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Treatment Options

The mainstay of VWD treatment is the replacement of the deficient protein (VWF)1. This will result in shortened bleeding time and correction of the coagulation abnormality.

There are several therapeutic options available to increase VWF levels. Among these are desmopressin acetate and plasma-derived antihemophilic factor/von Willebrand factor complex concentrate.

Desmopressin acetate, also known as desmopressin, is a synthetic analog to the vasoconstrictive hormone vasopressin. Desmopressin is first-line treatment for type 1 VWD. It should not be used in patients with type 2B since excessive platelet aggregation may be induced. Owing to variability in patient response, it is suggested that a test dose be performed prior to the use of desmopressin. Two dosage forms are available for the treatment of VWD: injection (DDAVP) and high-concentration nasal spray (Stimate® (desmopressin acetate) Nasal Spray, a CSL Behring product).

In patients unresponsive to desmopressin acetate or patients with type 2 (all variants) or type 3 VWD, the use of a plasma-derived concentrate product may be necessary to increase the plasma level of VWF. Humate-P® is a von Willebrand factor (VWF) replacement therapy indicated in adult and pediatric patients with VWD for treatment of spontaneous and trauma-induced bleeding episodes, and prevention of excessive bleeding during and after surgery in all VWD types.

Physicians sometimes prescribe oral contraceptives for women who have heavy menstrual bleeding because hormones can increase VWF and FVIII activity. Additionally, antifibrinolytic agents such as aminocaproic acid and tranexamic acid are useful to stop bleeding in VWD patients following minor surgery, tooth extraction, or an injury.

von Willebrand Disease Treatment Options
















 Drug/Type of Treatment How Taken Indicated for
Desmopressin acetate
(synthetic hormone)2 		Injection or nasal spray2 Most patients with type 1 and some with type 2A disease2; not indicated for patients with type 2B disease
Replacement therapy (concentrate of VWF and FVIII)2 Injection (IV)
  • Patients who cannot tolerate desmopressin (including patients with type 1 disease) or patients who need prolonged treatment2
  • Patients with any variant of type 2 disease and patients with type 3 (severe) VWD2
Oral contraceptives
(birth control pills) 		By mouth Women who have heavy menstrual bleeding2
Antifibrinolytic drugs (aminocaproic acid; tranexamic acid3) Injection or by mouth3 VWD patients to stop bleeding following minor surgery, tooth extraction, or an injury2; can be used alone or with desmopressin and replacement therapy2
Depending on the type of von Willebrand disease, the severity of an injury, the severity of nose, menstrual, or post-childbirth bleeding, or the extent of a medical or dental procedure, the physician may prescribe one or more of these treatments.

For more information about von Willebrand disease, please visit our Resources and Tools section.

Important Safety Information for Stimate®

Stimate Nasal Spray is indicated for patients with hemophilia A with Factor VIII coagulant activity levels greater than 5%. Desmopressin acetate is not indicated for the treatment of hemophilia A with Factor VIII coagulant activity levels equal to or less than 5%, or for the treatment of hemophilia B, or in patients who have Factor VIII antibodies.

Stimate® Nasal Spray is indicated for patients with mild to moderate classic type 1 von Willebrand disease (VWD) with Factor VIII levels greater than 5%. Stimate® Nasal Spray is not indicated for the treatment of severe classic VWD (Type I) and when there is evidence of an abnormal molecular form of Factor VIII antigen.

Stimate® Nasal Spray is contraindicated if you have a known hypersensitivity to desmopressin or any of the components of Stimate. Stimate should not be used by patients with type 2B VWD, since platelet aggregation may be induced. Stimate Nasal Spray is for intranasal use only.

To reduce the potential for water intoxication or hyponatremia, especially in the young and the elderly, patients using desmopressin may be advised to reduce fluid intake, ingesting only enough to satisfy thirst. Your doctor will monitor for a rare occurrence of an extreme decrease in plasma osmolality that could result in seizures, possibly leading to coma.

Other adverse reactions reported with use of injectable and/or intranasal desmopressin acetate include headache, nausea, somnolence (sleepiness), dizziness, chest pain, palpitations and tachycardia (rapid heartbeat), and severe allergic reactions, including anaphylaxis. See full prescribing information for a complete list of adverse reactions.

Please see full prescribing information.

 


Important Safety Information


Humate-P is indicated for treatment and prevention of bleeding in adult patients with hemophilia A (classical hemophilia). Humate-P is also indicated in adult and pediatric patients with von Willebrand disease (VWD) for (1) treatment of spontaneous and trauma-induced bleeding episodes, and (2) prevention of excessive bleeding during and after surgery. This applies to patients with severe VWD, and patients with mild and moderate VWD for whom use of desmopressin is known or suspected to be inadequate. Humate-P is not indicated for the prophylaxis of spontaneous bleeding episodes.

Humate-P is contraindicated in individuals with a history of anaphylactic or severe systemic response to antihemophilic factor or von Willebrand factor preparations.

Monitor for intravascular hemolysis and decreasing hematocrit values in patients with A, B, and AB blood groups who are receiving large or frequent doses. Also monitor VWF:RCo and FVIII levels in VWD patients, especially those undergoing surgery.

Thromboembolic events have been reported in VWD patients receiving coagulation factor replacement. Caution should be exercised and antithrombotic measures considered, particularly in patients with known risk factors for thrombosis.

Humate-P is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.

In patients receiving Humate-P in clinical studies for treatment of VWD, the most commonly reported adverse reactions observed by >5% of subjects are allergic-anaphylactic reactions, including urticaria (hives), chest tightness, rash, pruritus (itching), and edema (swelling). For patients undergoing surgery, the most common adverse reactions are postoperative wound or injection-site bleeding, and epistaxis (nosebleed).

Please see full prescribing information.

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.

The information provided herein is solely for use by physicians and healthcare professionals in the United States. The CSL Behring product listed may not have been approved in other countries and may not be available everywhere.

References

  1. Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351:683-694.
  2. National Heart, Lung, and Blood Institute Diseases and Conditions Index. How is von Willebrand disease treated? Available at: http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_Treatments.html. Accessed April 29, 2005.
  3. Medline Plus. MedlinePlus drug information: Antifibrinolytic agents (systemic). Available at: http://www.nlm.nih.gov/medlineplus/druginfo/uspdi/202058.html. Accessed April 29, 2005.
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