Coagulation Factor IX (recombinant)

What is Coagulation Factor IX (recombinant)?

• Coagulation Factor IX (recombinant) (ALPROLIX), Coagulation Factor IX (Recombinant), Fc Fusion Protein, is a recombinant DNA derived coagulation Factor IX concentrate used to help control and prevent bleeding in people with hemophilia B. Hemophilia B is also called congenital Factor IX deficiency.

What are the uses of this medicine?

This medicine is used in adults and children with hemophilia B (congenital Factor IX deficiency) for:

• On-demand treatment and control of bleeding episodes,
• Perioperative management of bleeding,
• Routine prophylaxis to reduce the frequency of bleeding episodes.

How does this medicine work?

• ALPROLIX is a recombinant, fusion protein that temporarily replaces the missing coagulation Factor IX needed for effective hemostasis.
• ALPROLIX contains the Fc region of human IgG1, which binds to the neonatal Fc receptor (FcRn).
• FcRn is part of a naturally occurring pathway that delays lysosomal degradation of immunoglobulins by cycling them back into circulation, and prolonging their plasma half-life.

Who Should Not Use this medicine ?

This medicine cannot be used in patients with:

• known history of hypersensitivity reactions, including anaphylaxis, to the product or its excipients

What drug interactions can this medicine cause?

• No formal clinical drug interaction studies have been performed with ALPROLIX.

Is this medicine FDA approved?

• It was approved for use in the United States in 2014.

How should this medicine be used?

Recommended Dosage: On-demand treatment and control of bleeding episodes:

• Each vial of ALPROLIX contains the labeled amount of coagulation Factor IX potency in international units (IU).
• On average, one unit per kilogram body weight of ALPROLIX increased the circulating Factor IX level by approximately 1% (IU/dL) in adults and children ≥6 years of age and by 0.6% (IU/dL) in children under 6 years of age.

• Minor and Moderate Type of Bleeding: Repeat every 48 hours as needed if there is further evidence of bleeding with target circulating fIX 30–60 (IU/dL).
• Major Type of Bleeding: Consider repeat dose after 6–10 hours, then every 24 hours for 3 days, then every 48 hours until healing achieved with target circulating fIX 80–100 (IU/dL).

Perioperative management:

• Minor surgery: a single infusion to reach FIX level of 50–80 IU/dL may be sufficient. Repeat as needed after 24–48 hours until bleeding stops and healing is achieved.
• Major surgery: initial infusion to reach FIX level of 60–100 IU/dL. Consider a repeat dose after 6–10 hours and then every 24 hours for the first 3 days, then every 48 hours until bleeding stops and healing is achieved.

Routine prophylaxis:

• For adults and adolescents ≥12 years of age, start at 50 IU/kg once weekly or 100 IU/kg once every 10 days.
• For children <12 years of age, start at 60 IU/kg once weekly.
• Adjust dosing regimen based on individual response.
• More frequent or higher doses may be needed in children <12 years of age.

Administration

• ALPROLIX should be administered as ordered by your healthcare provider.
• You should be trained on how to do infusions by your healthcare provider.
• Many people with hemophilia B learn to infuse their ALPROLIX by themselves or with the help of a family member.

For intravenous injection only

• Inspect the reconstituted ALPROLIX solution visually for particulate matter and discoloration prior to administration.
• Do not use if particulate matter or discoloration is observed.
• Do not administer reconstituted ALPROLIX in the same tubing or container with other medications.

Administration Steps:

• Attach the syringe to the connector end of the infusion set tubing by turning clockwise until it is securely in place.
• Depress the plunger until all air is removed from the syringe and ALPROLIX has reached the end of the infusion set tubing. Do not push ALPROLIX through the needle.
• Remove the protective needle cover from the infusion set tubing.
• Perform intravenous bolus infusion. The rate of administration should be determined by the patient's comfort level, and no faster than 10 mL per minute.
• After infusing ALPROLIX, remove and properly discard the infusion set.

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

This medicine is available in fallowing doasage form:

• As a lyophilized powder in single-dose vials containing nominally 250, 500, 1000, 2000, 3000, or 4000 international units (IU).

This medicine is available in fallowing brand namesː

• ALPROLIX

What side effects can this medication cause?

The most common side effects of this medicine include: Previously Treated Patients (PTPs):

• headache
• oral paresthesia
• obstructive uropathy

Previously Untreated Patients (PUPs):

• Factor IX inhibition
• injection site erythema
• hypersensitivity

What special precautions should I follow?

• Hypersensitivity reactions, including anaphylaxis, have been reported. Should symptoms occur, discontinue ALPROLIX and administer appropriate treatment.
• Neutralizing antibodies (inhibitors) to FIX have been reported with ALPROLIX. Perform an assay that measures Factor IX inhibitor concentration if plasma Factor IX level fails to increase as expected or if bleeding is not controlled with an appropriate dose.
• The use of Factor IX products has been associated with the development of thromboembolic complications.
• Nephrotic syndrome has been reported following immune tolerance induction with Factor IX-containing products in hemophilia B patients with Factor IX inhibitors and a history of allergic reactions to Factor IX.

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 no studies of ALPROLIX use in pregnant women to inform a drug-associated risk.

Can this medicine be used in children?

• Safety, efficacy, and pharmacokinetics of ALPROLIX have been evaluated in previously treated patients (PTPs) from the adult and adolescent study (12 to <17 years of age) and from the pediatric study (1 to 11 years of age).
• Safety of ALPROLIX has been evaluated in previously untreated patients (PUPs) less than 18 years of age (median: 0.6 year; range: 0.08–2 years) in the PUPs study.

What are the active and inactive ingredients in this medicine?

Active ingredient:

• eftrenonacog alfa

Inactive ingredients:

• histidine
• mannitol
• sucrose
• polysorbate 20

Who manufactures and distributes this medicine?

Manufactured by: Bioverativ Therapeutics Inc. Waltham, USA

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

• Store ALPROLIX vials at 2°C to 8°C (36°F to 46°F).
• Do not freeze.
• ALPROLIX vials may also be stored at room temperature up to 30°C (86°F) for a single 6-month period.

If you choose to store ALPROLIX at room temperature:

• Note on the carton the date on which the product was removed from refrigeration.
• Use the product before the end of this 6-month period or discard it.
• Do not return the product to the refrigerator.
• Do not use product or diluent after the expiration date printed on the carton, vial, or syringe.

After Reconstitution:

• Use the reconstituted product as soon as possible; however, you may store the reconstituted product at room temperature up to 30°C (86°F) for up to 3 hours. Protect the reconstituted product from direct sunlight. Discard any product not used within 3 hours after reconstitution.
• Do not use ALPROLIX if the reconstituted solution is cloudy, contains particles or is not colorless.

• Dailymed label info on Coagulation Factor IX (recombinant)
• FDA Coagulation Factor IX (recombinant)

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