Reye's Syndrome

  1. General information
    1. An acute encephalopathy with fatty degeneration of the liver
    2. Reye's syndrome is a true pediatric emergency: cerebral complication may reach an irreversible state
    3. Increased ICP secondary to cerebral edema is major factor contributing to morbidity and mortality
    4. Early recognition and prompt management reducing mortality
    5. Etiology unknown
  2. Medical management
    1. Proper initial staging essential.
    2. Treatment is supportive, based on stage of coma and level of blood ammonia.
    3. Treatment should take place in a pediatric intensive care unit.
  3. Assessment findings
    1. Child appears to be recovering from a viral illness, such as influenza or chickenpox, during which salicylates have been administered; symptoms then appear that follow a definite pattern, which has led to clinical staging.
      1. Stage I: sudden onset of persistent vomiting, fatigue, listlessness
      2. Stage II: personality and behavior changes, disorientation, confusion, hyperreflexia
      3. Stage III: coma, decorticate posturing
      4. Stage IV: deeper coma, decerebrate rigidity
      5. Stage V: seizures, absent deep tendon reflexes, respiratory reflexes, flaccid paralysis
    2. Pathophysiologic changes include
      1. Increased free fatty acid level
      2. Hyperammonemia due to reduction of enzyme that converts ammonia to urea
      3. Impaired liver function
      4. Structural changes of mitochondria in muscle and brain tissue
      5. Significant swelling of the brain
  4. Nursing interventions (depend on stage)
    1. Stage I: assess hydration status: monitor skin turgor, mucous membranes, I&O, urine specific gravity; maintain IV therapy.
    2. Stages I-V: assess neurologic status: monitor LOC, pupils, motor coordination, extremity movement, orientation, posturing, seizure activity.
    3. Stages II-V
      1. Assess respiratory status: note changing rate and pattern, presence of circumoral cyanosis, restlessness, agitation.
      2. Assess circulatory status: frequent vital signs, note neck vein distension, skin color and temperature, abnormal heart sounds.
      3. Support child/family.
        1. explain all treatments and procedures.
        2. incorporate family members in treatment as applicable.
        3. organize regular family and client-care conferences.
        4. use support services as needed.
      4. Provide additional parental and community education to ensure early recognition and treatment.