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Hereditary Angioedema

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

February 2018

Therapeutic area - Hereditary Angioedema

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

Preferred

  • Berinert

Nonpreferred

  • Cinryze
  • Firazyr
  • Haegarda
  • Kalbitor
  • Ruconest

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

Questions?

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

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