Commonly Used Brand Name(s)Berinert, Cinryze
Therapeutic ClassificationsImmune Modulator
C1 esterase inhibitor is used to treat or prevent hereditary angioedema (HAE). HAE is a rare disease that causes swelling of the face, hands, feet, throat, stomach, bowels, or sexual organs. People who have HAE have low levels of C1 esterase inhibitor in their body, and this medicine increases the amount of C1 esterase inhibitor in the body.
This medicine is available only with your doctor's prescription.
A nurse or other trained health professional will give you or your child this medicine. This medicine is given through a needle placed in one of your veins.
This medicine may sometimes be given at home to patients who do not need to be in the hospital or clinic. If you are using this medicine at home, your doctor or nurse will teach you how to prepare and inject the medicine. Be sure that you understand how to use the medicine.
Make sure family members or other people you are with know how to inject the medicine in case you are unable to do it yourself during an HAE attack.
Use a new needle and syringe each time you inject your medicine. Also, use a silicone-free syringe when using this medicine.
Check the injection kits regularly to make sure that the powder or liquid has not changed its color. Do not use this medicine if the powder or liquid has changed its color, or if there are solids in the mixed liquid.
Carry this medicine with you at all times for emergency use in case you have an HAE attack.
This medicine comes with a patient information leaflet. Read and follow the instructions carefully. Ask your doctor if you have any questions.
The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.
The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.
- For hereditary angioedema:
- For injection dosage form:
- Adults and children—Dose is based on body weight and must be determined by a doctor. The usual dose is 20 International Units (IU) per kilogram (kg) of body weight injected into a vein.
- Adults and teenagers—1000 units injected into a vein every 3 or 4 days.
- Children younger than 12 years of age—Use and dose must be determined by your doctor.
- For injection dosage form:
Call your doctor or pharmacist for instructions.
Use & StorageTOP
Keep out of the reach of children.
Do not keep outdated medicine or medicine no longer needed.
Ask your healthcare professional how you should dispose of any medicine you do not use.
Store the injection kits at room temperature, away from heat, moisture, and direct light. Keep the medicine in the original carton until ready to use. You may store the powder vial in the refrigerator. Do not freeze.
Do not reuse the remaining portion of the medicine that is left in the vial. Throw away the vial after you have used it.
In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:
Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.
Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of Berinert® in children.
Appropriate studies have not been performed on the relationship of age to the effects of Cinryze® in children 12 years of age and younger. Safety and efficacy have not been established.
Appropriate studies have not been performed on the relationship of age to the effects of C1 esterase inhibitor in the geriatric population. Safety and efficacy have not been established.
There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.
Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.
Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.
Other Medical ProblemsTOP
The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:
- Atherosclerosis (blood vessel disease) or
- Blood clots, history of—Use with caution. May make this condition worse.
It is very important that your doctor check the progress of you or your child at regular visits. This will allow your doctor to see if the medicine is working properly and to decide if you should continue to take it.
HAE attacks are life-threatening and require immediate medical attention. Check with your doctor right away, or go to an emergency room as soon as possible, even if you feel better after using this medicine.
This medicine may cause serious allergic reactions. These reactions can be life-threatening and require immediate medical attention. Call your doctor or nurse right away if you or your child have a rash, itching, hives, hoarseness, chest tightness, lightheadedness, dizziness or fainting, trouble breathing, trouble swallowing, or any swelling of your hands, face, or mouth after you receive this medicine.
This medicine may increase your risk of developing blood clots. Check with your doctor right away if you or your child have swelling and pain in your arms, legs, or stomach, chest pain, shortness of breath, loss of sensation, confusion, or problems with muscle control or speech.
This medicine is made from donated human blood. Some human blood products have transmitted certain viruses to people who have received them. The risk of getting a virus from medicines made of human blood has been greatly reduced in recent years. This is the result of required testing of human donors for certain viruses, and testing during the manufacture of these medicines. Although the risk is low, talk with your doctor if you have concerns.