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
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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

lyophilized powder for injection

  • 500 unit/vial
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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 adolescents and adults with hereditary angioedema
  • Neonates and children: Safety and efficacy not established
  • Adolescents: 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)  
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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

Previous anaphylaxis to product

Cautions

Risk of: severe hypersensitivity; thrombotic events may occur; transmission of infectious disease from product potentially possible

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Pregnancy & Lactation

Pregnancy Category: C

Lactation: excretion in milk unknown; use with caution

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 (1 dose): 0.68±0.08 U/mL
  • Peak Plasma Time (Single dose): 3.9 +/- 7.3 hr

Distribution

Berinert

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

Elimination

Berinert clearance (mL/kg/hr): 0.43-3.85 (adults); 0.98-1.1 (pediatrics)

Half-life

  • Cinryze: (1 dose) 56 +/- 36 hr
  • 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

Untampered vial may be stored at 2-25°C (36-77°F) for up to 1 year

Do not freeze

Protect from light

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Formulary

FormularyPatient Discounts

Adding plans allows you to compare formulary status to other drugs in the same class.

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:

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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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