C1 esterase inhibitor, human (Intravenous, Subcutaneous)

C1 esterase inhibitor is used to treat or prevent hereditary angioedema (HAE). HAE is a rare condition that causes swelling in the face, hands, feet, throat, stomach, bowels, or genitals. Individuals with HAE have low levels of C1 esterase inhibitor in their bodies. This medicine helps raise the levels of C1 esterase inhibitors in the body.

This medicine is available only with your doctor's prescription.

It is very important for your doctor to check your or your child's progress at regular visits to ensure that the medicine is working properly. Blood tests may be needed to look for unwanted effects.

HAE attacks are life-threatening and need immediate medical attention. Contact 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 experience 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 receiving this medicine.

This medicine may increase your risk of developing blood clots. Contact 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 (e.g., Creutzfeldt-Jakob Disease) to people who received them, although the risk is low. Human donors and donated blood are both tested for viruses to keep the transmission risk low. Discuss your concerns about this risk with your doctor.

In deciding to use a medicine, you and your doctor need to consider the risks versus the benefits. For this medicine, think about the following:

Allergies

Inform your doctor if you have ever had any unusual or allergic reactions to this medicine or any other medicines. Also, let your healthcare professional know about any other allergies you may have, such as to foods, dyes, preservatives, or animals. For non-prescription products, carefully read the label or package ingredients.

Pediatric

Current studies have not shown any specific problems in children that would limit the usefulness of Berinert®.

Current studies have not shown any specific problems in children 6 years and older that would limit the usefulness of Cinryze®. Safety and effectiveness have been established.

There have been no studies on the effects of Haegarda® in children younger than 8 years old. Safety and effectiveness have not been established.

Geriatric

There have been no studies on the effects of Berinert® in older adults. Safety and effectiveness have not been established.

Current studies have not shown any specific problems in older adults that would limit the usefulness of Haegarda® or Cinryze®. However, older patients are more likely to have age-related liver, kidney, or heart problems, which may require caution and a dose adjustment for patients taking Cinryze®.

Breast Feeding

There are no adequate studies in women to determine the risk to infants when using this medication while breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Interactions with Medicines

Some medicines should never be used together, while in other cases, two different medicines may be combined even if an interaction could happen. In these situations, your doctor may want to adjust the dose or take other precautions. Inform your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicines.

Interactions with Food/Tobacco/Alcohol

Certain medicines should not be taken at or around the time of eating food or specific types of food due to possible interactions. Using alcohol or tobacco with certain medicines may also lead to interactions. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other Medical Problems

Having other medical problems may affect how you can use this medicine. Make sure to tell your doctor if you have any other medical issues, especially:

• Atherosclerosis (blood vessel disease) or
• Blood clots, history of or
• Heart or blood vessel disease, history of—Use with caution. May worsen these conditions.

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 or tightness
• head congestion
• hoarseness or other voice changes
• nausea
• pain or tenderness around the eyes and cheekbones
• rash
• sore throat
• trouble breathing
• vomiting

Less common

• Bleeding gums
• collection of blood under the skin
• coughing up blood
• deep, dark purple bruise
• difficulty with swallowing
• dizziness
• hard lump
• headache
• increased menstrual flow or vaginal bleeding
• itching, pain, flushing, redness, or swelling of the skin
• nosebleeds
• paralysis
• prolonged bleeding from cuts
• red or black, tarry stools
• red or dark brown urine
• swelling
• unusually warm skin

Incidence not known

• Confusion
• difficulty with speaking
• 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

• Diarrhea
• headache
• pain
• stomach pain

Less common

• 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
• change in taste
• fever
• loss of taste
• muscle aches
• pain at the catheter site
• stuffy or runny nose
• 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.

In the U.S.

• Berinert
• Cinryze
• Haegarda

Available Dosage Forms:

• Powder for Solution

Therapeutic Class: Immune Modulator

Find detailed information on biosimilars for this medication.

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.

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