About HAE
What is hereditary angioedema (HAE)?
HAE is a rare, inherited disease that results in C1 esterase inhibitor (C1-INH) deficiency, the primary regulator of inflammation and vascular permeability. As a result of the deficiency, people living with HAE develop acute, recurrent episodes of localized edema, which may occur in the extremities, abdomen, face, and throat, and which can be fatal if not properly treated.1
Learn about diagnosing HAE
Learn about diagnosing HAE
How can I differentiate between HAE and conditions that present with similar symptoms?
HAE is often misdiagnosed because the symptoms mimic those of several common conditions, including allergic angioedema.1
It’s important to determine the difference between GI problems, allergic angioedema, and HAE because the pathophysiology and the treatment of the diseases are different.
HAE can be confirmed with blood tests to evaluate C4 serum concentration and C1-INH function.2
Learn 8 simple questions you can ask your patients to help determine if further testing is needed
It’s important to determine the difference between GI problems, allergic angioedema, and HAE because the pathophysiology and the treatment of the diseases are different.
HAE can be confirmed with blood tests to evaluate C4 serum concentration and C1-INH function.2
Learn 8 simple questions you can ask your patients to help determine if further testing is needed
About BERINERT
Which types of HAE does BERINERT treat?
BERINERT treats the two most common types of HAE (Type I and Type II).
Type I: The body does not produce enough C1 esterase inhibitor (C1-INH). Approximately 85% of people living with HAE have type I.1
Type II: The body produces C1-INH that does not function correctly. Approximately 15% of people living with HAE have type II.1
BERINERT has not been studied in HAE patients with normal C1-INH. This is a very rare form of HAE that occurs despite normal C1-INH levels and/or function.
Explore using C1-INH therapy to address C1-INH deficiency
Type I: The body does not produce enough C1 esterase inhibitor (C1-INH). Approximately 85% of people living with HAE have type I.1
Type II: The body produces C1-INH that does not function correctly. Approximately 15% of people living with HAE have type II.1
BERINERT has not been studied in HAE patients with normal C1-INH. This is a very rare form of HAE that occurs despite normal C1-INH levels and/or function.
Explore using C1-INH therapy to address C1-INH deficiency
How does BERINERT treat HAE?
Patients with HAE experience angioedema attacks because they lack functioning C1 esterase inhibitor (C1-INH).1 BERINERT addresses the root cause of HAE attacks by replacing missing or dysfunctional C1-INH to stop attacks.
BERINERT is the only C1-INH approved for on-demand treatment of acute abdominal, facial, or laryngeal attacks of HAE in adults and pediatrics.
BERINERT is an intravenous infusion that patients can self-administer after proper training by a healthcare professional.
Learn more about how C1-INH therapy addresses the root cause of HAE attacks
BERINERT is the only C1-INH approved for on-demand treatment of acute abdominal, facial, or laryngeal attacks of HAE in adults and pediatrics.
BERINERT is an intravenous infusion that patients can self-administer after proper training by a healthcare professional.
Learn more about how C1-INH therapy addresses the root cause of HAE attacks
How fast does BERINERT provide relief?
BERINERT provides fast onset of relief from HAE attacks in as little as:
Explore fast relief from HAE attacks with BERINERT
- 15 minutes for laryngeal attacks*
- 48 minutes for abdominal/facial attacks†
*
In an open-label study, onset of symptom relief was measured in 57 HAE patients (10–53 years of age) who had previously participated in the IMPACT 1 placebo-controlled clinical study following a single dose of BERINERT 20 IU/kg. Additional doses of C1-INH concentration were given for some attacks.
†
In a randomized, double‑blind, placebo-controlled clinical trial, onset of relief was experienced within 4 hours by 69.8% of BERINERT treatment group and 42.9% of placebo group. For subjects who did not respond within that time frame, study design allowed use of rescue medications as follows: placebo for 20 IU/kg body weight BERINERT treatment group and 20 IU/kg body weight BERINERT for placebo group.
Explore fast relief from HAE attacks with BERINERT
How does on-demand treatment benefit my patients?
On-demand treatment helps patients take control of their HAE by treating at the first sign of an HAE attack, which can occur suddenly, frequently, and without warning.
Because attacks can occur even in patients on prophylactic HAE therapy, the WAO Guidelines for the Management of HAE state that patients should have on-demand medication available at all times.2
Learn about rapid C1-INH uptake with self-administered IV infusion
Because attacks can occur even in patients on prophylactic HAE therapy, the WAO Guidelines for the Management of HAE state that patients should have on-demand medication available at all times.2
Learn about rapid C1-INH uptake with self-administered IV infusion
Has the safety of BERINERT been evaluated in pediatric patients?
Yes. A US-European Registry Study (N=18, age range 5–11 years) demonstrated safety similar to that observed in adults.
Safety and efficacy were also evaluated in placebo-controlled and open-label extension studies (N=12, age range 10–16 years).
Learn more about BERINERT safety in adults and pediatrics
Learn more about BERINERT safety in adults and pediatrics
What were the adverse reactions in the clinical trials?
No serious adverse events or adverse events leading to discontinuation of treatment occurred within 4 hours after study treatment.3
Adverse reactions occurring up to 4 hours after initial infusion in more than 4% of subjects, irrespective of causality, included nausea, dysgeusia, and abdominal pain.*
*
The following abdominal symptoms were identified in the protocol as associated with HAE abdominal attacks: abdominal pain, bloating, cramps, nausea, vomiting, and diarrhea.
What is the manufacturing process for BERINERT?
BERINERT is made from human blood. BERINERT and the human plasma from which it’s made have been carefully processed and screened to remove or inactivate known viruses. Because BERINERT is made from human blood, it may carry a risk of transmitting infectious agents, e.g., viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
Dosing and self-administration
How is BERINERT administered?
BERINERT is given as an intravenous infusion that, with training, can be self-administered or infused by a caregiver.
Get more information about reconstituting and self-administering BERINERT
Get more information about reconstituting and self-administering BERINERT
What is the recommended BERINERT dosage?
BERINERT offers a weight-based approach to provide an individualized dose of C1-INH during an attack.
The recommended BERINERT dosage is 20 units (IU) per kilogram body weight, with a recommended infusion rate of 4 mL/minute. For example, a 70-kg patient would require 1400 IU, infused over 7 minutes.
Learn more about individualized, weight-based dosing
The recommended BERINERT dosage is 20 units (IU) per kilogram body weight, with a recommended infusion rate of 4 mL/minute. For example, a 70-kg patient would require 1400 IU, infused over 7 minutes.
Learn more about individualized, weight-based dosing
What is the benefit of room-temperature storage?
When stored at temperatures of 2-30°C (36-86°F), BERINERT is stable for the period indicated by the expiration date on the carton and vial label.
Learn about BERINERT dosing and self-administration
Learn about BERINERT dosing and self-administration
Support, resources, and billing
What is BERINERT ConnectSM?
BERINERT Connect is a comprehensive support network for healthcare professionals and patients who depend on BERINERT. BERINERT Connect provides access to a range of individualized support programs with a single phone call.
You or your patients can call BERINERT Connect at 1-877-236-4HAE (1-877-236-4423), Monday–Friday, 8 AM–8 PM ET.
Explore services and support that BERINERT Connect offers
You or your patients can call BERINERT Connect at 1-877-236-4HAE (1-877-236-4423), Monday–Friday, 8 AM–8 PM ET.
Explore services and support that BERINERT Connect offers
Is there financial assistance to help my patients afford BERINERT?
While living with HAE carries a heavy burden, there is help. The BERINERT Co-Pay Program* is a financial assistance program designed to help patients pay for eligible out-of-pocket expenses.
Discover services and support for patients
*
Subject to terms and conditions of the copay assistance program. To read the full terms and conditions,
visit cslbehring.com/patients/support-and-assistance.
CSL Behring reserves the right to rescind, revoke, or amend the program at any time without notice.
How do I start a patient on BERINERT?
Starting a new patient on BERINERT is an easy process, and our BERINERT Care Coordinators can guide you and your staff the entire way.
The first step is to download and fill out the BERINERT Prescription Referral Form. Fax the form to BERINERT Connect at 1-866-415-2162.
If you have any questions while completing the BERINERT Prescription Referral Form, please contact your local CSL Behring representative, or call BERINERT Connect at 1-877-236-4HAE (1-877-236-4423).
Learn more about the helpful services of BERINERT Connect
The first step is to download and fill out the BERINERT Prescription Referral Form. Fax the form to BERINERT Connect at 1-866-415-2162.
If you have any questions while completing the BERINERT Prescription Referral Form, please contact your local CSL Behring representative, or call BERINERT Connect at 1-877-236-4HAE (1-877-236-4423).
Learn more about the helpful services of BERINERT Connect
How do I order BERINERT?
CSL Behring is dedicated to making BERINERT available to patients and healthcare professionals when and where it is needed. BERINERT is available through a range of national and regional specialty pharmacies and distributors.
For contact information for these organizations, please call BERINERT Connect at 1-877-236-4HAE (1-877-236-4423).
Get reimbursement and coding information
For contact information for these organizations, please call BERINERT Connect at 1-877-236-4HAE (1-877-236-4423).
Get reimbursement and coding information
What is the CSL Behring commitment to people affected by HAE?
CSL Behring is a world leader in specialty biopharmaceuticals and is committed to people around the world with rare and serious conditions, such as HAE. We are dedicated to making a difference in the HAE community through innovation, quality manufacturing, and support and education for patients and families affected by HAE.
References: 1. Zuraw BL. Clinical practice: hereditary angioedema. N Engl J Med. 2008;359(10):1027-1036. 2. Maurer M, Magert M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema—the 2017 revision and update [published online ahead of print]. Allergy. 2017. doi:10.1111/all.13384. 3. Craig TJ, Levy RJ, Wasserman RL, et al. Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks. J Allergy Clin Immunol. 2009;129(4):801-808.