11 Hereditary Angioedema Treatments: Berinert, Orladeyo, and More
Hereditary angioedema is a rare genetic disorder. Hereditary angioedema treatment may involve C1-INH replacement therapy and medications for related symptoms.
Berinert
Used for Hereditary Angioedema
Used for Hereditary Angioedema
Berinert is a medication injected intravenously (into the vein) to treat swelling and painful attacks in children and adults with hereditary angioedema (HAE). Berinert can be given at a healthcare facility, or at home if you or your caregiver are properly trained.
What is Berinert?
What is Berinert used for?
- Treatment of sudden hereditary angioedema (HAE) attacks that occur in the stomach, face, or throat in children and adults
How Berinert works
Berinert is a replacement for C1 esterase inhibitor, a protein in your blood that helps relieve swelling (inflammation). Hereditary angioedema (HAE) is a swelling condition caused by low levels of C1 esterase inhibitor. By raising and restoring the levels of C1 esterase inhibitor in the blood, Berinert can treat symptoms of sudden HAE attacks.
Drug facts
| Common Brands | Berinert |
|---|---|
| Drug Class | C1 esterase inhibitor replacement |
| Controlled Substance Classification | Not a controlled medication |
| Generic Status | No lower-cost generic available |
| Availability | Prescription only |
Side effects of Berinert
The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.
Common Side Effects
- Headache (4-7%)
- Change in taste (5%)
- Stomach pain or discomfort (2-5%)
- Rash (4%)
- Vaginal yeast infection (up to 4%)
- Nose or throat infection (2-4%)
- Flu-like symptoms (2-4%)
Less Common Side Effects
- Nausea
- Upper respiratory tract infection (e.g., cough, common cold)
Berinert serious side effects
Contact your healthcare provider immediately if you experience any of the following.
- Severe allergic reaction: hives, rash, chest tightness, difficulty breathing, wheezing, fast heartbeat, faintness, swelling of the tongue or throat
- Blood clot: pain, swelling, warmth, or discoloration of the arm or leg, unexplained shortness of breath, chest pain or discomfort, unexplained fast heartbeat, numbness or weakness on one side of the body
The following Berinert side effects have also been reported
Side effects that you should report to your care team as soon as possible:
- Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
- Blood clot—pain, swelling, or warmth in the leg, shortness of breath, chest pain
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
Pros and cons of Berinert
Pros
- Can be given at home if properly trained by a healthcare provider
- Can be used in children and adults
- Starts working fast to relieve HAE symptoms (within about 1 hour)
- Vials aren't made with latex, so don't have to worry if you have a latex allergy
Cons
- Has to be given by injection, which can be uncomfortable
- Must be mixed before use
- If given at home, need to contact provider each time medication is used
- Only available as a brand medication, so it might be expensive
Pharmacist tips for Berinert
- Don't use Berinert at home until you or your caregiver has been trained by a healthcare provider to identify symptoms of HAE attacks and are able to give the injection properly.
- Review the instructions on how to prepare and give the injections.
- Make sure the medication and the administration kit are not expired, damaged, or opened, and that the reconstituted (mixed) solution is clear and free of particles. Don't use the Berinert solution if it's cloudy, discolored, or contains particles.
- Use the reconstituted solution within 8 hours, because it doesn't have any preservatives to keep it sterile (free of bacteria or other microorganisms) for a long period of time. Each Berinert vial is only meant to be used once for this reason, so throw it away after you're done mixing the solution. Don't refrigerate or freeze the mixed solution.
- Record the lot number from the Berinert vial every time you use this medication, along with the date and time of the infusion. This will help your provider see how often you're using the medication. This is also helpful information for your provider in case you report any infections or allergic reactions to them.
- Make sure that you or your caregiver know how to use epinephrine (EpiPen) in case of an allergic reaction from Berinert. Talk to your provider if you're confused about the symptoms of your HAE attacks compared to an allergic reaction to this medication.
- Stop using Berinert and let your provider know right away if you experience an allergic reaction or signs of a blood clot (pain or swelling in an arm or leg, shortness of breath, chest pain, fast heart rate, numbness or weakness on one side of your body).
- Let your provider know if you're pregnant, planning to become pregnant, or breastfeeding while taking Berinert to discuss if this medication is safe for you and your baby.
- Before traveling, make sure you talk to your provider to get enough medication to last you through your trip.
Risks and warnings for Berinert
Berinert can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Severe allergic reaction
Berinert can cause severe allergic reactions, including anaphylaxis (closing of the throat), in some people. Your provider might prescribe epinephrine (Epipen) for you to have during your injection of this medication. Symptoms of an allergic reaction can be similar to those from an HAE attack, so talk with your provider about how to tell the difference. Get emergency medical help if you experience hives, chest tightness, difficulty breathing, wheezing, lightheadedness, fast heartbeat, or swelling of the tongue or throat.
Blood clots
- Risk factors: Current catheter or access device | History of blood clots | Buildup of fats in arteries | Taking oral contraceptives (birth control) or androgens | Obesity | Not mobile
Blood clots have occurred in people who take Berinert, even at the recommended dose. Let your provider know if you have any of the risk factors mentioned above, and they will determine if Berinert is right for you. Get emergency medical help if you experience any signs of blood clots, such as pain, swelling, warmth, or discoloration of the arm or leg, unexplained shortness of breath, chest pain or discomfort, unexplained fast heartbeat, or numbness or weakness on one side of the body.
Transmission of diseases
Although rare, Berinert has a risk of transmitting (spreading) certain viruses and diseases to you, because this medication is made from human blood. Before Berinert is made, blood donors are screened, the blood is tested for infections, and the blood product is processed to remove or inactivate certain viruses. Even though all these steps are taken to lower this risk, there is still a small risk of transmitting diseases. If you have concerns, speak to your provider.
Breathing problems due to HAE attack
Sudden HAE attacks can sometimes cause swelling in the throat and affect the airways. If you experience breathing problems during your HAE attacks, get medical help right after your Berinert treatment if you're administering it yourself. The healthcare team will be able to treat and monitor you appropriately.
Berinert dosage
Typical dosage for Berinert
Dosing will depend on your body weight. The typical dose is 20 IU/kg of body weight per HAE attack, injected into the vein at a speed of 4 mL/minute. If you're giving yourself Berinert at home, call your provider or get medical attention after your dose for further instructions.
*IU=international units
What are alternatives to Berinert?
There are a number of medications that your doctor can prescribe in place of Berinert. Compare a few possible alternatives below.
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