Initial Treatment
Givosiran may be considered medically necessary if all of the following conditions are met:
Individual is 18 years of age or older.
Individual has a diagnosis of acute hepatic porphyria (AHP) and confirmation of 1 of the following subtypes:
Acute intermittent porphyria (AIP)
Hereditary coproporphyria (HCP)
Variegate porphyria (VP)
Delta-aminolevulinic acid (ALA) dehydratase deficiency (ADP).
Documentation is provided that the individual has an elevated porphobilinogen (PBG)- or ALA in the urine or plasma within the past year.
Individual meets any 1 of the following criteria:
Individual has documented active symptomatic disease with at least 2 porphyria attacks within the last 6 months.
Individual is currently on prophylactic hemin treatment due to a history of severe or frequent porphyria attacks.
Individual will not be receiving prophylactic hemin treatment and givosiran concurrently.
Prescriber agrees to monitor liver function tests (LFTs).
Prescriber agrees to monitor renal function.
Initial authorization is for 12 months.
Continuation of Treatment
Incremental reauthorization for givosiran may be considered medically necessary if the following conditions are met:
Individual continues to meet the initial treatment criteria cited above.
Documentation is provided that the individual has experienced a clinical response to therapy (e.g., a reduction in rate of porphyria attacks or reduction in hemin requirements for acute attacks) since initiating therapy.
PBG or ALA concentration has not increased from baseline.
Individual does not have severe or clinically significant transaminase elevations, defined as alanine aminotransferase (ALT) greater than 5 times the upper limit of normal.
Reauthorization period is for 12 months.
Givosiran is considered investigational for all other indications.
