Appendix 1

Products for the management of VWD.

Generic name	Product	Formulation	Properties	Adverse reactions
Desmopressin (DDAVP)	Octostim	Solution for injection i.v., s.c. (15 micrograms/ml) Nasal spray i.n. (150 micrograms/dose)	Synthetic analogue of vasopressin. Release of endogenous FVIII, VWF and tissue plasminogen activator (t-PA).	Headache, nausea, temporary lowering of blood pressure with secondary tachycardia, facial flushing. Fluid retention, hyponatremia and seizures.
Plasma-derived VWF/FVIII complex	Haemate Haemate P	Sterile, lyophilized powder for injection i.v (1000 IE FVIII, 2400 IE VWF), (500 IE FVIII, 1200 IE VWF)	Plasma-derived factor concentrate. Pasteurization 10 h at 60°C. VWF:RCo/FVIII:C approx. 2.7.	Theoretical risk of prion transmission. Allergic / anaphylactic reactions. Development of alloantibodies.
Plasma-derived VWF/FVIII complex	Wilate	Sterile, lyophilized powder for injection i.v. (900 IE FVIII, 800 IE VWF), (450 IE FVIII, 400 IE VWF)	Plasma-derived factor concentrate. Solvent-detergent treatment, heating of the lyophilized product for 2 h at 100°C (PermaHeat). VWF:RCo/FVIII:C approx. 0.8.	Theoretical risk of prion transmission. Allergic / anaphylactic reactions. Development of alloantibodies.
Purified Plasma-derived VWF complex	Wilfactin Willfact	Sterile, lyophilized powder for injection i.v. (10 IE FVIII, 1000 IE VWF)	Plasma-derived. Solvent-detergent treatment, 35 nm nanofiltration and dry heating of the lyophilized product at 80°C for 72 h. VWF:RCo/FVIII:C approx. 60. Priming dose of FVIII may be necessary.	Theoretical risk of prion transmission. Allergic / anaphylactic reactions. Development of alloantibodies.
Recombinant VWF	Veyvondi	Sterile, lyophilized powder for injection i.v. (650 IU, 1300 IU)	1 IU/kg (VWF:RCo/VEYVONDI/vonicog alfa) raises the plasma VWF:RCo by 0.02 IU/mL (2%). Priming dose of FVIII necessary for acute bleeds if FVIII < 0.4 IU/dL.	Allergic / anaphylactic reactions. Development of alloantibodies
Tranexamic acid	Cyklokapron	Solution for injection i.v. and s.c.p. Tablets Dissolvable tablets Mouthwash*	Anti-fibrinolytic.	Nausea, vomiting, diarrhoea and abdominal pain.
Hormonal therapy	Estrogen containing oral contraceptive Progesteron containing intrauterine device (IUD) IUD	Tablet	Variable increase of VWF. Progesteron induced reduction in bleed.	Headache, nausea, weight gain, thrombosis, cancer. Headache, weight gain, risk for infection.
Transfusion	Platelets	Infusion		Allergic reactions, development of alloantibodies.

*Available Tranexamic formulations may vary in different Nordic countries.