BLEEDING DISORDER TREATMENTS: HEMOPHILIA A

BLEEDING DISORDER TREATMENTS: HEMOPHILIA A
Brand Strength Form Dose
ANTIHEMOPHILIC FACTOR VIII1
Advate 250 IU, 500 IU, 1000 IU, 1500 IU, 2000 IU, 3000 IU pwd for IV inj after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Hemorrhage (Mild): obtain 20–40% FVIII increase every 12–24hrs for 1–3 days until resolved; (Moderate): obtain 30–60% FVIII increase every 12–24hrs for 3 days or until resolved; (Major): obtain 60–100% FVIII increase every 8–24hrs until resolved. Peri-op: Minor: obtain 60–100% FVIII increase as single bolus within 1hr of surgery, then every 12–24hrs as needed; Major: pre- and post-op: obtain 80–120% FVIII increase; give pre-op and maintenance bolus, then repeat every 8–24hrs based on healing. Routine prophylaxis: 20–40 IU/kg every other day (3–4 times weekly); or may give every 3rd day. Max infusion rate 10mL/min.
Adynovate 250 IU, 500 IU, 750 IU, 1000 IU, 2000 IU PEGylated lyophilized pwd for IV inj after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Bleeding (Minor): obtain 20–40% FVIII increase every 12–24hrs until resolved; (Moderate): obtain 30–60% FVIII increase every 12–24hrs until resolved; (Major): obtain 60–100% FVIII increase every 8–24hrs until resolved. Peri-op: (Minor): obtain 60–100% FVIII increase; give 1hr before surgery, repeat after 24hrs if needed until bleeding resolved; (Major): obtain 80–120% FVIII increase (pre- and post-op); give 1hr before surgery, repeat every 8–24hrs (6–24hrs if <12yrs) until adequate wound healing. Routine prophylaxis (<12yrs): 55 IU/kg twice weekly; (≥12yrs): 40–50 IU/kg twice weekly; max 70 IU/kg. Max infusion rate 10mL/min.
Afstyla 250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Bleeding (Minor): obtain 20–40% FVIII increase; may repeat every 12–24hrs until resolved; (Moderate): obtain 30–60% FVIII increase; may repeat every 12–24hrs until resolved; (Major): obtain 60–100% FVIII increase; may repeat every 8–24hrs until resolved. Peri-op (Minor): obtain 30–60% FVIII increase; may repeat every 24hrs for ≥1 day until healed; (Major): obtain 80–100% FVIII increase; may repeat every 8–24hrs until adequately healed, then continue for ≥7 days to maintain Factor VIII activity of 30–60%. Routine prophylaxis(<12yrs): 30–50 IU/kg 2–3 times weekly (more frequent or higher doses may be required); (≥12yrs): 20–50 IU/kg 2–3 times weekly. Max infusion rate 10mL/min.
Helixate FS 250 IU, 500 IU, 1000 IU dried concentrate for IV infusion after reconstitution Adults and Children: Minor hemorrhage: 10–20 IU/kg; may repeat dose if needed. Moderate/major hemorrhage or minor surgery: 15–30 IU/kg; may repeat 1 dose at 12–24hrs if needed. Major/life-threatening hemorrhage, fractures or head trauma: initially 40–50 IU/kg, then 20–25 IU/kg every 8–12hrs. Major surgery: pre-op dose: 50 IU/kg2; may repeat after 6–12hrs initially, and for 10–14 days until completely healed.
Hemofil M 220–400 IU, 401–800 IU, 801–1700 IU, 1701–2000 IU dried concentrate for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Individualize. Hemorrhage (Mild): obtain 20–40% FVIII increase every 12–24hrs for 1–3 days until resolved; (Moderate): obtain 30–60% FVIII increase every 12–24hrs for 3 days or until resolved; (Life-threatening): obtain 60–100% FVIII increase every 8–24hrs until resolved. Surgery (Minor): obtain 60–80% FVIII increase as single infusion plus oral antifibrinolytic therapy within 1hr; (Major): pre- and post-op: obtain 80–100% FVIII increase; repeat every 8–24hrs based on healing. Max infusion rate 10mL/min.
Koate-DVI 250 IU, 500 IU, 1000 IU dried concentrate for IV infusion after reconstitution Adults: Hemorrhage (Mild): 10 IU/kg as single dose; (Moderate): 15–25 IU/kg, then 10–15 IU/kg every 8–12hrs if needed; (Severe): initially 40–50 IU/kg, then 20–25 IU/kg every 8–12hrs. Major surgery: pre-op dose: 50 IU/kg2; may repeat every 6–12hrs initially and for 10–14 days until healing complete.
Children: Not recommended.
Kogenate FS 250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Minor hemorrhage: 10–20 IU/kg; may repeat dose if needed. Moderate hemorrhage or minor surgery: 15–30 IU/kg; may repeat dose every 12–24hrs until resolved. Major hemorrhage, fractures or head trauma: initially 40–50 IU/kg, then 20–25 IU/kg every 8–12hrs until resolved. Major surgery:pre-op: 50 IU/kg2; repeat if needed after 6–12hrs initially, and for 10–14 days until completely healed. Routine prophylaxis (children): 25 IU/kg every other day.
Kovaltry 250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Individualize. Bleeding (Minor): obtain 20–40% FVIII increase; may repeat every 12–24hrs for ≥1 day until resolved or healing achieved; (Moderate): obtain 30–60% FVIII increase; may repeat every 12–24hrs for 3–4 days until resolved; (Major): obtain 60–100% FVIII increase; may repeat every 8–24hrs until resolved. Peri-op (pre- and post-op): Minor: obtain 30–60% FVIII increase; may repeat every 24hrs for ≥1 day until healed; Major: obtain 80–100% FVIII increase; may repeat every 8–24hrs until adequately healed, then continue for ≥7 days to maintain Factor VIII activity of 30–60%. Routine prophylaxis (≤12yrs): 25–50 IU/kg 2–3 times weekly or every other day; (>12yrs): 20–40 IU/kg 2–3 times weekly.
Monoclate-P 250 IU, 500 IU, 1000 IU, 1500 IU lyophilized concentrate for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Individualize. Mild hemorrhage: attain 30% FVIII increase as single dose. Moderate hemorrhage or minor surgery: initially 15–25 IU/kg, then 10–15 IU/kg every 8–12hrs if needed. Severe hemorrhage: initially 40–50 IU/kg, then 20–25 IU/kg every 8–12hrs. Major surgery: give 1st dose 1hr pre-op to attain 80–100% FVIII increase, then give a ½ dose 5hrs after 1st dose; maintain daily at ≥30% FVIII increase for 10–14 days post-op. Max infusion rate 2mL/min.
NovoEight 250 IU, 500 IU, 1000 IU, 1500 IU, 2000 IU, 3000 IU lyophilized pwd for IV inj after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired Factor VIII Increase × 0.5. Bleeding (Minor): obtain 20–40% FVIII increase every 12–24hrs for ≥1 day until resolved; (Moderate): obtain 30–60% FVIII increase every 12–24hrs for 3–4 days until resolved; (Major): obtain 60–100% FVIII increase every 8–24hrs for 7–10 days until resolved. Peri-op (Minor): obtain 30–60% FVIII increase every 24hrs for ≥1 day until healed; (Major): pre- and post-op: obtain 80–100% FVIII increase every 8–24hrs until adequate wound healing, then continue for ≥7 days to maintain FVIII activity of 30–60%. Routine prophylaxis (<12yrs): 25–60 IU/kg 3 times weekly or 25–50 IU/kg every other day; (≥12yrs): 20–50 IU/kg 3 times weekly or 20–40 IU/kg every other day.
Nuwiq 250 IU, 500 IU, 1000 IU, 2000 IU, 2500 IU, 3000 IU, 4000 IU lyophilized pwd for IV inj after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired Factor VIII Increase × 0.5. Bleeding (Minor): obtain 20–40% FVIII increase every 12–24hrs for ≥1 day until resolved; (Moderate to major): obtain 30–60% FVIII increase every 12–24hrs for 3–4 days or more until resolved; (Life-threatening): obtain 60–100% FVIII increase every 8–24hrs until resolved. Peri-op (pre- and post-op): Minor: obtain 30–60% FVIII increase every 24hrs for ≥1 day until healed; Major: obtain 80–100% FVIII increase every 8–24hrs until adequate wound healing, then continue for ≥7 days to maintain FVIII activity of 30–60%. Routine prophylaxis (2–11yrs): 30–50 IU/kg every other day or 3 times weekly; (≥12yrs): 30–40 IU/kg every other day. Max infusion rate 4mL/min.
Obizur3 500 Units lyophilized pwd for IV inj after reconstitution Adults: Minor and moderate bleed: initially 200 Units/kg every 4–12hrs; titrate subsequent doses to maintain 50–100 Units/dL. Major bleed: initially 200 Units/kg every 4–12hrs; titrate subsequent doses to maintain 100–200 Units/dL (to treat acute bleed) or 50–100 Units/dL (after acute bleed is controlled, if required).
Children: Not established.
Recombinate 250 IU, 500 IU, 1000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Hemorrhage (Mild): obtain 20–40% FVIII increase every 12–24hrs for 1–3 days until resolved; (Moderate): obtain 30–60% FVIII increase every 12–24hrs for 3 days or until resolved; (Life-threatening): obtain 60–100% FVIII increase every 8–24hrs until resolved. Surgery (Minor): obtain 60–80% FVIII increase as a single dose plus oral antifibrinolytic therapy within 1hr; (Major): pre- and post-op: obtain 80–100% FVIII increase; repeat every 8–24hrs based on healing. Max infusion rate 10mL/min.
Refacto 250 IU, 500 IU, 1000 IU, 2000 IU lyophilized pwd for IV infusion after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Individualize. Minor hemorrhage: obtain 20–40% FVIII increase every 12–24hrs for ≥1 day until resolved. Moderate hemorrhage and tooth extraction: obtain 30–60% FVIII increase every 12–24hrs for 3–4 days until adequate hemostasis (a single dose plus oral antifibrinolytic therapy within 1hr may be sufficient for tooth extraction). Major hemorrhage: obtain 60–100% FVIII increase every 8–24hrs until resolved; or, for surgery, until local hemostasis achieved. Prophylaxis: give ≥2 times weekly; children may need shorter dosage intervals or higher doses.
Xyntha4 250 IU, 500 IU, 1000 IU, 2000 IU lyophilized pwd for IV inj after reconstitution Adults and Children: One IU of FVIII per kg raises the plasma FVIII activity by ~2 IU/dL. Individualize. Bleeding (Minor): obtain 20–40 IU/dL or % of normal FVIII increase, repeat every 12–24hrs as needed for ≥1 day until resolved; (Moderate): 30–60 IU/dL or % of normal; repeat every 12–24hrs for 3–4 days or until hemostasis; (Major): 60–100 IU/dL or % of normal, repeat every 8–24hrs until resolved. Peri-op (Minor): 30–60 IU/dL or % of normal, repeat every 12–24hrs for 3–4 days or until hemostasis; (Major): 60–100 IU/dL or % of normal; repeat every 8–24hrs until hemostasis and wound healing occurs.
Xyntha Solofuse4 250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU lyophilized pwd in prefilled syringe for IV inj after reconstitution
ANTIHEMOPHILIC FACTOR VIII/Fc FUSION PROTEIN
Eloctate5 250 IU, 500 IU, 750 IU, 1000 IU, 1500 IU, 2000 IU, 3000 IU lyophilized pwd for IV inj after reconstitution Adults and Children: Dose (IU) = Body Weight (kg) × Desired % Factor VIII Increase × 0.5. Individualize. Hemorrhage (Minor/moderate): obtain 40–60% FVIII increase; give 20–30 IU/kg every 24–48hrs until resolved; (Major): obtain 80–100% FVIII increase; give 40–50 IU/kg every 12–24hrs until resolved (~7–10 days). Peri-op (Minor): obtain 50–80% FVIII increase; give 25–40 IU/kg every 24hrs for ≥1 day until healing achieved; (Major): obtain 80–120% FVIII increase (pre- and post-op); give pre-op dose (40–60 IU/kg) followed by repeat dose (40–50 IU/kg) after 8–24hrs, then every 24hrs to maintain target range; give until adequate wound healing, then continue for ≥7 days to maintain target range. Routine prophylaxis: 50 IU/kg every 4 days; may adjust to range of 25–65 IU/kg at 3–5 day intervals based on response. Max infusion rate 10mL/min.
ANTIHEMOPHILIC FACTOR VIII/VON WILLEBRAND FACTOR COMPLEX
Alphanate 250 IU, 500 IU, 1000 IU, 1500 IU lyophilized pwd for IV inj after reconstitution Adults: Hemorrhage (Minor): 15 FVIII IU/kg twice daily for 1–2 days; (Moderate): 25 FVIII IU/kg twice daily for 2–7 days; (Severe): 40–50 FVIII IU/kg twice daily for ≥3–5 days, then 25 FVIII IU/kg twice daily until healed (up to 10 days). Surgery: 40–50 FVIII IU/kg prior to surgery, then 25–50 FVIII IU/kg twice daily for 7–10 days or until healed. Max infusion rate ≤10mL/min.
Children: Not established.
Humate-P1 250 IU FVIII + 600 IU VWF, 500 IU FVIII + 1200 IU VWF, 1000 IU FVIII + 2400 IU VWF lyophilized pwd for IV infusion after reconstitution Adults: Minor bleed: 15 IU FVIII/kg (obtain 30% FVIII increase) once; if needed, may give ½ dose once or twice daily for 1–2 days. Moderate bleed: initially 25 IU FVIII/kg (obtain 50% FVIII increase), then 15 IU FVIII/kg (maintain 30% FVIII increase) every 8–12hrs for 1–2 days, then repeat for 1–2 times daily for a total of 7 days or until healed. Severe bleed: initially 40–50 IU FVIII/kg, then 20–25 IU FVIII/kg every 8hrs (maintain 80–100% FVIII increase) for 7 days, then repeat for 1–2 times daily for additional 7 days (maintain 30–50% FVIII increase). Max infusion rate 4mL/min.
Children: Not established.
ANTI-INHIBITOR COAGULANT COMPLEX
Feiba6,7 500 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).
DESMOPRESSIN
Stimate8 150mcg/spray soln for nasal spray Adults and Children: <11mos: Not recommended.
Give test dose prior to initiating therapy. >11mos: <50kg: 1 spray in one nostril (150mcg). ≥50kg: 1 spray per nostril (300mcg). May repeat dose based on clinical response. Pre-op: give 2hrs prior to procedure.
EMICIZUMAB-KXWH
Hemlibra6 30mg/mL, 60mg/0.4mL, 105mg/0.7mL, 150mg/mL soln for SC inj Adults and Children: Give by SC inj into upper outer arms, thighs, or any abdomen quadrant. 3mg/kg once weekly for 1st 4wks, then 1.5mg/kg once weekly.
RECOMBINANT COAGULATION FACTOR VIIa
NovoSeven RT6 1mg, 2mg, 5mg, 8mg lyophilized pwd for IV inj after reconstitution 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: FVIII=Factor VIII; VWF=von Willebrand factor

1 Confirm Factor VIII deficiency prior to treatment. Monitor for development of Factor VIII inhibitors.
2 Verify 100% Factor VIII activity prior to surgery.
3 Porcine sequence.
4 Contains polysorbate 80.
5 Higher or more frequent dosing may be needed in children <6yrs.
6 For hemophilia A with inhibitors.
7 Contains Factors II, IX, X (non-activated); Factor VII (activated); Factor VIII inhibitor bypassing activity; Prothrombin Complex Factors.
8 For Hemophilia A with Factor VIII levels >5%.

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)