Reye Syndrome

1. Introduction: Reye syndrome, first described as a disease entity in the mid-1960s, is a multisystem disorder primarily affecting children between 6 and 12 years of age. Although not truly a “communicable disease,” studies have confirmed a relationship between aspirin administration during a viral illness (chickenpox, flu) and the onset of Reye syndrome. The exact cause remains unknown. Reye syndrome is characterized by acute metabolic encephalopathy and fatty degeneration of the visceral organs, particularly the liver. Earlier diagnosis, more sophisticated monitoring equipment, and more aggressive treatment have greatly improved the survival rate of children with Reye syndrome; recovery is generally rapid in those children who do survive.
2. Assessment:
   1. Onset typically follows a viral illness, just as child appears to be recovering.
   2. Early signs and symptoms:
      1. Rapidly progressing behavioral changes: irritability, agitation, combativeness, hostility, confusion, apathy, lethargy.
      2. Vomiting, which becomes progressively worse.
   3. Rapidly progressive neurological deterioration:
      1. Cerebral edema and increased intracranial pressure.
      2. Alteration in level of consciousness from lethargy through coma, decerebrate posturing, and respiratory arrest.
   4. Liver dysfunction, necrosis, and failure:
      1. Elevated serum alanine aminotransferase (ALT) (serum glutamic-oxaloacetic transaminase [SGOT]), aspartate aminotransferase (AST) (serum glutamate pyruvate transaminase [SGPT]), lactate dehydrogenase (LDH), serum-ammonia levels.
      2. Severe hypoglycemia.
      3. Increased prothrombin time, coagulation defects, and bleeding.
3. Analysis/nursing diagnosis:
   1. Altered cerebral tissue perfusion related to cerebral edema and increased intracranial pressure.
   2. Altered hepatic tissue perfusion related to fatty degeneration of the liver.
   3. Risk for injury related to coagulation defects and bleeding.
   4. Knowledge deficit related to diagnosis, course of disease, treatment, and prognosis.
4. Nursing care plan/implementation:
   1. Goal: reduce intracranial pressure.
      1. Child is admitted to pediatric intensive care unit (PICU) for intensive nursing care, continuous observation, and monitoring.
      2. Monitor neurological status and vital signs continuously.
      3. Assist with/prepare for numerous invasive procedures, including endotracheal (ET) tube/mechanical ventilation and intracranial pressure monitor.
      4. Monitor closely for the development of seizures; institute seizure precautions.
      5. Position: elevate head of bed (HOB) 30-45 degrees.
      6. Administer medications as ordered:
         1. Osmotic diuretics (mannitol) to decrease I CP.
         2. Diuretics (Lasix) to decrease CSF production.
         3. Anticonvulsants (Dilantin, phenobarbital).
   2. Goal: restore and maintain fluid and electrolyte balance, including perfusion of liver.
      1. Administer IV fluids per physician’s order-usually 10% glucose (or higher).
      2. Strict I&O.
      3. Prepare for/assist with Foley catheter placement, CVP, ICP monitor, NG tube, etc.
      4. Monitor serum electrolyte lab values.
   3. Goal: prevent injury and possible bleeding.
      1. Observe child for petechiae, unusual bruising, oozing from body orifices or tubes, frank hemorrhage.
      2. Check all urine and stool for occult blood.
      3. Monitor lab values, including prothrombin time (PT), partial thromboplastin time (PTT), platelets.
      4. Administer blood products per physician’s order.
   4. Goal: provide parents with thorough understanding of Reye syndrome.
      1. Primary nurse assigned to provide care and follow through with teaching.
      2. Encourage parents’ presence, even in PICU-explain all equipment and procedures in simple, direct terms.
      3. Provide factual, honest, and complete information regarding disease, diagnosis, and prognosis.