Background: The recent recall of intranasal desmopressin (DDAVP) has impacted management of patients with von Willebrand disease (VWD) in the United States, leaving one available alternative for home administration: subcutaneous DDAVP preparation at 6 mcg/1.5 ml. With weight-based dosing at 0.3 mcg/kg/dose, individuals >20 Kg require multiple injections which decreases convenience and patient acceptance. Recognizing clinical need and limited alternatives, we adopted the practice of restricting DDAVP dosing to two subcutaneous injections (max dose=12mcg) regardless of patient weight. Since the data to support this practice is sparce, we reviewed the response of DDAVP challenge tests for individuals who had both subcutaneous and intranasal DDAVP. We speculated that subcutaneous route would cause additional stress response due to injection site pain and different pharmacodynamics patterns from intranasal.

Aims: Compare intra-patient DDAVP challenge response between subcutaneous and intranasal route.

Methods: This retrospective cohort study reviewed seven patients with VWD (type I and II) who underwent desmopressin challenge tests via intranasal and subcutaneous routes. Response variables included Von Willebrand Factor Antigen (VWF:Ag), VWF:Ristocetin activity (VWF:RCo), and factor VIII levels at baseline and 1 to 6 hours post-DDAVP administration. Response was defined as peak increment in VWF:RCo>1.5X baseline.

Results: All seven patients responded to subcutaneous DDAVP challenge test (Table 1, Figure 1). While the intra-patient responses via intranasal and subcutaneous route did not show significant difference in mean VWF:RCo peak to baseline ratio, p=0.179, three patients (ID# 1,2,7) who did not respond to intranasal DDAVP responded to subcutaneous, p=0.023. All five patients (ID#1,3,4,5,6) who received limited-dose DDAVP (12mg) responded as well. Study is limited by small sample size and reporting bias.

Conclusion(s): Our experience shows that subcutaneous DDAVP, maximum dosing 12mg, is an effective alternative to intranasal DDAVP. Subcutaneous route may even be an option for patients previously unresponsive to intranasal administration. Larger studies are needed to clarify our observation.

Table 1

Results of DDAVP challenge test with intranasal or subcutaneous route of administration. Values for subcutaneous administration are bolded for intra-patient comparison.
VWF: von Willebrand factor; RCo: Ristocetin cofactor activity; Antigen; FVIII: Factor VIII coagulant activity; DDAVP: 1-deamino-8-D-arginine vasopressin.

Figure 1

Graphical representation of VWF:RCo levels as a function of time post-DDAVP administration in the seven patients that underwent testing with both intranasal and subcutaneous routes. A single color is used per patient to compare intra-patient responses. Dashed lines correspond to subcutaneous responses while solid lines correspond to intranasal.

« Back to ISTH 2022 Congress

ISTH Congress Abstracts - https://abstracts.isth.org/abstract/hemostatic-response-to-subcutaneous-versus-intranasal-desmopressin-in-patients-with-von-willebrand-disease-vwd/