Reye’s syndrome /rāz″/ [Ralph D.K. Reye, Australian pathologist, 1912–1978] , a combination of acute encephalopathy and fatty infiltration of the internal organs that may follow acute viral infections. This syndrome has been associated with influenza B, chickenpox (varicella), the enteroviruses, and the Epstein-Barr virus. It usually affects people under 18 years of age, characteristically causing an exanthematous rash, vomiting, and confusion about 1 week after the onset of a viral illness. In the late stage there may be extreme disorientation followed by coma, seizures, and respiratory arrest. Laboratory tests reveal greater-than-normal amounts of SGOT and SGPT, bilirubin, and ammonia in the blood. A specimen obtained by liver biopsy shows fatty degeneration and confirms the diagnosis. Mortality varies between 20% and 80%, depending on the severity of symptoms. The cause of Reye’s syndrome is unknown; however, there appears to be an association with the administration of aspirin. Therefore aspirin is given only if prescribed by a physician for any condition in infants or children. Aspirin should not be given in cases of chickenpox or suspected influenza. No specific treatment is available. Insulin, antibiotics, and mannitol may be given. Blood gases, blood pH, and blood pressure are monitored frequently. Intensive supportive nursing care with meticulous monitoring of all vital functions and prompt correction of any imbalance are of extreme significance in the outcome of this syndrome. ▪ OBSERVATIONS: Reye’s syndrome is staged by the characteristics manifested. In stage I, the child is usually quiet, lethargic, and drowsy, with episodes of vomiting. Pupil reactions are brisk, and commands are followed. Serum lab values show evidence of liver dysfunction; EEG is type 1. In stage 2, there is evidence of deep lethargy, confusion, delirium, and combativeness. Reflexes are hyperreflexic, and hyperventilation is present. Pupillary reactions are sluggish. In stage 3, senses are obtunded, and light coma is present with seizure activity and decorticate rigidity. Pupillary light reaction is still intact. EEG is type II. In stage 4, the coma deepens with seizure activity and decerebrate rigidity. There is loss of oculocephalic reflexes, and pupils are fixed. EEG is type III or IV, and there is evidence of brain dysfunction. In stage 5, there is deep coma, loss of deep tendon reflexes, respiratory arrest, fixed and dilated pupils, and type IV EEG. Definitive diagnosis is established by liver biopsy or by a threefold rise in levels of serum aspartate aminotransferase, serum alanine aminotransferase, or serum ammonia. Survivors may exhibit a neuropsychological deficit. ▪ INTERVENTIONS: Deterioration is generally rapid and requires early diagnosis and aggressive treatment. Care in stages 1 and 2 is largely supportive, with frequent monitoring and evaluation of neurological status. Management of cerebral edema and increased intracranial pressure is a key focus in stages 3 through 5 and is monitored with an ICP monitor and treated by administration of mannitol, glycerol, and/or hyperventilation via endotracheal tube and ventilator. Pancuronium bromide is used to immobilize the child on a ventilator. Seizures are managed by IV phenytoin. NG tubes, urinary catheters, and peripheral IVs are placed to manage fluids. An arterial catheter is placed to permit continuous blood pressure measurement and monitor blood gases. A central venous catheter permits monitoring of blood volume and cardiac function and administration of hypertonic solutions. A pulmonary artery catheter may be inserted to monitor pulmonary artery pressure and cardiac output. A cooling blanket may be indicated to manage temperature. If increased intracranial pressure fails to respond to treatment, a decompressive craniotomy may be indicated. Prevention is targeted around routine vaccinations for influenza and varicella and avoidance of aspirin in children. ▪ PATIENT CARE CONSIDERATIONS: Nursing interventions during acute disease are continuous and intensive. Careful evaluation is required for neurological, cardiac, and respiratory systems. Management is required for multiple lines and tubes, including peripheral and central IV lines, arterial lines, CVP lines, ICP monitors, retention catheters, NG tubes, and endotracheal tubes. Management of ventilator settings is required. Careful fluid management and intake and output is required to reduce cerebral edema and prevent dehydration. Careful supportive care, including careful hygiene, positioning, and passive range of motion, is required to maintain skin integrity and prevent sequelae of immobility. Sensory stimulation is needed for the unconscious child. Emotional support for parents is essential because the sudden severity and intensity of the disease and the aggressive treatment is a source of extreme anxiety and fear. Prevention is targeted around education about maintaining routine vaccinations for influenza and varicella and the avoidance of aspirin in children.
