Response to 1-Deamino-8-<i>D</i>-Arginine Vasopressin in von Willebrand Disease

Abstract

The synthetic agent, l-deamino-8-D-arginine vasopressin (DDAVP), is generally regarded to be the treatment of choice for persons with type I von Willebrand disease (vWD), and may be useful in type IIA vWD as well. Several formulations of the drug are available, including a highly concentrated intranasal spray formulation which is ideal for home use. The degree of F VIII and vWF response to DDAVP varies among individuals, but is generally consistent in individual patients over time. Some have tachyphylaxis while others do not. While side effects are uncommon, DDAVP is a potent antidiuretic agent. Thus one should be aware of the possibility of hyponatremia and water intoxication if certain precautions are not observed.

DDAVP treatment in a child with von Willebrand disease type 2M

“…Therefore the recommendation to treat the mild quantitative defects, such as type 1 vWD, and a proportion of the qualitative defects, such as type 2A vWD, with DDAVP is widely accepted [7,10]. However, in rarely occurring defects and especially in type 2 de®ciencies many authors prefer replacement therapy with plasma products [1,3,5,11].…”

6 Factor VIII Concentrates, Factor VIII/von Willebrand Factor Concentrates, Factor IX Concentrates, Activated Prothrombin Complex Concentrates

“…The biological half-life of vWf is 6-12 h; infusion, subcutaneous injection, or nasal application of the vasopressin analogue DDAVP (1-desamino-8-D-arginin-vasopressin; desmopressin) releases vWf and factor VIII from the body's reservoirs and causes an approximately three-fold increase over initial plasma levels. Thus DDAVP can be employed to stop bleeding in mild forms of von Willebrand syndrome (type 1) and in mild hemophilia A during minor bleeding episodes or minor surgery [12,38].…”

“…In contrast, most patients with mild hemophilia A or von Willebrand syndrome type 1 can be treated with DDAVP, with the exception of severe bleedings or during major surgery. Prior to administration of DDAVP the rate of biologic response should be tested [22,38,54,59]. -Clotting factor concentrates are usually administered slowly as bolus i.v.…”

“…With the exception of severe bleeding or major surgery, most children beyond the age of 4 years with mild hemophilia or von Willebrand syndrome type 1 can be treated with the synthetic vasopressin analogue DDAVP at a dose of 0.3 µg/kg body weight or as nasal spray (dosage see expert information) [38,46,58]. Because of the danger of hyponatremia and cerebral seizures, DDAVP is not indicated in children under the age of 4 years.…”

“…With the exception of severe bleeding or major surgery, most patients with mild hemophilia or von Willebrand syndrome type 1 should be treated with the synthetic vasopressin analogue DDAVP (desmopressin) at a dose of 0.3 µg/kg body weight or as nasal spray (dosage see expert information) [38]. Life-threatening bleeding in patients with type 1, 2 or 3 von Willebrand syndrome should be treated with factor VIII/vWf concentrates.…”