Frequently Asked Questions Questions about Berinert? See what other patients have asked and how their questions have been answered.

Hereditary Angioedema FAQ

What is hereditary angioedema (HAE)?

HAE is a rare disease; it affects only about 1 in 10,000 to 50,000 individuals. It is an inherited disease.

HAE can cause considerable swelling in specific parts of the body, including the abdomen, face, and throat.

Because HAE symptoms can resemble other conditions, such as allergies or stomach problems, diagnosis is often delayed. Delay of diagnosis is common, so it's important to talk to your doctor about HAE as a possible cause of your symptoms.

What causes HAE?

People living with HAE are either missing a blood protein called C1 esterase inhibitor (C1-INH), or have normal levels of C1-INH but it is dysfunctional. When the body's need for C1-INH overwhelms its ability to keep up with demand, a sudden attack can occur.

What are the symptoms of HAE?

HAE attacks can cause swelling, or edema, in specific parts of the body, including the abdomen, face, and throat. Attacks often begin in childhood, becoming more severe over time. The number of episodes an individual may experience is unpredictable. Some people experience weekly attacks, while others may go years without one.

During an attack, abdominal, facial, and laryngeal swelling often gets worse over a period of 12 hours to 24 hours, then resolves within 72 hours. Unlike allergic reactions, there is no itching or redness with HAE. In addition to abdominal swelling and pain, patients often experience nausea, which can be accompanied by vomiting.

Attacks of HAE in the throat are the most dangerous, because swelling that closes off the airway may lead to death. Abdominal attacks cause acute pain in the abdomen, and sometimes also cause nausea, vomiting, and diarrhea. Peripheral attacks, such as attacks in hands or feet, can result in impaired function. 11

How do doctors diagnose HAE?

Hereditary angioedema can take a long time to diagnose, because some doctors may not be aware of the condition and HAE symptoms may resemble other conditions, such as allergies or stomach problems.

A series of blood tests that measure levels of serum C4 and C1-INH proteins are needed to confirm the disease. The tests determine HAE type.

HAE episodes are usually asymmetrical and localized to a single area of the body; however, simultaneous or closely spaced attacks can also occur.5

Attacks can also be migratory, starting in one location and then spreading to another before resolving. Migratory attacks may spread from site to site over the course of a few days. 2, 5

How is HAE treated?

Acute attacks in the abdomen, face, or throat may be treated by taking a C1-INH concentrate, such as Berinert, which is used to treat attacks on demand. Berinert is approved for patient self-administration after proper training by a healthcare professional.

Berinert treats two common types of HAE:

  • Type I: The body doesn't produce enough C1-1NH. Approximately 85% of people living with HAE have this type
  • Type II: The body produces C1-INH that does not function correctly. Approximately 15% of people living with HAE have Type II.

There is also another, very rare form of HAE, recently renamed as HAE with normal C1-INH (aka Type III). Learn more about HAE Type III: Learn more about HAE Type III

Self-administration at the first sign of an attack with fast and reliable Berinert can help put you in control of your HAE and increase your independence

Living with HAE FAQ

What are the challenges of living with HAE?

With day-to-day demands, living with HAE can be challenging. An attack can throw off your schedule, make it difficult to balance a job and other daily responsibilities; or even worse, put you in the hospital.

HAE attacks are unpredictable, painful, and potentially life-threatening, which can place a large burden of stress on you and your caregivers. You may be worried about the next attack and reluctant to make plans.

Living with HAE means understanding how your specific symptoms trigger attacks and how you and your physician can best manage HAE.11

How can I take control of my HAE?

First, you can learn to recognize your triggers, so you can avoid them. The actual causes of hereditary angioedema attacks have not been established and seem to differ from person to person. However, people living with HAE often report that certain triggers can lead to attacks. These triggers may include:

  • Stress or sickness
  • Hormonal changes
  • Mild trauma
  • Any dental work or surgical procedures
  • Certain medications, such as oral contraceptives containing estrogen and blood pressure medications, such as ACE inhibitors

To help identify your triggers, try keeping a treatment journal to record details about your attacks, including location, frequency, duration, and potential triggers. Be sure to share your journal with your doctor. Once triggers are identified, it's important to avoid situations that may bring them on. If stress is a trigger, try to avoid highly charged situations. Ask friends and family members for help and support when you feel overwhelmed, and use coping strategies that work for you. If certain medications are a problem, talk to your HAE specialist about alternatives.

What can I do if an attack happens unexpectedly?

For many people living with hereditary angioedema, an attack is not preventable. In addition, studies have shown that some patients receiving prophylactic therapy can experience breakthrough attacks. The best option is to be well prepared when an attack occurs.

Educate family, loved ones, and coworkers about your condition and always pack your on-demand medication (eg, Berinert) and infusion supplies before you travel. For extra preparation, the Berinert Expert Network (B.E.N.®) or the HAEA can provide you with information regarding which hospitals currently stock Berinert.

Keep a diagnosis letter from your doctor with you at all times, as well as an emergency patient information card that communicates your medical needs—in the event that you're unable to relate important medical information.

Work with your doctor and your caregivers to develop an individualized action plan, so you'll know what to do when an attack happens. Make sure you carry enough Berinert with you at all times to treat up to 2 attacks.11

What are my treatment options?

A physician who is experienced in the treatment of HAE can help you determine the right treatment to manage your HAE. If you need help finding a physician experienced in the treatment of HAE in your area, contact the HAEA.

HAE treatment can be divided into 2 categories: prophylaxis and acute therapy.

In the case of an HAE patient, prophylaxis means to attempt to prevent HAE attacks. Prophylaxis treatment options include low-dose androgen therapy, antifibrinolytic agents, and C1-INH treatment indicated for prophylaxis.

However, some HAE patients find that prophylaxis therapy is not enough to keep their HAE under control. Breakthrough attacks can occur. That is why there is an acute therapy option for HAE patients. For acute therapy, Berinert is currently one of the treatment options available. Talk to your doctor about which treatment may be right for you.

Berinert FAQ

What is Berinert?

Berinert is the only C1 esterase inhibitor (C1-INH) approved for on-demand treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adults and children.

Berinert is different from prophylactic treatment—it is approved for on-demand patient self-administration at the first sign of an attack. Berinert is a safe and effective therapy that works when and where you need it. That means it should be used only when you have an acute attack–and not as part of a preventive regimen.

Can I administer Berinert to myself?

Yes, Berinert is the only on-demand C1-INH approved for self-administration after proper training by a healthcare professional. Self-administering Berinert simply allows you to treat your HAE at the first sign of an attack for fast relief.

With Berinert self-administration you can reduce the length of your HAE attacks. You also have the flexibility to treat your HAE attacks wherever you happen to be.

With proper training, patients can infuse Berinert on their own or with help from a family member or other caregiver. Following setup, administration takes about 7 to 8 minutes for an average-sized adult.

How do I know when to administer?

Sometimes, early HAE symptoms appear that signal the onset of an HAE attack. It is important to treat at the first sign of an HAE attack. The sooner you treat, the sooner you will feel relief from the symptoms.

How do I determine the correct dose?

Because each dose of Berinert is calculated based on your weight, you use just the right amount of medicine—individualized for YOUR body. Because you use Berinert only to treat your HAE attacks, you only use the RIGHT amount of medicine, WHEN you need it. Berinert uses weight-based dosing of 20 IU/kg body weight, which ensures you receive the correct dose of C1-INH during an attack. No dose adjustments are required for children. Dosing is based on weight for all patients on Berinert. Talk to your doctor about establishing your dosing regimen.

How quickly does Berinert work?

Berinert provides fast, sustained relief from HAE attacks. In clinical studies, about half of patients having an abdominal or facial attack experienced an onset of symptom relief in about 48 minutes with Berinert; it was more than four hours before half the patients in the placebo group experienced onset of symptom relief. In an extension study, about half experiencing a laryngeal attack felt an onset of relief in less than 15 minutes

Is Berinert safe?

Berinert is made from human blood plasma. Berinert and the human plasma from which it's made have been processed and screened to remove or inactivate known viruses. The screening steps include:

  • Pasteurization (heat treatment for 10 hours) in aqueous solution
  • Hydrophobic interaction chromatography
  • Virus nanofiltration

Berinert is highly purified. However, because Berinert is made from human plasma, the risk that it may transmit infectious agents cannot be completely eliminated.

The safety of Berinert was proven in both adults and children. The most serious adverse reaction reported in subjects who received Berinert in clinical studies was an increase in the severity of pain associated with HAE. Dysgeusia, a distortion of the sense of taste, was the most common adverse reaction reported in over 4% of subjects and more frequently than in the placebo group.

How is Berinert supplied?

Berinert doesn't require refrigeration and can be stored for up to 30 months, making it easy for you to keep it at home or carry with you.

Is support available if I need it?

Yes, there is reliable support available 24/7. The Berinert Expert Network (B.E.N.®) team can help arrange training for you through qualified specialty pharmacy providers, and will provide training materials as well. B.E.N. also gathers many resources in one place to provide assistance, information, and support—all with a single phone call (1-877-236-4423), including:

  • Assisting you in starting your Berinert therapy
  • Working through insurance questions and issues
  • Connecting you with the HAE community
  • Enrolling you in support programs for patients
  • Giving you an opportunity to share your experiences with your peers via the B.E.N. P.A.L.S. patient advocate program
  • Helping to reduce your out-of-pocket co-pay costs through the Co-Pay BENefit™ program
  • Providing peace of mind through CSL Behring's AssuranceSM and Assistance programs

Is CSL Behring committed to the HAE patient community?

CSL Behring is a world leader in specialty biopharmaceuticals and is committed to people around the world with rare and serious conditions, such as hereditary angioedema (HAE). We're dedicated to making a difference in the HAE community through education, services, and other resources for patients and families affected by the condition.

In addition to the B.E.N. resource network, we sponsor AllAboutHAE.com, an online HAE resource with useful information, such as an interactive treatment journal.

We are a proud supporter of the US Hereditary Angioedema Association, a nonprofit patient advocacy organization dedicated to expediting US approval of safer and more effective HAE therapies. The organization provides a wide range of services that include clinical trial placement, physician referrals, education, and individualized patient case management.

CSL Behring is also committed to those who are uninsured or underinsured, as well as those who experience a gap in coverage. To learn more about our assistance programs, please visit the Resources and Support section.

Starting Berinert FAQ

What are the first steps?

If your doctor suspects that you may have hereditary angioedema (HAE), he or she will order a series of blood tests, which is the only way HAE can be confirmed. Your doctor may then refer you to a specialist who knows about HAE, such as an allergist or immunologist.

If your doctor has trouble locating a specialist, you may want to contact the US Hereditary Angioedema Association. This organization maintains a robust list of doctors across the country who treat people living with HAE.

How do I know if Berinert is right for me?

If you are experiencing acute abdominal, facial, or laryngeal attacks of hereditary angioedema, you may be ready to explore treatment options or look for an alternative to what you are currently taking. Berinert is one therapy you may want to consider, but only you and your doctor can tell you if Berinert is right for you.

What should I discuss with my doctor?

  1. Ask your doctor how Berinert works.
  2. Ask your doctor about the intravenous infusion process and if self-administration is appropriate for you.
  3. Explain specifics about your attacks (such as frequency and location) and how you work to manage them so your doctor can determine which treatment option is right for you.
  4. Discuss other current HAE medications, including side effects, dosing, storage, and how effective they may be in relieving or preventing an attack.
  5. Tell your doctor about all of the medications you're taking, including over-the-counter medicines, vitamins, and herbal supplements.
  6. Download the HAE treatment plan to help facilitate this conversation.

How can I recognize when an HAE attack is starting?

HAE attacks may occur suddenly and without warning. However, many patients with HAE notice symptoms at the very early stage of an attack.

Some common signs of an HAE attack may include sudden mood changes, rash, irritability, aggressiveness, anxiety, extreme fatigue, or a tingling sensation of the skin where the swelling will begin.

Talk with your doctor to learn more about recognizing the first signs of an HAE attack and when to administer Berinert.

How can I learn to self-administer Berinert?

Just like hundreds of Berinert patients, with the proper training, you can infuse Berinert on your own or with help from a family member or other caregiver. One-on-one training from a nurse or other healthcare professional can help you develop confidence to safely and effectively self-administer Berinert at the first sign of an HAE attack.

Remember—never attempt to self-administer Berinert unless you've been taught to do so by a healthcare professional.

If you self-administer to treat a laryngeal attack, immediately seek medical attention afterward.

Berinert provides numerous self-administration training and practice materials to help patients become accustomed to the procedure. These materials are provided to the patient through a specialty-pharmacy training nurse. Speak with your doctor, or BEN to arrange a training session.

Let us take the hassle out of taking control of your HAE therapy. B.E.N. can assist you in starting or self-administering your Berinert therapy.

Support Services FAQ

What is the Berinert Co-Pay BENefit™?

Living with HAE can be a heavy burden to bear. But it shouldn't also be a financial burden. Help take control of your HAE attacks and the cost of treatment. The Berinert Co-Pay BENefit* is a financial assistance program designed to cover your eligible out-of-pocket co-pay expenses—up to $12,000 per year. Contact B.E.N. to enroll.

*The Berinert Co-Pay BENefit is available for new or existing Berinert patients. Patients must be 12 years of age or older, a U.S. resident, and insured in the U.S. Patients covered by state- or federally funded programs, such as Medicare, Medicaid, and Veterans Health Insurance, are NOT eligible.

What is the CSL Behring AssuranceSM program?

A gap in insurance shouldn't mean a gap in treatment. CSL Behring Assurance is designed to help ensure that people who rely on our therapies can continue to receive treatment even if they experience a lapse in third-party, private health insurance. Contact B.E.N. to enroll.

What is the Patient Assistance Program?

Periodically, patients with chronic illnesses are faced with difficult insurance situations that may prevent access to life-sustaining therapies. To help ensure that patients receive the treatment they need, the Patient Assistance program provides medically necessary therapy to qualified individuals who are uninsured, underinsured, or unable to afford their prescribed therapy. Contact B.E.N. to determine eligibility.

How can I connect with other HAE patients like me?

BEN can put you in touch with B.E.N. P.A.L.S.® (Peer Advocates for Learning and Support). Our B.E.N. P.A.L.S. are patients who have personal experience with hereditary angioedema (HAE) and Berinert. They offer you encouragement, assistance, and advice by:

  • Helping you better understand and manage your Berinert therapy
  • Sharing their own experiences with Berinert and life with HAE
  • Answering nonmedical questions*

*Peer supporters are not healthcare professionals or medical experts. For medical questions patients should contact their physicians.

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Important Safety Information

Berinert®, C1 Esterase Inhibitor (Human), is used in adults and children to treat swelling and/or painful attacks of hereditary angioedema (HAE) affecting the abdomen, face or throat. The safety and efficacy of Berinert in preventing HAE attacks have not been established.

Do not use Berinert if you have experienced life-threatening allergic reactions or severe hypersensitivity to the product. Inform your healthcare provider of all medications you are taking and of any medical conditions, especially any history of blood-clotting problems.

Blood clots have occurred in patients receiving Berinert. Tell your healthcare provider if you have a history of heart or blood vessel disease, stroke, or blood clots, or if you have thick blood, an indwelling catheter/access device in a vein, or have been immobile for some time. Certain medications, such as birth control pills, may also increase your risk of clotting problems.

Report to your physician or an emergency room any signs and symptoms of a blood clot, including pain and/or swelling or discoloration of an arm or leg, with warmth over affected area; unexplained shortness of breath; chest pain or discomfort that worsens on deep breathing; rapid pulse; and numbness or weakness on one side of the body.

In addition, report immediately any signs or symptoms of allergic reactions to Berinert, including hives, chest tightness, wheezing, difficulty breathing, turning blue, faintness, facial swelling and fast heartbeat.

If you have been trained to self-administer Berinert, immediately prepare the prescribed dose at the first symptoms of an attack. Seek immediate medical attention and do not begin to self-adminster if an HAE attack has progressed to a point where you will be unable to prepare or administer a dose of Berinert.

If you self-administer to treat a laryngeal attack, immediately seek medical attention afterward. If you self-administer for an abdominal attack, inform your physician so that other possible causes can be ruled out.

Call your doctor right away if swelling is not controlled after use of Berinert.

In clinical studies, the most serious adverse reaction reported in subjects who received Berinert was an increased severity of the pain associated with HAE. In the placebo-controlled clinical trial, the most common adverse reaction reported more often among subjects who received Berinert than those receiving placebo was dysgeusia (a bad taste in mouth). Tell your healthcare provider about any side effect that bothers you or does not go away.

Because Berinert is made from human blood, the risk that it may transmit infectious agents, including viruses and theoretically, the agents of Creutzfeldt-Jakob Disease (CJD) and its variant form (vCJD), cannot be completely eliminated.

Please see full prescribing information for Berinert, including the patient product information.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

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