Management of outpatients

Patients with VWD should regularly visit a hemophilia center having clinicians experienced both in VWD and in the coagulation laboratory that measures VWF and FVIII activities among other coagulation variables. It is desirable to manage and follow-up patients with VWD and other bleeding disorders with help of a computerized registry that includes relevant clinical and laboratory information. This is already introduced, or ongoing, in most countries/centers in the Nordic region according to the EUHANET policy. Patients with severe VWD (especially type 3), or those with frequent or severe bleeds should be observed once or twice a year or more often if required. Milder patients may be observed less regularly, e.g., with 2-3 years intervals. The comorbidities associated with bleeding tendency, such as hypertension, renal impairment and anemia should be monitored on a regular basis. Drugs that are impairing hemostasis should be avoided. A list of interfering drugs, with focus on platelet function, is found in the NHC Nordic guidelines for the diagnostics of inherited platelet disorders (see appendix 2).

All patients should be given an “identity” or “bleeder’s” card to be kept available, and inform about the bleeding disorder, the initial treatment in case of trauma or bleed and contact information to the hemophilia center. The patient file should provide a risk alert of the VWD diagnosis and emergency contacts to the hemophilia center.