Cirrhosis
Adult-Gerontology Acute Care Practice Guidelines
Definition
A.Cirrhosis: Chronic inflammation leading to fibrosis/scarring of the liver. Causes of injury may be viral, autoimmune, metabolic, drug induced, or due to alcohol and/or fat.
1.Compensated: Mild portal hypertension but normal synthetic function (bilirubin, prothrombin time [PT], albumin, creatinine).
2.Decompensated: Progression of disease due to complications of portal hypertension which include variceal hemorrhage, hepatic encephalopathy, ascites, spontaneous bacterial peritonitis (SBP).
a.Median survival in decompensated cirrhosis is 6 months or less.
b.MELD score—model to predict prognosis in patients with cirrhosis using the bilirubin, creatinine, and international normalized ratio (INR).
Incidence
A.Centers for Disease Control and Prevention (CDC) data from 2015 reports 3.9 million people in the United States with cirrhosis (see Figure 4.1).
B.National Institutes of Health (NIH)—cirrhosis is the 12th leading cause of death in the United States.
Pathogenesis
A.Most common causes of cirrhosis in the United States include: Hepatitis C, alcoholic liver disease, and nonalcoholic fatty liver disease (NAFLD). These three causes account for 80% of patients on the liver transplant list during the years 2004 to 2013.
B.Other less common causes include: Viral hepatitis B, hemochromatosis, autoimmune disease, primary biliary cholangitis, primary sclerosing cholangitis, drug-induced liver injury (DILI), Wilson’s disease, Alpha 1 antitrypsin deficiency, celiac disease, polycystic liver disease, sarcoidosis, and right-sided heart failure.
FIGURE 4.1 Common causes of cirrhosis.
Source: Chaney, A. (2017). Fast Facts about GI and Liver Diseases for Nurses: What APRNs need to know in a nutshell. New York, NY: Springer Publishing Company.
Predisposing Factors
A.Combination of more than one factor may lead to an accelerated progression to fibrosis.
1.Heavy alcohol use.
2.Viral hepatitis.
3.Fatty liver disease.
4.Genetic or metabolic disorder—that is, Wilson’s, Alpha 1 antitrypsin, hemochromatosis.
5.Autoimmune disease.
6.Hepatic congestion, that is, heart failure.
Subjective Data
A.Common complaints/symptoms.
1.Nonspecific—anorexia, weight loss, fatigue.
2.Decompensated—jaundice, abdominal distension, confusion, gastrointestinal (GI) bleeding.
B.Common/typical scenario.
1.May be incidental finding on lab tests or imaging without any symptoms.
2.There may be an acute presentation with hepatic decompensation.
C.Family and social history.
1.Detailed history of alcohol use, drug use, sex partners, body piercings, tattoos.
2.Inquire about family history of autoimmune disease, liver disease, and liver cancer.
3.Living situation—household contacts with infected individuals with viral hepatitis.
4.Occupation—potential exposure to viral hepatitis.
5.Travel—recent travel or country of origin.
D.Review of systems.
1.May present with jaundice, pruritus, easy bruising, hematemesis, melena, hematochezia, ascites, lower extremity edema, confusion, or sleep disturbances.
Physical Examination
A.May have no physical findings.
B.In cirrhosis, one may see:
1.Jaundice.
2.Spider angiomata on the neck or chest.
3.Gynecomastia.
4.Ascites—associated with edema in the scrotum and/or lower extremities.
5.Firm nodular liver.
6.Splenomegaly.
7.Palmar erythema.
8.Digital clubbing.
9.Asterixis: Bilateral flapping of dorsiflexed hands.
10.Low blood pressure.
Diagnostic Tests
A.Labs: Complete blood count (CBC), comprehensive metabolic panel (CMP), PT, INR.
1.All liver tests may be abnormal. In decompensated disease, rising bilirubin, low albumin, elevated INR, and creatinine indicate synthetic dysfunction and progression of cirrhosis.
2.Thrombocytopenia: Related to portal hypertension and splenomegaly; platelets less than 150/L.
3.Anemia: Due to GI blood loss, chronic disease, renal disease, bone marrow suppression, or hemolysis.
4.Leukopenia: Secondary to hypersplenism.
5.Hyponatremia: Inability to excrete free water.
B.Imaging.
1.Ultrasound (US): Shrunken nodular liver with or without splenomegaly/ascites.
2.US with Doppler: Portal hypertension, portal vein thrombosis, or collateral flow may be present.
3.MRI: Can identify cirrhosis, hepatocellular carcinoma, or other liver masses. It is the preferred study for cancer surveillance and staging.
C.Liver biopsy.
1.It is the gold standard to diagnose or identify the cause of cirrhosis.
2.Fibroscan is a noninvasive tool to stage fibrosis.
Differential Diagnosis
A.Cirrhosis related to chronic hepatic inflammation due to:
1.Viral: Hepatitis A, B, C.
2.Alcohol.
3.Fatty liver.
4.Autoimmune: Autoimmune hepatitis, celiac, primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC).
5.Genetic/metabolic: Wilson’s, hemochromatosis, Alpha 1 antitrypsin, polycystic liver disease.
B.Extrahepatic causes of cirrhosis.
1.Congestive heart failure.
2.Sarcoidosis.
3.Budd Chiari.
4.Portal and splenic vein thrombosis.
Evaluation and Management Plan
A.General plan.
1.See Figure 4.2.
2.Ascites and SBP.
a.Ascites: Abdominal accumulation of fluid due to high portal pressures. It is typically treated with sodium restriction and diuretics. Refractory cases require paracentesis or transjugular intrahepatic portosystemic shunt (TIPS).
b.SBP is an infection of the ascitic fluid unrelated to perforated viscus. Patients with high MELD scores are at most risk, and failure to identify the diagnosis can lead to refractory sepsis. Cultures will show high absolute polymorphonuclear leukocyte counts greater than 250 cells/mm.
3.Encephalopathy.
a.Impairment in cognitive and neuromuscular function associated with decompensated cirrhosis.
b.Disturbance in sleep pattern, insomnia, and hypersomnia are common early features before more typical levels of consciousness alterations.
c.Manifestations range from mild confusion to severe somnolence and coma.
d.Treatment is based on underlying cause/trigger (e.g., GI bleeding, infection, dehydration, medication noncompliance).
4.GI bleeding.
a.Acute variceal hemorrhage is the result of portal hypertension and usually occurs in the upper GI tracts. Other causes of GI bleeding in patients with liver disease include peptic ulcers, portal hypertensive gastropathy, and gastric antral vascular ectasias (GAVE).
b.Bleeding presents as hematemesis and/or melena.
c.Plan: Stabilization of patient.
i.Blood and blood products: Goal hemoglobin of 7 to 9 g/dL.
ii.Restoration of intravascular volume: Intravenous (IV) fluids.
iii.Treatment with endoscopic evaluation and interventions to stop the bleeding. Epinephrine, banding, Argon plasma coagulation, and clipping may be used alone or in combination.
iv.Patients at high risk of rebleeding may be considered a candidate for TIPS.
v.Bleeding that is refractory to traditional measures may require esophageal tamponade. Short-term only.
vi.Prevention and management of complications (e.g., sepsis, renal failure, aspiration pneumonia) desired.
5.Hepatorenal syndrome (HRS).
a.Chronic or acute kidney injury related to advanced hepatic failure and portal hypertension. Must exclude other causes (e.g., shock, nephrotoxic drugs, dehydration). Can be precipitated by an acute insult such as SBP or GI bleeding. Without therapy, most patients with HRS die within weeks of the onset of renal impairment.
i.Type I HRS: More severe with rapid onset and poor prognosis. May reverse if cause of hepatic disease is treatable, that is, alcohol cessation, antiviral therapy.
ii.Type II HRS: Less rapid progression.
b.Plan.
i.Correction of underlying hepatic cause, if able.
ii.Medications to raise the mean arterial pressure and improve renal perfusion.
iii.Monitor fluid status closely.
iv.Dialysis as a bridge to transplant or renal recovery.
v.May need dual renal/liver transplant.
B.Patient/family teaching points.
1.Ascites and SBP.
a.Ascitic fluid may reaccumulate despite diuretic therapy and will likely require multiple paracenteses to manage fluids and mitigate side effects such as shortness of breath and edema.
b.Daily weights.
c.2,000 mg sodium restricted diet.
d.Laboratory monitoring of electrolytes and renal function for safe dosing of diuretics.
e.Report any fever, abdominal pain, or altered mental status that may indicate SBP.
FIGURE 4.2 Comprehensive management of patients with cirrhosis.
f.Prophylactic antibiotics for SBP is lifelong therapy or until transplant.
2.Encephalopathy.
a.Review with family/caregiver to be alert for confusion, memory lapses, mood changes, speech abnormalities, slowed movements, gait disturbance, or day/night reversal.
b.Clearance of neurotoxins such as ammonia is important. This is primarily achieved through the stool. Medications for treatment target that means of clearance through promotion of several bowel movements daily.
3.GI bleeding.
a.High risk of recurrent bleeding within 6 weeks of initial episode.
b.If banding was performed, expect a sore throat for a few days. Soft diet recommended.
c.Review signs of bleeding: Vomiting blood or coffee ground emesis, black tarry or maroon-colored stools, weakness, lightheadedness.
d.If TIPS was placed, may see worsening encephalopathy.
e.Avoid NSAIDs.
4.HRS.
a.Avoid nephrotoxic drugs.
b.Stop diuretic therapy.
c.May require dialysis while awaiting transplant.
d.Teach SQ injections for home therapy (e.g., octreotide).
C.Pharmacotherapy.
1.Ascites and SBP.
a.Combination diuretics, usually Lasix and Aldactone, to balance potassium. Diuretic starting dose is 20 mg Lasix with 50 mg Aldactone, which then increases using the same ratio.
b.SBP is typically treated with third generation cephalosporin such as ceftriaxone or cefotaxime, typically for 5 days. At discharge, switch to long-term prophylaxis using Cipro 500 mg po daily or Bactrim DS one tablet daily.
2.Encephalopathy.
a.Lactulose: Synthetic disaccharide that is a mainstay of therapy and causes a purging of ammonia through the bowels. This has been shown to improve symptoms in 70% to 80% of patients. Typical dose is 30 to 45 mL orally, two to four times per day. Dose is titrated to achieve two to three bowel movements per day. If comatose, it may be given as an enema.
b.Xifaxan: Also known as rifaximin. A non-absorbable antibiotic that reduces the risk of hepatic encephalopathy recurrence. This is usually added to lactulose therapy or for patients who are intolerant of lactulose side effects. Dose is usually 550 mg orally twice daily.
c.Benzodiazepines are contraindicated with cirrhotic patients who have encephalopathy.
3.GI bleeding.
a.Vasoactive medication: Initiated at the time of presentation to decrease portal blood flow, that is, octreotide. Usually given 2 to 5 days.
b.Proton pump inhibitor.
c.Prophylactic antibiotics: Broad spectrum antibiotics such as ceftriaxone 1 g IV daily for 7 days to prevent bacterial infections, which can occur in 20% of hospitalized cirrhotic patients (e.g., urinary tract infection [UTI], bacteremia, respiratory infections).
d.A nonselective beta blocker is often prescribed to reduce portal pressures for prevention of rebleeding after vasoactive medication has been discontinued. Titrate dose to a heart rate of 60 bpm. Propranolol or nadolol are commonly used.
4.HRS.
a.Combined therapy with the goal to improve renal and systemic hemodynamics.
b.Midodrine: Selective Alpha 1 adrenergic agonist; 7.5 to 15 mg orally three times a day.
c.Octreotide: Somatostatin analog; IV infusion: 50 mcg/hr or SQ 100 mcg three times a day.
d.Albumin: Volume expander; IV bolus: 1 g per kg of body weight per day.
D.Discharge instructions.
1.Ascites and SBP.
a.Low sodium diet.
b.Take diuretics as prescribed.
c.Lab testing as recommended.
d.Follow-up with hepatologist.
e.Call office or report to emergency department (ED) with abdominal pain, distension, shortness of breath, fever, or altered mental status.
2.Encephalopathy.
a.Medication compliance is reinforced.
b.Encourage two to three bowel movements daily for control of encephalopathy.
c.Call the office or report to the ER with worsening confusion, fever, slurred speech, or lethargy.
d.Follow-up with hepatologist.
3.GI bleeding.
a.If esophageal band ligation was performed, will need a repeat esophagogastroduodenoscopy (EGD) every 4 weeks until eradicated.
b.Medication compliance with beta blockers.
c.Monitor CBC.
d.Follow-up visit with hepatology.
4.HRS.
a.Compliance with medical regimen of dialysis and medications.
b.Close monitoring of electrolytes and renal function.
c.Follow-up with hepatology.
Follow-Up
A.Follow-up with hepatology.
B.Patients will need hepatocellular carcinoma and esophageal varices screening.
1.Imaging every 6 months: Alternating US and MRI of the abdomen.
2.Variceal surveillance: EGD every 2 to 3 years.
C.Labs every 3 months: CBC, CMP, INR, alpha fetoprotein (AFP).
D.Will need vaccination for hepatitis A and B if not immune.
Consultation/Referral
A.MELD score of 10 or higher will be referred to a transplant center.
B.If alcohol-related cirrhosis, must complete a substance abuse program.
C.Social services may need to help with insurance coverage if transplant is indicated.
Special/Geriatric Considerations
A.Transplant eligibility varies between centers, but usually age greater than 70 is contraindicated.
B.Cirrhosis is considered a high risk for abdominal, cardiac, or orthopedic surgeries due to high portal pressures and coagulopathies. It is usually determined by the surgeon on a case-by-case basis.
Bibliography
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Centers for Disease Control and Prevention. (2016, October 6). Chronic liver disease and cirrhosis.
Retrieved from https://www.cdc.gov/nchs/fastats/liver-disease.htm
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