Complement C1 esterase inhibitor
Medically reviewed on January 10, 2018
What is complement C1 esterase inhibitor?
Complement C1 esterase inhibitor is a man-made form of a protein that occurs naturally in the blood stream and helps control swelling in the body. People with a condition called hereditary angioedema do not have enough of this protein. Hereditary angioedema can cause attacks of swelling and symptoms such as stomach problems or trouble breathing.
Complement C1 esterase inhibitor is used in people with hereditary angioedema. The Berinert brand of this medicine is used to treat attacks of angioedema. The Cinryze and Haegarda brands are used to prevent attacks of angioedema.
Complement C1 esterase inhibitor may also be used for purposes not listed in this medication guide.
You should not use this medicine if have ever had a life-threatening allergic reaction to complement C1 esterase inhibitor.
Before taking this medicine
You should not use this medicine if you have ever had a life-threatening allergic reaction to complement C1 esterase inhibitor.
If possible before you receive complement C1 esterase inhibitor, tell your doctor if you have ever had:
a stroke or blood clot; or
an allergy to rabbits or rabbit products (meat, fur, pelt).
It is not known whether this medicine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.
It is not known whether this medicine passes into breast milk or if it could affect the nursing baby. Tell your doctor if you are breast-feeding.
Complement C1 esterase inhibitor is made from human plasma (part of the blood) which may contain viruses and other infectious agents. Donated plasma is tested and treated to reduce the risk of it containing infectious agents, but there is still a small possibility it could transmit disease. Talk with your doctor about the risks and benefits of using this medication.
Do not give this medicine to a child without medical advice.
How should I use complement C1 esterase inhibitor?
Follow all directions on your prescription label. Do not use this medicine in larger or smaller amounts or for longer than recommended.
The Cinryze and Berinert brands of this medicine are injected into a vein through an IV. Haegarda is injected under the skin.
You may be shown how to use injections at home. Do not give yourself this medicine if you do not understand how to use the injection and properly dispose of needles, IV tubing, and other items used.
Cinryze or Haegarda are usually given every 3 or 4 days to prevent angioedema attacks.
Read all patient information, medication guides, and instruction sheets provided to you. Ask your doctor or pharmacist if you have any questions.
Complement C1 esterase inhibitor is a powder medicine that must be mixed with a liquid (diluent) before using it. If you are using Cinryze home, you will need to use two vials of Cinryze to make up a single dose. Follow all directions carefully. Be sure you understand how to properly mix and store the medicine.
Gently swirl but do not shake the mixed medicine or you may ruin it. Prepare your dose only when you are ready to give yourself an injection.
The powder and diluent mixture should be clear and colorless. Mixed Cinryze should be clear or slightly blue in color. Do not use the mixed medicine if it has changed colors or has particles in it. Call your pharmacist for new medicine.
Each single-use vial (bottle) of this medicine is for one use only. Throw it away after one use, even if there is still medicine left inside.
Complement C1 esterase inhibitor contains no preservative. Once you have pierced the rubber top of a vial with a needle, you must use that vial right away or throw it away.
Store the unmixed dry powder in a refrigerator or at cool room temperature. Protect from light and do not freeze. Throw away any unused vial after the expiration date on the label has passed.
After mixing Berinert or Haegarda with the diluent, use the injection right away, or store the mixture at room temperature and use it within 8 hours.
After mixing Cinryze with the diluent, use the injection right away or store the mixture at room temperature and use it within 3 hours.
Use a disposable needle and syringe only once. Follow any state or local laws about throwing away used needles and syringes. Use a puncture-proof "sharps" disposal container (ask your pharmacist where to get one and how to throw it away). Keep this container out of the reach of children and pets.
What happens if I miss a dose?
Call your doctor for instructions if you miss a dose of Berinert or Haegarda.
What happens if I overdose?
Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.
What should I avoid after using complement C1 esterase inhibitor?
Follow your doctor's instructions about any restrictions on food, beverages, or activity.
Complement C1 esterase inhibitor side effects
Stop your IV infusion and get emergency medical help if you have signs of an allergic reaction: hives; chest tightness, fast heartbeats; wheezing or difficult breathing, blue lips or gums; feeling light-headed; or swelling of your face, lips, tongue, or throat.
Call your doctor at once if you have:
sudden numbness or weakness on one side of the body;
pain, swelling, warmth, or redness in an arm or leg;
sudden severe headache, confusion, problems with vision, speech, or balance;
chest pain while taking deep breaths; or
Common side effects may include:
altered sense of taste;
runny or stuffy nose, sneezing, watery eyes;
This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
See also: Side effects (in more detail)
Complement C1 esterase inhibitor dosing information
Usual Adult Dose for Hereditary Angioedema:
For routine prophylaxis against angioedema attacks in HAE patients.
Cinryze: 1,000 Units by intravenous infusion at a rate of 1 mL per minute (1,000 units/ 10 mL) every 3 or 4 days.
For treatment of acute abdominal, facial or laryngeal attacks of HAE:
Berinert: 20 units per kg body weight intravenously at a rate of approximately 4 mL per minute (500 units/10 mL).
Usual Pediatric Dose for Hereditary Angioedema:
For treatment of acute abdominal, facial or laryngeal attacks of HAE:
13 years and older:
Berinert: 20 units per kg body weight intravenously at a rate of approximately 4 mL per minute (500 units/10 mL)
What other drugs will affect complement C1 esterase inhibitor?
Other drugs may interact with complement C1 esterase inhibitor, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all your current medicines and any medicine you start or stop using.
Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.
Copyright 1996-2018 Cerner Multum, Inc. Version: 5.01.
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