I. Berinert [C1 Esterase Inhibitor (Human)]
When a benefit, coverage of Berinert may be approved when all of the following criteria are met (1. through 6.):
A. HAE Type I & II
1. Berinert is being used for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in patients greater than or equal to five (5) years of age .
2. A 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).
3. Past medical 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
4. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
5. Members should not be on two claims for acute therapy processed on the same day.
6. Documentation of the member’s weight has been provided.
When a benefit, coverage of Berinert may be approved when all of the following criteria are met (1. through 7.):
B. HAE Type III
1. Berinert is being used for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in patients ≥ 5 years of age.
2. 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
3. Clinical documentation of at least one of the following (a. or b.):
a. Family history of HAE
b. FXII mutation
4. Past medical 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. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
6. Members should not be on two claims for acute therapy processed on the same day.
7. Documentation of the member’s weight has been provided.
II. Cinryze [C1 Esterase Inhibitor (Human)]
When a benefit, coverage of Cinryze may be approved when all of the following criteria are met (1. through 6.):
A. HAE Type I & II
1. Cinryze is being used for routine prophylaxis management against angioedema attacks in adult, adolescent, and pediatric patients (≥ 6 years of age) with hereditary angioedema (HAE).
2. A 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).
3. Past medical 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
4. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
5. Members should not be on two claims for acute therapy processed on the same day.
6. Documentation of the member’s weight has been provided.
When a benefit, coverage of Cinryze may be approved when all of the following criteria are met (1. through 7.):
B. HAE Type III
1. Cinryze is being used for routine prophylaxis management against angioedema attacks in adult, adolescent, and pediatric patients (≥ 6 years of age) with hereditary angioedema (HAE).
2. 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
3. Clinical documentation of at least one of the following (a. or b.):
a. Family history of HAE
b. FXII mutation
4. Past medical 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. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
6. Members should not be on two claims for acute therapy processed on the same day.
7. Documentation of the member’s weight has been provided.
III. Firazyr (icatibant)
When a benefit, coverage of Firazyr may be approved when all of the following criteria are met (1. through 6.):
A. HAE Type I & II
1. Firazyr is being used for treatment of acute attacks of hereditary angioedema (HAE) in adults (≥ 18 years of age).
2. A 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).
3. Past medical 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
4. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
5. Members should not be on two claims for acute therapy processed on the same day.
6. Documentation of the member’s weight has been provided.
When a benefit, coverage of Firazyr may be approved when all of the following criteria are met (1. through 7.):
B. HAE Type III
1. Firazyr is being used for treatment of acute attacks of hereditary angioedema (HAE) in adults (≥ 18 years of age).
2. 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
3. Clinical documentation of at least one of the following (a. or b.):
a. Family history of HAE
b. FXII mutation
4. Past medical 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. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
6. Members should not be on two claims for acute therapy processed on the same day.
7. Documentation of the member’s weight has been provided.
IV. Haegarda [C1 Esterase Inhibitor (Human)]
When a benefit, coverage of Haegarda may be approved when all of the following criteria are met (1. through 6.):
A. HAE Type I & II
1. Haegarda is being used for prophylaxis management against angioedema attacks in patients ≥ 12 years of age with hereditary angioedema (HAE).
2. A 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).
3. Past medical 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
4. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
5. Members should not be on two claims for acute therapy processed on the same day.
6. Documentation of the member’s weight has been provided.
When a benefit, coverage of Haegarda may be approved when all of the following criteria are met (1. through 7.):
B. HAE Type III
1. Haegarda is being used for prophylaxis management against angioedema attacks in patients ≥ 12 years of age with hereditary angioedema (HAE).
2. 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
3. Clinical documentation of at least one of the following (a. or b.):
a. Family history of HAE
b. FXII mutation
4. Past medical 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. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
6. Members should not be on two claims for acute therapy processed on the same day.
7. Documentation of the member’s weight has been provided.
V. Ruconest [C1 Esterase Inhibitor (Recombinant)]
When a benefit, coverage of Ruconest may be approved when all of the following criteria are met (1. through 6.):
A. HAE Type I & II
1. Ruconest is being used for treatment of acute hereditary angioedema (HAE) attacks in patients ≥ 13 years of age.
2. A 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).
3. Past medical 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
4. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
5. Members should not be on two claims for acute therapy processed on the same day.
6. Documentation of the member’s weight has been provided.
When a benefit, coverage of Ruconest may be approved when all of the following criteria are met (1. through 7.):
B. HAE Type III
1. Ruconest is being used for treatment of acute hereditary angioedema (HAE) attacks in patients ≥ 13 years of age.
2. 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
3. Clinical documentation of at least one of the following (a., or b.):
a. Family history of HAE
b. FXII mutation
4. Past medical 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. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
6. Members should not be on two claims for acute therapy processed on the same day.
7. Documentation of the member’s weight has been provided.
VI. Takhzyro (lanadelumab-flyo)
When a benefit, coverage of Takhzyro may be approved when all of the following criteria are met (1. through 6.):
A. HAE Type I & II
1. Takhzyro is being used for prophylaxis to prevent attacks of hereditary angioedema (HAE) in patients 12 years of age and older.
2. A 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).
3. Past medical 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
4. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
5. Members should not be on two claims for acute therapy processed on the same day.
6. Documentation of the member’s weight has been provided.
When a benefit, coverage of Takhzyro may be approved when all of the following criteria are met (1. through 7.):
B. HAE Type III
1. Takhzyro is being used for prophylaxis to prevent attacks of hereditary angioedema (HAE) in patients 12 years of age and older.
2. 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
3. Clinical documentation of at least one of the following (a., or b.):
a. Family history of HAE
b. FXII mutation
4. Past medical 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. Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor blockers) have been evaluated and discontinued when appropriate.
6. Members should not be on two claims for acute therapy processed on the same day.
7. Documentation of the member’s weight has been provided.
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.