Berinert

What is Berinert?

• Berinert (C1 esterase inhibitor (human)) is a plasma-derived C1 Esterase Inhibitor (Human) used for the treatment of Hereditary Angioedema (HAE).

What are the uses of this medicine?

Berinert (C1 esterase inhibitor (human)) is an injectable medicine used to treat swelling and/or painful HAE attacks in adults and children with Hereditary Angioedema (HAE).

Hereditary Angioedema (HAE):

• HAE is caused by the poor functioning or lack of a protein called C1 that is present in your blood and helps control inflammation (swelling) and parts of the immune system.
• BERINERT contains C1 esterase inhibitor, a protein that helps control C1.
• Early HAE symptoms appear anywhere from minutes to one to two days before the attack worsens. HAE attacks can last hours to several days, and range in severity. Itching is not a typical feature of HAE attacks.
• Facial attacks — These HAE attacks can occur in areas around the eyes and mouth, and result from local edema of tissue beneath the skin (subcutaneous).
• Abdominal attacks — These HAE attacks appear as pain (colic), nausea, vomiting, and/or diarrhea. These symptoms result from the swelling of walls of the gastrointestinal tract.
• Laryngeal HAE attacks — Laryngeal edema (swelling of the voice box, difficulty breathing, loss of voice, and noisy breathing on inspiration), can occur by itself, or with swelling of the lips, tongue, uvula (the piece of mouth tissue that hangs down from the top of the mouth over the back of the tongue), and soft palate (the soft tissue at the back of the mouth). Removing a tooth and oral surgery can trigger a laryngeal attack. Laryngeal swelling can develop in minutes or hours.
• Many HAE attacks involve only one location of the body at a time, although combination attacks, such as cutaneous attacks that spread to involve the larynx (the voice box), can occur.

Limitation of use:

• The safety and efficacy of BERINERT for prophylactic therapy have not been established.

How does this medicine work?

• C1 esterase inhibitor is a normal constituent of human plasma and belongs to the group of serine protease inhibitors (serpins) that includes antithrombin III, alpha1-protease inhibitor, alpha2-antiplasmin, and heparin cofactor II.
• C1 esterase inhibitor, which is usually activated during the inflammatory process, inactivates its substrate by covalently binding to the reactive site.
• C1 esterase inhibitor is the only known inhibitor for the subcomponent of the complement component 1 (C1r), C1s, coagulation factor XIIa, and kallikrein.
• Additionally, C1 esterase inhibitor is the main inhibitor for coagulation factor XIa of the intrinsic coagulation cascade.
• HAE patients have low levels of endogenous or functional C1 esterase inhibitor.
• HAE attacks may be primarily mediated through contact system activation.
• Suppression of contact system activation by C1 esterase inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate this vascular permeability by preventing the generation of bradykinin.
• Administration of BERINERT to patients with C1 esterase inhibitor deficiency replaces the missing or malfunctioning protein in patients.

Who Should Not Use this medicine ?

This medicine cannot be used in patients who:

• have experienced life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product.

What drug interactions can this medicine cause?

• No formal drug interaction studies have been conducted with BERINERT.

Is this medicine FDA approved?

• Initial U.S. Approval: 2009

How should this medicine be used?

Recommended dosage:

• Administer BERINERT at a dose of 20 International Units (IU) per kg body weight by intravenous injection.
• Doses lower than 20 IU/kg body weight should not be administered.

Administration:

• For intravenous use only.
• Store the vial in the original carton in order to protect from light.
• Store at 2-30°C (36-86°F).
• Do not freeze.
• Reconstitute BERINERT prior to use using the Sterile Water for Injection, USP provided.
• Use the silicone-free syringe provided for administration of BERINERT.
• Administer at room temperature within 8 hours of reconstitution.
• Inject at a rate of approximately 4 mL per minute.
• Do not mix BERINERT with other medicinal products or solutions.
• Appropriately trained patients may self-administer upon recognition of an HAE attack.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As 500 International Units lyophilized concentrate in a single-dose vial for reconstitution with 10 mL of Sterile Water for Injection, USP.

This medicine is available in fallowing brand namesː

• BERINERT

What side effects can this medication cause?

The most common side effects of this medicine include: Allergic reactions may occur with BERINERT which may include:

• wheezing
• difficulty breathing
• chest tightness
• turning blue (look at lips and gums)
• fast heartbeat
• swelling of the face
• faintness
• rash
• hives

Signs of a blood clot include:

• pain and/or swelling of an arm or leg with warmth over the affected area
• discoloration of an arm or leg
• unexplained shortness of breath
• chest pain or discomfort that worsens on deep breathing
• unexplained rapid pulse
• numbness or weakness on one side of the body.

What special precautions should I follow?

• Hypersensitivity reactions may occur. Epinephrine should be immediately available to treat any acute severe hypersensitivity reactions following discontinuation of administration.
• Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of C1 Esterase Inhibitor (Human) products, including BERINERT, following administration in patients with HAE. Risk factors may include the presence of an indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, and immobility. Benefits of treatment of HAE attacks should be weighed against the risks of TE events in patients with underlying risk factors. Monitor patients with known risk factors for TE events during and after BERINERT administration.
• TE events have also been reported following administration of a C1 Esterase Inhibitor (Human) product when used for unapproved indications at higher than recommended doses.
• Because BERINERT is made from human blood, it may carry a risk of transmitting infectious agents, e.g., viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
• Following self-administration of BERINERT for laryngeal HAE attacks, advise patients to immediately seek medical attention.

What to do in case of emergency/overdose?

Symptoms of overdosage may include:

• Thrombosis
• The maximum dose administered in clinical studies in hereditary angioedema was 20 IU/kg body weight.

Management of overdosage:

• If an overdose occurs, general supportive measures should be instituted as deemed necessary by the treating physician.

Can this medicine be used in pregnancy?

• It is not known if BERINERT can harm your unborn baby.
• BERINERT should be given to a pregnant woman only if clearly needed.

Can this medicine be used in children?

• The safety profile observed in the pediatric population was similar to that observed in adults.
• Compared to adults, when adjusted for baseline, the half-life of BERINERT was shorter and clearance (on per kg basis) was faster in children. The clinical implication of this difference is not known.

What are the active and inactive ingredients in this medicine?

Active ingredient:

• HUMAN C1-ESTERASE INHIBITOR

Inactive ingredients:

• Glycine
• Sodium Chloride
• SODIUM CITRATE, UNSPECIFIED FORM

Who manufactures and distributes this medicine?

Manufactured by:

• CSL Behring GmbH
• 35041 Marburg, Germany
• US License No. 1765

Distributed by:

• CSL Behring LLC
• Kankakee, IL 60901 USA

What should I know about storage and disposal of this medication?

• Keep BERINERT in its original carton to protect from light until ready to use.
• When stored at temperatures of 2-30°C (36-86°F), BERINERT is stable for the period indicated by the expiration date on the carton and vial label.
• Do not freeze.

• Dailymed label info on Berinert
• FDA Berinert

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