Clinical Context
A pediatric or adult patient with confirmed mucopolysaccharidosis VII (Sly syndrome) presents for enzyme replacement therapy using vestronidase alfa-vjbk (J3397, 1 mg). The patient arrives to an infusion center or hospital outpatient infusion suite accompanied by a caregiver for baseline vitals and assessment. Prior to infusion, nursing documents weight, reviews recent labs (including liver function tests and renal function), confirms no recent febrile illness, and reviews pre-medication orders for hypersensitivity risk (e.g., antihistamine, corticosteroid, antipyretic) per the prescribing clinician.
The infusion is administered intravenously over the recommended infusion time with continuous nursing assessment for infusion-related reactions. Vital signs are recorded at baseline, periodically during the infusion, and after completion. If an infusion reaction occurs, the nurse follows the facility protocol which may include temporary infusion interruption, administration of rescue medications, and documentation of modifier(s) if applicable. Post-infusion, the patient is observed for a specified monitoring period and discharged with follow-up appointments for ongoing dosing cycles typically every two weeks as defined by the treatment plan.