Description and Brand Names

Drug information provided by: Merative, Micromedex®

US Brand Name

  1. Berinert
  2. Cinryze
  3. Haegarda

Descriptions


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.

This product is available in the following dosage forms:

  • Powder for Solution

Before Using

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.

Pediatric

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 younger than 8 years of age. Safety and efficacy have not been established.

Geriatric

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®.

Breastfeeding

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.

Drug Interactions

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:

  • 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.

Proper Use

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.

Do not inject into skin areas that are itchy, swollen, painful, red, bruised, or have scars or stretch marks.

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.

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 hereditary angioedema:
    • For injection dosage form:
      • Berinert®:
        • 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.
      • Cinryze®:
        • 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.
      • Haegarda®:
        • Adults and children 8 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 8 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 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.

Precautions

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, trouble breathing, 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 (eg, Creutzfeldt-Jakob Disease) 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.

Side Effects

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

  1. Chest pain or tightness
  2. head congestion
  3. hoarseness or other voice changes
  4. nausea
  5. pain or tenderness around the eyes and cheekbones
  6. rash
  7. sore throat
  8. trouble breathing
  9. vomiting

Less common

  1. Bleeding gums
  2. collection of blood under the skin
  3. coughing up blood
  4. deep, dark purple bruise
  5. difficulty with swallowing
  6. dizziness
  7. hard lump
  8. headache
  9. increased menstrual flow or vaginal bleeding
  10. itching, pain, flushing, redness, or swelling of the skin
  11. nosebleeds
  12. paralysis
  13. prolonged bleeding from cuts
  14. red or black, tarry stools
  15. red or dark brown urine
  16. swelling
  17. unusually warm skin

Incidence not known

  1. Confusion
  2. difficulty with speaking
  3. dizziness
  4. double vision
  5. fast heartbeat
  6. general tiredness and weakness
  7. hives
  8. inability to move the arms, legs, or facial muscles
  9. numbness or tingling in the face, arms, or legs
  10. pain, redness, or swelling in the arm or leg
  11. puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
  12. slow speech
  13. 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

  1. Diarrhea
  2. headache
  3. pain
  4. stomach pain

Less common

  1. Bleeding, blistering, burning, coldness, discoloration of skin, feeling of pressure, hives, infection, inflammation, itching, lumps, numbness, pain, rash, redness, scarring, soreness, stinging, swelling, tenderness, tingling, ulceration, or warmth at the injection site
  2. change in taste
  3. fever
  4. loss of taste
  5. muscle aches
  6. pain at the catheter site
  7. stuffy or runny nose
  8. unusual tiredness or weakness

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.

Portions of this document last updated: Feb. 01, 2024

Original article: https://www.mayoclinic.org/ar/drugs-supplements/c1-esterase-inhibitor-human-intravenous-route-subcutaneous-route/precautions/drg-20072383