria in the gut and cross the blood-brain barrier (Dooley et al, 2011). Several tools are used to assess HE, including the West-Haven scale (Ortiz et al, 2007).
There may be precipitating factors in patients with cirrhosis (Box 2), which need to be identified and treated accordingly, although in 20-30% of patients no precipitating cause is found (Dooley et al, 2011). HE negatively affects quality of life and can be distressing for patients and their families. It also affects decision-making processes, so mental capacity needs to be assessed regularly.
Treatment of HE aims to cut ammonia levels in the gastrointestinal tract, using aperients such as lactulose, which assist with evacuating bowel contents. However, it can be difficult for patients to manage this, and side-effects can reduce adherence. Patients are advised to titrate
the aperient dose so they aim to have at least two soft, bulky stools a day without developing diarrhoea. Rifaximin, a minimally absorbed, broad-spectrum antibiotic has been effective at treating HE and reducing hospital admissions (Felicilda-Reynaldo, 2012).
Variceal bleeding
Another potentially life-threatening complication, variceal bleeding, is the presenting feature in >25% of patients with cirrhosis. Hypertension in the portal venous system can lead to oesophageal, gastric and duodenal varices (abnormally dilated blood vessels), which can rupture, resulting in haemorrhage. Although hospital mortality rates linked to variceal bleeding have fallen, the mortality rate in patients with ELD is around 30% (Ginès et al, 2012). Control of bleeding is achieved in 95% of patients using endoscopic banding; TIPSS may also be considered to reduce portal hypertension, as described earlier (European Association for the Study of the Liver, 2010).
Malnutrition
Malnutrition is often seen in patients with the complications of liver disease and becomes more marked in those with ELD, where muscle loss is evident - even in patients who are obese. In patients like Ms Brown, our case study in Box 1, the discomfort associated with large-volume ascites often results in loss of appetite. The dietitian’s role is vital: nutritional intervention can improve survival rates and quality of life (Iwasa et al, 2013). For patients such as Ms Brown who have developed decompensated cirrhosis, liver transplantation may be the only long-term treatment option. Outcomes after liver transplant have improved significantly, with a 68-77% five-year survival rate (NHS Blood and Transplant, 2014a).
Several prognostic scoring systems can be used to identify patients who may be considered for a liver transplant. The UK uses the United Kingdom Model for End-Stage Liver Disease (UKELD), a scoring system combining blood tests for bilirubin, INR, creatinine and serum sodium (Asrani and Kim, 2010); patients with NASH cirrhosis would need a UKELD of >49 to be considered for the liver-transplant waiting list (NHSBT, 2014b). However, these scoring models do not reflect other adverse factors such as quality of life and difficult-to-treat variceal bleeding (Dooley et al, 2011). There are guidelines regarding eligibility for, and contraindications to, liver transplantation complications (NHSBT, 2014b). Ms Brown was considered for a transplant but did not fulfil the criteria due to cardiovascular comorbidities, which suggested she would not survive the arduous surgical procedure and potential post-operative complications.
End-of-life care in liver disease
Murray et al (2005) outlined three distinct illness trajectories for people with progressive chronic illness:
Steady progression with a clear terminal phase, for example in cancer;
Gradual decline punctuated with episodes of acute deteriorati
Liver disease is the fifth major cause of mortality in England and Wales, and one of the few causes of premature death that is increasing (Fig 1), yet the most common causes - obesity, undiagnosed viral infection with hepatitis B and C, and harmful drinking - are avoidable. Rates of chronic liver disease (CLD) and cirrhosis in people aged under 65 years have increased by around 20% over the last 10 years in the UK, but many European countries have seen cases of CLD fall in the same period (Davies, 2012).As there is no national strategy to improve the detection and treatment of liver disease, the All-Party Parliamentary Hepatology Group (2014) has recommended 20 actions to do this. The National Confidential Enquiry into Patient Outcome and Death’s (2013) report on hospital deaths from alcohol-related liver disease revealed a wide range of missed opportunities when caring for these patients:Death was considered avoidable in 10% of the cases reviewed;Care was rated as less than good in more than half of patients admitted.Nurses from all areas of practice must be able to offer health information and education to patients to raise awareness of liver disease and promote healthy-living strategies. As the mortality and morbidity associated with CLD and cirrhosis increase, nurses need to develop their knowledge and skills in caring for people who have advanced liver disease. The Royal College of Nursing has developed a competency framework for caring for people with liver disease (RCN, 2013).Table 1 lists common causes of liver disease, and some of the tests and investigations used to confirm its presence (British Liver Trust, 2007). Ultrasound imaging may be used to assess the size and shape of the liver and surrounding organs, detect the presence of ascites, observe the direction of blood flow through the portal vein and detect focal abnormalities indicative of hepatocellular carcinoma. More imaging with computerised tomography or magnetic resonance imaging may be required.Non-alcohol related steatohepatitis (NASH)NASH is the accumulation of fat - mainly triglyceride, cholesterol and phospholipids - over 5-10% of liver weight. The term “non-alcohol related fatty liver disease” was introduced to explain the varying degree of severity of liver fibrosis in patients not exposed to alcohol. NASH is the more severe form and can progress over years to cirrhosis; it now accounts for 10-20% of all cases of cirrhosis (Dooley et al, 2011). NASH is part of a metabolic syndrome that includes obesity (although not all patients are obese), diabetes mellitus, hypertension and cardiovascular disease; these comorbidities may complicate care.Treatment of NASH is aimed at weight loss and dietary intake, tight control of blood glucose in patients with diabetes, and management of hyperlipidaemia and hypertension. A multiprofessional team approach, with dietitians, diabetes specialist nurses and NASH pathways between primary and secondary care, optimises treatment outcomes.End-stage liver disease (ELD)Liver cirrhosis occurs as a result of any CLD. Repeated damage to the hepatocytes results in the development of fibrosis and nodular tissue. This alters the liver’s cellular structure, impedes function and affects the blood flow in and around the liver, leading to portal hypertension. Once cirrhosis has developed, it is usually irreversible and can lead to liver failure (Sargent, 2006).The liver is a vital organ with many functions including: metabolising carbohydrates, fats and bilirubin; storing glycogen; and cleansing blood. The cirrhotic liver may be able to function adequately - termed a “compensated” liver - but once the functions start to deteriorate and complications of portal hypertension arise, it is “decompensated” and the patient has ELD.Common presentations of decompensated cirrhosis are:Jaundice (icterus);Ascites;Hepatic encephalopathy;Variceal bleeding;Sepsis, including spontaneous bacterial peritonitis, septicaemia, chest infection, urinary tract infection;Lethargy or weakness;Anaemia and chronic gastrointestinal blood loss;Nausea and vomiting;Pruritis (itching);Malnutrition; andPeripheral muscle loss.Complications of ELDCommon complications of ELD include those experienced by Ms Brown (Box 1), such as ascites, hepatic encephalopathy, variceal bleeding and malnutrition.AscitesAscites is fluid that collects in the peritoneal cavity. Many factors are involved in its development including increased pressure in the portal venous system resulting from cellular and blood-vessel changes in the cirrhotic liver (European Association for the Study of the Liver, 2010). Ascites occurs in 60% of patients with compensated cirrhosis within a 10-year period (EASL, 2010); it can cause abdominal discomfort, breathlessness and restricted mobility due to the large volume of fluid collected.Initial treatment of ascites in patients with normal renal function is to use potassium-sparing diuretics such as spironolactone. Patients are encouraged to cut their sodium intake (not adding salt at the table or in cooking, and avoiding high-salt foods), and may need support with this. Spironolactone may be used with frusemide (Dooley et al, 2011). Renal and weight monitoring is vital to ensure symptom relief and early detection of diuretic side-effects such as electrolyte imbalance and dehydration.In 10-15% of patients, ascites does not respond to diuretic therapy; these patients may need paracentesis (a drainage catheter is inserted into the peritoneal cavity). This may be required regularly and carries risks of infection, pain and bleeding. Patients who need regular paracentesis may be considered for transjugular intrahepatic portosystemic shunt and stent (TIPSS), in which a stent is placed into the cirrhotic liver to facilitate portal blood returning to systemic circulation. However, TIPSS is contraindicated in patients who have developed hepatic encephalopathy (EASL, 2010).Hepatic encephalopathy (HE)HE is a complex and potentially reversible neuropsychiatric syndrome seen in patients with ELD. It may be episodic and is a result of the cirrhotic liver’s inability to break down nitrogen-based substances that arise from the bacteria in the gut and cross the blood-brain barrier (Dooley et al, 2011). Several tools are used to assess HE, including the West-Haven scale (Ortiz et al, 2007).There may be precipitating factors in patients with cirrhosis (Box 2), which need to be identified and treated accordingly, although in 20-30% of patients no precipitating cause is found (Dooley et al, 2011). HE negatively affects quality of life and can be distressing for patients and their families. It also affects decision-making processes, so mental capacity needs to be assessed regularly.
Treatment of HE aims to cut ammonia levels in the gastrointestinal tract, using aperients such as lactulose, which assist with evacuating bowel contents. However, it can be difficult for patients to manage this, and side-effects can reduce adherence. Patients are advised to titrate
the aperient dose so they aim to have at least two soft, bulky stools a day without developing diarrhoea. Rifaximin, a minimally absorbed, broad-spectrum antibiotic has been effective at treating HE and reducing hospital admissions (Felicilda-Reynaldo, 2012).
Variceal bleeding
Another potentially life-threatening complication, variceal bleeding, is the presenting feature in >25% of patients with cirrhosis. Hypertension in the portal venous system can lead to oesophageal, gastric and duodenal varices (abnormally dilated blood vessels), which can rupture, resulting in haemorrhage. Although hospital mortality rates linked to variceal bleeding have fallen, the mortality rate in patients with ELD is around 30% (Ginès et al, 2012). Control of bleeding is achieved in 95% of patients using endoscopic banding; TIPSS may also be considered to reduce portal hypertension, as described earlier (European Association for the Study of the Liver, 2010).
Malnutrition
Malnutrition is often seen in patients with the complications of liver disease and becomes more marked in those with ELD, where muscle loss is evident - even in patients who are obese. In patients like Ms Brown, our case study in Box 1, the discomfort associated with large-volume ascites often results in loss of appetite. The dietitian’s role is vital: nutritional intervention can improve survival rates and quality of life (Iwasa et al, 2013). For patients such as Ms Brown who have developed decompensated cirrhosis, liver transplantation may be the only long-term treatment option. Outcomes after liver transplant have improved significantly, with a 68-77% five-year survival rate (NHS Blood and Transplant, 2014a).
Several prognostic scoring systems can be used to identify patients who may be considered for a liver transplant. The UK uses the United Kingdom Model for End-Stage Liver Disease (UKELD), a scoring system combining blood tests for bilirubin, INR, creatinine and serum sodium (Asrani and Kim, 2010); patients with NASH cirrhosis would need a UKELD of >49 to be considered for the liver-transplant waiting list (NHSBT, 2014b). However, these scoring models do not reflect other adverse factors such as quality of life and difficult-to-treat variceal bleeding (Dooley et al, 2011). There are guidelines regarding eligibility for, and contraindications to, liver transplantation complications (NHSBT, 2014b). Ms Brown was considered for a transplant but did not fulfil the criteria due to cardiovascular comorbidities, which suggested she would not survive the arduous surgical procedure and potential post-operative complications.
End-of-life care in liver disease
Murray et al (2005) outlined three distinct illness trajectories for people with progressive chronic illness:
Steady progression with a clear terminal phase, for example in cancer;
Gradual decline punctuated with episodes of acute deteriorati
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