C1 Esterase Inhibitor (Recombinant)

What is C1 Esterase Inhibitor (Recombinant)?

• C1 Esterase Inhibitor (Recombinant) (RUCONEST) used for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).

What are the uses of this medicine?

• This medicine is used for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).

Limitation of Use:

• Effectiveness was not established in HAE patients with laryngeal attacks.

How does this medicine work?

• C1 esterase inhibitor (C1INH) is a normal constituent of human blood and is one of the serine protease inhibitors (serpins).
• The primary function of C1INH is to regulate the activation of the complement and contact system pathways.
• Regulation of these systems is performed through the formation of complexes between the protease and the inhibitor, resulting in inactivation of both and consumption of the C1INH.
• C1INH exerts its inhibitory effect by irreversibly binding several proteases (target proteases) of the contact and complement systems.
• The effect of RUCONEST on the following target proteases was assessed in vitro: activated C1s, kallikrein, factor XIIa and factor XIa. Inhibition kinetics were found to be comparable with those observed for plasma-derived human C1INH.
• HAE patients have low levels of endogenous or functional C1INH.
• Although the events that induce attacks of angioedema in HAE patients are not well defined, it is thought that contact system activation, and resulting increased vascular permeability lead to the clinical manifestation of HAE attacks.
• Suppression of contact system activation by C1INH through the inactivation of plasma kallikrein and factor XIIa is thought to modulate vascular permeability by preventing the generation of bradykinin.
• Administration of RUCONEST increases plasma levels of functional C1INH activity.

Who Should Not Use this medicine ?

This medicine cannot be used in patients with:

• a history of allergy to rabbits or rabbit-derived products.
• a history of life-threatening immediate hypersensitivity reactions to C1 esterase inhibitor preparations, including anaphylaxis.

What drug interactions can this medicine cause?

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

Is this medicine FDA approved?

• This medicine is approved in the year 2014.

How should this medicine be used?

Recommended Dosage:

• The recommended dose of RUCONEST is 50 U per kg with a maximum of 4200 U to be administered as a slow intravenous injection over approximately 5 minutes.
• If the attack symptoms persist, an additional (second) dose can be administered at the recommended dose level.
• Do not exceed 4200 U per dose.
• No more than two doses should be administered within a 24 hour period.

Administration

• Reconstitute each vial (2100 U) by adding 14 mL sterile Water for Injection per vial to obtain a solution of 150 U per mL.
• Inspect RUCONEST visually for particulate matter and discoloration after reconstitution and prior to administration. The reconstituted solution should be colorless, clear, and free from visible particles. Do not use if the solution is cloudy, colored, or contains particulates.
• RUCONEST vial is for single-use only.
• Use the reconstituted product immediately, or within 8 hours stored at 2°C - 8°C (36°F - 46°F). Discard partially used vials.
• Do not freeze the reconstituted solution.
• Do not mix RUCONEST with other medicinal products. Administer RUCONEST by a separate infusion line.
• Use aseptic technique when administering RUCONEST.
• Follow recommended venipuncture guidelines for initiating intravenous therapy.
• Administer RUCONEST by slow intravenous injection over approximately 5 minutes.
• For self-administration, provide the patient with instructions and training for intravenous injection outside of a clinic setting so patients may self-administer RUCONEST upon recognition of symptoms of an HAE attack.
• After administration, immediately discard any unused product and all used disposable supplies in accordance with local requirements.

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

This medicine is available in fallowing doasage form:

• As 2100 U Lyophilized powder for reconstitution for injection in a single-use vial.

This medicine is available in fallowing brand namesː

• RUCONEST

What side effects can this medication cause?

The most common side effects of this medicine include:

• headache
• nausea
• diarrhea

The serious adverse reaction reported in clinical trials was anaphylactic reaction.

What special precautions should I follow?

• Severe hypersensitivity reactions may occur. Discontinue RUCONEST and administer appropriate treatment.
• Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of plasma derived C1 esterase inhibitor products in patients with risk factors.
• Monitor patients with known risk factors for TE events during and after RUCONEST administration.

What to do in case of emergency/overdose?

• In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

• Overdose related information is also available online at poisonhelp.org/help.
• In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.

Can this medicine be used in pregnancy?

• There are, however, no adequate and well-controlled studies in pregnant women.
• Because animal reproduction studies are not always predictive of human response, RUCONEST should only be used during pregnancy if clearly needed.

Can this medicine be used in children?

• The safety and efficacy of RUCONEST were evaluated in 17 adolescent patients (13-17 years of age) treated for 52 HAE attacks.

What are the active and inactive ingredients in this medicine?

Active ingredient:

• CONESTAT ALFA

Inactive ingredients:

• SUCROSE
• SODIUM CITRATE, UNSPECIFIED FORM
• CITRIC ACID MONOHYDRATE

Who manufactures and distributes this medicine?

• Packager: Santarus, Inc.

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

• Store in the original package in order to protect from light.
• Shelf life: 48 months when stored at 2°C to 25°C (36°F to 77°F).
• Do not freeze.
• Each vial of RUCONEST should be reconstituted with 14 mL Water for Injection (not supplied). The reconstituted solution contains 150 U/mL rhC1INH and is clear and colorless.
• Each vial of RUCONEST is for single use only. RUCONEST contains no preservative. Any product that has been reconstituted should be used immediately, or within 8 hours stored at 2°C to 8°C (36°F to 46°F). Discard partially used vials after treatment.

• Dailymed label info on C1 Esterase Inhibitor (Recombinant)
• FDA C1 Esterase Inhibitor (Recombinant)

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