Avoid ASA, NSAIDs, heparin and coordinate with hematology prior to any invasive or surgical procedures.
Multiple therapeutic options exist for prophylaxis or treatment of bleeding.  The majority of therapy utilizes Humate-P and/or desmopressin
VWF and factor VIII concentration is the first line therapy for<unk>  bleeding patients. It is contraindicated for any patient with prior history of anaphylaxis to Humate-p
Loading dose 40 to 60 IU/kg, then 40 to 50 IU/kg every 8 to 12 hours for 3 days to keep the trough level of VWF:RCo >50%; then 40 to 50 IU/kg daily for a total of up to 7 days of treatment. 
For severe bleeding the loading dose is increased to 50 to 75 IU/kg
Consider if replacement therapy instituted and persistent bleeding
Induces release of vWF from endothelial storage sites
0.3mcg/kg  IV (max 20mcg) over 30min
Analogue of the amino acid lysine making it an inhibitor for proteolytic enzymes like plasmin, the enzyme responsible for fibrinolysis.
Consider in type 3<unk>  patients who have developed antibodies to VWF replacement
Increased risk of thrombosis, especially in patients with coronary artery<unk> 
For major procedures follow factor VIII levels with plan to keep troughs over 80% Not desmopressin responsive:Humate-P to achieve peak over 120% and troughs of 80%.  Levels below 30%: 40-50 IU/kg followed by 20 IU/kg every 12 hoursLevels above 30%: 20-40 IU/kg every day
Von Willebrand disease
Too few
Factor V Leiden
Thrombocytopenia
Hemophilia
Nonfunctional
Hereditary hemorrhagic telangiectasia
Aspirin