Berinert: 7 things you should know
Medically reviewed by Carmen Fookes, BPharm. Last updated on Feb 27, 2023.
1. How it works
- Berinert is a brand (trade) name for a man-made form of a protein called C1 esterase inhibitor, which naturally occurs in our plasma. Berinert may be used to treat attacks of hereditary angioedema (HAE) in adults and children. HAE is a rare genetic condition that occurs in people with low levels of certain proteins (C1-INH and C4) in their bodies.
- The C1 esterase inhibitor protein helps regulate several cascade systems within our body, such as the complement system, the intrinsic coagulation system and the coagulation cascade (the process that causes our blood to clot), and the fibrinolytic system (the process that removes and degrades clots after blood vessels are repaired). It also helps control bradykinin production (bradykinin is a protein fragment that increases the movement of fluid through blood vessel walls, resulting in swelling and inflammation).
- People with hereditary angioedema (HAE) have low levels of naturally occurring C1-INH in their blood. During an attack, these levels of C1-INH become so low that angioedema (severe swelling in the limbs, face, intestinal tract, and airway) occurs. Berinert adds working C1-INH, which brings levels of C1 back up and closer to normal, reducing bradykinin production, decreasing the permeability of blood vessels, and reducing swelling.
- Berinert belongs to the class of medicines known as C1 esterase inhibitors. It may also be called a hereditary angioedema agent
- May be used to treat acute abdominal, facial, or laryngeal hereditary angioedema (HAE) attacks in adults and children.
- Reduces swelling in the limbs, face, intestinal tract, and airway.
- The recommended dosage is 20 units per kg body weight by intravenous injection. Do not use dosages lower than this.
- May be used as often as needed to treat attacks. Not recommended as a preventive treatment; other treatments such as Haegarda are preventive treatments.
- 95% of Berinert infusions are self-administered at home or in other non-healthcare settings. An infusion takes about 7 or 8 minutes to self-administer. Do not try to self-administer without the appropriate training and approval from your health professional.
- Berinert may also be administered by caregivers or health professionals.
- Has a similar safety profile in children as in adults.
- Can be kept in the refrigerator or at room temperature (store it between 2-30°C [36-86°F]). Protect from light. Do not freeze.
- Available in a 500 IU vial for reconstitution with 10mL of sterile water for injection.
If you are between the ages of 18 and 60, take no other medication or have no other medical conditions, side effects you are more likely to experience include:
- An increase in the severity of pain associated with HAE is the most serious side effect reported with Berinert. Dysgeusia (experiencing a sour, sweet, bitter, or metallic taste), and injection site reactions have also been reported in more than 4% of people. Other side effects, such as headache, rash, and nasopharyngitis, are uncommon.
- Thromboembolic events, such as basilar artery thrombosis, multiple pulmonary microemboli, and thrombosis have been reported postmarketing. Thrombosis has been reported in people receiving dosages greater than 20 IU/kg when used for unapproved indications in newborns and in young children with congenital heart anomalies before or after cardiac surgery.
- Severe hypersensitivity reactions (severe allergies) have been reported and epinephrine should be available for immediate treatment if a reaction occurs. Signs of a hypersensitivity reaction include hives, rash, fast heartbeat, difficulty breathing (wheezing), chest tightness, feeling light-headed or faint, and swelling of your face, lips, tongue, or throat that occur during or soon after the administration of Berinert. Note that hypersensitivity reactions may have similar symptoms to HAE attacks. If a hypersensitivity reaction occurs, discontinue Berinert and administer appropriate treatment, such as epinephrine, if necessary.
- Serious thromboembolic events (blood clots) have been reported with C1 esterase inhibitor products, such as Berinert, The risk is greater in those with an indwelling catheter, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, or immobility. Weigh up the benefits of using Berinert in these people versus the risk of an event.
- Because Berinert is made from human blood there is a risk of transmitting infectious agents, such as viruses, and theoretically, the Creutzfeldt-Jakob disease vector.
- People self-administering Berinert for acute laryngeal HAE attacks require follow-up medical attention in an appropriate healthcare facility because of the potential for airway obstruction.
- Requires reconstitution before self-administration. The dosage of Berinert is based on body weight, so it is individualized for each person.
- Not suitable for people who have developed life-threatening, immediate hypersensitivity reactions, including anaphylaxis, to Berinert or any similar products.
- There is also the potential for immunogenicity and antibody formation.
- People can be taught how to self-administer Berinert.
- Not to be used for the prevention of HAE. Other medications, such as Haegarda, do this.
- There is a lack of data regarding the use of Berinert in pregnant women and it should be given only if needed. In a retrospective case series of 20 pregnant women, aged 20 to 35 years, who received repeated doses of up to 3,500 IU per HAE attack, no complications during delivery nor harmful effects on the 34 neonates were reported. It is not known if Berinert is excreted into human milk.
- No generic is available.
Note: In general, seniors or children, people with certain medical conditions (such as liver or kidney problems, heart disease, diabetes, seizures) or people who take other medications are more at risk of developing a wider range of side effects. View complete list of side effects
4. Bottom Line
- Berinert is used to treat hereditary angioedema (HAE) in adults and children and people can be taught how to self-administer it intravenously at home. An increase in the severity of pain associated with HAE, taste disturbances, and injection site reactions are the most common side effects reported. 62.8% of people experienced an onset of symptom relief within 1 hour of Berinert administration.
- If you want to learn how to self-administer Berinert, ask your doctor or nurse to give you a demonstration before you try doing it yourself. Do not try to self-administer it without the appropriate training and approval from your health professional.
- To self-administer Berinert, take the vial of Berinert and the sterile water vial out of the box and check the expiry date. Do not use if the protective caps are missing or the vials are out of date. Wash and dry your hands and clean your work surfaces. Put on gloves if you have been told to do so. Remove the flip caps, wipe them with an alcohol swab, and allow them to dry before opening the Mix2 vial transfer kit and pushing the blue spike of the transfer kit into the vial of sterile water.
- Remove the clear packaging from the other end and push the other spike of the transparent adapter through the center of the vial of Berinert. The water will automatically transfer into the vial of Berinert. With everything still attached, gently swirl the Berinert vial to mix the contents. Make sure the powder dissolves completely. Disconnect the vials and take the transfer set off the top of the Berinert vial. Using the syringe provided, draw air into the syringe and inject this into the Berinert vial. While the syringe is still attached to the vial turn it upside down and draw the concentrate into the syringe by gently drawing back on the plunger. If you are not using the solution immediately, it may be stored at room temperature for up to 8 hours. Do not put it in the refrigerator.
- Unscrew the syringe containing the reconstituted Berinert from the vial and attach an infusion set to it. Make sure the butterfly clip is open and gently push down on the plunger to fill the tubing with Berinert. Apply a tourniquet to your arm above the infusion site. Clean the skin above the vein in your inner elbow with an alcohol wipe. Allow the site to dry. Take the needle sheath off the infusion kit. Hold both ends of the butterfly between the thumb and the index finger with the slanted opening of the needle pointed upwards. Insert the butterfly needle into your vein, and insert it as flat to your forearm as possible. Using sterile tape or a transparent dressing, hold the needle in place.
- Make sure the needle is in a vein by gently pulling back on the plunger and checking to see if blood comes into the tubing. If blood is present, the needle is in the vein. If no blood is present, remove the needle and try inserting it into the vein again. Once the needle is in a vein, remove the tourniquet and inject the Berinert at a rate of 4ml per minute. After all the Berinert has been administered, remove the butterfly needle. Hold pressure on the site with a piece of cotton wool. Dispose of all the injection equipment in an appropriate sharps bin. Record the details of your infusion in your Berinert Journal. An infusion takes about 7 or 8 minutes to self-administer.
- Your doctor should discuss with you what to do if you have any signs of an allergic reaction while you are administering Berinert or soon after, for example, stopping the infusion if it is still running and administering epinephrine. Signs of an allergic reaction may include hives, rash, fast heartbeat, difficulty breathing (wheezing), chest tightness, feeling light-headed or faint, and swelling of your face, lips, tongue, or throat.
- Report any signs of a blood clot, such as swelling, pain, discoloration, a feeling of warmth, in an arm or leg, shortness of breath, chest pain or discomfort that worsens on deep breathing, numbness or weakness on one side of the body.
- Because Berinert is a blood product, it does carry a risk for transmission of infectious agents such as viruses, but this is unlikely because the risk of transmitting disease has been reduced by carefully selecting blood donors, testing donors for infections, and inactivating viruses during the manufacturing process.
- Talk to your healthcare provider about the risk of taking this drug during pregnancy or during breastfeeding especially if you are considering trying to become pregnant. Tell your doctor if you inadvertently become pregnant. There is limited information about the use of Berinert in pregnant women.
6. Response and effectiveness
- Studies have indicated Berinert starts working in 20% of people within 15 minutes, in approximately 45% of people by 30 minutes, and in approximately 70% of people in under 1.5 hours. 62.8% of people experienced an onset of symptom relief within 1 hour of Berinert administration.
- Nobody reported a worsening of HAE symptoms between 2 and 4 hours of Berinert administration compared to 28.6% of those receiving a placebo.
- The average number of vomiting episodes was 6 within 4 hours of the start of Berinert treatment compared to 35 episodes with a placebo.
- 30.2% of people receiving Berinert also received analgesics or antiemetics during their HAE attack compared with 54.8% of those receiving a placebo.
- The plasma concentration of C1 esterase inhibitor in healthy volunteers is approximately 270 mg/L7.
Medicines that interact with Berinert may either decrease its effect, affect how long it works, increase side effects, or have less of an effect when taken with Berinert. An interaction between two medications does not always mean that you must stop taking one of the medications; however, sometimes it does. Speak to your doctor about how drug interactions should be managed.
Common medications that may interact with Berinert include:
- anti-cancer medications such as carfilzomib
- evening primrose oil
- hormones, such as estrogen, estradiol, nandrolone, or testosterone
- thrombolytics such as alteplase, anistreplase, or urokinase.
Note that this list is not all-inclusive and includes only common medications that may interact with Berinert. You should refer to the prescribing information for Berinert for a complete list of interactions.
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- Drug class: hereditary angioedema agents
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Related treatment guides
- Berinert (human c1-esterase inhibitor) Updated 09/2021. CSL Behring https://www.drugs.com/pro/berinert.html
Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use Berinert only for the indication prescribed.
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.
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