Commonly Used Brand Name(s)Berinert, Cinryze, Haegarda
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 genitals. People who have HAE have low levels of C1 esterase inhibitor in their body. This medicine helps increase the amount of C1 esterase inhibitors 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. It is given through a needle placed into one of your veins or as a shot under your skin.
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.
This medicine comes with a patient information leaflet. Read and follow the instructions carefully. Ask your doctor or pharmacist if you have any questions.
You will be shown the body areas where this shot can be given. Use a different body area each time you give yourself a shot. Keep track of where you give each shot to make sure you rotate body areas. This will help prevent skin problems.
Make sure family members or other people you are with know how to inject the medicine in case you are unable to do it by 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 it is discolored or if there are particles in the mixed liquid.
Carry this medicine with you at all times for emergency use in case you have an HAE attack.
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 children 12 years of age and older—1000 units (U) injected into a vein every 3 or 4 days. Your doctor may adjust your dose as needed. However, the dose is usually not more than 2500 U every 3 or 4 days.
- Children 6 to 11 years of age—500 U injected into a vein every 3 or 4 days. Your doctor may adjust your dose as needed. However, the dose is usually not more than 1000 U every 3 or 4 days.
- Children younger than 6 years of age—Use and dose must be determined by your doctor.
- Adults and children 12 years of age and older—Dose is based on body weight and must be determined by your doctor. The dose is usually 60 units per kilogram (kg) of body weight injected under the skin 2 times a week (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 also store the powder vial in the refrigerator. Do not freeze.
You may store the mixed liquid at room temperature. Use Berinert® and Haegarda® within 8 hours of mixing them, and use Cinryze® within 3 hours of mixing it. Do not refrigerate or freeze the mixed liquid.
Do not use leftover medicine. Throw away the vial after you have used it.
Throw away used needles in a hard, closed container which the needles cannot poke through. Keep this container away from children and pets.
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 performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of Cinryze® in children 6 years of age and older. Safety and efficacy have been established.
Appropriate studies have not been performed on the relationship of age to the effects of Haegarda® 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 Berinert® in the geriatric population. Safety and efficacy have not been established.
Appropriate studies performed to date have not demonstrated geriatric-specific problems that would limit the usefulness of Haegarda® or Cinryze® in the elderly. However, elderly patients are more likely to have age-related liver, kidney, or heart problems, which may require caution and an adjustment in the dose for patients receiving Cinryze®.
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 or
- Heart or blood vessel disease, history of—Use with caution. May make these conditions worse.
It is very important that your doctor check your or your child's progress at regular visits to make sure that the medicine is working properly. Blood tests may be needed to check for unwanted effects.
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, which 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, although the risk is low. Human donors and donated blood are both tested for viruses to keep the transmission risk low. Talk with your doctor if you have concerns about this risk.