VWD by Type

VWD is classified into three major categories—Types 1, 2, and 3—based on the kind of abnormality to VWF that caused it1:

  • In Type 1, the most common, VWF levels are low (partial lack of factor)
    • Type 1 also includes an extremely rare variant called Vicenza
  • In Type 2, the VWF does not work correctly
    • Type 2 is further divided into four subtypes (2A, 2B, 2M, and 2N) based on the different ways each interferes with the proper function of VWF
  • In Type 3, the rarest and most severe, VWF is almost completely absent

The difference between VWD Types 1, 2, and 3

The chart below shows how the different types of VWD differ from one another in terms of their effect on VWF levels, prevalence, and severity of symptoms and how they are inherited.

VWD by Type1
Type 1 Low 75% Mild DOMINANT:
Requires only 1
parent to pass on
the gene
Type 2 Normal, but VWF
does not work
correctly
15%-25% Moderate,
occasionally
severe
DOMINANT:
Requires only 1
parent to pass on
the gene*
Type 3 Little or none Less than 5% Severe RECESSIVE:
Requires that
both parents pass
on the gene
*Except Type 2N VWD.

References:

  1. National Heart, Lung, and Blood Institute. The Diagnosis, Evaluation, and Management of von Willebrand Disease. Bethesda, MD: National Institutes of Health; 2007. NIH Publication No. 08-5832.

Important Safety Information

Stimate® (desmopressin acetate) Nasal Spray, 1.5 mg/mL is a treatment used to stop some types of bleeding in people with mild hemophilia A or mild to moderate von Willebrand disease (VWD) Type 1. Stimate Nasal Spray should not be used in children under 11 months of age.

All patients using Stimate Nasal Spray are at risk of water intoxication, fluid overload and low sodium levels in the blood. Follow your healthcare provider’s instructions on limiting the amount of fluid you drink when using Stimate Nasal Spray, as too much fluid intake can lead to serious adverse reactions, including seizures, coma, and even death. Fluid restrictions are especially important for children and elderly patients, as they are at higher risk for these reactions.

See the patient information leaflet in the prescribing information for Stimate Nasal Spray for symptoms that could mean your blood sodium level is low—including headache, hallucinations, confusion, restlessness, weight gain and muscle spasms. Immediately report any of these symptoms to your physician or, if necessary, an emergency department. Also contact your doctor immediately if you have uncontrolled bleeding.

Before being prescribed Stimate Nasal Spray, make sure your doctor knows about all your medical conditions and about any medications you are taking. Use Stimate Nasal Spray exactly as your healthcare provider has instructed.

Side effects of Stimate Nasal Spray generally come from having too much water in the body. The most common include facial flushing, nasal congestion, runny nose, nose bleed, sore throat, cough, and upper respiratory infections. Tell your healthcare provider if you experience a side effect that does not go away.

Please see full prescribing information for Stimate Nasal Spray

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.