MANAGE C1-INH DEFICIENCY WITH HAEGARDA
HAEGARDA replaces missing or dysfunctional C1-INH
- Functional C1‑INH levels ≥40% of normal are proposed to reduce the risk of HAE attacks1
- HAEGARDA replaces missing or dysfunctional C1-INH, which regulates the normal production of bradykinin
What made me try HAEGARDA? It’s the same protein my body is lacking.

Subcutaneous C1-INH builds and maintains steady-state C1-INH functional activity2*
- Administered subcutaneously, HAEGARDA 60 IU/kg maintained steady-state C1-INH functional levels above 40%.2
- Steady state is expected within 3 to 4 doses2

* The plasma levels of C1-INH functional activity were evaluated in patients with type 1 or type 2 HAE in a Phase 3, placebo-controlled, crossover study. Patients received twice-weekly subcutaneous injections of HAEGARDA 40 IU/kg or 60 IU/kg for 16 weeks. The model-derived outcome is the steady-state C1-INH functional activity vs time.2
ROOT CAUSE OF HAE:
DEFICIENT OR DYSFUNCTIONAL C1-INH3
Missing or nonfunctioning C1-INH is the root cause of HAE4
- C1-INH inactivates factors Xlla, plasmin, and kallikrein, thus preventing bradykinin overproduction5
- Low-level of dysfunctional C1-INH allows bradykinin production to go unregulated6
- Excess bradykinin increases vascular permeability, resulting in angioedema4
- HAE with normal C1-INH levels and function is very rare; lack of pivotal clinical trials hinders ability to clearly establish diagnosis7
OVERVIEW OF HEREDITARY ANGIOEDEMA (HAE)
Epidemiology
- HAE is rare: affecting ~1 in 50K individuals. (1 in 10K – 1 in 150K worldwide)8
- HAE is an autosomal dominant genetic disorder resulting in deficiency (type I-85%) or dysfunction (type II-15%) of the C1-INH protein8
- C1-INH—a serine protease inhibitor—regulates the complement, contact, and coagulation cascades8
- Deficiency of functional C1-INH allows for uncontrolled activation of the contact system, resulting in increased vascular permeability and the classic symptoms of swelling8
Age of onset may vary
- Symptoms may occur at any age, but initial onset usually begins during childhood or adolescence (median = 6 years of age). Fifty percent of female and male patients are symptomatic at 12 and 13 years of age, respectively9
- HAEGARDA is now approved for adults and children 6 years and older
Symptoms
- Unpredictable angioedema attacks of swelling in various body parts10
- Debilitation due to extreme pain, vomiting, nausea, and, if the airway is affected, asphyxiation10
- Patients may experience symptoms for 10 or more years before HAE is identified11
I remember the first time I went into the emergency room and the doctor said, ‘You have HAE’ and…it was such a load removed from my shoulders because it was a doctor who knew what I had, and was able to treat it.
Common locations of swelling8

The World Allergy Organization (WAO) guidelines recommend the use of C1-INH for HAE for long-term prophylaxis.9

What Makes HAEGARDA Different
HAEGARDA is the HAE product that reduced HAE attacks by 95%.†
LEARN MORE† Median reduction in the number of attacks with HAEGARDA 60 IU/kg vs placebo.
THE WORLD ALLERGY ORGANIZATION GUIDELINES RECOMMENDATIONS9
C1-INH has been used for 35 years, and is the WAO-recommended first-line therapy
- The WAO recommends C1-INH as first-line preventive therapy for HAE for long-term prophylaxis4
- C1-INH is the preferred therapy for pregnant or breastfeeding HAE patients
FOR YOUR PATIENTS
To me, any therapy that replaces what you're actually missing and keeps it at a constant level, makes sense.
HAE TREATMENT GOALS FOR HCPs9
On-demand treatment goal‡
- Minimize the duration and severity of an attack that has started
- HAEGARDA is not approved for on-demand use
‡ Short-term or preprocedural prophylaxis can also be administered before an event that may trigger an HAE attack (eg, medical or invasive dental procedure).
See On-demand Treatment of Acute HAE Attacks
LEARN MORELong-term prophylaxis (LTP) treatment goals
- Prevent attacks and reduce severity if they occur
- Minimize the patient's burden of disease
World Allergy Organization/European Academy of Allergy and Clinical Immunology
guidelines on prophylaxis
"We recommend that patients are evaluated for long-term prophylaxis at every visit. Disease burden and patient
preference should be taken into consideration. Evidence Grade D; strength of
recommendation strong, 100% agreement."