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Coverage criteria policies

Hereditary angioedema (HAE) drug therapy - Minnesota Health Care Programs

These services may or may not be covered by your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

Administrative Process

Hereditary angioedema medications require prior authorization from HealthPartners Pharmacy Administration.

Approval Criteria

Berinert

  1. Patient has a diagnosis of hereditary angioedema; and,
  2. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics.
  3. Max dosage: 10 vials per 34 days

Cinryze

Initial approval criteria:

  1. Patient has been diagnosed with hereditary angioedema (HAE); and,
  2. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; and,
  3. Patient has a history of 1 of the following criteria for long-term HAE prophylaxis:
    1. History of 2 or more severe HAE attacks per month (e.g., airway swelling, debilitating cutaneous or gastrointestinal episodes); or,
    2. Patient is disabled more than 5 days per month by HAE; or,
    3. History of recurrent laryngeal attacks caused by HAE; and,
  4. Patient has tried and failed or has a contraindication to prophylaxis with Takhzyro; and,
  5. Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance; and,
  6. Treatment of patient with “on-demand” therapy (e.g., Kalbitor, Firazyr, Ruconest, or Berinert) did not provide satisfactory control or access to “on-demand therapy” is limited; and,
  7. Patient has tried and failed, is intolerant to, or has a contraindication to attenuated (17 alpha-alkylated) androgens (e.g., danazol) for HAE prophylaxis; and,
  8. Confirmation that the patient is avoiding all of the following possible triggers for HAE attacks:
    1. Helicobacter pylori infections (confirmed by lab test); and,
    2. Estrogen-containing oral contraceptive agents or hormone replacement therapy; and,
    3. Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors; and,
  9. The drug is prescribed according to the FDA approved regimen or meet the following dosing guidelines:
    1. Cinryze maximum dose of 1,000 units every 3 to 4 days, patients requiring doses exceeding this limit will require trial of an alternate HAE prophylaxis therapy

Renewal approval criteria:

  1. Patient continues to meet initial criteria; and,
  2. Significant improvement (at least 50 percent reduction) in severity and duration of attacks have been achieved and sustained; and,
  3. Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: severe hypersensitivity reactions, thromboembolic events, etc.

Max dosage: 20 vials per 34 days

Firazyr®

  1. Patient must be at least 18 years of age; and,
  2. Patient has been diagnosed with hereditary angioedema (HAE); and,
  3. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; and,
  4. Patient is receiving only one agent indicated for the treatment of acute HAE attacks; and,
  5. Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance; and,
  6. One of the following three criteria is met:
    1. Patient has a documented intolerance to or treatment failure with Berinert; or,
    2. Access to Berinert is limited due to a verifiable drug shortage as reported in the FDA Drug Shortages Database; or,
  7. Patient resides in a geographical region of the state with limited access to acute care facility; and,
  8. Patient has tried and failed, is intolerant to, or has a contraindication to attenuated (17 alpha-alkylated) androgens (e.g., danazol) for HAE prophylaxis; and,
  9. Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
    1. Helicobacter pylori infections (confirmed by lab test); and,
    2. Estrogen-containing oral contraceptive agents or hormone replacement therapy; and,
    3. Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors; and,
  10. The drug is prescribed according to the FDA approved regimen.

Max dosage: 3 syringes

Haegarda®

Initial approval criteria:

  1. Patient must be at least 12 years of age; and,
  2. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; and,
  3. Patient has a history of 1 of the following criteria for long-term HAE prophylaxis:
    1. History of 2 or more severe HAE attacks per month (e.g., airway swelling, debilitating cutaneous or gastrointestinal episodes); or,
    2. Patient is disabled more than 5 days per month by HAE; or,
    3. History of recurrent laryngeal attacks caused by HAE; and,
  4. Treatment of patient with “on-demand” therapy (e.g., Kalbitor, Firazyr, Ruconest, or Berinert) did not provide satisfactory control or access to “on-demand therapy” is limited; and,
  5. Patient has tried and failed, is intolerant to, or has a contraindication to attenuated (17 alpha-alkylated) androgens (e.g., danazol) for HAE prophylaxis; and,
  6. Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
    1. Helicobacter pylori infections (confirmed by lab test); and,
    2. Estrogen-containing oral contraceptive agents or hormone replacement therapy; and,
    3. Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors; and,
  7. The drug is prescribed according to the FDA approved regimen.

Renewal approval criteria:

  1. Patient continues to meet initial criteria; and,
  2. Significant improvement (at least 50 percent reduction) in severity and duration of attacks have been achieved and sustained; and,
  3. Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: severe hypersensitivity reactions, thromboembolic events, etc.

Kalbitor

  1. Patient must be at least 12 years of age; and,
  2. Patient has been diagnosed with hereditary angioedema (HAE); and,
  3. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; and,
  4. Patient is receiving only one agent indicated for the treatment of acute HAE attacks; and,
  5. Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance; and,
  6. One of the following two criteria is met:
    1. Patient has a documented intolerance to or treatment failure with Berinert; or,
    2. Access to Berinert is limited due to a verifiable drug shortage as reported in the FDA Drug Shortages Database; and,
  7. Patient has tried and failed, is intolerant to, or has a contraindication to attenuated (17 alpha-alkylated) androgens (e.g., danazol) for HAE prophylaxis; and,
  8. Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
    1. Helicobacter pylori infections (confirmed by lab test); and,
    2. Estrogen-containing oral contraceptive agents or hormone replacement therapy; and,
    3. Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors.
  9. The drug is prescribed according to the FDA approved regimen.

Max dosage: two 30mg doses

Ruconest

  1. Patient must be at least 13 years of age; and,
  2. Patient has been diagnosed with hereditary angioedema (HAE); and,
  3. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; and,
  4. Patient is receiving only one agent indicated for the treatment of acute HAE attacks; and,
  5. Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance; and,
  6. One of the following two criteria is met:
    1. Patient has a documented intolerance to or treatment failure with Berinert, Firazyr, and Kalbitor; or,
    2. Access to Berinert, Firazyr, and Kalbitor is limited due to a verifiable drug shortage as reported in the FDA Drug Shortages Database; and,
  7. Patient has tried and failed, is intolerant to, or has a contraindication to attenuated (17 alpha-alkylated) androgens (e.g., danazol) for HAE prophylaxis; and,
  8. Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
    1. Helicobacter pylori infections (confirmed by lab test); and,
    2. Estrogen-containing oral contraceptive agents or hormone replacement therapy; and,
    3. Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors.
  9. Max dosage: 4 vials

Takhyzro®

Initial approval criteria:

  1. Patient must be at least 12 years of age; and,
  2. Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; and,
  3. Patient has a history of 1 of the following criteria for long-term HAE prophylaxis:
    1. History of 2 or more severe HAE attacks per month (e.g., airway swelling, debilitating cutaneous or gastrointestinal episodes); or,
    2. Patient is disabled more than 5 days per month by HAE; or,
    3. History of recurrent laryngeal attacks caused by HAE; and,
  4. Treatment of patient with “on-demand” therapy (e.g., Kalbitor, Firazyr, Ruconest, or Berinert) did not provide satisfactory control or access to “on-demand therapy” is limited; and,
  5. Patient has tried and failed, is intolerant to, or has a contraindication to attenuated (17 alpha-alkylated) androgens (e.g., danazol) for HAE prophylaxis; and,
  6. Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
  7. Helicobacter pylori infections (confirmed by lab test); and,
    1. Estrogen-containing oral contraceptive agents or hormone replacement therapy; and,
    2. Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors; and,
  8. The drug is prescribed according to the FDA approved regimen

Renewal approval criteria:

  1. Patient continues to meet initial criteria; and,
  2. Significant improvement (at least 50 percent reduction) in severity and duration of attacks have been achieved and sustained; and,
  3. Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: severe hypersensitivity reactions, thromboembolic events, etc.

Max dosage: two 300 mg doses

Definitions

Hereditary angioedema (HAE) is a rare genetic disorder resulting from an inherited deficiency or dysfunction of the C1 inhibitor. It is characterized by recurrent episodes of angioedema, without urticaria or pruritis, which most often affect the skin or the mucosal tissues of the upper respiratory and gastrointestinal tracts.

The exact mechanism(s) that lead to angioedema are not fully understood. The role of C1 inhibitor in regulating the production of the proinflammatory mediator bradykinin appears to be critical in the pathogenesis of HAE. Kallikrein is a mediator in the production of bradykinin.

Effective therapies may replace the inhibitor (administration of C1-inhibitor concentrate or fresh frozen plasma) or increase its hepatic synthesis (administration of androgens). During acute attacks the administration of kallikrein inhibitors may reduce the production of bradykinin.

Cinryze® is a C1 inhibitor, FDA-approved for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE).

Berinert® is a C1 inhibitor, FDA-approved for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adult and adolescent patients.

Ruconest® is a C1 inhibitor, FDA-approved for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE). Effectiveness was not established in HAE patients with laryngeal attacks.

Kalbitor® is a plasma kallikrein inhibitor, FDA-approved for the treatment of acute attacks of hereditary angioedema (HAE) in patients 12 years and older.

Firazyr® is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of hereditary angioedema (HAE) in adults 18 years of age and older.

Haegarda® is a plasma-derived concentrate of C1 esterase inhibitor (human), indicated for routine prophylaxis to prevent Hereditary Angioedema (HAE) attacks in adolescent and adult patients.

Codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

HCPCS Codes

Codes

Description

C9399

Unclassified drugs or biologicals (Hospital Outpatient Use ONLY) (use before 10/1/2019)

J0593

Injection, lanadelumab-flyo, (Takhzyro), 1 mg (effective 10/1/2019)

J0596

Injection, c-1 esterase inhibitor (recombinant), Ruconest, 10 units

J0597

Injection, C-1 esterase inhibitor (human), Berinert, 10 units

J0598

Injection, C-1 esterase inhibitor (human), Cinryze, 10 units

J0599

Injection, c-1 esterase inhibitor (human), (Haegarda), 10 units

J1290

Injection, ecallantide, 1 mg (Kalbitor)

J1744

Injection, icatibant, 1 mg (Firazyr)

J3590

Unclassified biologics (use before 10/1/2019)

NDC Codes

Codes

Description

63833082502

Berinert 500 unit kit

63833083501

Berinert 500(10ML) VIAL (EA)

42227008105

Cinryze 500 UNIT Solution Reconstituted

47783010101

Kalbitor 10 MG/ML Solution

54092070202

Firazyr 30 MG/3ML Solution

54092070203

Firazyr 30 MG/3ML Solution

68012035001

Ruconest 2100 UNIT Solution Reconstituted

68012035002

Ruconest 2100 UNIT Solution Reconstituted

71274035001

Ruconest 2100 UNIT Solution Reconstituted

71274035002

Ruconest 2100 UNIT Solution Reconstituted

63833082802

Haegarda 2000 UNIT Solution Reconstituted

63833082902

Haegarda 3000 UNIT Solution Reconstituted

47783064401

Takhzyro 300 MG/2ML Solution

CPT Copyright American Medical Association. All rights reserved.  CPT is a registered trademark of the American Medical Association.

Products

This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.

References

  1. https://mn.gov/dhs/partners-and-providers/policies-procedures/minnesota-health-care-programs/provider/types/rx/pa-criteria/hereditary-angioedema.jsp

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Policy activity

  • 01/01/2017 - Date of origin
  • 07/01/2019 - Effective date
Review date
  • 11/2018
Revision date
  • 05/06/2019

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