Pharmacy Management Drug Policy
Hereditary Angioedema (HAE)
Proprietary of the Insurance Plan Page 4 of 7
E. Firazyr specific criteria
1. Must have a diagnosis of hereditary angioedema (progress notes required)
2. Must be used for acute episodic attacks--Safety and efficacy as prophylactic therapy
has not been established and therefore will not be covered
3. Firazyr has potential to attenuate antihypertensive effects of ACE inhibitors and should
not be administered to patients taking ACE inhibitors since there are no clinical trials in
this population
4. Standard dosing is 30mg subcutaneously injected into abdominal area; if response is
inadequate or symptoms recur additional injections of 30mg may be administered at
intervals of at least 6 hours with no more than 3 doses per 24 hour period
5. Quantity limit of #3 (3ml each) pre-filled syringes – 9ml/30 days.
6. Authorization will be for 6 months at a time
a. Recertification will require documentation of decrease in severity or
duration of attacks. Documentation including frequency of administration will also
be required at time of recertification to monitor for appropriate use.
7. Safety and Efficacy in anyone under 18 years old is not established and therefore will
not be covered.
8. Firazyr can be self-administered upon recognition of an HAE attack after training under
the guidance of a healthcare professional and therefore will be covered under the
pharmacy benefit.
F. Ruconest specific criteria
1. Must have a diagnosis of acute attacks associated with hereditary angioedema
(progress notes required)
2. Ruconest will not be covered for laryngeal attacks as efficacy has not been established
in patients with laryngeal HAE attacks
3. Safety and efficacy in children under the age of 13 has not been established and
therefore will not be covered
4. Ruconest will be reviewed under the medical benefit when administered by a health
care professional. If the member transitions to self-administration, then Ruconest can
be authorized under the pharmacy benefit.
5. Must have documentation of therapeutic failure or severe adverse reaction to Berinert or
Firazyr
6. Dose is 50 IU per kg by IV injection
a. Maximum dose is 4200 IU with no more than 2 doses within a 24 hour time
period
b. Quantity limit of 100 ml per 30 days
c. Authorization on the medical benefit will be for 1 month initially then every 6
months
d. Authorization on the pharmacy benefit will be for 6 months at a time
e. Recertification will require documentation of decrease in severity or duration of
attacks. Documentation including frequency of administration will also be
required at time of recertification to monitor for appropriate use
7. Based on national shortages of other medications that are FDA approved for HAE
prophylaxis and emerging clinical data, requests for Ruconest for hereditary
angioedema prophylaxis will be allowed with the following dosing:
Pharmacy Management Drug Policy
Hereditary Angioedema (HAE)
Proprietary of the Insurance Plan Page 5 of 7
50 IU per kg for patients less than 84 kg or 4200 IU for patients greater than or equal to
84 kg twice weekly
a. Quantity limit of 16 vials per 28 days
b. Approvals will be short term (2 months) until supply of other medications that are
FDA approved for HAE prophylaxis is adequate.
G. Haegarda specific criteria
1. Must have a diagnosis of hereditary angioedema (progress notes required)
2. Must be used as a prophylaxis – not for acute treatment
3. For long term prophylaxis, the patient must meet the following:
a. History of at least two severe HAE attacks (i.e. airway swelling, debilitating
cutaneous or gastrointestinal episodes) per month that resulted in loss of
work/school productivity or ER/unscheduled doctor visits OR disability for > 5 days
per month due to HAE AND
b. Patient has a documented contraindication, severe intolerance, or therapeutic
failure to 17 alpha-alkylated androgens (e.g. danazol) for HAE prophylaxis AND
c. Treatment with acute therapy (i.e. Kalbitor, Firazyr, Berinert or Ruconest) did not
result in meaningful outcomes such as decreased severity of attacks, avoidance
of hospitalization, etc
4. Haegarda will be allowed for short-term prophylaxis if being requested prior to medical,
surgical or dental procedure. Approval will be for one month only.
5. Haegarda can be self-administered after training under the guidance of a healthcare
professional and therefore will be covered under the pharmacy benefit.
6. Standard dosage is 60 International Units (IU) per kg body weight by subcutaneous
injection twice weekly.
a. Quantity limit of 16 vials per 28 days (defined as the combined total amount of
2000 unit vials AND 3000 unit vials)
b. Requests will require documentation of patient’s weight to verify requested
dosing is in accordance to the FDA approved dosing.
c. Requests for dosing every three days will require documentation of a previous
trial of twice weekly dosing that did not lead to a decrease in the severity,
duration and/or frequency of attacks.
7. Authorization will be for 6 months at a time.
a. Recertification will require documentation of decrease in the severity, duration,
and/or frequency of attacks. Documentation including frequency of administration
will also be required at time of recertification to monitor for appropriate use.
8. Safety and efficacy in children under the age of 13 has not been established and
therefore will not be covered
POLICY GUIDELINES:
1. Prior-authorization is contract dependent.
2. A normal C4 particularly during an edema attack should make one question the diagnosis of HAE
3. Some medications may trigger or worsen angioedema events in patients with HAE and should be
avoided including estrogen contraceptives, hormone replacement therapy, and ACE-Inhibitors.