Bleeding Disorder Treatments: von Willebrand Disease- MPR

Bleeding Disorder Treatments: von Willebrand Disease

BLEEDING DISORDER TREATMENTS: VON WILLEBRAND DISEASE
Brand	Strength	Form	Dose
ANTIHEMOPHILIC FACTOR VIII/VON WILLEBRAND FACTOR COMPLEX
Alphanate1	250 IU, 500 IU, 1000 IU, 1500 IU, 2000 IU	lyophilized pwd for IV inj after reconstitution	Adults: Pre-op: 60 VWF:RCo IU/kg, then 40–60 VWF:RCo IU/kg every 8–12hrs if needed; may reduce dose after 3rd post-op day; treat until healed. Max infusion rate ≤10mL/min. Children: Initially 75 VWF:RCo IU/kg, then 50–75 VWF:RCo IU/kg every 8–12hrs if needed; may reduce dose after 3rd post-op day; treat until healed. Max infusion rate ≤10mL/min.
Humate-P	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 and Children: Type 1 (Mild): major bleed: initially 40–60 IU/kg, then 40–50 IU/kg every 8–12hrs for 3 days, then once daily for a total of 7 days. Type 1 (Moderate or severe): minor bleed: 40–50 IU/kg for 1–2 doses; major bleed: initially 50–75 IU/kg, then 40–60 IU/kg every 8–12hrs for 3 days, then once daily for a total of 7 days. Types 2 and 3: minor bleed: 40–50 IU/kg for 1–2 doses; major bleed: initially 60–80 IU/kg, then 40–60 IU/kg every 8–12hrs for 3 days, then once daily for a total of 7 days. Max infusion rate: 4mL/min. Surgery: see full labeling.
Wilate	500 IU FVIII + 500 IU VWF:RCo, 1000 IU FVIII + 1000 IU VWF:RCo	pwd for IV inj after reconstitution	<5yrs: Contact manufacturer. ≥5yrs: Minor bleed: 20–40 IU/kg once, then 20–30 IU/kg every 12–24hrs as needed. Major bleed: 40–60 IU/kg once, then 20–40 IU/kg every 12–24hrs as needed. Surgery (give loading dose within 3hrs before); Minor: 30–60 IU/kg once, then 15–30 IU/kg (or half the loading dose) every 12–24hrs; Major: 40–60 IU/kg once, then 20–40 IU/kg (or half the loading dose) every 12–24hrs. Usual duration: minor hemorrhage/surgery: 3 days; major hemorrhage: 5–7 days; major surgery: ≥6 days.
DESMOPRESSIN
DDAVP²	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.
VON WILLEBRAND FACTOR
Vonvendi3	650 IU VWF:RCo, 1300 IU VWF:RCo	lyophilized pwd for IV inj after reconstitution	Adults: ≥18yrs: Minor bleed:initially 40–50 IU/kg, then every 8–24hrs as needed. Major bleed:initially 50–80 IU/kg, then 40–60 IU/kg every 8–24hrs for 2–3 days as needed. Routine prophylaxis: initially 40–60 IU/kg twice weekly; adjust dose up to 60 IU/kg twice weekly if breakthrough bleeding occurs in joints or if severe bleeding occurs. Surgery: see full labeling. Max infusion rate: 4mL/min. Children: <18yrs: not established.
NOTES
Key: FVIII = Factor VIII; VWF = von Willebrand factor; VWF:RCo = von Willebrand factor Ristocetin cofactor 1 Not for severe VWD (Type 3) undergoing major surgery. 2 For mild-moderate Type 1 VWD with Factor VIII >5% only. 3 Give recombinant FVIII with first infusion if baseline plasma FVIII level <40% or is unknown. Recombinant FVIII may also be needed for subsequent infusions or prior to surgery. 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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