this present day [12:<12 years of age is supported by data in older pediatric patients. 1 Additional pharmacokinetic and safety data are available in a limited number of pediatric patients 2 11 years of age. 1 Efficacy in patients> <2 years of age may be extrapolated from pharmacokinetic data. 1 No specific safety issues identified in patients> <12 years of age. 1 Geriatric Use Insufficient experience in patients ≥65 years of age to determine whether geriatric patients respond differently than younger patients. 1 Common Adverse Effects Headache, oral paresthesia. 1 Factor IX (Recombinant), Fc fusion protein Pharmacokinetics Absorption Plasma Concentrations Exhibits dose-proportional pharmacokinetics. 8 9 Rapid onset of action with peak plasma concentrations attained immediately after a dose. 5 Mean recovery of factor IX approximately 1 unit/dL for each 1 unit/kg of factor IX (recombinant), Fc fusion protein administered. 1 Compared with adults, patients> <12 years of age appear to have lower incremental recovery of factor IX. 1 In one study, incremental recovery was approximately 0.6, 0.7, or 0.9 units/dL per each unit/kg administered in pediatric patients 2 5 years, 6 11 years, or 12 17 years of age, respectively. 1 Distribution Extent Not known whether factor IX (recombinant), Fc fusion protein is distributed into milk. 1 Elimination Half-life Following administration of a single 50-unit/kg dose in patients with hemophilia B, mean terminal half-life was 86.52 hours; average time to reach 1% factor IX activity was 11.5 days. 1 Compared with adults, patients> <12 years of age appear to have higher body weight-adjusted clearance and shorter half-life. 1 In one study, half-life was approximately 66, 72, or 84 hours in patients 2 5 years, 6 11 years, or 12 17 years of age, respectively. 1 In a comparative pharmacokinetic analysis, terminal half-life of factor IX (recombinant), Fc fusion protein was substantially longer than that of a conventional recombinant factor IX preparation (82 versus 33.8 hours). 4 9 Stability Storage Parenteral Powder for Injection 2 8°C; do not freeze or expose to direct sunlight. 1 Freezing may damage prefilled diluent syringe. 1 May store at room temperature ( 30°C) for a single period of up to 6 months within expiration dates. 1 Do not place product back under refrigeration after warming to room temperature. 1 Reconstituted Solution May store reconstituted solution at room temperature ( 30°C) prior to administration; protect from direct sunlight. 1 Use solution within 3 hours of reconstitution; do not refrigerate reconstituted solution. 1 Actions Biosynthetic (recombinant DNA-origin) preparation of blood coagulation factor IX covalently linked to the Fc domain of IgG 1 . 1 7 10 13 16 Fc portion prolongs half-life of factor IX through interaction with the Fc neonatal receptor. 1 5 7 8 9 13 Factor IX portion is similar in structure and function to endogenous factor IX. 1 Patients with hemophilia B have decreased levels of endogenous factor IX, resulting in a hemorrhagic tendency and clinical manifestations such as bleeding into soft tissues, muscles, joints, and internal organs. 1 8 171 Clinical severity and frequency of bleeding generally correlate with degree of deficiency of factor IX activity. 171 Patients with mild hemophilia B generally have >5% of normal activity, those with moderate disease generally have 1 5% of normal activity, and those with severe disease have> <1% of normal activity. 171 Administration of factor IX (recombinant), Fc fusion protein to patients with hemophilia B increases plasma levels of factor IX and temporarily corrects coagulation defect. 1 Also may shorten aPTT, which is typically prolonged in patients with hemophilia B. 1 Produced by recombinant DNA technology in a human embryonic kidney (HEK) cell line; undergoes several purification processes (e.g., nanofiltration, column chromatography) and is manufactured without human and animal proteins. 1 8 13 155 156 Advice to Patients Importance of advising patients to read the manufacturer-provided patient information and instructions for use. 1 Importance of patients reporting to their clinician any adverse reactions or other issues following administration of factor IX (recombinant), Fc fusion protein. 1 Importance of discontinuing therapy and informing a clinician if any manifestations of hypersensitivity (e.g., hives, chest tightness, wheezing, difficulty breathing, swelling of the face) or anaphylaxis occur. 1 Possible development of inhibitors; advise patients to inform clinician if they experience a lack of response to factor IX (recombinant), Fc fusion protein therapy. 1 Importance of informing clinician of existing or contemplated concomitant therapy, including prescription and OTC drugs, as well as any concomitant illnesses. 1 Importance of women informing their clinician if they are or plan to become pregnant or plan to breast-feed. 1 Importance of informing patients of other important precautionary information. 1 (See Cautions.) Preparations Excipients in commercially available drug preparations may have clinically important effects in some individuals; consult specific product labeling for details. Please refer to the ASHP Drug Shortages Resource Center for information on shortages of one or more of these preparations. Factor IX (Recombinant), Fc Fusion Protein Routes Dosage Forms Strengths Brand Names Manufacturer Parenteral For injection, for IV use only number of units indicated on label (nominally 250, 500, 1000, 2000, or 3000 units) Alprolix (with prefilled diluent syringe; available with vial adapter) Biogen AHFS DI Essentials. Copyright 2017, Selected Revisions February 24, 2017. American Society of Health-System Pharmacists, Inc., 4500 East-West Highway, Suite 900, Bethesda, Maryland 20814. References 1. Biogen Idec. Alprolix coagulation factor IX (recombinant), Fc fusion protein prescribing information. Cambridge, MA; 2016 Feb. 2. Biogen Idec. Alprolix coagulation factor IX (recombinant), Fc fusion protein instructions for use. Cambridge, MA; 2014 Mar . 3. Food and Drug Administration. Orphan designations pursuant to Section 526 of the Federal Food and Cosmetic Act as amended by the Orphan Drug Act (P.L. 97-414). Rockville, MD. From FDA web site. 4. Powell JS, Pasi KJ, Ragni MV et al. Phase 3 study of recombinant factor IX Fc fusion protein in hemophilia B. N Engl J Med . 2013; 369:2313-23. [PubMed 24304002] 5. Shapiro AD, Ragni MV, Valentino LA et al. Recombinant factor IX-Fc fusion protein (rFIXFc) demonstrates safety and prolonged activity in a phase 1/2a study in hemophilia B patients. Blood . 2012; 119:666-72. [PubMed 22110246] 6. Powell JS, Apte S, Chambost H et al. Long-acting recombinant factor IX Fc fusion protein (rFIXFc) for perioperative management of subjects with haemophilia B in the phase 3 B-LONG study. Br J Haematol . 2014; :. [PubMed 25208598] 7. Mancuso ME, Mannucci PM. Fc-fusion technology and recombinant FVIII and FIX in the management of the hemophilias. Drug Des Devel Ther . 2014; 8:365-71. [PubMed 24729686] 8. Valentino LA. Recombinant FIXFc: a novel therapy for the royal disease?. Expert Opin Biol Ther . 2011; 11:1361-8. [PubMed 21780945] 9. Ducore JM, Miguelino MG, Powell JS. Alprolix (recombinant Factor IX Fc fusion protein): extended half-life product for the prophylaxis and treatment of hemophilia B. Expert Rev Hematol . 2014; 7:559-71. [PubMed 25142322] 10. Peters RT, Low SC, Kamphaus GD et al. Prolonged activity of factor IX as a monomeric Fc fusion protein. Blood . 2010; 115:2057-64. [PubMed 20056791] 11. Miguelino MG, Powell JS. Clinical utility and patient perspectives on the use of extended half-life rFIXFc in the management of hemophilia B. Patient Prefer Adherence . 2014; 8:1073-83. [PubMed 25143713] 12. Powell J, Shapiro A, Ragni M et al. Switching to recombinant factor IX Fc fusion protein prophylaxis results in fewer infusions, decreased factor IX consumption and lower bleeding rates. Br J Haematol . 2014; :. [PubMed 25209873] 13. McCue J, Osborne D, Dumont J et al. Validation of the manufacturing process used to produce long-acting recombinant factor IX Fc fusion protein. Haemophilia . 2014; 20:e327-35. [PubMed 24811361] 14. Sommer JM, Buyue Y, Bardan S et al. Comparative field study: impact of laboratory assay variability on the assessment of recombinant factor IX Fc fusion protein (rFIXFc) activity. Thromb Haemost . 2014; 112:. [PubMed 25144892] 15. Wyeth. BeneFIX coagulation factor IX (recombinant) prescribing information. Philadelphia, PA; 2012 March. 16. Shapiro A. Development of long-acting recombinant FVIII and FIX Fc fusion proteins for the management of hemophilia. Expert Opin Biol Ther . 2013; 13:1287-97. [PubMed 23930915] 17. Carcao M. Changing paradigm of prophylaxis with longer acting factor concentrates. Haemophilia . 2014; 20 Suppl 4:99-105. [PubMed 24762284] 155. Medical and Scientific Advisory Council (MASAC), National Hemophilia Foundation. MASAC recommendation regarding the use of recombinant clotting factor products with respect to pathogen transmission (May 6, 2014). MASAC recommendation #226. From National Hemophilia Foundation website. 156. Medical and Scientific Advisory Council (MASAC), National Hemophilia Foundation. MASAC recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders (revised April 2014). MASAC recommendation #225. From National Hemophilia Foundation website. 171. World Federation of Hemophilia. Guidelines for the management of hemophilia 2nd edition. 2012. From the World Federation of Hemophilia website. 176. Medical and Scientific Advisory Council (MASAC), National Hemophilia Foundation. MASAC recommendation concerning prophylaxis (regular administration of clotting factor concentrate to prevent bleeding) (November 4, 2007). MASAC recommendation #179. From National Hemophilia Foundation website. 185. Carcao MD, Aledort L. Prophylactic factor replacement in hemophilia. Blood Rev . 2004; 18:101-13. [PubMed 15010149] 186. Nilsson IM, Berntorp E, Löfqvist T et al. Twenty-five years experience of prophylactic treatment in severe haemophilia A and B. J Intern Med . 1992; 232:25-32. [PubMed 1640190] 187. Medical and Scientific Advisory Council (MASAC), National Hemophilia Foundation. MASAC recommendations regarding factor concentrate prescriptions and formulary development and restrictions (March 12, 2005). MASAC recommendation #159. From National Hemophilia Foundation website. Next Interactions Print this page Add to My Med List More about coagulation factor ix Side Effects During Pregnancy Dosage Information Drug Interactions Support Group En Español 2 Reviews Add your own review/rating Drug class: miscellaneous coagulation modifiers Consumer resources Coagulation factor IX ... +7 more Professional resources Coagulation Factor IX (Recombinant, Human), Albumin Fusion Protein (rIX-FP) (AHFS Monograph) Factor IX (Human), Factor IX Complex (Human) (AHFS Monograph) Factor IX (Recombinant) (AHFS Monograph) Factor IX (Human) (Wolters Kluwer) Other brands: BeneFix , Alprolix , Alphanine SD , Idelvion , ... +2 more Related treatment guides Factor IX Deficiency Hemophilia B> ]} FEATURED: CAR-T Cell Therapy Overview Mechanism of Action KTE-C19 Studies KTE-C19 Cancer Targets Adverse Events Manufacturing Drug Status Rx Availability Prescription only C Pregnancy Category Risk cannot be ruled out N/A CSA Schedule Not a controlled drug Drug Class Miscellaneous coagulation modifiers Related Drugs Factor IX Deficiency tranexamic acid , BeneFix , Cyklokapron , Profilnine SD , Alphanine SD , factor ix complex , More... 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