Life-Limiting Conditions in Adult Patients · 4 min read
Liver Failure
Definition and Classification
Liver failure represents the loss of hepatic function sufficient to cause clinical manifestations. It is classified as acute or chronic based on the presence or absence of pre-existing liver disease and the time course of deterioration.
Acute liver failure (ALF) occurs in patients without pre-existing liver disease and is characterized by coagulopathy (INR greater than 1.5) and encephalopathy within 26 weeks of symptom onset. Hyperacute failure develops within 7 days, acute failure within 8 to 28 days, and subacute failure within 5 to 26 weeks.
Chronic liver failure results from progressive liver disease over months to years, typically culminating in cirrhosis. Acute-on-chronic liver failure (ACLF) describes acute deterioration in a patient with known chronic liver disease, often precipitated by infection, bleeding, or other insults.
Etiology
Acute liver failure causes include acetaminophen toxicity (most common in the United States and United Kingdom), other drug-induced liver injury, viral hepatitis (particularly hepatitis A, B, and E), autoimmune hepatitis, Wilson disease, Budd-Chiari syndrome, ischemic hepatitis, and pregnancy-related conditions. In many cases, the etiology remains indeterminate.
Chronic liver disease leading to cirrhosis results from alcohol-associated liver disease, chronic viral hepatitis B and C, nonalcoholic steatohepatitis (NASH), autoimmune conditions, hereditary hemochromatosis, alpha-1 antitrypsin deficiency, primary biliary cholangitis, primary sclerosing cholangitis, and cardiac cirrhosis.
Pathophysiology
The liver performs hundreds of essential functions including synthesis of clotting factors, albumin, and other proteins; metabolism of drugs, hormones, and toxins; bile production and bilirubin conjugation; ammonia detoxification; glucose homeostasis; and immune function.
Liver failure disrupts all these functions. Coagulopathy results from decreased production of clotting factors. Hypoalbuminemia causes ascites and edema. Accumulation of ammonia and other toxins produces hepatic encephalopathy. Impaired bilirubin conjugation leads to jaundice. Portal hypertension from cirrhosis causes varices, splenomegaly, and ascites.
Hepatorenal syndrome represents functional renal failure in advanced liver disease, resulting from splanchnic vasodilation and renal vasoconstriction. Hepatopulmonary syndrome causes hypoxemia from intrapulmonary vascular dilatations. Cirrhotic cardiomyopathy impairs cardiac function.
Clinical Manifestations
Early symptoms of liver disease include fatigue, anorexia, nausea, right upper quadrant discomfort, and pruritis. As disease progresses, patients develop jaundice, dark urine, pale stools, easy bruising, and spider angiomata.
Decompensated cirrhosis manifests with ascites causing abdominal distension and discomfort, peripheral edema, hepatic encephalopathy ranging from subtle cognitive changes to coma, variceal bleeding presenting as hematemesis or melena, spontaneous bacterial peritonitis, and hepatorenal syndrome.
Physical examination findings include icterus, spider angiomata, palmar erythema, gynecomastia, testicular atrophy, asterixis, caput medusae, hepatomegaly or small shrunken liver in advanced cirrhosis, splenomegaly, ascites, and peripheral edema.
Disease Trajectory and Prognostication
Chronic liver disease follows a variable trajectory. Patients may remain compensated for years before developing complications. Once decompensation occurs, median survival without transplantation is 2 years. However, the course is characterized by acute exacerbations with partial recoveries, making prognostication challenging.
Prognostic models include the Model for End-Stage Liver Disease (MELD) score, which uses bilirubin, INR, and creatinine to predict 90-day mortality. MELD-Na incorporates sodium levels. Child-Pugh classification uses ascites, encephalopathy, albumin, bilirubin, and INR to categorize patients as Class A, B, or C with corresponding survival estimates.
Indicators of poor prognosis include refractory ascites, recurrent hepatic encephalopathy, hepatorenal syndrome, variceal bleeding despite optimal management, hepatopulmonary syndrome with significant hypoxemia, hepatocellular carcinoma beyond transplant criteria, MELD score greater than 20, and Child-Pugh Class C cirrhosis.
Hospice and Palliative Care Considerations
Liver failure presents unique palliative care challenges. Patients experience significant symptom burden including pain, fatigue, pruritis, dyspnea, anorexia, and cognitive impairment from encephalopathy. The unpredictable trajectory complicates advance care planning and prognostic discussions.
Ascites management includes sodium restriction, diuretics (spironolactone and furosemide), and therapeutic paracentesis. Large-volume paracentesis provides symptom relief but carries risks and requires albumin replacement. Transjugular intrahepatic portosystemic shunt (TIPS) may reduce ascites but worsens encephalopathy.
Hepatic encephalopathy management involves lactulose to reduce ammonia absorption, rifaximin as adjunctive therapy, identification and treatment of precipitants, and avoidance of sedating medications. Encephalopathy significantly impacts quality of life for patients and caregivers.
Pain management requires careful medication selection. Acetaminophen is acceptable at reduced doses (2 grams daily maximum). Nonsteroidal anti-inflammatory drugs are contraindicated due to renal and bleeding risks. Opioids require dose reduction and longer dosing intervals due to impaired hepatic metabolism.
Pruritis management includes cholestyramine, rifampin, naltrexone, and sertraline. Topical agents provide limited relief. Severe pruritis significantly impacts sleep and quality of life.
Psychosocial considerations include the impact of encephalopathy on decision-making capacity, caregiver burden of managing complex medical regimens, guilt associated with alcohol-related liver disease, and grief over loss of transplant candidacy.
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