ph-0514
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Givlaari™ (givosiran)

Policy Number: PH-0514

 

Subcutaneous

 

Last Review Date: 01/05/2021

Date of Origin: 12/13/2019

Dates Reviewed: 12/2019, 01/2021

FOR PEEHIP Members Only -Coverage excludes the provider-administered medication(s) outlined in this drug policy from being accessed through a specialty pharmacy. It must be obtained through buy and bill.

  1. Length of Authorization

Coverage will be provided for 6 months initially and may be renewed annually thereafter.

  1. Dosing Limits

A.  Quantity Limit (max daily dose) [NDC Unit]:

  • Givlaari 189 mg/mL in a single-dose vial for injection: 2 vials every month

B.  Max Units (per dose and over time) [HCPS Unit]:

  • 576 billable units every month
  1. Initial Approval Criteria1

Coverage is provided in the following conditions:

  • Patient is at least 18 years of age; AND

Universal Criteria 1,3-5

Submission of medical records related to the medical necessity criteria is REQUIRED on all requests for authorizations. Records will be reviewed at the time of submission. Please provide documentation via direct upload through the PA web portal or by fax.

  • Patient will avoid known triggers of porphyria attacks (i.e., alcohol, smoking, exogenous hormones, hypocaloric diet/fasting, certain medications such as barbiturates, hydantoins, sulfa-antibiotics, anti-epileptics, etc.); AND
  • Patient has not had or is not anticipating a liver transplant; AND

Acute Hepatic Porphyria (AHP) † Ф 1,3-5

  • Patient has a definitive diagnosis of acute hepatic porphyria* (including acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, or ALA dehydratase deficient porphyria) as evidenced by one of the following:
    • Patient has had elevated urinary or plasma PBG (porphobilinogen) and ALA (delta-aminolevulinic acid) levels within the previous year; OR
    • Patient has a mutation in an affected gene as identified on molecular genetic testing; AND
  • Patient has a history of at least two documented porphyria attacks (i.e., requirement of hospitalization, urgent healthcare visit or intravenous administration of hemin) OR one severe attack with CNS involvement (e.g., hallucinations, seizures, etc.) during the previous six months; AND
  • Patients currently receiving prophylactic intravenous hemin therapy will discontinue hemin within 3 to 6 months following initiation of givosiran

*Acute Hepatic Porphyria

Urine delta-aminolevulinic acid (ALA)

Urine porphobilinogen (PBG)

Urine porphyrins

Gene

Acute Intermittent Porphyria (AIP)

Elevated

Elevated

Increased uroporphyrin

HMBS

Hereditary Coproporphyria (HCP)

Elevated

Elevated

Increased coproporphyrin

CPOX

Variegate Porphyria (VP)

Elevated

Elevated

Increased coproporphyrin

PPOX

ALA Dehydratase-Deficiency Porphyria (ADP)

Elevated

Normal

Increased coproporphyrin

ALAD

FDA Approved Indication(s); Compendium Recommended Indication(s); Ф Orphan Drug

  1. Renewal Criteria 1

Coverage can be renewed based upon the following criteria:

  • Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: anaphylactic reactions, severe hepatic toxicity, severe renal toxicity, severe injection site reactions, etc.; AND
  • Disease response as evidenced by a decrease in the frequency of acute porphyria attacks,  and/or hospitalizations/urgent care visits, and/or a decrease requirement of hemin intravenous infusions for acute attacks; AND
  • Patient has a reduction/normalization of biochemical markers (i.e., ALA, PBG) compared to baseline; AND
  • Patient will not use in combination with prophylactic intravenous hemin therapy
  1. Dosage/Administration 1

Indication

Dose

Acute Hepatic Porphyria (AHP)

For administration by a healthcare professional as a subcutaneous injection only.

  • Administer 2.5 mg/kg via subcutaneous injection once monthly.  Dosing is based on actual body weight.
  1. Billing Code/Availability Information

HCPCS Code:

  • J0223 – Injection, givosiran, 0.5 mg: 1 billable unit=0.5 mg

NDC:

  • Givlaari 189 mg/mL in a single-dose vial for injection: 71336-1001-xx
  1. References
  1. Givlaari [package insert]. Cambridge, MA; Alnylam Pharm., Inc., November 2019. Accessed November 2020.
  2. Whatley SD, Badminton MN. Acute Intermittent Porphyria. 2005 Sep 27 [Updated 2013 Feb 7]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1193/.
  3. Anderson KE. Porphyrias: An overview. Mahoney DH, Means RT, ed. UpToDate. Waltham, MA: UpToDate Inc. https://www.uptodate.com/contents/porphyrias-an-overview?search=Acute%20Hepatic%20Porphyria&sectionRank=2&usage_type=default&anchor=H148718266&source=machineLearning&selectedTitle=1~123&display_rank=1#H148718266 (Accessed on November 24, 2020).
  4. Balwani M, Gouya L, Rees D, et al. GS-14-ENVISION, a phase 3 study to evaluate efficacy and safety of givosiran, an investigational RNAi therapeutic targeting aminolevulinic acid synthase 1, in acute hepatic porphyria patients. J Hepatology:Apr 2019; Vol 70; Iss. 1, Suppl;pps e81–e82
  5. Balwani M, Sardh E, Ventura P,et al.; ENVISION Investigators. Phase 3 Trial of RNAi Therapeutic Givosiran for Acute Intermittent Porphyria. N Engl J Med. 2020 Jun 11;382(24):2289-2301.

Appendix 1 – Covered Diagnosis Codes

ICD-10

ICD-10 Description

E80.20

Unspecified porphyria

E80.21

Acute intermittent (hepatic) porphyria

E80.29

Other porphyria

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determination (NCD), Local Coverage Determinations (LCDs), and Local Coverage Articles (LCAs) may exist and compliance with these policies is required where applicable. They can be found at: http://www.cms.gov/medicare-coverage-database/search/advanced-search.aspx. Additional indications may be covered at the discretion of the health plan.

Medicare Part B Covered Diagnosis Codes (applicable to existing NCD/LCD/LCA): N/A

Medicare Part B Administrative Contractor (MAC) Jurisdictions

Jurisdiction

Applicable State/US Territory

Contractor

E (1)

CA, HI, NV, AS, GU, CNMI

Noridian Healthcare Solutions, LLC

F (2 & 3)

AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ

Noridian Healthcare Solutions, LLC

5

KS, NE, IA, MO

Wisconsin Physicians Service Insurance Corp (WPS)

6

MN, WI, IL

National Government Services, Inc. (NGS)

H (4 & 7)

LA, AR, MS, TX, OK, CO, NM

Novitas Solutions, Inc.

8

MI, IN

Wisconsin Physicians Service Insurance Corp (WPS)

N (9)

FL, PR, VI

First Coast Service Options, Inc.

J (10)

TN, GA, AL

Palmetto GBA, LLC

M (11)

NC, SC, WV, VA (excluding below)

Palmetto GBA, LLC

L (12)

DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA)

Novitas Solutions, Inc.

K (13 & 14)

NY, CT, MA, RI, VT, ME, NH

National Government Services, Inc. (NGS)

15

KY, OH

CGS Administrators, LLC

 

 

 

GIVLAARI™ (givosiran) Prior Auth Criteria
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