Bleeding Disorder Treatments: Hemophilia A- MPR

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.
Esperoct	500 IU, 1000 IU, 1500 IU, 2000 IU, 3000 IU	lyophilized pwd for IV infusion after reconstitution	Adults and Children: <12yrs: Bleeding: 65 IU/kg once; (Minor): one dose is sufficient; (Moderate): an additional dose may be given after 24hrs; (Major): additional dose(s) may be given every 24hrs. Perioperative: 65 IU/kg once; (Minor): additional dose(s) can be given after 24hrs; (Major): additional dose(s) can be given approx. every 24hrs for the first week, then approx. every 48hrs until wound has healed. Routine prophylaxis: 65 IU/kg twice weekly; then may adjust individually based on bleeding episodes. ≥12yrs: Bleeding (Minor): 40 IU/kg once; (Moderate): 40 IU/kg once; an additional dose may be given after 24hrs; (Major): 50 IU/kg once; additional dose(s) may be given every 24hrs. Perioperative: 50 IU/kg once; (Minor): additional dose(s) can be given after 24hrs; (Major): additional dose(s) can be given approx. every 24hrs for the first week, then approx. every 48hrs until wound has healed. Routine prophylaxis: initially 50 IU/kg every 4 days; then may adjust individually based on bleeding episodes. Also to achieve a specific target FVIII activity level, calculate dose using: Dosage Required (IU) = Body Weight (kg) × Desired FVIII Increase (IU/dL or % of Normal) × 0.5.
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.
Jivi	500 IU, 1000 IU, 2000 IU, 3000 IU	lyophilized pwd for IV inj after reconstitution	Adults and Children: Dose (IU) = Body Weight (kg) × Desired Factor VIII Increase × Reciprocal of expected recovery (or observed recovery). Bleeding (Minor): obtain 20–40% FVIII increase; give 10–20 IU/kg every 24–48hrs until resolved; (Moderate): obtain 30–60% FVIII increase; give 15–30 IU/kg every 24–48hrs until resolved; (Major): obtain 60–100% FVIII increase; give 30–50 IU/kg every 8–24hrs until resolved. Perioperative (Minor): obtain 30–60% (pre- and post-op) FVIII increase; give 15–30 IU/kg every 24hrs for ≥1 day until healed; (Major): obtain 80–100% (pre- and post-op) FVIII increase; give 40–50 IU/kg every 12–24hrs until adequate healing completed, then continue for ≥7 days to maintain FVIII activity of 30–60%. Routine prophylaxis: initially 30–40 IU/kg twice weekly; may adjust to 45–60 IU/kg every 5 days based on bleeding episodes, then may further adjust individually. Max infusion rate 2.5mL/min. Max dose per infusion: 6000 IU.
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.
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. Dose (IU) = Body Weight (kg) × Desired % FVIII Increase × 0.5. Individualize. Bleeding (Minor): obtain 20–40% FVIII increase every 12–24hrs for ≥1 day depending on severity; (Moderate): obtain 30–60% FVIII increase every 12–24hrs for 3–4 days or until hemostasis achieved; (Major): obtain 60–100% FVIII increase every 8–24hrs until resolved. Peri-op (Minor): obtain 30–60% FVIII increase every 12–24hrs for 3–4 days or until hemostasis achieved (for tooth extraction: a single dose plus oral antifibrinolytic therapy within 1hr may be sufficient); (Major): obtain 60–100% FVIII increase every 8–24hrs until resolved or adequate healing achieved. Routine prophylaxis (<12yrs): initially 25 IU/kg every other day (more frequent or higher doses may be required); (≥12yrs): initially 30 IU/kg 3 times weekly. Adjust dose based on response.
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/Fc-VWF-XTEN FUSION PROTEIN
Altuviiio⁹	250 IU, 500 IU, 750 IU, 1000 IU, 2000 IU, 3000 IU, 4000 IU	lyophilized pwd for IV inj after reconstitution	Adults and Children: Dose (IU) = Body Weight (kg) x Desired Factor VIII Increase (IU/dL or % normal) x 0.5. Minor and moderate bleed: 50 IU/kg once; use 30 IU/kg if bleeding episode occurs within 2–3 days after prophylactic dose; may consider additional doses of 30 or 50 IU/kg every 2–3 days. Major bleed: 50 IU/kg once; may consider additional doses of 30 or 50 IU/kg every 2–3 days. May resume prophylaxis after ≥72 hours interval. Perioperative (Minor): 50 IU/kg once; may consider additional doses of 30 or 50 IU/kg after 2–3 days; (Major): 50 IU/kg once; may give additional doses of 30 or 50 IU/kg every 2–3 days, as clinically needed. Routine prophylaxis: 50 IU/kg once weekly. Max infusion rate 6mins/vial (<20kg); 2–3mins/vial (≥20kg); 1–2mins/vial (adults and adolescents).
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; (Major): 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 30–50 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.
Wilate	500 IU FVIII + 500 IU VWF:RCo, 1000 IU FVIII + 1000 IU VWF:RCo	pwd for IV inj after reconstitution	<12yrs: contact manufacturer. ≥12yrs: Minor bleed: 30–40 IU/kg every 12–24hrs for ≥1 day. Moderate bleed: 30–40 IU/kg every 12–24hrs for ≥3–4 days. Major bleed: 35–50 IU/kg every 12–24hrs for ≥3–4 days. Life-threatening: 35–50 IU/kg every 8–24hrs until resolved. Routine prophylaxis: 20–40 IU/kg every 2–3 days.
ANTI-INHIBITOR COAGULANT COMPLEX
Feiba^6,7	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
DDAVP⁸	4mcg/mL	soln for inj or IV infusion after dilution	Adults and Children: <3mos:not recommended. ≥3mos: 0.3mcg/kg (max 20mcg) IV over 15–30mins. Pre-op: give 30mins before scheduled procedure. If used to reduce spontaneous or traumatic bleeding, may repeat doses after 8–12hrs and once daily thereafter based on clinical response
EMICIZUMAB-KXWH
Hemlibra	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 first 4wks, then 1.5mg/kg once weekly, or 3mg/kg once every 2wks, or 6mg/kg once every 4wks.
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.
Sevenfact⁶	1mg, 5mg	lyophilized pwd for IV infusion after reconstitution	Adults: Give by IV bolus over ≤2mins. Mild/moderate bleeding: 75mcg/kg every 3hrs until hemostasis is achieved; or initially 225mcg/kg, if hemostasis is not achieved within 9hrs, additional 75mcg/kg may be given every 3hrs as needed to achieve hemostasis. Consider alternative treatments if bleeding is not controlled within 24hrs of first administration. Severe bleeding: initially 225mcg/kg, if hemostasis is not achieved after 6hrs, give 75mcg/kg every 2hrs until achieved. Both: cumulative daily doses >900mcg/kg have not been studied. Children: <12yrs: not established.
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 coagulant activity levels >5% without factor VIII antibodies. ⁹ Not for von Willebrand disease. 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. (Rev. 7/2023)

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