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	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
Stimate2	150mcg/spray	soln for nasal spray	Adults and Children: <11mos: not recommended.Give test dose prior to initiation. >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.
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. Max infusion rate: 4mL/min. Surgery: see full labeling. 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. 1/2020)

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