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Ultomiris (ravulizumab-cwvz)

Policy Number: PH-0427

 

(Intravenous)

Document Number: IC-0427

Last Review Date: 02/04/2019

Date of Origin: 02/04/2019

Dates Reviewed: 02/2019

 

  1. Length of Authorization

Coverage will be provided for twelve months and may be renewed.

  1. Dosing Limits
  1. Quantity Limit (max daily dose) [Pharmacy Benefit]:

Ultomiris 300 mg/30 mL single-dose vial: 10 vials on day zero followed by 13 vials starting on day 14 and every 8 weeks thereafter

  1. Max Units (per dose and over time) [Medical Benefit]:

Indication

Loading Doses

Maintenance Dose

PNH

3,000 mg on Day 0

3,600 mg on Day 14 and every 8 weeks thereafter

  1. Initial Approval Criteria

Coverage is provided in the following conditions:

  • Patient does not have a systemic infection; AND
  • Patients must be administered a meningococcal vaccine at least two weeks prior to initiation of therapy and revaccinated according to current medical guidelines for vaccine use; AND
  • Prescriber is enrolled in the Ultomiris Risk Evaluation and Mitigation Strategy (REMS) program; AND
  • Will not be used in combination with other complement-inhibitor therapy (i.e., eculizumab); AND

Paroxysmal Nocturnal Hemoglobinuria (PNH) †

  • Patient is 18 years or older; AND
  • Diagnosis must be accompanied by detection of PNH clones of at least 5% by flow cytometry diagnostic testing; AND
    • Demonstrate the presence of at least 2 different glycosylphosphatidylinositol (GPI) protein deficiencies (e.g. CD55, CD59, etc.) within at least 2 different cell lines (granulocytes, monocytes, erythrocytes); AND
  • Patient has one of the following indications for therapy:
  • Presence of a thrombotic event
  • Presence of organ damage secondary to chronic hemolysis
  • Patient is pregnant and potential benefit outweighs potential fetal risk
  • Patient is transfusion dependent
  • Patient has high LDH activity (defined as ≥1.5 x ULN) with clinical symptoms
  • Documented baseline values for one or more of the following (necessary for renewal): serum lactate dehydrogenase (LDH), hemoglobin level, and packed RBC transfusion requirement

FDA Approved Indication(s)

  1. Renewal Criteria

Coverage may be renewed based upon the following criteria:

  • Patient continues to meet the criteria identified in section III; AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: serious meningococcal infections (septicemia and/or meningitis), infusion reactions, serious infections, thrombotic microangiopathy complications (TMA), etc.; AND
  • Disease response indicated by one or more of the following:
  • PNH
    • Decrease in serum LDH from pretreatment baseline
    • Stabilization/improvement in hemoglobin level from pretreatment baseline
    • Decrease in packed RBC transfusion requirement from pretreatment baseline
  1. Dosage/Administration

Indication

Dose

Paroxysmal nocturnal hemoglobinuria (PNH)

Complement-Inhibitor Therapy Naïve*

Administer the loading dose based on weight. Two weeks later, begin maintenance doses at a once every 8-week interval:

Body Weight Range

Loading Dose (mg)

Maintenance Dose (mg)

≥40 kg - <60 kg

2,400

3,000

≥60 kg - <100 kg

2,700

3,300

≥100 kg

3,000

3,600

*Switch Therapy from eculizumab: administer the loading dose of Ultomiris 2 weeks after the last eculizumab infusion, and then administer maintenance doses once every 8 weeks, starting 2 weeks after loading dose administration, as indicated above.

  1. Billing Code/Availability Information

HCPCS code:

J3590 – Unclassified biologic

NDC:

Ultomiris 300 mg/30 mL single-use vials for injection: 25682-0022-xx         

  1. References
  1. Ultomiris [package insert]. Boston, MA; Alexion Pharmaceuticals, Inc; December 2018. Accessed January 2019.
  2. Guidelines for the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria and related disorders by flow cytometry. Borowitz MJ, Craig FE, DiGiuseppe JA, Illingworth AJ, Rosse W, Sutherland DR, Wittwer CT, Richards SJ. Cytometry B Clin Cytom. 2010 Jul;78(4):211-30. doi: 10.1002/cyto.b.20525.
  3. Parker C, Omine M, Richards S, et al. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005 Dec 1. 106(12):3699-709.
  4. Sahin F, Akay OM, Ayer M, et al. Pesg PNH diagnosis, follow-up and treatment guidelines.  Am J Blood Res. 2016;6(2): 19-27.

Appendix 1 – Covered Diagnosis Codes

 

ICD-10

ICD-10 Description

D59.5

Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli]

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) and Local Coverage Determinations (LCDs) 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): 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