• Berinert is a man-made form of a protein called C1 esterase inhibitor (C1-INH), that naturally occurs in our blood.
• Berinert is injected intravenously (into a vein) to treat acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adults and children.
• Berinert is the only C1-INH that is approved for self-administration for acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adults and children.
• 95% of Berinert infusions are self-administered at home or in other non-health care 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. It may also be administered by caregivers or health professionals.

Berinert is a brand of complement C1 esterase inhibitor (C1-INH). C1-INH is a man-made form of a protein in blood that helps control swelling in the body and is used to treat people with a condition called hereditary angioedema who lack C1-INH.

Hereditary angioedema is a genetic disorder that causes attacks of severe swelling in the limbs, face, intestinal tract, and airway.

What is Berinert?

Berinert is a man-made form of the C1-INH. The main purpose of C1-INH is to regulate the coagulation pathway (the process that causes our blood to clot), the fibrinolytic system (the process that removes and degrades clots after blood vessels are repaired), and to control the production of bradykinin, which is a protein fragment that increases the movement of fluid through blood vessel walls, resulting in swelling and inflammation.

How is Berinert given?

Berinert is given by intravenous infusion (slowly into the vein), as soon as possible in people experiencing an acute attack of HAE. It can either be given by a trained nurse or doctor or people (or their caregivers) can be taught how to self-administer it themselves. 95% of Berinert infusions are self-administered at home or in other non-health care settings. An infusion takes about 7 or 8 minutes to self-administer.

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 without the appropriate training and approval from your health professional. To self-administer Berinert:

1. 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.
2. 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.
3. 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.
4. With everything still attached, gently swirl the Berinert vial to mix the contents. Make sure the powder dissolves completely.
5. 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.
6. 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.
7. 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.
8. 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, insert as flat to your forearm as possible. Using sterile tape or a transparent dressing, hold the needle in place.
9. Make sure the needle is in a vein by gently pulling back on the plunger and check 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.
10. 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.
11. Record the details of your infusion in your Berinert Journal.

How does Berinert work?

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 is used to treat attacks of angioedema in people with HAE.