Dosing and uses of Bebulin VH, Profilnine SD (Factor IX complex)
Adult dosage forms and strengths
Lyophilized powder for reconstitution
Derived from human plasma
Standardized in terms of Factor IX content and each vial is labeled for the Factor IX content indicated in International Units (IU)
Bebulin, Bebulin VH
- Purified, sterile, stable, freeze-dried concentrate of the coagulation Factors IX (Christmas Factor) as well as II (Prothrombin) and X (Stuart Prower Factor) and low amounts of Factor VII
- Contains small amounts of heparin (0.15 IU heparin or less per IU Factor IX)
Profilnine Sd
- Solvent Detergent Treated, is a sterile, lyophilized concentrate of Factor IX (antihemophilic factor B), Factor II (prothrombin), Factor X (Stuart-Prower Factor), and low levels of Factor VII (proconvertin) derived from human plasma
- Factor II (<150 Units per 100 Factor IX Units), Factor X (<100 Units per 100 Factor IX Units), and Factor VII (<35 Units per 100 Factor IX Units)
- Does not contain heparin
Hemophilia B
Indicated for prevention and control of hemorrhagic episodes
Bebulin, Bebulin VH: Number of Factor IX IU required = body weight (kg) x desired increase in plasma Factor IX (%) x 1.2 IU/kg
Profilnine SD: Number of Factor IX IU required = body weight (kg) x desired increase in plasma Factor IX (%) x 1 IU/kg
Management of bleeding
- Typical initial doses suggested below; usually two-thirds of initial dose given for maintenance Minor: Single dose of 25-35 IU/kg IV x 1 dose; may repeat dose after 24 hr
- Moderate: 40-55 IU/kg/day IV x 2 days or until adequate wound healing
- Major: 60-70 IU/kg/day IV x 2-3 days or until adequate wound healing
Management of surgical procedures
- Minor: 50-60 IU/kg IV on day of surgery, decrease to 25-55 IU/kg/day during initial postop period (ie, 1st to 2nd week)
- Major: 70-95 IU/kg IV on day of surgery, decrease to 35-70 IU/kg/day during initial postop period (ie, 1st to 2nd week), THEN 25-35 IU/kg/day late postop period (ie, 3rd week onwards)
Dosing Considerations
A 1% increase in Factor IX (0.01 IU)/IU administered/kg can be expected
The dose required to establish hemostasis will vary with each patient and depend on the circumstances
Not indicated for treatment of Factor VII deficiency
Clinical studies have not been conducted showing benefit for treating coagulation factor deficiencies other than Factor IX deficiency
Pediatric dosage forms and strengths
Safety and efficacy not established
Bebulin VH, Profilnine SD (Factor IX complex) adverse (side) effects
Frequency not defined
Formation of circulating antibodies inhibiting Factor IX
Postmarketing Reports
Blood and lymphatic system disorders: Disseminated intravascular coagulation
Immune system disorder: Anaphylactic/anaphylactoid reactions, hypersensitivity
Nervous system disorders: Headache
Cardiac disorders: Tachycardia
Vascular disorders: Thromboembolic events (including DVT, PE, thrombotic stroke), flushing
Respiratory, thoracic, and mediastinal disorders: Dyspnea, bronchospasm, wheezing, cough
Gastrointestinal disorders: Abdominal pain, nausea
Skin and subcutaneous tissue disorders: Angioedema, facial edema, rash, pruritus
Renal and urinary disorders: Nephrotic syndrome
General disorders and administration site conditions: Infusion site reactions, including pain at infusion site
Warnings
Contraindications
Hypersensitivity
Bebulin, Bebulin VH: Known allergy to heparin or history of heparin-induced thrombocytopenia
Cautions
Made from pooled human plasma, it may carry a risk of transmitting infectious agents (eg, viruses), and theoretically, the Creutzfeldt-Jakob disease (CJD) agent
Thromboembolic events (DVT, PE, thrombotic stroke) as well as DIC reported
Factor IX complex products may partically counteract the effect of warfarin treatment; does NOT include adequate FVII levels to be used to reverse warfarin-induced bleeding
Formation of circulating antibodies inhibiting Factor IX reported
Pregnancy and lactation
Pregnancy category: C
Lactation: Unknown if distributed in human breast milk
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.
Pharmacology of Bebulin VH, Profilnine SD (Factor IX complex)
Mechanism of action
Mixture of vitamin K-dependent clotting factors; temporarily increases the plasma levels of Factor IX, thus minimizing the hazards of hemorrhage in patients with hemophilia B
Administration
IV Preparation
Remove lyophilized powder and diluent from refrigerator and bring to room temperature
Reconstitute lyophilized product with provided diluent
Do not refrigerate after reconstitution
The solution does not contain a preservative and must be used within 3 hr of reconstitution
IV Administration
Infuse at IV rate comfortable to the patient, not to exceed 2 mL/min
Discard partially used vials
Storage
Unopened vials (Bebulin)
- Stable until date indicated on package
- Store between 2-8°C [34-46°F]) in original carton and protect from light
- Do not freeze
Unopened vials (Profilnine SD)
- Stable for 3 years, up to the expiration date printed on its label, provided storage temperature does not exceed 25°C [77°F])
- Do not freeze
