Hemophilia Products

Policy ID: I-9904-004

Section: Injections

Effective Date: January 01, 2023

Revised Date: September 11, 2023

Revision Effective Date: October 01, 2023

Last Reviewed: September 12, 2023

Applies To: Commercial and Medicaid Expansion

Description

Hemophilia is an X-linked genetic disorder caused by a deficiency or absence in one of the clotting factors in the plasma.

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.

A hemophilia product should be billed through a member's pharmacy benefit but may be considered medically necessary to bill through a member's medical benefit if the following criteria is met:

ONE of the following:
- The individual is receiving the hemophilia product for training and education purposes; OR
- The individual's primary insurance requires medical claim processing; OR
- A medical reason is provided as to why an individual cannot self-administer (i.e., physical disability, anxiety/panic attack, previous severe reaction to the hemophilia product; OR
- The individual is hospitalized; AND
The requested hemophilia product is Advate, Adynovate, Afstyla, Alphanate, Altuviiio, Eloctate, Esperoct, Hemofil M, Humate-P, Jivi, Koate, Kogenate, Kovaltry, Novoeight, Nuwiq, Recombinate, Vonvendi, Wilate or Xyntha; AND
- ONE of the following:
  - The individual has a diagnosis of hemophilia A (also known as Factor VIII deficiency or classic hemophilia) AND ONE of the following:
    - The individual is currently experiencing a bleed AND BOTH of the following:
      - The individual is out of medication; AND
      - The individual needs to receive a ONE-TIME emergency supply of medication; OR
    - BOTH of the following:
      - The requested agent is FDA approved or compendia supported for a diagnosis of hemophilia A; AND
      - The requested agent is being used for ONE of the following:
        Prophylaxis AND the individual will NOT be using the requested agent in combination with Hemlibra (emicizumab-kxwh) ; OR
        As a component of Immune Tolerance Therapy (ITT)/Immune Tolerance Induction (ITI) AND BOTH of the following:
        The individual will NOT be using the requested agent in combination with Hemlibra (emicizumab-kxwh); AND
        ONE of the following (medical records required):
        The individual has NOT had more than 33 months of ITT/ITI therapy; OR
        Information has been provided supporting the continued use of ITT/ITI therapy (i.e., the individual has had a greater than or equal to 20% decrease in inhibitor level over the last 6 months and needs further treatment to eradicate inhibitors); OR
        On-demand use for bleeds; OR
        Peri-operative management of bleeding; OR
  - The individual has a diagnosis of von Willebrand disease (VWD) AND ALL of the following:
    - The requested agent is FDA approved or compendia supported for a diagnosis of von Willebrand disease; AND
    - ONE of the following:
      - The individual is currently experiencing a bleed AND BOTH of the following:
        The individual is out of medication; AND
        The individual needs to receive a ONE-TIME emergency supply of medication; OR
      - The individual has type 1, 2A, 2M or 2N VWD AND ONE of the following:
        The individual has tried and had an inadequate response to desmopressin (e.g., DDAVP injection, Stimate nasal spray); OR
        The individual did not respond to a DDAVP trial with 1- and 4-hour post infusion bloodwork; OR
        The individual has an intolerance or hypersensitivity to desmopressin; OR
        The individual has an FDA labeled contraindication to desmopressin; OR
        The prescriber has provided information supporting why the individual cannot use desmopressin (e.g., shortage in marketplace); OR
      - The individual has type 2B or 3 VWD; AND
    - The requested agent will be used for ONE of the following:
      - Prophylaxis AND ONE of the following
        The requested agent is Vonvendi AND ONE of the following:
        The individual has severe Type 3 VWD; OR
        The individual has another subtype of VWD AND the subtype is FDA approved for prophylaxis use; OR
        The requested agent is NOT Vonvendi; OR
      - On-demand use for bleeds; OR
      - Peri-operative management of bleeding; AND
- If the individual has an FDA approved indication, ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with a nonsteroidal anti-inflammatory agent (NSAID) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this patient; AND
- The prescriber must provide the actual prescribed dose with ALL of the following:
  - Individual's weight; AND
  - Intended use/regimen: (e.g., prophylaxis, ITT/ITI, on-demand, peri-operative); AND
  - If the individual has a diagnosis of hemophilia A BOTH of the following:
    - Severity of the factor deficiency (i.e., severe is less than 1% factor activity, moderate is greater than or equal to one (1) to less than or equal to 5% factor activity, mild is greater than five (5) to 40% factor activity); AND
    - Inhibitor status; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with another agent in the same category (e.g., Factor VIII agents, Factor VIII and von Willebrand Factor combination agents) included in this program; OR
  - Information has been provided supporting the use of more than one unique agent in the same category (medical records required); OR

The requested hemophilia product is AlphaNine, Alprolix, BeneFIX, Idelvion, Ixinity, Mononine, Profilnine SD, Rebinyn or Rixubis; AND
- The individual has a diagnosis of hemophilia B (also known as Factor IX deficiency, Christmas disease) AND ONE of the following:
  - The individual is currently experiencing a bleed AND BOTH of the following:
    - The individual is out of medication; AND
    - The individual needs to receive a ONE-TIME emergency supply of medication; OR
  - The requested agent is being used for ONE of the following:
    - Prophylaxis; OR
    - On-demand use for bleeds; OR
    - Peri-operative management of bleeding; AND
- If the individual has an FDA approved indication, ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this individual; AND
- The prescriber must provide the actual prescribed dose with ALL of the following:
  - Individual's weight; AND
  - Severity of the factor deficiency (i.e., severe is less than 1% factor activity, moderate is greater than or equal to one (1) to less than or equal to 5% factor activity, mild is greater than five (5) to 40% factor activity); AND
  - Inhibitor status; AND
  - Intended use/regimen: prophylaxis, on-demand, peri-operative; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with another Factor IX agent included in this program; OR
  - Information has been provided supporting the use of more than one unique Factor IX agent (medical records required); OR

The requested hemophilia product is NovoSeven RT; AND
- ONE of the following:
  - The individual has a diagnosis of hemophilia A AND BOTH of the following:
    - The individual has inhibitors to Factor VII; AND
    - The requested agent is being used for ONE of the following:
      - On-demand use for bleeds AND ONE of the following:
        The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
        The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
      - Prophylaxis AND ALL of the following:
        ONE of the following:
        The individual has tried and had an inadequate response to Immune Tolerance Induction (ITI) [Immune Tolerance Therapy (ITT)]; OR
        The individual has an inhibitor level greater than or equal to 200 BU (lab records required); OR
        Information has been provided indicating why the individual is not a candidate for ITI; AND
        The individual will NOT be using the requested agent in combination with Hemlibra; AND
        The individual will NOT be using the requested agent in combination with Feiba [activated prothrombin complex (aPCC)] used for prophylaxis (on-demand use of aPCC is acceptable); OR
      - Peri-operative management of bleeding; OR
      - As a component of Immune tolerance induction (ITI)/Immune tolerance therapy (ITT) AND ONE of the following:
        The individual has NOT had more than 33 months of ITT/ITI therapy; OR
        Information has been provided supporting the continued use of ITT/ITI therapy (i.e., the individual has had a greater than or equal to 20% decrease in inhibitor level over the last six (6) months and needs further treatment to eradicate inhibitors) (medical records required); OR
  - The individual has a diagnosis of hemophilia B AND BOTH of the following:
    - The individual has inhibitors to Factor IX; AND
    - The requested agent is being used for ONE of the following:
      - On-demand use for bleeds AND ONE of the following:
        The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
        The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
      - Prophylaxis AND BOTH of the following:
        ONE of the following:
        The individual has tried and had an inadequate response to Immune Tolerance Induction (ITI) [Immune Tolerance Therapy (ITT)]; OR
        The individual has an inhibitor level greater than or equal to 200 BU (lab records required); OR
        Information has been provided indicating why the individual is not a candidate for ITI; AND
        The individual will NOT be using the requested agent in combination with Feiba [activated prothrombin complex (aPCC)] used for prophylaxis (on-demand use of aPCC is acceptable); OR
      - Peri-operative management of bleeding; OR
      - As a component of Immune tolerance induction (ITI)/Immune tolerance therapy (ITT) AND ONE of the following:
        The individual has NOT had more than 33 months of ITT/ITI therapy; OR
        Information has been provided supporting the continued use of ITT/ITI therapy (i.e., the individual has had a greater than or equal to 20% decrease in inhibitor level over the last six (6) months and needs further treatment to eradicate inhibitors) (medical records required); OR
  - The individual has a diagnosis of congenital Factor VII deficiency AND the requested agent will be used for ONE of the following:
    - On-demand use for bleeds AND ONE of the following:
      - The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
      - The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
    - Prophylaxis; OR
    - Perioperative use; OR
  - The individual has a diagnosis of Glanzmann's thrombasthenia AND BOTH of the following:
    - The individual is refractory to platelet transfusions; AND
    - The requested agent will be used for ONE of the following:
      - On-demand use for bleeds AND ONE of the following:
        The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
        The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
      - Perioperative use; OR
  - The individual has a diagnosis of acquired hemophilia AND the requested agent will be used for ONE of the following
    - On-demand use for bleeds AND ONE of the following:
      - The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
      - The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
    - Perioperative use; OR
  - The individual has another FDA approved indication for the requested agent and route of administration; OR
  - The individual has another indication that is supported in compendia for the requested agent and route of administration; AND
- If the individual has an FDA approved indication, ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual will NOT be using the requested agent in combination with another Factor VIIa agent; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this individual; OR

The requested hemophilia product is Sevenfact; AND
- ONE of the following:
  - The individual has a diagnosis of hemophilia A AND BOTH of the following:
    - The individual has inhibitors to Factor VIII; AND
    - The requested agent is being used for on-demand use for bleeds; OR
  - The individual has a diagnosis of hemophilia B AND BOTH of the following:
    - The individual has inhibitors to Factor IX; AND
    - The requested agent is being used for on-demand use for bleeds; OR
  - The individual has another FDA approved indication for the requested agent and route of administration; AND
- If the individual has an FDA approved indication, ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual will NOT be using the requested agent in combination with another Factor VIIa agent; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this individual; AND
- ONE of the following:
  - The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
  - The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR

The requested hemophilia product is Coagadex; AND
- ONE of the following:
  - The individual has a diagnosis of hereditary Factor X deficiency AND ONE of the following:
    - The individual is currently experiencing a bleed AND BOTH of the following:
      - The individual is out of medication; AND
      - The individual needs to receive a ONE-TIME emergency supply of medication; OR
    - The requested agent will be used for prophylaxis treatment AND ONE of the following:
      - The individual has severe or moderate Factor X deficiency (Factor X level less than or equal to 5%); OR
      - The individual has mild Factor X deficiency (Factor X level 6-10%) AND the prescriber has provided information supporting prophylaxis use of the requested agent (medical records required); OR
  - The requested agent will be used as on-demand treatment to control bleeding episodes AND BOTH of the following:
    - The prescriber has communicated with the individual (via any means) and has verified that the individual does NOT have more than five (5) on-demand doses on hand; AND
    - ONE of the following:
      - The individual has tried and had an inadequate response to aminocaproic acid or tranexamic acid; OR
      - The individual has an intolerance or hypersensitivity to aminocaproic acid or tranexamic acid; OR
      - The individual has an FDA labeled contraindication to BOTH aminocaproic acid AND tranexamic acid; OR
      - The prescriber has provided information to support the use of the requested agent over BOTH aminocaproic acid AND tranexamic acid; OR
  - The requested agent will be used as perioperative management of bleeding AND BOTH of the following:
    - The individual has mild (Factor X level 6-10%) or moderate (Factor X level 1-5%) hereditary Factor X deficiency; AND
    - ONE of the following:
      - The individual has tried and had an inadequate response to aminocaproic acid or tranexamic acid; OR
      - The individual has an intolerance or hypersensitivity to aminocaproic acid or tranexamic acid; OR
      - The individual has an FDA labeled contraindication to BOTH aminocaproic acid AND tranexamic acid; OR
      - The prescriber has provided information to support the use of the requested agent over BOTH aminocaproic acid AND tranexamic acid; AND
- The prescriber is a specialist (e.g., hematologist) in the area of the individual's diagnosis or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual does NOT have liver disease; AND
- The individual does NOT have vitamin K deficiency; AND
- The individual will NOT be using the requested agent in combination with an indirect or direct Factor Xa inhibitor [e.g., apixaban (Eliquis), dalteparin (Fragmin), edoxaban (Savaysa), enoxaparin (Lovenox), fondaparinux (Arixtra), rivaroxaban (Xarelto) or warfarin (Coumadin)]; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this individual; OR

The requested hemophilia product is Fibryga or RiaSTAP; AND
- The individual has a diagnosis of fibrinogen deficiency (factor I deficiency) AND ONE of the following:
  - The individual is currently experiencing a bleed AND BOTH of the following:
    - The individual is out of medication; AND
    - The individual needs to receive a ONE-TIME emergency supply of medication; OR
  - ALL of the following:
    - The individual has a fibrinogen level of less than 150 mg/dL; AND
    - The individual does NOT have dysfibrinogenemia; AND
    - The requested agent will be used as on-demand treatment to control acute bleeding episodes; AND
    - The prescriber has communicated with the individual (via any means) and has verified that the individual does NOT have more than 5 on-demand doses on hand; AND
- The prescriber is a specialist (e.g., hematologist) in the area of the individual's diagnosis or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this individual.

Length of Approval: One (1) month

The use of hemophilia products 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.

Procedure Codes

J7170

J7175

J7177

J7178

J7179

J7182

J7183

J7185

J7186

J7187

J7189

J7190

J7192

J7193

J7194

J7195

J7200

J7201

J7202

J7203

J7204

J7205

J7207

J7208

J7209

J7210

J7211

J7212

J7213

J7214

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the United States Food and Drug Administration (U.S. FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines.

NOTE: Related policy I-9010 Emicizumab-kxwh (Hemlibra) - J7170

Covered Diagnosis Code for: J7170, J7182, J7185, J7190, J7192, J7204, J7205, J7207, J7208, J7209, J7210, J7211

D66

Covered Diagnosis code for J7175

D68.2

Covered Diagnosis Codes for J7177, J7178

D68.2

D68.8

O03.6

O08.1

O45.011

O45.012

O45.013

O45.019

O46.011

O46.012

O46.013

O46.019

O67.0

O72.3

Covered Diagnosis Codes for J7179, J7183

D68.00

D68.01

D68.020

D68.021

D68.022

D68.023

D68.029

D68.03

D68.04

D68.09

Covered Diagnosis Codes for J7186, J7187

D66

D68.00

D68.01

D68.020

D68.021

D68.022

D68.023

D68.029

D68.03

D68.04

D68.09

Covered Diagnosis Codes for J7189

D66

D67

D68.2

D68.311

Covered Diagnosis code for J7193, J7194, J7195, J7200, J7201, J7202, J7203,

D67

Covered Diagnosis codes for J7212

D66

D68.4

Covered Diagnosis code for J7312

D68.311

Professional Statements and Societal Positions Guidelines

Not Applicable

ND Committee Review

Internal Medical Policy Committee 11-29-2022 - Effective January 1, 2023

Adopted new precertification policy for hemophilia products for when pharmacy benefit billing is not feasible

Internal Medical Policy Committee 5-23-2023 - Effective June 1, 2023

Updated Factor VIII and von Willebrand Factor criteria
Added new drug Altuviiio

Internal Medical Policy Committee 7-26-2023 - Effective July 1, 2023

Added new code J7213 for Ixinity to the policy

Internal Medical Policy Committee 9-12-2023 Effective October 01, 2023

Added new code, J7214 for Altuviiio, to the policy
Updated NSAIDs criteria where applicable

Links

References (PDF)

Disclaimer

Current medical policy is to be used in determining a Member's contract benefits on the date that services are rendered. Contract language, including definitions and specific inclusions/exclusions, as well as state and federal law, must be considered in determining eligibility for coverage. Members must consult their applicable benefit plans or contact a Member Services representative for specific coverage information. Likewise, medical policy, which addresses the issue(s) in any specific case, should be considered before utilizing medical opinion in adjudication. Medical technology is constantly evolving, and the Company reserves the right to review and update medical policy periodically.

Policy ID: I-9904-003

Section: Injections

Effective Date: January 01, 2023

Revised Date: June 21, 2023

Revision Effective Date: July 01, 2023

Last Reviewed: July 26, 2023

Archived Date: June 30, 2023

Applies To: Commercial and Medicaid Expansion

Description

Hemophilia is an X-linked genetic disorder caused by a deficiency or absence in one of the clotting factors in the plasma.

Criteria

Coverage is subject to the specific terms of the member's benefit plan.

A hemophilia product should be billed through a member's pharmacy benefit but may be considered medically necessary to bill through a member's medical benefit if the following criteria is met:

ONE of the following:
- The individual is receiving the hemophilia product for training and education purposes; OR
- The individual's primary insurance requires medical claim processing; OR
- A medical reason is provided as to why an individual cannot self-administer (i.e., physical disability, anxiety/panic attack, previous severe reaction to the hemophilia product; OR
- The individual is hospitalized; AND
The requested hemophilia product is Advate, Adynovate, Afstyla, Alphanate, Altuviiio, Eloctate, Esperoct, Hemofil M, Humate-P, Jivi, Koate, Kogenate, Kovaltry, Novoeight, Nuwiq, Recombinate, Vonvendi, Wilate or Xyntha; AND
- ONE of the following:
  - The individual has a diagnosis of hemophilia A (also known as Factor VIII deficiency or classic hemophilia) AND ONE of the following:
    - The individual is currently experiencing a bleed AND BOTH of the following:
      - The individual is out of medication; AND
      - The individual needs to receive a ONE-TIME emergency supply of medication; OR
    - BOTH of the following:
      - The requested agent is FDA approved or compendia supported for a diagnosis of hemophilia A; AND
      - The requested agent is being used for ONE of the following:
        Prophylaxis AND the individual will NOT be using the requested agent in combination with Hemlibra (emicizumab-kxwh) ; OR
        As a component of Immune Tolerance Therapy (ITT)/Immune Tolerance Induction (ITI) AND BOTH of the following:
        The individual will NOT be using the requested agent in combination with Hemlibra (emicizumab-kxwh); AND
        ONE of the following:
        The individual has NOT had more than 33 months of ITT/ITI therapy; OR
        Information has been provided supporting the continued use of ITT/ITI therapy (i.e., the individual has had a greater than or equal to 20% decrease in inhibitor level over the last 6 months and needs further treatment to eradicate inhibitors); OR
        On-demand use for bleeds; OR
        Peri-operative management of bleeding; OR
  - The individual has a diagnosis of von Willebrand disease (VWD) AND ALL of the following:
    - The requested agent is FDA approved or compendia supported for a diagnosis of von Willebrand disease; AND
    - ONE of the following:
      - The individual is currently experiencing a bleed AND BOTH of the following:
        The individual is out of medication; AND
        The individual needs to receive a ONE-TIME emergency supply of medication; OR
      - The individual has type 1, 2A, 2M or 2N VWD AND ONE of the following:
        The individual has tried and had an inadequate response to desmopressin (e.g., DDAVP injection, Stimate nasal spray); OR
        The individual did not respond to a DDAVP trial with 1- and 4-hour post infusion bloodwork; OR
        The individual has an intolerance or hypersensitivity to desmopressin; OR
        The individual has an FDA labeled contraindication to desmopressin; OR
        The prescriber has provided information supporting why the individual cannot use desmopressin (e.g., shortage in marketplace); OR
      - The individual has type 2B or 3 VWD; AND
    - The requested agent will be used for ONE of the following:
      - Prophylaxis AND ONE of the following
        The requested agent is Vonvendi AND ONE of the following:
        The individual has severe Type 3 VWD; OR
        The individual has another subtype of VWD AND the subtype is FDA approved for prophylaxis use; OR
        The requested agent is NOT Vonvendi; OR
      - On-demand use for bleeds; OR
      - Peri-operative management of bleeding; AND
- ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with a nonsteroidal anti-inflammatory agent (NSAID) (e.g., aspirin, ibuprofen) other than cyclooxygenase-2 (COX-2) inhibitors (e.g., celecoxib) NOTE: for the purposes of this criteria COX-2 inhibitors will be accepted for concomitant use; OR
  - The prescriber has provided information in support of using an NSAID for this patient; AND
- The prescriber must provide the actual prescribed dose with ALL of the following:
  - Individual's weight; AND
  - Intended use/regimen: (e.g., prophylaxis, ITT/ITI, on-demand, peri-operative); AND
  - If the individual has a diagnosis of hemophilia A BOTH of the following:
    - Severity of the factor deficiency (i.e., severe is less than 1% factor activity, moderate is greater than or equal to one (1) to less than or equal to 5% factor activity, mild is greater than five (5) to 40% factor activity); AND
    - Inhibitor status; AND
- ONE of the following:
  - The individual will NOT be using the requested agent in combination with another agent in the same category (e.g., Factor VIII agents, Factor VIII and von Willebrand Factor combination agents) included in this program; OR
  - Information has been provided supporting the use of more than one unique agent in the same category (medical records required); OR

The requested hemophilia product is AlphaNine, Alprolix, BeneFIX, Idelvion, Ixinity, Mononine, Profilnine SD, Rebinyn or Rixubis; AND
- The individual has a diagnosis of hemophilia B (also known as Factor IX deficiency, Christmas disease) AND ONE of the following:
  - The individual is currently experiencing a bleed AND BOTH of the following:
    - The individual is out of medication; AND
    - The individual needs to receive a ONE-TIME emergency supply of medication; OR
  - ONE of the following:
    - The requested agent is Rebinyn, AND is being used for one of the following:
      - On-demand use for bleeds; OR
      - Peri-operative management of bleeding; OR
    - The requested agent is being used for one of the following:
      - Prophylaxis; OR
      - On-demand use for bleeds; OR
      - Peri-operative management of bleeding; AND
- ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen); AND
- The prescriber must provide the actual prescribed dose with ALL of the following:
  - Individual's weight; AND
  - Severity of the factor deficiency (i.e., severe is less than 1% factor activity, moderate is greater than or equal to one (1) to less than or equal to 5% factor activity, mild is greater than five (5) to 40% factor activity); AND
  - Inhibitor status; AND
  - Intended use/regimen: prophylaxis, on-demand, peri-operative; AND
- ONE of the following:
- The individual will NOT be using the requested agent in combination with another Factor IX agent included in this program; OR
- Information has been provided supporting the use of more than one unique Factor IX agent (medical records required); OR

The requested hemophilia product is NovoSeven RT; AND
- ONE of the following:
  - The individual has a diagnosis of hemophilia A AND BOTH of the following:
    - The individual has inhibitors to Factor VII; AND
    - The requested agent is being used for ONE of the following:
      - On-demand use for bleeds AND ONE of the following:
        The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
        The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
      - Prophylaxis AND ALL of the following:
        ONE of the following:
        The individual has tried and had an inadequate response to Immune Tolerance Induction (ITI) [Immune Tolerance Therapy (ITT)]; OR
        The individual has an inhibitor level greater than or equal to 200 BU (lab records required); OR
        Information has been provided indicating why the individual is not a candidate for ITI; AND
        The individual will NOT be using the requested agent in combination with Hemlibra; AND
        The individual will NOT be using the requested agent in combination with Feiba [activated prothrombin complex (aPCC)] used for prophylaxis (on-demand use of aPCC is acceptable); OR
      - Peri-operative management of bleeding; OR
      - As a component of Immune tolerance induction (ITI)/Immune tolerance therapy (ITT) AND ONE of the following:
        The individual has NOT had more than 33 months of ITT/ITI therapy; OR
        Information has been provided supporting the continued use of ITT/ITI therapy (i.e., the individual has had a greater than or equal to 20% decrease in inhibitor level over the last six (6) months and needs further treatment to eradicate inhibitors) (medical records required); OR
  - The individual has a diagnosis of hemophilia B AND BOTH of the following:
    - The individual has inhibitors to Factor IX; AND
    - The requested agent is being used for ONE of the following:
      - On-demand use for bleeds AND ONE of the following:
        The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
        The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
      - Prophylaxis AND BOTH of the following:
        ONE of the following:
        The individual has tried and had an inadequate response to Immune Tolerance Induction (ITI) [Immune Tolerance Therapy (ITT)]; OR
        The individual has an inhibitor level greater than or equal to 200 BU (lab records required); OR
        Information has been provided indicating why the individual is not a candidate for ITI; AND
        The individual will NOT be using the requested agent in combination with Feiba [activated prothrombin complex (aPCC)] used for prophylaxis (on-demand use of aPCC is acceptable); OR
      - Peri-operative management of bleeding; OR
      - As a component of Immune tolerance induction (ITI)/Immune tolerance therapy (ITT) AND ONE of the following:
        The individual has NOT had more than 33 months of ITT/ITI therapy; OR
        Information has been provided supporting the continued use of ITT/ITI therapy (i.e., the individual has had a greater than or equal to 20% decrease in inhibitor level over the last six (6) months and needs further treatment to eradicate inhibitors) (medical records required); OR
  - The individual has a diagnosis of congenital Factor VII deficiency AND the requested agent will be used for ONE of the following:
    - On-demand use for bleeds AND ONE of the following:
      - The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
      - The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
    - Prophylaxis; OR
    - Perioperative use; OR
  - The individual has a diagnosis of Glanzmann's thrombasthenia AND BOTH of the following:
    - The individual is refractory to platelet transfusions; AND
    - The requested agent will be used for ONE of the following:
      - On-demand use for bleeds AND ONE of the following:
        The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
        The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
      - Perioperative use; OR
  - The individual has a diagnosis of acquired hemophilia AND the requested agent will be used for ONE of the following
    - On-demand use for bleeds AND ONE of the following:
      - The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
      - The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR
    - Perioperative use; OR
  - The individual has another FDA approved indication for the requested agent and route of administration; OR
  - The individual has another indication that is supported in compendia for the requested agent and route of administration; AND
- ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual will NOT be using the requested agent in combination with another Factor VIIa agent; AND
- The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen); OR

The requested hemophilia product is Sevenfact; AND
- ONE of the following:
  - The individual has a diagnosis of hemophilia A AND BOTH of the following:
    - The individual has inhibitors to Factor VIII; AND
    - The requested agent is being used for on-demand use for bleeds; OR
  - The individual has a diagnosis of hemophilia B AND BOTH of the following:
    - The individual has inhibitors to Factor IX; AND
    - The requested agent is being used for on-demand use for bleeds; OR
  - The individual has another FDA approved indication for the requested agent and route of administration; AND
- ONE of the following:
  - The individual's age is within FDA labeling for the requested indication for the requested agent; OR
  - The prescriber has provided information in support of using the requested agent for the individual's age for the requested indication; AND
- The prescriber is a specialist in the area of the individual's diagnosis [e.g., prescriber working in a hemophilia treatment center (HTC), hematologist with hemophilia experience] or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual will NOT be using the requested agent in combination with another Factor VIIa agent; AND
- The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen); AND
- ONE of the following:
  - The prescriber communicated with the individual (via any means) regarding the frequency and severity of the individual's bleeds and has verified that the individual does not have greater than five (5) on-demand doses on hand; OR
  - The prescriber has provided information in support of the individual having more than five (5) on-demand doses on hand (supportive reasoning required); OR

The requested hemophilia product is Coagadex; AND
- ONE of the following:
  - The individual has a diagnosis of hereditary Factor X deficiency AND ONE of the following:
    - The individual is currently experiencing a bleed AND BOTH of the following:
      - The individual is out of medication; AND
      - The individual needs to receive a ONE-TIME emergency supply of medication; OR
    - The requested agent will be used for prophylaxis treatment AND ONE of the following:
      - The individual has severe or moderate Factor X deficiency (Factor X level less than or equal to 5%); OR
      - The individual has mild Factor X deficiency (Factor X level 6-10%) AND the prescriber has provided information supporting prophylaxis use of the requested agent (medical records required); OR
  - The requested agent will be used as on-demand treatment to control bleeding episodes AND BOTH of the following:
    - The prescriber has communicated with the individual (via any means) and has verified that the individual does NOT have more than five (5) on-demand doses on hand; AND
    - ONE of the following:
      - The individual has tried and had an inadequate response to aminocaproic acid or tranexamic acid; OR
      - The individual has an intolerance or hypersensitivity to aminocaproic acid or tranexamic acid; OR
      - The individual has an FDA labeled contraindication to BOTH aminocaproic acid AND tranexamic acid; OR
      - The prescriber has provided information to support the use of the requested agent over BOTH aminocaproic acid AND tranexamic acid; OR
  - The requested agent will be used as perioperative management of bleeding AND BOTH of the following:
    - The individual has mild hereditary Factor X deficiency (Factor X level 6-10%); AND
    - ONE of the following:
      - The individual has tried and had an inadequate response to aminocaproic acid or tranexamic acid; OR
      - The individual has an intolerance or hypersensitivity to aminocaproic acid or tranexamic acid; OR
      - The individual has an FDA labeled contraindication to BOTH aminocaproic acid AND tranexamic acid; OR
      - The prescriber has provided information to support the use of the requested agent over BOTH aminocaproic acid AND tranexamic acid; AND
- The prescriber is a specialist (e.g., hematologist) in the area of the individual's diagnosis or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual does NOT have liver disease; AND
- The individual does NOT have vitamin K deficiency; AND
- The individual will NOT be using the requested agent in combination with an indirect or direct Factor Xa inhibitor [e.g., apixaban (Eliquis), dalteparin (Fragmin), edoxaban (Savaysa), enoxaparin (Lovenox), fondaparinux (Arixtra), rivaroxaban (Xarelto) or warfarin (Coumadin)]; AND
- The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen); AND

The requested hemophilia product is Fibryga or RiaSTAP; AND
- The individual has a diagnosis of fibrinogen deficiency (factor I deficiency) AND ONE of the following:
  - The individual is currently experiencing a bleed AND BOTH of the following:
    - The individual is out of medication; AND
    - The individual needs to receive a ONE-TIME emergency supply of medication; OR
  - ALL of the following:
    - The individual has a fibrinogen level of less than 150 mg/dL; AND
    - The individual does NOT have dysfibrinogenemia; AND
    - The requested agent will be used as on-demand treatment to control acute bleeding episodes; AND
    - The prescriber has communicated with the individual (via any means) and has verified that the individual does NOT have more than 5 on-demand doses on hand; AND
- The prescriber is a specialist (e.g., hematologist) in the area of the individual's diagnosis or the prescriber has consulted with a specialist in the area of the individual's diagnosis; AND
- The individual will NOT be using the requested agent in combination with nonsteroidal anti-inflammatory agents (NSAIDs) (e.g., aspirin, ibuprofen).

Length of Approval: One (1) month

Procedure Codes

J7170

J7175

J7177

J7178

J7179

J7182

J7183

J7185

J7186

J7187

J7189

J7190

J7192

J7193

J7194

J7195

J7200

J7201

J7202

J7203

J7204

J7205

J7207

J7208

J7209

J7210

J7211

J7212

J7213

J7199

NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines.

NOTE: Related policy I-9010 Emicizumab-kxwh (Hemlibra) - J7170

Covered Diagnosis Code for: J7170, J7182, J7185, J7190, J7192, J7204, J7205, J7207, J7208, J7209, J7210, J7211

D66

Covered Diagnosis code for J7175

D68.2

Covered Diagnosis Codes for J7177, J7178

D68.2

D68.8

O03.6

O08.1

O45.011

O45.012

O45.013

O45.019

O46.011

O46.012

O46.013

O46.019

O67.0

O72.3

Covered Diagnosis Codes for J7179, J7183

D68.00

D68.01

D68.020

D68.021

D68.022

D68.023

D68.029

D68.03

D68.04

D68.09

Covered Diagnosis Codes for J7186, J7187

D66

D68.00

D68.01

D68.020

D68.021

D68.022

D68.023

D68.029

D68.03

D68.04

D68.09

Covered Diagnosis Codes for J7189

D66

D67

D68.2

D68.311

Covered Diagnosis code for J7193, J7194, J7195, J7200, J7201, J7202, J7203,

D67

Covered Diagnosis codes for J7212

D66

D68.4

Covered Diagnosis code for J7312

D68.311

Professional Statemnts and Societal Positions Guidelines

Not Applicable

ND Committee Review

Internal Medical Policy Committee 11-29-2022 - Effective January 1, 2023

Adopted new precertification policy for hemophilia products for when pharmacy benefit billing is not feasible

Internal Medical Policy Committee 5-23-2023 - Effective June 1, 2023

Updated Factor VIII and von Willebrand Factor criteria
Added new drug Altuviiio

Internal Medical Policy Committee 7-26-2023 - Effective July 1, 2023

Added new code J7213 for Ixinity to the policy

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