Understanding this condition will help you provide optimal care for patients and their families.

Transcription

1 Cirrhosis A complex cascade of care ILLUSTRATION BY STEVE OH/PHOTOTAKE Understanding this condition will help you provide optimal care for patients and their families. By Ann Crawford, PhD, RN, CNS, CEN, and Helene Harris, MSN, RN Cirrhosis is a complex disorder; the initial insult causes a cascade of events that will affect the patient both physically and emotionally. The nurse should have a thorough understanding of the disease and its manifestations in order to provide optimal care for patients and their families. To that end, this article provides an overview of the disease, its complications, appropriate treatments, and nursing interventions. Liver function The liver is the largest visceral organ in the body. It s located below the diaphragm, primarily in the right upper quadrant of the abdomen, nestled up and under the lower aspect of the rib cage. 1 The left and right lobes comprise functioning components (lobules) containing cells called hepatocytes. The hepatic artery and the portal vein are the major blood vessels for liver perfusion. Over 400 functions of the liver may be classified within three main categories: storage, protection, and metabolism. The liver stores several minerals and vitamins including the fat-soluble vitamins (A, D, E, and K). Within its protective role, the liver contains Kupffer cells, which destroy harmful bacteria and other harmful matter. It also detoxifies potentially dangerous substances, including alcohol and drugs. 1,2 The liver is involved in many 26 l Nursing2013CriticalCare l Volume 8, Number 4

2 metabolic pathways, including carbohydrate, fat, and protein metabolism. 1,2 Cirrhosis pathophysiology Cirrhosis is a slowly developing, chronic condition where the functional hepatic tissue is replaced by fibrous connective tissue. It s commonly associated with alcoholism and chronic viral hepatitis, although it has other causes such as inherited metabolic liver diseases. This widespread and permanent scarring (fibrosis) of the liver tissue can obstruct bile ducts and compress blood vessels and lymphatic channels. Obstruction of the biliary channels leads to biliary stasis, while the interruption of the normal blood flow patterns leads to portal hypertension and its complications. The progressive degeneration and destruction of hepatocytes ultimately leads to liver failure. Cirrhosis is considered compensated as long as the liver is able to perform essential functions with limited signs and symptoms. The onset of complications and significant clinical manifestations heralds the onset of decompensated cirrhosis. The process may take weeks to years to progress to end-stage liver disease. 1,3,4 Signs and symptoms Due to the slow progression of the disease, the early signs and symptoms of cirrhosis are often vague and nonspecific. Patients may complain of fatigue, nausea, vomiting, weight loss, anorexia, and abdominal pain. As the disease progresses, the patient will present with multisystem manifestations of severe liver dysfunction and its complications, including portal hypertension and gastrointestinal (GI) bleeding. (See Clinical manifestations of cirrhosis and its complications.) Complications of cirrhosis Complications of cirrhosis include ascites, portal hypertension, variceal hemorrhage, spontaneous bacterial peritonitis, hepatic encephalopathy, and hepatorenal syndrome. 2,3,5 Ascites, or the accumulation of fluid in the peritoneal cavity, is the most common complication of cirrhosis. Ascites is directly related to the increased hydrostatic pressure caused by portal hypertension forcing fluid from the vascular compartment into the peritoneal cavity. Hypoalbu minemia, secondary Clinical manifestations of cirrhosis and its complications 1,2,3 (Symptoms may vary based on disease progression) Skin Ecchymoses Peripheral edema Jaundice Palmar erythema Pruritus Petechiae Spider angiomas Caput medusae GI Abdominal pain/ tenderness Ascites Anorexia Weight changes Fatty stools Acholic (clay-colored) stools Esophageal varices Hepatosplenomegaly Nausea/vomiting GI bleeding Fetor hepaticus Cardiovascular Dysrhythmias Portal hypertension Neurologic Asterixis Decreased mentation Emotional lability Depression Paresthesias Altered sleep/wake cycles Sensory disturbances Pulmonary Dyspnea Hyperventilation Hypoxemia Renal Hepatorenal syndrome Increased urine bilirubin Dark, amber urine Hematology Anemia Leukopenia Thrombocytopenia Other manifestations Gynecomastia Testicular atrophy Decreased libido and infertility to decreased hepatic synthesis of plasma proteins, is also a contributing factor. Hypoalbuminemia causes a decrease in plasma colloid osmotic pressure, which results in a fluid shift from the vascular compartment into the peritoneal cavity. 1,6 This third-space fluid shift may lead to intravascular hypovolemia and hemodynamic instability. Intravascular hypovolemia also activates the reninangiotensin-aldosterone system as a compensatory mechanism to retain sodium and water and increase intravascular volume. Because of the hypoalbuminemia, this fluid increases the ascites. 4,5,6 Portal hypertension (PHT) is a persistent elevation in pressure in the portal venous system (pressure in the portal venous system is normally about 3 mm Hg). Although PHT is an increase to at least 10 mm Hg, a portal pressure of approximately 12 mm Hg is generally required for fluid retention. July l Nursing2013CriticalCare l 27

3 Cirrhosis: A complex cascade of care In cirrhosis, PHT occurs due to impeded or obstructed blood flow through the portal venous system secondary to hepatic fibrosis. Sustained PHT causes the development of collateral veins and the formation of varices (dilated collateral veins). Varices can form in the esophagus, stomach, abdominal wall, and rectum. If these varices rupture, the patient can experience life-threatening hemorrhage. 3,5 Variceal hemorrhage occurs in about 30% of patients with compensated cirrhosis, with the number increasing to 60% in those patients with decompensated cirrhosis. 1 Variceal hemorrhage occurs in 25% to 40% of patients with cirrhosis. Although survival has improved with appropriate treatment, mortality remains high. Each episode of active variceal hemorrhage is associated with 30% mortality. Patients who survive an episode of active bleeding have a 70% risk of recurrent hemorrhage within 1 year. Spontaneous bacterial peritonitis (SBP) is an infection of ascitic fluid without evidence of a secondary intra-abdominal infection source. Infectious agents are believed to originate from increased intestinal permeability and bacterial translocation. Bacterial peritonitis is noted in about 30% of patients with ascites. 5,6 Signs and symptoms include fever, chills, and abdominal tenderness and pain. 1 Hepatic encephalopathy. There are many factors facilitating the development of hepatic encephalopathy, although the exact pathogenesis is not clearly defined. Some hypotheses include changes in the neurotransmitter pathways and changes in cerebral blood flow. The most probable cause is portal venous blood bypassing the liver and being shunted directly into the circulatory system without hepatic detoxification. 5 Hyperammonemia is common in patients with hepatic encephalopathy, but there is no correlation between serum ammonia levels and encephalopathy severity. Other possible risk factors for hepatic encephalopathy in patients with cirrhosis include hypovolemia, hypokalemia, GI bleeding, and certain drug toxicities. A high-protein diet may Ascites, or the accumulation of fluid in the peritoneal cavity, is the most common complication of cirrhosis. also be a contributing factor for the development of hepatic encephalopathy. 1 Signs and symptoms include memory impairment, decreased attention, mental status changes, mood swings, and sleep disturbances. Late signs include altered reflexes and decreased level of consciousness, including coma. 2,4 Hepatorenal syndrome (HRS) refers to the development of renal failure in a patient with advanced liver disease and is an end-stage complication of cirrhosis. The prognosis for these patients is poor. HRS is a result of decreased renal perfusion secondary to severe liver disease. The patient will have oliguria, an elevated blood urea nitrogen and serum creatinine, and an increased urine osmolarity. HRS can be precipitated by SBP, GI hemorrhage, or hepatic encephalopathy. 5 Diagnosis Diagnosis of cirrhotic liver disease, with its potential complications, is made through a combination of patient history, physical assessment findings, and diagnostic study results. (See Abnormal lab findings in liver dysfunction and its complications.) Abdominal ultrasound is often used to detect hepatomegaly, splenomegaly, and ascites in an individual with suspected liver disease, as well as to assess for biliary calculi and/or biliary tract obstruction. Contrast-enhanced computed tomography and magnetic resonance imaging detect structural changes within the liver tissue and surrounding areas. Liver biopsies facilitate diagnosis and allow monitoring of disease progression, although bleeding is a risk. Esophagogastroduodenoscopy can visualize the upper GI tract for abnormalities, such as bleeding varices, and endoscopic retrograde cholangiopancreatography can visualize the biliary tract for abnormalities, such as biliary calculi. 1,3 Treatment Treatment for cirrhosis is centered on appropriate interventions to manage and treat the clinical 28 l Nursing2013CriticalCare l Volume 8, Number 4

4 manifestations and complications associated with liver dysfunction and to prevent further damage to the liver. Liver tissue does have the ability to regenerate to a certain extent, dependent upon the level of damage to the cell. Any treatment that slows disease progression can have positive effects on overall patient health. Patients with cirrhosis are often malnourished, so adherence to a healthy diet is essential in all stages of the disease. These patients need a diet with high-carbohydrate, moderate-fat, and high-protein foods. This must be revised, however, to a lowprotein diet in the presence of encephalopathy. Raw shellfish should be avoided, as it may contain harmful bacteria. In addition, vitamin supplements may be added due to the inability of the damaged liver to store vitamins. Patients should abstain from alcohol and illicit substances, and must adhere to their healthcare provider s instructions when taking prescription and over-the-counter medications as they may adversely affect liver function. 1,5 Most interventions for ascites focus on maintaining a negative sodium balance, including sodium restriction and diuretics. Sodium restriction and diuretic therapy help to decrease extracellular fluid volume to reverse abnormal fluid retention. A paracentesis may be required for ascites refractory to diuretics and sodium restriction. I.V. albumin administration helps to increase intravascular osmotic pressure to prevent further fluid shifts into the extravascular spaces. 1,2,6,8 Beta-adrenergic blockers are administered to treat PHT and prevent GI variceal bleeding. 8 The use of beta-blockers may decrease PHT by 15-20% and bleeding episodes in the upper GI tract by 40%. 5 Endoscopic band ligation of varices may also be performed to prevent variceal bleeding. Patients with variceal hemorrhage have a high mortality and should be treated emergently. Interventions include short-term antibiotic prophylaxis, pharmacologic therapy with somatostatin or its analogues, and endoscopic variceal ligation or sclerotherapy. As liver function deteriorates, fluid retention increases the risk of SBP. Treatment for SBP includes administration of I.V. antibiotics and albumin. Albumin infusion has been associated with a significant decrease in renal dysfunction and a significant reduction in mortality in patients with SBP. Abnormal lab findings in liver dysfunction and its complications 1,3 Serum enzymes Elevated aspartate aminotransferase Elevated alanine aminotransferase Elevated lactate dehydrogenase Elevated serum alkaline phosphatase Serum bilirubin Elevated total bilirubin Elevated direct (conjugated) bilirubin Elevated indirect (unconjugated) bilirubin Serum proteins Decreased serum total protein Decreased serum albumin Elevated serum globulin Complete blood cell count Decreased red blood cell count Decreased platelets Decreased hemoglobin Decreased hematocrit Serum electrolytes Hypernatremia Dilutional hyponatremia Hypocalcemia Hypokalemia Other lab results Elevated serum ammonia Prolonged prothrombin time Increased international normalized ratio Blood urea nitrogen (BUN) elevated with renal dysfunction Serum creatinine elevated with renal dysfunction Elevated urine bilirubin Elevated urine urobilirubin Decreased fecal urobilirubin Treatment of hepatic encephalopathy includes the identification and treatment of any underlying cause, such as GI bleeding, along with measures to decrease the serum ammonia level. Drug therapy to treat hyperammonemia includes administration of the nonabsorbable disaccharide, lactulose. Lactulose can be given orally or as an enema. Dosage is titrated so that the patient has two to three soft stools per day. If lactulose alone is not effective, then nonabsorbable antibiotics such as neomycin or rifaximin may be administered to decrease intestinal ammonia production. 1,5 Hepatic encephalopathy may improve as other complications of cirrhosis are treated. HRS is treated by giving medications that will increase renal perfusion. A combination of I.V. July l Nursing2013CriticalCare l 29

5 Cirrhosis: A complex cascade of care albumin and the vasopressor midodrine and/or octreotide is beneficial for almost two-thirds of patients with HRS. Patients not responding to pharmacologic therapy may need hemodialysis. 1,5 Additional interventions focus on identifying and treating the underlying cause of cirrhosis to limit disease progression. For example, treatment of chronic hepatitis C virus (HCV) infection with antivirals such as peginterferon combined with ribavirin may help to decrease cellular injury. In addition, tell patients to abstain from alcohol and other substances toxic to the liver tissue. 2 When the liver disease is end-stage and its complications can t be controlled by treatment, liver transplantation may be considered. Nursing care of the patient with cirrhosis Nursing care of the patient with cirrhosis is complex. The patient may not have any complaints of discomfort during the early stages of the disease; however, as the disease progresses, the patient will have numerous health-related problems, so nurses need to know the manifestations of cirrhosis and its effect on other body systems. Obtain a thorough health history including occupational history, alcohol use, drugs (prescription and illicit), and exposure to toxic chemicals, as well as any risk factors for HCV, such as prior needlestick injury. Question patients admitting to alcohol and/or drug use to determine specifics of use. To elicit honest answers without causing potential embarrassment to the patient, use a nonjudgmental attitude when interviewing patients. 1,2 Perform a thorough head-to-toe physical assessment. Assess neurological status to determine level of consciousness and identity mental status changes and changes in mood patterns. Assess cardiovascular status and monitor for any signs of bleeding, such as hematemesis, melena, or hypovolemia. Monitor vital signs regularly, identifying and communicating any significant changes or trends to the healthcare provider. Monitor respiratory effort, breathing pattern, and lung sounds for effective oxygenation. 1,2 Assess skin for abnormalities, such as jaundice and ecchymoses. Assess for abdominal tenderness/ pain and for the presence of ascites. Assess dietary intake, while encouraging adherence to a sodiumrestricted diet. Monitor intake and output, documenting urine and stool characteristics, in addition to daily weight and abdominal girth measurements. Monitor for adverse reactions and effectiveness of medications, assist with ambulation, and implement patient safety measures. Monitor lab results and report any abnormalities to the healthcare provider. Patient and family education Teach patients with cirrhosis and their families about the disease and its manifestations, effects, treatment plans, and continued care needs. Encourage patients to eat a healthy diet with sufficient calories and carbohydrates to avoid protein breakdown, and to avoid alcohol and other hepatotoxic substances. Ensure that patients have an understanding of their medications actions, uses, and adverse reactions, as well as which medications are contraindicated. Emphasize the importance of keeping scheduled appointments with their healthcare provider and of notifying the healthcare provider if symptoms worsen. 1,2 Summary Cirrhosis is a complex condition with multisystem effects. By understanding its underlying pathophysiology and how it affects quality of life you can be an advocate for the patient. With thorough assessment and appropriate interventions, you may be able to prevent and identify potentially lifethreatening complications of cirrhosis. REFERENCES 1. Ignatavicius D, Workman L. Medical-Surgical Nursing: Patient-Centered Collaborative Care. 7th ed. St. Louis, MO: Elsevier Saunders; Urden LD, Stacy KM, Lough ME. Critical Care Nursing: Diagnosis and Management. 6th ed. St. Louis, MO: Mosby Elsevier; Lefton H, Rosa A, Cohen M. Diagnosis and epidemiology of cirrhosis. Med Clin North Am. 2009;93(4): Porth CM, Matfin G. Pathophysiology: Concepts of Altered Health States. 8th ed. Philadelphia, PA: Lippincott Williams & Wilkins; Dong MH, Saab S. Complications of cirrhosis. Dis Mon. 2008;54(7): Kashani A, Landaverde C, Medici V, Rossaro L. Fluid retention in cirrhosis: pathophysiology and management. QJM. 2008;101(2): Moreau R. Hyponatremia in cirrhosis. Pathophysiology, prevalence, prognostic value, treatment. Acta Gastroenterol Belg. 2008;71(4): Adams M, Koch R. Pharmacology: Connections to Nursing Practice. Upper Saddle River, NJ: Pearson; Hodgson B, Kizior R. Saunders Nursing Drug Handbook. St. Louis, MO: Elsevier Saunders; Ann Crawford is a professor in the College of Nursing at University of Mary Hardin-Baylor in Belton, Tex. Helene Harris is a clinical educator at Central Texas Veterans Health Care System in Temple, Tex. The authors have disclosed that they have no financial relationship related to this article. DOI /01.CCN f 30 l Nursing2013CriticalCare l Volume 8, Number 4