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Trade Names:
AlphaNine SD
- Powder for injection Dried plasma fraction of Factor IX

Trade Names:
BeneFix
- Powder for injection Nonpyrogenic lyophilized. Purified protein produced by recombinant DNA

Trade Names:
Mononine
- Powder for injection Lyophilized concentrate of Factor IX, plasma-derived

Trade Names:
Profilnine SD
- Powder for injection Lyophilized concentrate of Factor IX, plasma-derived

Trade Names:
Proplex T
- Powder for injection Plasma derived concentrate of clotting Factors II, VII, IX, and X

Trade Names:
Bebulin VH
- Powder for injection Purified freeze-dried concentrate of coagulation Factor IX, II, X, and VII (low amounts). Heat-treated

Mechanism of Action

Pharmacology

Restores hemostasis in patients with Factor IX deficiency.

Pharmacokinetics

Elimination

Mean biologic t _½ is about 18.8 h (adults) and 20.2 h (children).

Indications and Usage

Control and prevention of hemorrhagic episodes in patients with hemophilia B (factor IX deficiency) ( Proplex T only); bleeding episodes in patients with inhibitors to Factor VIII, prevention or control of bleeding episodes in patients with factor VII deficiency.

Contraindications

BeneFix : Hypersensitivity to hamster protein. Mononine : Hypersensitivity to mouse protein. Proplex T : Patients with signs of fibrinolysis and patients with disseminated intravascular coagulation.

Dosage and Administration

IV Dose based on patient condition, degree of deficiency, and desired level of Factor IX to be achieved. Dosing guideline: 1 units/kg ( BeneFix : 1.2 units/kg for adults; 1.4 units/kg for children younger 15 yr of age; Bebulin : 1.2 units/kg) times body weight (kg) times desired increase (% of normal). Dosing interval and duration of therapy determined by type of hemorrhage or procedure to be performed.

IV 20 to 30 units/kg 1 to 2 times per week.

IV 0.5 units/kg times body weight (kg) times desired increase (% of normal), repeated every 4 to 6 h as needed.

IV 75 units/kg.

General Advice

• For IV administration only. Not for intradermal, subcutaneous, IM, or intra-arterial administration.
• Allow vials of lyophilized powder and diluent to reach room temperature before reconstituting.
• Transfer contents of diluent vial to dry powder using transfer needle; point transfer needle toward wall of dry powder vial to prevent excessive foaming ( Mononine : direct diluent over entire surface of dry powder cake). Vacuum in dry powder vial will draw diluent into vial. Gently rotate or swirl vial to dissolve powder.
• Administer reconstituted solution within 3 h of reconstitution. Reconstituted solution may be stored at room temperature prior to administration. Do not refrigerate reconstituted solution.
• Withdraw reconstituted solution into sterile disposable plastic syringe using sterile filter spike. Do not inject air into BeneFix or Mononine vials. If contents of more than 1 vial are being used, contents of multiple vials may be drawn into same syringe using separate, unused filter spike.
• Administer reconstituted solution through infusion set tubing. Limit the amount of blood entering tubing. If red blood cell agglutination is observed in the tubing or syringe, discard all material (tubing, syringe, and factor IX solution) and resume administration with a new package.
• Rates of infusion vary with product: Profilnine SD , less than 10 mL/min; Mononine , up to 225 units/min; Proplex T , 2 to 3 mL/min; Bebulin VH , no greater than 2 mL/min; AlphaNine SD , less than 10 mL/min; Benefix , over several min. Rate of administration is determined by patient's comfort level.
• If headache, flushing, changes in heart rate or BP appear, stop the infusion until symptoms subside, then resume at a slower rate.
• Discard any unused solution. Do not save for future use. Discard administration equipment after single use.
• Do not use reconstituted solution if diluent does not transfer completely into dry powder vial. It is acceptable for a small amount of diluent to remain in the diluent vial after transfer.
• Reconstituted solution should be clear and colorless. Do not use if solution is discolored, cloudy, or contains particulate matter.

Storage/Stability

Store powder for injection and diluents in refrigerator (36° to 46°F). Protect diluent vials from freezing. BeneFix may be stored at room temperature (not exceeding 77°F) for up to 6 mo. Profilnine SD may be stored at room temperature (not exceeding 86°F) for up to 3 mo. Mononine may be stored at room temperature (not exceeding 77°F) for up to 1 mo. AlphaNine SD may be stored at room temperature (not exceeding 86°F) for up to 1 mo.

Drug Interactions

May increase risk of thrombosis.

Laboratory Test Interactions

None well documented.

Adverse Reactions

Cardiovascular

MI, pulmonary embolism (high doses); DIC; hypotension, thrombosis (postmarketing).

CNS

Dizziness; drowsiness; headache; lethargy; lightheadedness; somnolence.

Dermatologic

Hives; rash; urticaria.

EENT

Visual disturbance.

GI

Altered taste; diarrhea; nausea; vomiting.

Genitourinary

Renal infarct.

Hypersensitivity

Allergic rhinitis; anaphylactoid or anaphylactic reactions (including fever, itching, urticarial rash, nausea, retching, dyspnea, anaphylactic shock), angioedema (postmarketing).

Lab Tests

ALT and AST elevations; increased alkaline phosphatase.

Local

Burning, cellulitis, discomfort, pain, phlebitis; or stinging at IV site.

Respiratory

Dry cough; lung disorder; dyspnea, laryngeal edema (postmarketing).

Miscellaneous

Burning sensation in jaw and skull; chest tightness; chills; fever; flushing; hypoxia; photosensitivity; shaking; tingling; cyanosis, inadequate therapeutic response, inhibitor development (postmarketing).

Precautions

Pregnancy

Category C .

Lactation

Undetermined.

Children

Safety and efficacy of Profilnine SD and AlphaNine SD have not established in pediatric patients 16 yr of age and younger.

Hypersensitivity

Allergic type hypersensitivity reactions, including anaphylaxis, have been reported. Evaluate patient experiencing allergic reaction for presence of factor IX inhibitor.

Immune tolerance induction

Nephrotic syndrome has been reported following immune tolerance induction with factor IX concentrates in hemophilia B patients with factor IX inhibitors and a history of allergic reactions to factor IX. Safety and efficacy of using BeneFix for immune tolerance induction has not been established.

Latex sensitivity

Diluent vial for BeneFix and certain components of Proplex T packaging may contain dry natural latex rubber that may cause allergic reactions in latex sensitive individuals.

Thromboembolic complications

Because use of factor IX complex concentrates has been associated with development of thromboembolic complications, the use of factor IX-containing products may be potentially hazardous in patients with signs of fibrinolysis or with disseminated intravascular coagulation. Use with caution when administering to patients with liver disease, patients post-operatively, neonates, or to patients at risk of thromboembolic phenomena or DIC.

Viral infections (eg, hepatitis)

All products except BeneFix are prepared from pooled units of human plasma and may carry a risk of transmitting infectious agents.

Patient Information

• Review benefits of therapy and risks, including potential to transmit disease and unknown infectious agents.
• Advise patient or caregiver that medication will be prepared and administered by a health care professional in a health care setting.
• Review signs and symptoms of hypersensitivity reactions (eg, hives, angioedema, chest tightness, difficulty breathing or unexplained shortness of breath, wheezing, faintness, rapid heart beat). Instruct patient to contact health care professional and/or seek immediate emergency care depending on the severity of the reaction.