complement C1 esterase inhibitor
Generic Name: complement C1 esterase inhibitor (KOM ple ment C1 ES ter ase in HIB it or)
Brand Name: Berinert, Cinryze, Ruconest (obsolete)
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 brand is used to prevent attacks of angioedema.
Complement C1 esterase inhibitor may also be used for purposes not listed in this medication guide.
What is the most important information I should know about complement C1 esterase inhibitor?
You should not use this medicine if have ever had a life-threatening allergic reaction to complement C1 esterase inhibitor.
What should I discuss with my health care provider before using complement C1 esterase inhibitor?
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:
a history of stroke or blood clot; or
if you are allergic 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 complement C1 esterase inhibitor passes into breast milk or if it could harm a nursing baby. Tell your doctor if you are breast-feeding a baby.
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.
Complement C1 esterase inhibitor is injected into a vein through an IV. A healthcare provider will give you a Berinert in a clinic or hospital setting. You may be shown how to use Cinryze in an IV at home. Do not self-inject this medicine if you do not fully understand how to give the injection and properly dispose of used needles, IV tubing, and other items used to inject the medicine.
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.
Mixed Berinert 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, is cloudy, or has any particles in it. Call your doctor for a new prescription.
Cinryze is usually given every 3 or 4 days to prevent angioedema attacks. An IV infusion takes about 10 minutes to complete.
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.
Each single use vial (bottle) of this medicine is for one use only. Throw away after one use, even if there is still some medicine left in it after injecting your dose.
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.
Store the unmixed dry powder in a refrigerator or in a dark cool place. Keep the medicine protected from light and do not allow it to freeze. Throw away any unused vial after the expiration date on the label has passed.
After mixing Berinert with the diluent, use it right away or store the mixture at room temperature and use it within 8 hours.
After mixing Cinryze with the diluent, use it right away or store the mixture at room temperature and use it within 3 hours.
What happens if I miss a dose?
Use the missed dose as soon as you remember. Skip the missed dose if it is almost time for your next scheduled dose. Do not use extra medicine to make up the missed dose.
Since Berinert is used when needed, it does not have a daily dosing schedule. Call your doctor promptly if your symptoms do not improve after using this medicine.
What happens if I overdose?
Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.
Overdose symptoms may include sudden headache, numbness, weakness, or problems with your speech, vision, or balance.
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 any of these signs of an allergic reaction: hives; wheezing or difficult breathing; feeling like you might pass out; or swelling of your face, lips, tongue, or throat.
Call your doctor at once if you have any other serious side effect, such as:
new or worsening pain;
sudden numbness or weakness, especially on one side of the body;
sudden severe headache, confusion, problems with vision, speech, or balance;
stomach pain or swelling;
chest pain, sudden cough, rapid breathing, fast heart rate; or
pain, swelling, warmth, or redness in one or both legs.
Common side effects may include:
altered sense of taste;
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 each of your health care providers about all medicines you use now and any medicine you start or stop using.
More about C1 esterase inhibitor (human)
- C1 esterase inhibitor (human)
- C1 esterase inhibitor (recombinant)
- C1 inhibitor (human)
- Complement C1 esterase inhibitor
- C1 esterase inhibitor recombinant Intravenous (Advanced Reading)
- C1 esterase inhibitor, human Intravenous (Advanced Reading)
Related treatment guides
Where can I get more information?
- Your doctor or pharmacist can provide more information about complement C1 esterase inhibitor.
- 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.
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