OH Epic formulary, available to order through therapy plan, restricted to:

• Indication: Treatment of non–central nervous system manifestations of acid sphingomyelinase deficiency (ASMD)
• Service line: Pediatric GI/Hepatology/Genetics
• Formulary location: Outpatient infusion
• Service location: Outpatient infusion
• Patient population: Pediatric patients
• Prior authorization required: Yes
• Restriction/criteria of use (all will apply unless specified):
• Diagnosis of acid sphingomyelinase deficiency, confirmed via enzyme assay, and judged by the provider to be ASMD type B or type A/B.
• Patient does not have acute or rapidly progressive neurologic abnormalities.
• Patient does not require use of invasive ventilatory support or requires noninvasive ventilatory support while awake and for greater than 12 hours a day.
• Females of reproductive potential: negative pregnancy test. Advise females of reproductive potential to use effective contraception during treatment and for 14 days after the last dose if Xenpozyme is discontinued.

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• Johns Hopkins Guides