FULMINANT HEPATIC FAILURE

DEFINITION: Fulminant Hepatic failure (FHF) is usually defined as the severe impairment of hepatic functions or severe necrosis of hepatocytes with the development of hepatic encephalopathy within 8 weeks of first symptoms of illness in the absence of preexisting liver disease. It is characterized by mental confusion, emotional instability, restlessness, bleeding manifestations and even coma. However, unlike in adults, encephalopathy may be absent, late, or unrecognized in children. Thus, the emphasis in children is placed on the presence of significant coagulopathy in the absence of sepsis or disseminated intravascular coagulation. ETIOLOGY. Various etiologies have been found to cause fulminant hepatic failure depending on the age at which the condition occurs. TABLE 1. THE LIKELY ETIOLOGIES FOR FULMINANT HEPATIC FAILURE FOR DIFFERENT AGE GROUPS INFANCY (0-6 MONTHS) Congenital viral infections: Echovirus Herpesvirus CMV Hepatitis B Galactosemia LATE INFANCY (6-36 MONTHS) Hepatitis A Hepatitis B Delta hepatitis EB virus Drugs, toxins CHILDHOOD Hepatitis A Hepatitis B Delta hepatitis EB virus Drugs, toxins Reyes Syndrome

(Source: del Mundo,F, et. al. (2000). Textbook of pediatrics and child health. (4 th Ed.).Quezon City: JMC Press, Inc., pp. 801)

TABLE 2. HEPATIC ENCEPALOPATHY: CAUSES AND PRECIPITATING FACTORS Decrease in hepatocellular function Hypoxia Infection Diuretics (produce Hypokalemia, alkalosis and hypovolemia) Depressants: phenobarbital, narcotics, tranquilizers and sedatives) GI bleeding Medications containing ammonium and amino compounds Paracentesis Increased protein intake

Constipation Dehydration Hypokalemia Portosystemic and portacaval shunts

(Source: Black, J.M. and Hawks, J.H. (2008). Medical-surgical nursing: Clinical management and positive outcomes. (8th Ed.). Singapore: Elsevier Pte Ltd., Volume 1,pp.1166)

CLINICAL MANIFESTATIONS:

Deepening jaundice (with bilirubin >20mg/dL)

falling aminotransferases ascites hypoglycemia low BUN low albumin low cholesterol leukocytosis thrpmbocytopenia

Persistent anorexia Vomiting fever mental status changes hyperventilation respiratory alkalosis shrinking liver size

vitamin K resistant hypoprothrombinemia

Patients with FHF go into various stages of hepatic encephalopathy. TABLE 3. STAGES OF HEPATIC ENCEPALOPATHY A PATIENT WITH FHF WILL UNDERGO. STAGES STAGE I Disturbances of consciousness or motor function MANIFESTATIONS fatigue restlessness irritability decreased intellectual performance decreased attention span diminished short-term memory personality changes sleep pattern reversal

STAGE

II

Drowsy,

but

Deterioration in handwriting

responsive to commands

Asterixis flapping tremor of the fingers and the wrist elicited by dorsiflexion of the wrist with the arm extended and forearm fixed Drowsiness Confusion Lethargy Fetor hepaticus mildly fruity, fecal or musty odor, specific for hepatic failure

STAGE III stuporous responsive to painful stimuli

but

Severe confusion Enability to follow commands Deep somnolence, but arousable Coma Unresponsive to painful stimuli Possible decorticate or decerebrate posturing

STAGE IV unresponsive to painful Stimuli

(Source: Black, J.M. and Hawks, J.H. (2008). Medical-surgical nursing: Clinical management and positive outcomes. (8th Ed.). Singapore: Elsevier Pte Ltd., Volume 1,pp.1167)

LABORATORY/ DIAGNOSTIC TESTS: 1. ABG analysis to determine acidosis/alkalosis. 2. Blood Chemistry to determine components in the blood (e.g. glucose, urea) 3. CBC or hematocrit to monitor presence of infection and anemia 4. CT scan of the head or MRI 5. EEG 6. Liver function tests 7. Prothrombin time 8. Serum ammonia levels 9. Sodium level in the blood 10. Potassium level in the blood 11. BUN and creatinine to see how the kidneys are working MEDICAL MANAGEMENT:

1.

Identify and Treat Precipitating Causes. Factors that may precipitate or severely aggravate hepatic encephalopathy in clients with severe liver disease are listed on Table 2. Protein may be total eliminated in the diet, with an intake of only fruit juices and IV fluids, although this radical restriction leads to the catabolism of the clients own protein stores. The usual protein restriction is 20 40 g daily. The client with chronic hepatic encephalopathy may need to adjust to a long-term, low-protein diet (50-60g/day). Vegetable and dairy protein may be tolerated than meats. These proteins contain fewer ammonia-forming amino acids than those in meat. A diet high in vegetables and products also helps to prevent constipation, thus further reducing ammonia production. Hypokalemia can be handled using IV KCl or KPO4 in severe cases. Stop giving potassium - losing diuretics as these may aggravate the already existing hypokalemia. Hyperkalemia on the other hand may be severe to require dialysis. Hypoglycemia usually respond to 10% IV dextrose infusion unless the hepatic dysfunction is severe. Metabolic acidosis can be combated by ensuring adequate blood volume. Intravenous albumin may at times be needed to expand the plasma volume. Dialysis is resorted if no response is observed. An H2 receptor antagonist is given to keep gastric pH > 5.0. Vitamin K is given either therapeutically at 1mg/kg/dose OD, not exceeding 10mg/day or prophylactically at the same dose every 5-7 days.

2. Reduce Nitrogenous Waste (ammonia) in Blood and Bacteria in

Colon. Neomycin and lactulose are given to reduce bacteria in the intestinal tract. Because it is not absorbed into the circulation, neomycin exerts a powerful effect on the intestinal bacteria that are responsible for ammonia production. Undesirable side effects result from the depletion of intestinal flora (e.g. diarrhea, vitamin K deficiency). Also, because it is nephrotoxic, its use must be avoided in clients with renal insufficiency. Lactulose, which helps decrease blood ammonia levels by reducing absorption of ammonia, is given to produce 2-4 stools a day. Antibiotics are administered to inhibit growth of GI bacteria and oral magnesium sulfate or enemas are given after hemorrhage to clean out the intestines.

3. Maintain Fluid Volume Balance. IV fluids are administered, carefully monitoring the quantity and rate of administration. NURSING MANAGEMENT: ASSESSMENT 1. Use interviewing and psychophysiologic status. assessment techniques to evaluate

2. Observe client for personality changes with labile feelings states

and elicit liver flap or flapping (asterixis) by asking client to dorsiflex the hand with the rest of the arm resting on the bed. In asterixis, the hand cannot be held steady. 3. Jaundice is the presenting symptom in most pediatric clients. A prodromw of flulike illness that may precede jaundice. Fever, anorexia and vomiting, abdominal pain and fetor hepaticus are associated clinical findings. Infants initially may present with poor feeding, irritability, and disturbances in sleep rhythms, with frank features of encephalopathy manifesting only later. 4. Assess change in the level of mental functioning.

INTERVENTIONS
1. Promote

Low-Protein diet. It is important that the client understand the importance of the reduced protein diet to have the motivation to remain on this diet. for Gastrointestinal Hemorrhage. Assess for manifestations of GI bleeding, checking for bright red blood in the stool or for black, tarry stools. To reverse the progression of manifestations, constipation must be prevented. Administer catharthics and enemas to hasten the exit of protein material from the intestines. Infusion of platelets is recommended if platelets are less than 50,000/cumm. Fresh frozen plasma is administered only in cases of active bleeding.

2. Monitoring

3. Encourage Bowel Cleansing. The client may need to learn to

manage diarrhea, a possible side effect related to laxative action of lactulose or neomycin sulfate. Intervention in severe hepatic encephalopathy commonly combines neomycin therapy with protein restriction and bowel cleansing. Administer prescribed maintenance doses of neomycin and provide low-protein diet for clients with chronic hepatic encephalopathy.
4. In

addition, administer oral lactulose, a combination of galactose and fructose that passes through the intestine unchanged to decrease the ammonia by trapping ammonia ions and allowing their evacuation from the bowel. The appropriate lactulose dosage causes two to four soft stool evacuation daily. if severe diarrhea occurs, the dosage is reduced to prevent further electrolyte imbalance. to prevent further hepatic injury and reduced renal perfusion. Deliver IV fluids evenly overtime. Monitor vital signs frequently. If necessary, measure urine output hourly. Restriction of fluids and salt and the administration of an aldosterone antagonists are recommended. Fluid restriction is 60-80mL/kg/day.

5. Fluid balance must be achieved, maintained, and monitored

6. Prevent

Hypoxemia. Hypoxemia may precipitate hepatic encephalopathy by damaging the hepatic cells. To prevent and treat this, attend to respiratory interventions (e.g. maintain patent airway). Hyperventilation to maintain PaCO2 25 to 30 mmHg may have to be administered for >5 min in patients with significant cerebral edema. protein from tissue catabolism, necessitates rapid intervention. The client is particularly vulnerable to nosocomial (hospital-acquired) infections. Wash your hands thoroughly and take other measures to prevent cross-contamination.

7. Prevent Infection. Concurrent infection, with accumulation of

8. Prevent Ammonia Toxicity and Hypokalemia. Be alert to

possible harmful accumulation of ammonia as a result of diuretic therapy. Hypokalemia from the use of diuretics contributes to hepatic encephalopathy by increasing ammonia production in the kidney.

9. Avoid

Sedation. Agents with CNS-depressant effects may precipitate coma and their use should be avoided. If agitation occurs in early encephalopathy, administer agents that are excreted partially through the kidney. Administer phenobarbital with caution. Know which opioids, tranquilizers and sedatives are biotransformed by the liver; they are often contraindicated in clients with decreased hepatic function. Administration of benzodiazepine antagonists (flumazenil). It is designed to block the benzodiazepine receptor and thus reverse the sedative effect. Prevent Complications of Immobility. Prevent pneumonia and skin breakdown by turning client frequently and promoting lung aeration. As the body accumulates metabolic substances, physiologic disturbances develop that may produce a state of agitation. Therefore, protect client from self-injury (e.g. lowering the bed and padding side rails).

10.

11. Review all medications along with scheduled times of administration and their intended and adverse side effects. Potential medications include lactulose and vitamin supplements. 12. Explain the importance of well-balanced, nutritional diet of the client with specific information about limitations on dietary protein, sodium, and water. Teach family members and significant others about the need to encourage eating and still maintain food intake within prescribed limits. 13. Teach that the clients home may need to be altered to adjust for limitations in mobility. Safety precautions should be taken to help prevent injury to the client. The clients bedroom should be near the bathroom if receiving diuretics. 14. The clients status should be followed closely. Be sure that the clients caregivers are aware of any changes that require immediate medical attention. They should also know that diagnostic testing at regular intervals is continued to monitor status of liver.

BIBLIOGRAPHY:

Black, J.M. and Hawks, J.H. (2008). Medical-surgical nursing: Clinical management and positive outcomes. (8th Ed.). Singapore: Elsevier Pte Ltd.,

Del Mundo,F, et. al. (2000). Textbook of pediatrics and child health. (4th Ed.).Quezon City: JMC Press, Inc.,)