Guideline-recommended treatments for HAE available in Canada that are supported by high-level evidence (3)
| HAE-specific treatment | Brand name | Mechanism of action | Approved indications in Canada | Dose and route of administration | Age indications |
|---|---|---|---|---|---|
| Plasma-derived C1-INH* | Berinert | Replaces C1-INH | Acute treatment | 20 U/kg IV as needed | Children, adolescents and adults |
| Cinryze | Replaces C1-INH | Long-term prophylaxis | 1000 U IV every 3–4 d | Adolescents and adults | |
| Haegarda | Replaces C1-INH | Long-term prophylaxis | 60 U/kg body weight twice weekly (every 3–4 d) | Adolescents and adults | |
| Icatibant | Firazyr | Synthetic selective and specific antagonist of bradykinin 2 receptor | Acute treatment | 30 mg SC injection as needed; dose-adjusted for adolescents < 65 kg and children ≥ 2 yr† | Children, adolescents and adults |
| Lanadelumab | Takhzyro | Fully human monoclonal antibody that binds plasma kallikrein and inhibits its proteolytic activity | Long-term prophylaxis | 300 mg SC injection every 2 wk A dosing interval of 300 mg every 4 wk may be considered if the patient is well controlled (e.g., attack free) for more than 6 mo | Adolescents and adults |
Note: IV = intravenous, SC = subcutaneous.
↵* Treatment of choice during pregnancy, delivery and breast-feeding.
↵† 12 kg to 25 kg: 10 mg (1.0 mL); 26 kg to 40 kg: 15 mg (1.5 mL); 41 kg to 50 kg: 20 mg (2.0 mL); 51 kg to 65 kg: 25 mg (2.5 mL); > 65 kg: 30 mg (3.0 mL).
Adapted from: Betschel S, Badiou J, Binkley K, et al. The International/Canadian Hereditary Angioedema guideline. Allergy Asthma Clin Immunol 2019;15:72.