Bleeding Disorder Treatments: Hemophilia B

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BLEEDING DISORDER TREATMENTS: Hemophilia B
BLEEDING DISORDER TREATMENTS: HEMOPHILIA B
Brand Strength Form Dose
ANTI-INHIBITOR COAGULANT COMPLEX
Feiba1,2 500 units,1000 units, 2500 units lyophilized pwd for IV infusion after reconstitution Adults and Children: Joint hemorrhage: 50–100units/kg every 12hrs until improved. Mucous membrane bleed: 50–100units/kg every 6hrs for ≥1 day or until resolved. Soft tissue hemorrhage: 100units/kg every 12hrs until resolved. Other severe hemorrhage (eg, CNS bleeds): 100units/kg every 6–12hrs until resolved. Pre-op: 50–100units/kg once immediately prior to surgery. Post-op: 50–100units/kg every 6–12hrs until resolved and healed. Routine prophylaxis: 85units/kg every other day. All: Max 200units/kg/day (100units/kg/dose).
COAGULATION FACTOR IX
AlphaNine SD7 250 IU, 500 IU, 1000 IU, 1500 IU lyophilized concentrate for IV infusion after reconstitution Adults: Dose (IU) = Body Weight (kg) × Desired Factor IX increase × 1 IU/kg. Individualize. Mild hemorrhage: increase FIX 20–30% (20–30 IU/kg twice daily) for 1–2 days or until resolved. Moderate hemorrhage: increase FIX 25–50% (25–50 IU/kg twice daily) for 2–7 days. Major hemorrhage: increase FIX 50% (30–50 IU/kg twice daily) for 3–5 days then maintain at 20% (20 IU/kg twice daily) for up to 10 days. Surgery: pre-op: increase FIX 50–100% (50–100 IU/kg twice daily), then maintain at 50–100% for 7–10 days or until healed. Max infusion rate: 10mL/min.
Children: See full labeling.
BeneFix3 250 IU, 500 IU, 1000 IU, 2000 IU lyophilized pwd for IV infusion after reconstitution Adults: Dose (IU) = Body Weight (kg) × Desired Factor IX increase × 1.3 IU/kg. Minor hemorrhage: 20–30% increase every 12–24hrs for 1–2 days. Moderate hemorrhage: 25–50% increase every 12–24hrs for 2–7 days until resolved. Major hemorrhage: 50–100% increase every 12–24 hrs for 7–10 days.
Children: <15yrs: see full labeling. Dose (IU) = body weight (kg) × % FIX increase × 1.4 IU/kg.
Mononine3 500 IU, 1000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired Factor IX increase × 1 IU/kg. Individualize. Minor spontaneous hemorrhage, prophylaxis: increase FIX 15–25%; give up to 20–30 IU/kg once; may repeat in 24hrs if needed. Major trauma, surgery: increase FIX 25–50%; give up to 75 IU/kg every 18–30hrs for up to 10 days.
Rebinyn4 500 IU, 1000 IU, 2000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Minor/moderate bleed: 40 IU/kg as single dose. Major bleed: 80 IU/kg as single dose. Both: may give additional doses of 40 IU/kg. Peri-op: Minor: 40 IU/kg as single dose; repeat if needed; Major: 80 IU/kg as single dose; if needed, repeat doses of 40 IU/kg (in 1–3 day intervals) within first week, then may extend to once weekly until bleeding stops and healing achieved.
Rixubis 250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU lyophilized pwd for IV infusion after reconstitution Adults: Initial dose = body weight (kg) x desired FIX increase × reciprocal of observed recovery (IU/dL per IU/kg). Dose in previously treated patients (IU) = body weight (kg) x desired FIX increase × 1.1 dL/kg. Individualize. Minor bleed: 20–30% required every 12–24hrs for ≥1 day until healing achieved. Moderate bleed: 25–50% required every 12–24hrs for 2–7 days until bleeding stops and healing achieved. Major bleed: 50–100% required every 12–24hrs for 7–10 days until bleeding stops and healing achieved. Peri-op: Minor: 30–60% required every 24hrs for ≥1 day until healing achieved; Major: 80–100% required every 8–24hrs for 7–10 days until bleeding stops and healing achieved. Routine prophylaxis (PTPs): 40–60 IU/kg twice weekly; titrate if needed. Max infusion rate 10mL/min.
Children: Initial dose = body weight (kg) x desired FIX increase × reciprocal of observed recovery (IU/dL per IU/kg). Dose in PTPs (IU) = body weight (kg) x desired FIX increase x 1.4 dL/kg. Individualize. Minor, moderate, major bleed: see Adults: Peri-op: see Adults: Routine prophylaxis (PTPs): 60–80 IU/kg twice weekly; titrate if needed. Max infusion rate 10mL/min.
COAGULATION FACTOR IX/ALBUMIN FUSION PROTEIN
Idelvion5 250 IU, 500 IU, 1000 IU, 2000 IU lyophilized pwd for IV inj after reconstitution Adults and Children: Dose (IU) = body weight (kg) × desired FIX increase x reciprocal of recovery (IU/dL per IU/kg). Individualize. Minor/moderate bleed: 30–60% required; repeat every 48–72hrs for ≥1 day until bleeding stops and healing achieved. Major bleed: 60–100% required; repeat every 48–72hrs for 7–14 days until bleeding stops and healing achieved; give maintenance dose weekly. Peri-op: Minor: 50–80% required; repeat every 48–72hrs for ≥1 day or until healing achieved; Major: initially 60–100% required every 48–72hrs for 7–14 days or until bleeding stops and healing achieved; may repeat every 48–72hrs for the 1st week or until healing achieved; give maintenance dose 1–2 times weekly. Routine prophylaxis (<12yrs): 40–55 IU/kg every 7 days; (≥12yrs): 25–40 IU/kg every 7 days (may give 50–75 IU/kg every 14 days if well tolerated). Max infusion rate: 10mL/min.
COAGULATION FACTOR IX/Fc FUSION PROTEIN
Alprolix6 250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU, 4000 IU lyophilized pwd for IV inj after reconstitution Adults and Children: Dose (IU) = body weight (kg) × desired FIX increase x reciprocal of recovery (IU/kg per IU/dL). Individualize. Minor/moderate bleed: 30–60% required; repeat every 48hrs if needed. Major bleed: 80–100% required; consider repeat dose after 6–10hrs, then every 24hrs for the 1st 3 days; then may reduce dose and frequency to every 48hrs or longer until bleeding stops and healing achieved. Peri-op: Minor: 50–80% required as single dose; repeat as needed after 24–48hrs until bleeding stops and healing achieved; Major: initially 60–100% required; consider repeat dose after 6–10hrs, then every 24hrs for the 1st 3 days; then may reduce dose and frequency to every 48hrs or longer until bleeding stops and healing achieved. Routine prophylaxis (<12yrs): initially 60 IU/kg once weekly; (≥12yrs): initially 50 IU/kg once weekly or 100 IU/kg once every 10 days. Max infusion rate 10mL/min.
COAGULATION FACTOR IX COMPLEX
Bebulin7,8 500 –700 IU freeze-dried concentrate for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired Factor IX increase × 1.2 IU/kg. Minor bleed: 20% increase (25–35 IU/kg) for 1 dose. Moderate bleed: 40% increase (40–55 IU/kg) once, give 2nd dose after 24hrs (continue until resolved). Major bleed: ≥60% increase (60–70 IU/kg) once daily for 2–3 days or until healed. Surgery: give loading dose 1hr prior to surgery; may repeat every 12hrs initially then every 24hrs in late post-op period. Minor: initially 40–60% (50–60 IU/kg), then 20–40% (25–55 IU/kg) for 1–2wks. Major: initially ≥60% increase (70–95 IU/kg) then 20–60% (35–75 IU/kg) for 1–2wks, then 20% (25–35 IU/kg) until healed. Prophylaxis: 20–30 IU/kg 1–2 times weekly. Max infusion rate: 2mL/min.
Profilnine SD7 500 IU, 1000 IU, 1500 IU lyophilized concentrate for IV infusion after reconstitution Adults: Dose (IU) = Body Weight (kg) × Desired Factor IX increase × 1 IU/kg. Individualize. Mild to moderate hemorrhage: increase FIX 20–30% as single dose. Serious hemorrhage: increase FIX 30–50% once daily. Surgery: increase FIX 30–50% for ≥1wk post-op. Dental extraction: increase FIX 50% immediately prior to procedure; may give additional doses if bleeding recurs. Max infusion rate: 10mL/min.
Children: See full labeling.
COAGULATION FACTOR VIIa
NovoSeven RT1 1mg, 2mg, 5mg, 8mg lyophilized pwd for IV inj after recon-stitution Adults and Children: Give by IV bolus only. Individualize. Bleeding: 90mcg/kg every 2hrs, adjust until hemostasis achieved; post-hemostatic dose: continue at 3–6hrs intervals for severe bleeds. Peri-op: initially 90mcg/kg prior to surgery, repeat at 2hr intervals during surgery. Post-op: Minor: every 2hrs for 48hrs, then every 2–6hrs until healed; Major: every 2hrs for 5 days, then every 4hrs until healed.
NOTES

Key: FIX = Factor IX; PTP = previously treated patients

1 For Hemophilia B with inhibitors.

2 Contains Factors II, IX, X (non-activated); Factor VII (activated); Factor VIII inhibitor bypassing activity; Prothrombin Complex Factors.

3 May need higher doses if FIX inhibitors present or low FIX recovery.

4 GlycoPEGylated.

5 Dose adjustment may be needed in children <12yrs.

6 More frequent or higher doses may be needed in children <12yrs (esp. <6yrs).

7 Contains Factor II, Factor VII, Factor X (non-therapeutic levels in AlphaNine).

8 Contains heparin.

Not an inclusive list of medications and/or official indications. Please see drug monograph at www.eMPR.com and/or contact company for full drug labeling.

(Created 4/2018)

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