Givosiran (Givlaari) — Coverage Criteria for Acute Hepatic Porphyria
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Medical policy governing coverage criteria for givosiran (Givlaari) for treatment of adults with acute hepatic porphyria under specified benefit plans; applies to providers seeking authorization for therapy. Affects patients with diagnoses of AHP and clinicians/administrators requesting prior authorization.
No material clinical or coverage changes in this revision.
Coverage Criteria for Givosiran (Givlaari)
Initial Therapy
Covered when ALL of the following are met for Initial Therapy:
From Initial Therapy section
From Initial Therapy section
From Initial Therapy and Instructions for Use
Exclusion criterion
Concurrent prophylactic hemin is not permitted
Specialist involvement required
FDA labeling required (dose per label)
Authorization time limit
Continuation Therapy
Covered when ALL of the following are met for Continuation Therapy:
From Continuation Therapy section
Response required for reauthorization
Exclusion criterion
Concurrent prophylactic hemin is not permitted
Specialist involvement required
FDA labeling required (dose per label)
Reauthorization time limit
Coverage for givosiran (Givlaari) excludes patients who have had a liver transplant. The policy lists "Patient has not had a liver transplant" as a required criterion for both Initial Therapy and Continuation Therapy, and members who have undergone liver transplantation therefore do not meet the eligibility requirements for coverage.
When assessing requests, reviewers should verify documentation of transplant history in the medical record (for example, operative reports, transplant clinic notes, or problem list entries). If a liver transplant is documented, the request should be denied because the patient does not satisfy the explicit exclusion in the coverage criteria.
Concurrent use of prophylactic hemin is not permitted while a patient is receiving givosiran. The policy explicitly requires that the patient will not receive concomitant prophylactic hemin treatment while on Givlaari for both Initial Therapy and Continuation Therapy, and patients receiving prophylactic hemin at the time of request do not meet policy criteria.
Providers must attest that baseline hemin administration (prophylactic or treatment) and attack history have been documented and that prophylactic hemin will be discontinued prior to initiating givosiran. Requests that indicate ongoing prophylactic hemin use should be denied or held until documentation shows hemin has been stopped in accordance with the policy requirement.
Coding and Diagnosis References
| J0223 | Injection, givosiran, 0.5 mg |
| E80.0 | Hereditary erythropoietic porphyria |
| E80.1 | Porphyria cutanea tarda |
| E80.20 | Unspecified porphyria |
| E80.21 | Acute intermittent (hepatic) porphyria |
| E80.29 | Other porphyria |
Provider Requirements and Authorization Actions
Prior Authorization Required
Prior authorization is required for Givlaari (givosiran). Initial authorizations are limited to no more than 12 months and must document the diagnosis and criteria supporting medical necessity.
- Initial authorization: up to 12 months
- Prescribed by or in consultation with a hematologist or AHP specialist
- Dosing per FDA labeling (2.5 mg/kg SC once monthly, based on actual body weight)
Step Therapy / Sequencing
Step therapy is not expressed as a required prior trial of a specific branded product, but coverage requires either a documented attack frequency or current prophylactic hemin use. Members must meet the stated clinical entry conditions before authorization will be approved.
- Clinical entry: ≥2 documented porphyria attacks in the prior 6 months, OR current prophylactic hemin use
- Note: Patients receiving prophylactic hemin may be considered under 'Initial Therapy' criteria but concurrent prophylactic hemin is not permitted while on Givlaari
Required Documentation
Providers must supply attestation and baseline documentation prior to initiation and at reauthorization to demonstrate treatment need and response.
- Provider attestation documenting baseline hemin administration requirements (prophylactic or treatment) and baseline rate and/or number of porphyria attacks prior to initiating givosiran
- Documentation of diagnosis consistent with AHP subtypes (e.g., acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, ALA dehydratase deficient porphyria)
- For continuation: documentation of prior Givlaari use and evidence of positive clinical response while on therapy
- Prescriber specialty or consultation note (hematologist or AHP expert)
Triggers for Denial
Authorizations may be denied if required documentation or clinical criteria are not met.
- Missing or insufficient documentation of AHP diagnosis
- Failure to document the required baseline attack history (e.g., fewer than 2 attacks in the prior 6 months when required)
- Documentation showing the member is receiving concurrent prophylactic hemin while requesting Givlaari
- Evidence of prior liver transplant
Background on Acute Hepatic Porphyria and Givosiran
Acute hepatic porphyria (AHP) refers to a family of ultra-rare genetic disorders caused by deficiencies in enzymes of hepatic heme biosynthesis. These conditions lead to accumulation of neurotoxic intermediates such as delta-aminolevulinic acid (ALA) and porphobilinogen (PBG), which are associated with recurrent neurovisceral attacks characterized by severe abdominal pain and neurologic and gastrointestinal symptoms.
Givosiran (Givlaari) is an ALAS1-directed small interfering RNA that lowers hepatic ALAS1 mRNA, reducing ALA and PBG levels and thereby decreasing the frequency of porphyria attacks. The drug is indicated for adults with AHP meeting the coverage criteria described in this policy.
Key Definitions
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