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C1 esterase inhibitor, human (Intravenous route)

Pronunciation:

C1 ES-ter-ase in-HIB-i-ter, HUE-man

Brand Names:

  • Berinert
  • Cinryze

Dosage Forms:

  • Powder for Solution

Classifications:

Therapeutic—

Immune Modulator

Uses of This Medicine:

C1 esterase inhibitor is used to treat or prevent hereditary angioedema (HAE) in adults and teenagers. 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.

Before Using This Medicine:

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:

Allergies—

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.

Children—

Appropriate studies have not been performed on the relationship of age to the effects of C1 esterase inhibitor in children 12 years of age and younger. Safety and efficacy have not been established.

Older adults—

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.

Pregnancy—

Pregnancy CategoryExplanation
All TrimestersCAnimal studies have shown an adverse effect and there are no adequate studies in pregnant women OR no animal studies have been conducted and there are no adequate studies in pregnant women.

Breast-feeding—

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.

Other medicines—

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.

Other interactions—

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 problems—

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:

  • Blood clots, history of—Use with caution. May make this condition worse.

Proper Use of This Medicine:

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.

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.

Dosing—

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 injection dosage form:
    • For hereditary angioedema:
      • Adults and teenagers—
        • Berinert®: Dose is based on body weight and must be determined by a doctor. The usual dose is 20 units per kilogram (kg) of body weight injected into a vein.
        • Cinryze®: 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.

Missed dose—

Call your doctor or pharmacist for instructions.

Storage—

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.

You may store the mixed liquid at room temperature. Use Berinert® within 8 hours of mixing it, and use Cinryze® within 3 hours of mixing it. Do not refrigerate or freeze the mixed liquid.

Do not reuse the remaining portion of the medicine that is left in the vial. Throw away the vial after you have used it.

Precautions While Using This Medicine:

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, shortness of breath, 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.

Side Effects of This Medicine:

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor or nurse immediately if any of the following side effects occur:

More common
Chest pain
head congestion
hoarseness or other voice changes
nausea and vomiting
pain or tenderness around the eyes and cheekbones
rash
shortness of breath or troubled breathing
sore throat
tightness of the chest
Less common
Flushing or redness of the skin
itching
unusually warm skin
Incidence not known
Confusion
difficulty with speaking
difficulty with swallowing
dizziness
double vision
fast heartbeat
general tiredness and weakness
hives
inability to move the arms, legs, or facial muscles
numbness or tingling in the face, arms, or legs
pain, redness, or swelling in the arm or leg
puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
slow speech
trouble speaking, thinking, or walking

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common
Abdominal or stomach pain
diarrhea
headache
pain
Less common
Change in taste
loss of taste
pain at the catheter site

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.


Last Updated: 11/4/2014

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