C1 inhibitor human (Rx)

Brand and Other Names:Berinert, Cinryze, more...Haegarda
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

IV, lyophilized powder for reconstitution injection

  • 500 unit/vial (Berinert, Cinryze)

SC, lyophilized powder for reconstitution (Haegarda)

  • 2000 IU/vial
  • 3000 IU/vial

Hereditary Angioedema Acute Treatment

Berinert

  • Indicated for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adults and children
  • 20 units/kg IV infused slowly, not to exceed 4 mL/min
  • Patients may self administer after completing education for IV administration

Hereditary Angioedema Prophylaxis

Cinryze

  • Indicated for routine prophylaxis against angioedema attacks in adolescents and adults patients with hereditary angioedema
  • 1,000 units IV q3-4Days; infuse over 10 minutes (ie, 1 mL/min)

Haegarda

  • Indicated for routine prophylaxis against angioedema attacks in adolescents and adults with hereditary angioedema
  • 60 IU/kg SC twice weekly (every 3-4 days)  

Kidney Transplant Rejection (Orphan)

Cinryze: Orphan designation for treatment of acute antibody-mediated rejection following kidney transplantation

Sponsor

  • Shire ViroPharma, Inc; 300 Shire Way; Lexington, Massachusetts

Delayed Graft Function (Orphan)

Prevention and/or treatment of delayed graft function after solid organ transplantation

Orphan indication sponsor

  • Pharming Technologies B.V.; Archimedesweg 4, Netherlands

Capillary Leakage Syndrome (Orphan)

Orphan indication sponsor

  • Pharming Technologies B.V.; Archimedesweg 4, Netherlands

Dosage Forms & Strengths

IV, lyophilized powder for reconstitution injection

  • 500 unit/vial (Berinert, Cinryze)

SC, lyophilized powder for reconstitution (Haegarda)

  • 2000 IU/vial
  • 3000 IU/vial

Hereditary Angioedema Acute Treatment

Berinert

  • Indicated for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adults and children
  • 20 units/kg IV infused slowly, not to exceed 4 mL/min

Hereditary Angioedema Prophylaxis

Cinryze

  • Indicated for routine prophylaxis against angioedema attacks in adults, adolescents and pediatric patients (≥6 years) with HAE
  • <6 years: Safety and efficacy not established
  • 6-11 years
    • 500 units IV q3-4Days; infuse over 5 minutes (ie, 5 mL/min)
    • Adjust dose according to individual response, up to 1,000 units q3-4Days
  • ≥12 years
    • 1,000 units IV q3-4Days; infuse over 10 minutes (ie, 1 mL/min)
    • For patients who have not responded adequately, consider doses up to 2,500 U (not to exceed 100 U/kg) q3 or 4Days based on individual patient response  

Haegarda

  • Indicated for routine prophylaxis against angioedema attacks in adults, adolescents and pediatric patients (≥6 years) with HAE
  • 60 IU/kg SC twice weekly (every 3-4 days)  
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Adverse Effects

Berinert

>4%: nausea, vomiting, diarrhea, dysgeusia, headache, abdominal pain, muscle spasms, and pain

Rare: laryngeal edema, swelling (shoulder and chest), exacerbation of hereditary angioedema, and laryngospasm

Cinryze

>5%: URI, sinusitis, rash, & headache

Rare: death to non-catheter related foreign body embolus, pre-eclampsia, stroke, exacerbation of HAE attacks

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Warnings

Contraindications

History of life-threatening immediate hypersensitivity reactions (ie, anaphylaxis)

Cautions

Severe hypersensitivity reactions may occur; if hypersensitivity occurs, discontinue infusion and institute appropriate treatment; immediately have epinephrine available for treatment of acute severe hypersensitivity reaction (see Contraindications)

Serious arterial and venous thromboembolic (TE) events reported; risk factors may include presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, and immobility; monitor patients with known risk factors for TE events during and after drug administration

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Pregnancy & Lactation

Pregnancy

There are no data with C1 inhibitor human use in pregnant women to inform a drug associated risk

Animal reproduction studies have not been conducted

It is unknown whether drug can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity; administer to a pregnant woman only if clearly needed

Lactation

There are no data regarding the presence of C1 inhibitor human in human milk, the effects on the breastfed infant, or the effects on milk production

Pregnancy Categories

A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA: Information not available.

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Pharmacology

Mechanism of Action

Serine proteinase inhibitor

Increases plasma concentration of C1 inhibitor activity

HAE patients have low levels of endogenous or functional C1 inhibitor: presentation of HAE attacks are thought to be mediated primarily through contact system activation

C1 inhibitor suppresses contact system activation through inactivation of plasma kallikrein and Factor XIIa modulating vasc permeability by preventing bradykinin generation

Absorption

Cinryze

  • Peak plasma concentration: 0.68 units/mL (single dose); 0.85 units/mL (double dose)
  • Peak plasma time: 3.9 hr (single dose); 2.7 hr (double dose)
  • AUC: 74.5 units·hr/mL (single dose); 95.9 units·hr/mL (double dose)

Distribution

Berinert

  • Vd (mL/kg): 14.1-56.1 (adults); 31.9-54.0 (pediatrics)

Elimination

Clearance

  • Berinert (mL/kg/hr): 0.43-3.85 (adults); 0.98-1.1 (pediatrics)
  • Cinryze: 0.85 mL/min (single dose); 1.17 mL/min (double dose)

Half-life

  • Cinryze: 36 hr (single dose); 62 hr (double dose)
  • Berinert: 7.4-22.8 hr (adults); 7.4-22.5 hr (pediatrics)
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Administration

IV Preparation

Do not use if turbid or discolored (should be colorless to slight blue)

Bring to room temp, cleanse stoppers with germicidal solution, allow to dry

Use double-ended transfer needle; invert diluent vial containing 5 mL SWI over upright & slightly angled vial of drug; then rapidly insert free end of needle through the drug's vial stopper at its center; vial vacuum will draw in diluent

Do NOT use product if there is no vacuum in vial

Disconnect vials, discard diluent vial along w/ transfer needle, gently swirl to mix

Cinryze: Each reconstituted vial contains 5 mL of 100 U/mL solution

Cinryze: Reconstitute 2 vials for one dose

Berinert: Vials are reconstituted with 10 mL dilutent supplied

IV Administration

Administer at room temp within 3 hr (Cinryze) or 8 hr (Berinert) after reconstitution

To withdraw, introduce air in vial & withdraw drug solution

Attach needle or infusion set with winged adapter

Cinryze: Infuse at 1 mL/min over 10 min

Berinert: Infuse at no faster than 4 mL/min

SC Preparation

Use silicone-free syringe for reconstitution and administration

Ensure lyophilized powder and sterile water for injection (SWI) are at room temperature

2000 IU vial: Must reconstitute with 4 mL SWI

3000 IU vial: Must reconstitute with 6 mL SWI

Use Mix2Vial according to package instructions to reconstitute lyophilized powder

Do not shake vial (gently swirl until powder dissolved)

Once reconstituted, use SC solution within 8 hr

SC Administration

Attach the syringe containing reconstituted solution to a hypodermic needle or SC infusion set and administer by SC injection in abdominal area or other SC injection sites

Adapt SC injection/infusion rate to patient comfort level

Rotate injection sites

Storage

Berinert, Cinryze

  • Untampered Unopened vial may be stored at 2-25°C (36-77°F) for period stated on the vial/container up to 1 year
  • Do not freeze
  • Protect from light

Haegarda

  • Unopened vial
    • Store at up temperature up to 30°C (86°F) until expiration date
    • Protect from light, by keeping vial in original container until ready to reconstitute
    • Do not freeze
  • Reconstituted solution
    • Store reconstituted solution at room temperature for up to 8 hr
    • Do not freeze
    • Single-use vials; discard any unused solution
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Images

BRAND FORM. UNIT PRICE PILL IMAGE
Cinryze intravenous
-
500 unit (5 mL) vial
Berinert intravenous
-
500 unit (10 mL) kit

Copyright © 2010 First DataBank, Inc.

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Patient Handout

Patient Education
C1 esterase inhibitor intravenous

C1-ESTERASE INHIBITOR (TREATMENT) - INJECTION

COMMON BRAND NAME(S): Berinert

USES: This medication is used to treat the swelling problems that occur with a certain immune disease passed down through families (hereditary angioedema-HAE). HAE is caused by low levels or improper function of a natural substance produced by the body (C1-inhibitor). Symptoms such as rapid swelling of the hands, feet, limbs, face, tongue, or throat can occur. Swelling of the intestinal tract may cause symptoms such as abdominal cramps, diarrhea, or vomiting. Attacks of swelling may occur without reason. However, anxiety, stress, sickness, and surgery may trigger attacks in some people. This medication increases the level of C1-inhibitor to help treat attacks of sudden swelling due to hereditary angioedema.

HOW TO USE: Read the Patient Information Leaflet if available from your pharmacist before you start using C1-esterase inhibitor and each time you get a refill. If you have any questions, ask your doctor or pharmacist.This medication is given by injection into a vein, at the first signs of an HAE attack, as directed by your doctor. The dosage is based on weight and your medical condition.If you are using this medication at home, learn all preparation and usage instructions from your health care professional. The solution is normally clear and colorless. Before using, check this product visually for particles or discoloration. If either is present, do not use the liquid. Learn how to store and discard medical supplies safely.After giving yourself a dose of this medication, you should get medical help right away for follow-up treatment.Tell your doctor if your condition does not improve or if it worsens.

SIDE EFFECTS: A bad taste in the mouth may occur. Other side effects are uncommon. If you notice any unusual side effects, tell your doctor or pharmacist promptly.Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.This medication may increase the risk for blood clots, especially when used at high doses. Get medical help right away if you notice any of the following rare but serious side effects: new swelling/pain in the arm/leg/abdomen, chest pain, loss of feeling or ability to move, trouble speaking.A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

PRECAUTIONS: Before using C1-esterase inhibitor, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.Before using this medication, tell your doctor or pharmacist your medical history, especially of: blood clots (such as in the lungs, legs).This medication is made from human blood. Even though the blood is carefully tested, and this medication goes through a special manufacturing process, there is an extremely small chance that you may get infections from the medication (for example, virus infections such as hepatitis). Consult your doctor or pharmacist for more information.Before traveling, talk to your doctor to make sure you have enough of this medication.Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.It is unknown if this drug passes into breast milk. Consult your doctor before breast-feeding.

DRUG INTERACTIONS: Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

OVERDOSE: If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call their local poison control center at 1-800-222-1222. Canada residents can call a provincial poison control center.

NOTES: Do not share this medication with others.

MISSED DOSE: Not applicable.

STORAGE: Store the unmixed vial in the original carton between 36-77 degrees F (2-25 degrees C) away from light. Do not freeze. Use this medication immediately after mixing or within the time period indicated in the product instructions. Discard any unused portion. Keep all medications away from children and pets.Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.

Information last revised August 2021. Copyright(c) 2022 First Databank, Inc.

IMPORTANT: HOW TO USE THIS INFORMATION: This is a summary and does NOT have all possible information about this product. This information does not assure that this product is safe, effective, or appropriate for you. This information is not individual medical advice and does not substitute for the advice of your health care professional. Always ask your health care professional for complete information about this product and your specific health needs.

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Formulary

FormularyPatient Discounts

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To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.

Adding plans allows you to:

  • View the formulary and any restrictions for each plan.
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The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
NC NOT COVERED – Drugs that are not covered by the plan.
Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.