Criteria
Coverage is subject to the specific terms of the member's benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
Givosiran (Givlaari) may be considered medically necessary when the following criteria are met:
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The individual has a diagnosis of acute hepatic porphyria (AHP) [i.e., acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), ALA-dehydratase deficiency porphyria (ADP)] confirmed by at least ONE of the following (lab results required):
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Elevated urinary or plasma porphobilinogen (PBG) and/or 5-aminolevulinic acid (ALA) levels
OR
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Genetic confirmation of a mutation in ONE of the following genes:
- hydroxymethylbilane synthase [HMBS (diagnostic for AIP)]
- coproporphyrinogen oxidase [CPOX (diagnostic for HCP)]
- protoporphyrinogen oxidase [PPOX (diagnostic for VP)]
-
ALA dehydratase [ALAD (diagnostic for ADP)]
AND
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The individual has symptoms consistent with AHP occurring during acute attacks or chronically (e.g., abdominal pain, back pain, chest pain, nausea, vomiting, weakness, tachycardia, hypertension, constipation, anxiety, depression, diarrhea)
AND
-
The prescriber has evaluated the individual's baseline (prior to therapy with the requested agent) average number of acute AHP attacks
AND
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The individual has implemented and maintained dietary and lifestyle changes to help prevent acute attacks
AND
-
Medications known to cause or exacerbate acute attacks (e.g., estrogen/progesterone, barbiturates, sulfonamides) have been evaluated and discontinued when appropriate
AND
-
The individual will NOT be using the requested agent in combination with prophylactic hemin
AND
-
If the individual has an FDA labeled indication, then ONE of the following:
-
The individual's age is within FDA labeling for the requested indication for the requested agent
OR
-
There is support for using the requested agent for the individual's age for the requested indication
AND
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The prescriber is a specialist in the area of the individual's diagnosis (e.g., hematologist, hepatologist) or the prescriber has consulted with a specialist in the area of the individual's diagnosis
AND
-
The individual does NOT have any FDA labeled contraindications to the requested agent
AND
- The requested quantity (dose) is within FDA labeled dosing for the requested indication.
Reauthorization Criteria
Givosiran (Givlaari) may be considered medically necessary when the following criteria are met:
-
The individual has been previously approved for the requested agent through Blue Cross Blue Shield of North Dakota's Medical Drug Review process [Note: individuals not previously approved for the requested agent will require initial evaluation review]
AND
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The individual has had clinical benefit with the requested agent (e.g., reduction in frequency of acute attacks)
AND
-
The individual has continued to maintain dietary and lifestyle changes to help prevent acute attacks
AND
-
Medications known to cause or exacerbate acute attacks (e.g., estrogen/progesterone, barbiturates, sulfonamides) have been evaluated and discontinued when appropriate
AND
-
The individual will NOT be using the requested agent in combination with prophylactic hemin
AND
-
The prescriber is a specialist in the area of the individual's diagnosis (e.g., hematologist, hepatologist) or the prescriber has consulted with a specialist in the area of the individual's diagnosis
AND
-
The individual does NOT have any FDA labeled contraindications to the requested agent
AND
- The requested quantity (dose) is within FDA labeled dosing for the requested indication.
The use of givosiran (Givlaari) for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.
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