| Pharmacy Policy Bulletin |
| Hereditary Angioedema - Commercial and Healthcare Reform | |
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| Number: J-0424 | Category: Prior Authorization |
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Line(s) of Business:
Commercial |
Benefit(s):
Commercial: Prior Authorization 1. Miscellaneous Specialty Drugs Oral = Yes w/ Prior Authorization 2. Miscellaneous Specialty Drugs Injectable = Yes w/ Prior Authorization Healthcare Reform: Not Applicable |
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Region(s):
All |
Additional Restriction(s):
None |
| Drugs Products |
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| FDA-Approved Indications: |
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| Background: |
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| Approval Criteria |
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I. Berinert [C1 Esterase Inhibitor (Human)] A. HAE Type I & II When a benefit, coverage of Berinert may be approved when all of the following criteria are met (1. through 8.): 1. The member is 5 years of age or older. 2. Berinert is being used for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE). 3. The member has low C4 level less than or equal to 14mg/dL or a C4 below the lower limit of the laboratory’s reference range and one (1) of the following criteria (a. or b.): a. C1 inhibitor (C1INH) antigenic level less than or equal to 19 mg/dL (normal range: 19 to 37 mg/dL) or below the lower limit of the laboratory’s reference range. b. A normal C1INH antigenic level (C1INH greater than or equal to 19 mg/dL) and a low C1INH functional level (functional C1INH less than 50% or below the laboratory’s reference range). 4. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 5. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 6. The member should not have two claims for acute therapy processed on the same day. 7. The prescriber provided documentation of the member’s weight. 8. If the member is 18 years of age or older, the member has experienced therapeutic failure, intolerance, or contraindication to generic icatibant.
B. HAE Type III When a benefit, coverage of Berinert may be approved when all of the following criteria are met (1. through 9.): 1. The member is 5 years of age or older. 2. Berinert is being used for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE). 3. The prescriber provided clinical laboratory performance of at least one (1) of the following (a., b., or c.): a. C4 within normal limits (normal range: 14 to 40 mg/dL) or C4 within normal limits of the laboratory's normal reference range b. C1INH antigen within normal limits (normal range:19 to 37 mg/dL) or C1INH antigenic level within normal limits of the laboratory's normal reference range c. C1INH functional within normal limits of the laboratory's normal reference range 4. The prescriber provided clinical documentation of at least one (1) of the following (a. or b.): a. Family history of HAE b. FXII mutation 5. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 6. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 7. The member should not have two claims for acute therapy processed on the same day. 8. The prescriber provided documentation of the member’s weight. 9. If the member is 18 years of age or older, the member has experienced therapeutic failure, intolerance, or contraindication to generic icatibant.
II. Cinryze [C1 Esterase Inhibitor (Human)] A. HAE Type I & II When a benefit, coverage of Cinryze may be approved when all of the following criteria are met (1. through 7.): 1. The member is 6 years of age or older. 2. Cinryze is being used for routine prophylaxis management against angioedema attacks of hereditary angioedema (HAE). 3. The member has low C4 level less than or equal to 14mg/dL or a C4 below the lower limit of the laboratory’s reference range and one (1) of the following criteria (a. or b.): a. C1 inhibitor (C1INH) antigenic level less than or equal to 19 mg/dL (normal range: 19 to 37 mg/dL) or below the lower limit of the laboratory’s reference range. b. A normal C1INH antigenic level (C1INH greater than or equal to 19 mg/dL) and a low C1INH functional level (functional C1INH less than 50 % or below the laboratory’s reference range). 4. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 5. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 6. The member should not have two claims for acute therapy processed on the same day. 7. The prescriber provided documentation of the member’s weight.
B. HAE Type III When a benefit, coverage of Cinryze may be approved when all of the following criteria are met (1. through 8.): 1. The member is 6 years of age or older. 2. Cinryze is being used for routine prophylaxis management against angioedema attacks in adult, adolescent, and pediatric patients with hereditary angioedema (HAE). 3. The prescriber provided clinical laboratory performance of at least one (1) of the following (a., b., or c.): a. C4 within normal limits (normal range:14 to 40 mg/dL) OR C4 within normal limits of the laboratory's normal reference range b. C1INH antigen within normal limits (normal range:19 to 37 mg/dL) OR C1INH antigenic level within normal limits of the laboratory's normal reference range c. C1INH functional within normal limits of the laboratory's normal reference range 4. The prescriber provided clinical documentation of at least one (1) of the following (a. or b.): a. Family history of HAE b. FXII mutation 5. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 6. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 7. The member should not be on two claims for acute therapy processed on the same day. 8. The prescriber provided documentation of the member’s weight.
III. Firazyr (icatibant) A. HAE Type I & II When a benefit, coverage of brand Firazyr or generic icatibant may be approved when all of the following criteria are met (1. through 8.): 1. The member is 18 years of age or older. 2. Firazyr is being used for treatment of acute attacks of hereditary angioedema (HAE). 3. The member has low C4 level less than or equal to 14mg/dL or a C4 below the lower limit of the laboratory’s reference range and one (1) of the following criteria (a. or b.): a. C1 inhibitor (C1INH) antigenic level less than or equal to 19 mg/dL (normal range:19 to 37 mg/dL) or below the lower limit of the laboratory’s reference range. b. A normal C1INH antigenic level (C1INH greater than or equal to 19 mg/dL) and a low C1INH functional level (functional C1INH less than 50 % or below the laboratory’s reference range). 4. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 5. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 6. The member should not be on two claims for acute therapy processed on the same day. 7. The prescriber provided documentation of the member’s weight. 8. If the brand Firazyr is being requested, the member has experienced therapeutic failure, intolerance, or contraindication to generic icatibant.
B. HAE Type III When a benefit, coverage of brand Firazyr or generic icatibant may be approved when all of the following criteria are met (1. through 9.): 1. The member is 18 years of age or older. 2. Firazyr is being used for treatment of acute attacks of hereditary angioedema (HAE). 3. The prescriber provided clinical laboratory performance of at least one (1) of the following (a., b., or c.): a. C4 within normal limits (normal range: 14 to 40 mg/dL) OR C4 within normal limits of the laboratory's normal reference range b. C1INH antigen within normal limits (normal range:19 to 37 mg/dL) OR C1INH antigenic level within normal limits of the laboratory's normal reference range c. C1INH functional within normal limits of the laboratory's normal reference range 4. The prescriber provided clinical documentation of at least one (1) of the following (a. or b.): a. Family history of HAE b. FXII mutation 5. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 6. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 7. The members should not have two claims for acute therapy processed on the same day. 8. The prescriber provided documentation of the member’s weight. 9. If the brand Firazyr is being requested, the member has experienced therapeutic failure, intolerance, or contraindication to generic icatibant.
IV. Haegarda [C1 Esterase Inhibitor (Human)] A. HAE Type I & II When a benefit, coverage of Haegarda may be approved when all of the following criteria are met (1. through 7.): 1. The member is 12 years of age or older. 2. Haegarda is being used for prophylaxis management against angioedema attacks of hereditary angioedema (HAE). 3. The member has low C4 level less than or equal to 14mg/dL or a C4 below the lower limit of the laboratory’s reference range and one (1) of the following criteria (a. or b.): a. C1 inhibitor (C1INH) antigenic level less than or equal to 19 mg/dL (normal range:19 to 37 mg/dL) or below the lower limit of the laboratory’s reference range. b. A normal C1INH antigenic level (C1INH greater than or equal to 19 mg/dL) and a low C1INH functional level (functional C1INH less than 50 % or below the laboratory’s reference range). 4. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 5. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 6. The members should not have two claims for acute therapy processed on the same day. 7. The prescriber provided documentation of the member’s weight.
B. HAE Type III When a benefit, coverage of Haegarda may be approved when all of the following criteria are met (1. through 8.): 1. The member is 12 years of age or older. 2. Haegarda is being used for prophylaxis management against angioedema attacks of hereditary angioedema (HAE). 3. The prescriber provided clinical laboratory performance of at least one (1) of the following (a., b., or c.): a. C4 within normal limits (normal range:14 to 40 mg/dL) or C4 within normal limits of the laboratory's normal reference range b. C1INH antigen within normal limits (normal range:19 to 37 mg/dL) or C1INH antigenic level within normal limits of the laboratory's normal reference range c. C1INH functional within normal limits of the laboratory's normal reference range 4. The prescriber provided clinical documentation of at least one (1) of the following (a. or b.): a. Family history of HAE b. FXII mutation 5. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 6. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 7. The member should not have two claims for acute therapy processed on the same day. 8. The prescriber provided documentation of the member’s weight.
V. Ruconest [C1 Esterase Inhibitor (Recombinant)] A. HAE Type I & II When a benefit, coverage of Ruconest may be approved when all of the following criteria are met (1. through 8.): 1. The member is 13 years of age or older. 2. Ruconest is being used for treatment of acute hereditary angioedema (HAE) attacks. 3. The member has low C4 level less than or equal to 14mg/dL or a C4 below the lower limit of the laboratory’s reference range and one (1) of the following criteria (a. or b.): a. C1 inhibitor (C1INH) antigenic level less than or equal to 19 mg/dL (normal range: 19 to 37 mg/dL) or below the lower limit of the laboratory’s reference range. b. A normal C1INH antigenic level (C1INH greater than or equal to 19 mg/dL) and a low C1INH functional level (functional C1INH less than 50 % or below the laboratory’s reference range). 4. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 5. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 6. The member should not have two claims for acute therapy processed on the same day. 7. The prescriber provided documentation of the member’s weight. 8. If the member is 18 years of age or older, the member has experienced therapeutic failure, intolerance, or contraindication to generic icatibant.
B. HAE Type III When a benefit, coverage of Ruconest may be approved when all of the following criteria are met (1. through 9.): 1. The member is 13 years of age or older. 2. Ruconest is being used for treatment of acute hereditary angioedema (HAE) attacks. 3. The prescriber provided clinical laboratory performance of at least one (1) the following (a., b., or c.): a. C4 within normal limits (normal range:14 to 40 mg/dL) OR C4 within normal limits of the laboratory's normal reference range b. C1INH antigen within normal limits (normal range:19 to 37 mg/dL) OR C1INH antigenic level within normal limits of the laboratory's normal reference range c. C1INH functional within normal limits of the laboratory's normal reference range 4. The prescriber provided clinical documentation of at least one (1) of the following (a. or b.): a. Family history of HAE b. FXII mutation 5. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 6. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 7. The member should not have two claims for acute therapy processed on the same day. 8. The prescriber provided documentation of the member’s weight. 9. If the member is 18 years of age or older, the member has experienced therapeutic failure, intolerance, or contraindication to generic icatibant.
VI. Takhzyro (lanadelumab-flyo) A. HAE Type I & II When a benefit, coverage of Takhzyro may be approved when all of the following criteria are met (1. through 7.): 1. The member is 12 years of age or older. 2. Takhzyro is being used for prophylaxis to prevent attacks of hereditary angioedema (HAE) in patients 12 years of age and older. 3. The member has low C4 level less than or equal to 14mg/dL or a C4 below the lower limit of the laboratory’s reference range and one of the following criteria (a. or b.): a. C1 inhibitor (C1INH) antigenic level less than or equal to 19 mg/dL (normal range:19 to 37 mg/dL) or below the lower limit of the laboratory’s reference range. b. A normal C1INH antigenic level (C1INH greater than or equal to 19 mg/dL) and a low C1INH functional level (functional C1INH less than 50 % or below the laboratory’s reference range). 4. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 5. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 6. The member should not have two claims for acute therapy processed on the same day. 7. The prescriber provided documentation of the member’s weight.
B. HAE Type III When a benefit, coverage of Takhzyro may be approved when all of the following criteria are met (1. through 8.): 1. The member is 12 years of age or older. 2. Takhzyro is being used for prophylaxis to prevent attacks of hereditary angioedema (HAE). 3. The prescriber provided clinical laboratory performance of the following (a., b., or c.): a. C4 within normal limits (normal range:14 to 40 mg/dL) or C4 within normal limits of the laboratory's normal reference range b. C1INH antigen within normal limits (normal range:19 to 37 mg/dL) or C1INH antigenic level within normal limits of the laboratory's normal reference range c. C1INH functional within normal limits of the laboratory's normal reference range 4. The prescriber provided clinical documentation of at least one (1) of the following (a. or b.): a. Family history of HAE b. FXII mutation 5. The member has a history of at least one (1) symptom of moderate or severe angioedema attack (e.g. airway swelling, severe abdominal pain, facial swelling, nausea and vomiting, painful facial distortion) in absence of concomitant hives or use of medication to cause angioedema 6. The member’s medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate. 7. The members should not have two claims for acute therapy processed on the same day. 8. The prescriber provided documentation of the member’s weight.
VII. For Commercial and HCR members enrolled in a West Virginia Plan, an exception to the step therapy within this policy may be made based on Policy J-513 – West Virginia – Step Therapy Override Exception – Commercial and Healthcare Reform. |
| Limitations of Coverage |
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I. Coverage of Berinert, Cinryze, Firazyr (icatibant), Haegarda, Ruconest and Takhzyro for disease states outside of their FDA-approved indications should be denied based on the lack of clinical data to support their effectiveness and safety in other conditions. II. For Commercial or HCR members with a closed formulary, a non-formulary product will only be approved if the member meets the criteria for a formulary exception in addition to the criteria outlined within this policy.
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| Authorization Duration |
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| Automatic Approval Criteria |
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None |
| Version: J-0424-007 |
| Effective Date Begin: 01/01/2020 |
| Effective End Begin: 06/21/2020 |
| Original Date: 03/04/2015 |
| Review Date: 08/07/2019 |
References:
1. Cinryze [package insert]. Shire ViroPharma, Inc. Lexington, MA. 2008. June 2018.
2. DRUGDEX System (Micromedex 2.0). Greenwood Village, CO: Truven Health Analytics: 2019.
3. Berinert [package insert]. CSL Behring, Kankakee, IL: 2009. September 2016.
4. Ruconest [package insert]. Santaris. Raleigh, NC. 2014. February 2015.
5. Haegarda [package insert].Kankakee, IL: CSL Behring, LLC; June 2017.
6. Firazyr [package insert]. Lexington, MA: Shire Orphan Therapies, LLC; November 2015.
7. Takhzyro [package insert]. Lexington, MA: Shire LLC; August 2018.
8. Banerji A. Lanadelumab for prevention of attacks in hereditary angioedema: results from the phase 3 HELP study. American College of Allergy, Asthma and Immunology Meeting. 2017, 74: 1-18
9. Banerji et al. Inhibiting plasma kallikrein for hereditary angioedema prophylaxis. N Engl J Med. 2017; 376(8):717-728.
10. Craig T, Pursun A, Bork K, et al. WAO guideline for the management of hereditary angioedema. Available at: Accessed January 25, 2016.
11. Zuraw BL, Banerji A, Bernstein JA, Busse PJ, et al. US Hereditary Angioedema Association Medical Advisory Board 2013 Recommendations for the Management of Hereditary Angioedema Due to C1 Inhibitor Deficiency. J Allergy Clin Immunol.2013;1:458-67.
12. Gainer JV, Morrow JD, Loveland A, et al. Effect of Bradykinin-Receptor Blockade on the Response To Angiotensin-Converting-Enzyme Inhibitor In Normotensive And Hypertensive Subjects. NEJM. 1998; 339(18): 1285-1291.
13. Zuraw BL, Bork K, Binkley KE, et al. Hereditary angioedema with normal C1 inhibitor function: Consensus of an international expert panel. Allergy Asthma Proc. 2012;33 Suppl 1:S145-S156.
14. Bowen T, Cicardi M, Farkas H, et al. 2010. International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol 2010;6:24.