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    Hereditary Angioedema

    Drugs - Berinert, Cinryze, Firazyr, Haegarda, Kalbitor, Ruconest, Takhzyro

    June 2019

    Therapeutic area - Hereditary Angioedema

    MHCP Preferred Drug List - Hereditary Angioedema; all required prior authorization

    Preferred

    • Berinert

    Nonpreferred

    • Cinryze
    • Firazyr
    • Haegarda
    • Kalbitor
    • Ruconest
    • Takhzyro

    Berinert

    Approval Criteria

    Patient has a diagnosis of hereditary angioedema

    Quantity Limits

    10 vials per 34 days

    Billing for Berinert

    • Berinert doses administered in an outpatient health care center or facility must be billed on a professional claim using J0597 (buy-and-bill)
    • Berinert doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement. Supporting documentation, including but not limited to medication administration record, must be provided at time of request
    • If Berinert doses are to be administered at home for “on-demand” use, documentation must be provided demonstrating that the patient has been taught self-infusion techniques

    Cinryze

    Initial approval criteria

    • Patient has been diagnosed with hereditary angioedema (HAE) AND
    • Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics AND
    • Patient has a history of 1 of the following criteria for long-term HAE prophylaxis:
      • History of 2 or more severe HAE attacks per month (e.g., airway swelling, debilitating cutaneous or gastrointestinal episodes) OR
      • Patient is disabled more than 5 days per month by HAE OR
      • History of recurrent laryngeal attacks caused by HAE AND
    • Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance AND
    • 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
    • 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
    • Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
      • Helicobacter pylori infections (confirmed by lab test) AND
      • Estrogen-containing oral contraceptive agents OR hormone replacement therapy AND
      • Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors

    Renewal approval criteria

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

    Quantity Limit

    20 vials per 34 days

    Billing for Cinryze

    • Cinryze doses administered in an outpatient health care center or facility must be billed on a professional claim using J0598 (buy-and-bill)
    • If Cinryze doses are to be administered at home for “on-demand” use, documentation must be provided demonstrating that the patient has been taught self-infusion techniques
    • Cinryze doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement. Supporting documentation, including but not limited to medication administration record, must be provided at time of request

    Firazyr

    Approval Criteria

    • Patient must be at least 18 years of age AND
    • Patient has been diagnosed with hereditary angioedema (HAE) AND
    • Patient is receiving only ONE agent indicated for the treatment of acute HAE attacks AND
    • Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance AND
    • Patient has a documented intolerance to OR treatment failure with Berinert OR
    • Access to Berinert is limited due to a verifiable drug shortage as reported in the FDA Drug Shortages Database OR
    • Patient resides in a geographical region of the state with limited access to acute care facility
    • 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
    • Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
      • Helicobacter pylori infections (confirmed by lab test) AND
      • Estrogen-containing oral contraceptive agents OR hormone replacement therapy AND
      • Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors

    Quantity limit

    3 syringes 

    Billing for Firazyr

    • Firazyr doses, if approved to be administered by a qualified health care professional in an outpatient health care center or facility, must be billed on a professional claim using J1744 (buy-and-bill)
    • Firazyr doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement. Supporting documentation, including but not limited to medication administration record, must be provided at time of request
    • If Firazyrt doses are to be administered at home for “on-demand” use, documentation must be provided demonstrating that the patient has been taught self-injection techniques

    Haegarda

    Initial approval criteria

    • Patient must be at least 12 years of age AND
    • Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics AND
    • Patient has a history of 1 of the following criteria for long-term HAE prophylaxis:
      • History of 2 or more severe HAE attacks per month (e.g., airway swelling, debilitating cutaneous or gastrointestinal episodes) OR
      • Patient is disabled more than 5 days per month by HAE OR
      • History of recurrent laryngeal attacks caused by HAE AND
    • 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
    • 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
    • Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
      • Helicobacter pylori infections (confirmed by lab test) AND
      • Estrogen-containing oral contraceptive agents OR hormone replacement therapy AND
      • Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors

    Renewal approval criteria

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

    Billing for Haegarda

    Haegarda doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement.

    Kalbitor

    Approval Criteria

    • Patient must be at least 12 years of age AND
    • Patient has been diagnosed with hereditary angioedema (HAE) AND
    • Patient is receiving only ONE agent indicated for the treatment of acute HAE attacks AND
    • Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance AND
    • Patient has a documented intolerance to OR treatment failure with Berinert OR
    • Access to Berinert is limited due to a verifiable drug shortage as reported in the FDA Drug Shortages Database
    • 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
    • Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
      • Helicobacter pylori infections (confirmed by lab test) AND
      • Estrogen-containing oral contraceptive agents OR hormone replacement therapy AND
      • Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors

    Quantity Limits

    Two 30 mg doses 

    Billing for Kalbitor

    • Kalbitor doses, administered in health care facility, must be billed on a professional claim using J1290 (buy-and-bill)
    • Kalbitor doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement. Supporting documentation, including but not limited to medication administration record, must be provided at time of request

    Ruconest

    Approval Criteria

    • Patient must be at least 13 years of age AND
    • Patient has been diagnosed with hereditary angioedema (HAE) AND
    • Patient is receiving only ONE agent indicated for the treatment of acute HAE attacks AND
    • Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance AND
    • Patient has a documented intolerance to OR treatment failure with Berinert, Firazyr, and Kalbitor OR
    • Access to Berinert, Firazyr, and Kalbitor is limited due to a verifiable drug shortage as reported in the FDA Drug Shortages Database AND
    • 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
    • Confirmation that the patient is avoiding the following possible triggers for HAE attacks:
      • Helicobacter pylori infections (confirmed by lab test) AND
      • Estrogen-containing oral contraceptive agents OR hormone replacement therapy AND
      • Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors

    Quantity Limit

    4 vials

    Billing for Ruconest

    • Ruconest doses administered in an outpatient health care center or facility must be billed on a professional claim using J0596 (buy-and-bill)
    • Ruconest doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement. Supporting documentation, including but not limited to medication administration record, must be provided at time of request
    • If Ruconest doses are to be administered at home for “on-demand” use, documentation must be provided demonstrating that the patient has been taught self-infusion techniques

    Takhzyro

    Approval Criteria

    • Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics AND
    • Patient must be ≥12 years of age AND
    • Documentation demonstrating that patient has been provided a treatment plan that include treatment for acute attacks and trigger avoidance AND
    • 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
    • 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
    • Takhzyro will not be used in combination with C1 inhibitor prophylaxis (e.g., Cinryze or Haegarda) AND
    • Patient is avoiding the following possible triggers for HAE attacks:
      • Helicobacter pylori infections (confirmed by lab test) AND
      • Estrogen-containing oral contraceptive agents OR hormone replacement therapy AND
      • Antihypertensive agents containing angiotensin-converting enzyme (ACE) inhibitors

    Renewal criteria

    • Patient continues to meet initial criteria AND
    • Significant improvement (at least 50 percent reduction) in severity and duration of attacks have been achieved and sustained AND
    • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include the following: severe hypersensitivity reactions, thromboembolic events, etc.
    • Patients who have demonstrated improvement/stabilization of disease and are well-controlled (e.g., attack free) for > 6 months may consider a trial of every 4 week dosing.

    Quantity limits

    • 2 vials per 28 days

    Billing for Takhzyro

    • Takhzyro doses administered in an inpatient setting or as part of an inpatient stay is not eligible for outpatient reimbursement
    • If Takhzyro doses are to be administered by the patient or caregiver, documentation must be provided demonstrating that the patient or caregiver has been taught self-injection techniques

    Questions?

    MHCP Provider Call Center 651-431-2700 or 800-366-5411

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