Learn more about your von Willebrand disease

Learn more about your von Willebrand disease

For more information about von Willebrand disease, visit these websites, or read these publications: the National Heart, Lung and Blood Institute website (http://www.nhlbi.nih.gov/health/health-topics/topics/vwd/, https://www.nhlbi.nih.gov/health/health-topics/topics/vwd/signs, https://www.nhlbi.nih.gov/health/health-topics/topics/vwd/diagnosis), the Hemophilia of Georgia website (http://www.hog.org/handbook/section/2/the-inheritance of-von-willebrand-disease), the National Hemophilia Foundation website (https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Von-Willebrand-Disease), and Castaman G. Blood Transfus. 2011;9:s9-s13.

What is von Willebrand disease?

Von Willebrand disease (VWD) is an inherited bleeding disorder. People with VWD have a deficiency in a protein called von Willebrand factor (VWF). VWF is a glue-like protein that helps cells in your blood, called platelets, clump together and form a plug that prevents blood loss following an injury. People with VWD cannot make this plug because their body does not make enough VWF, or what they do make is not effective. VWD is the most common bleeding disorder. Up to 1% of the population may have VWD

VWD is the most common bleeding disorder in the United States.

What causes von Willebrand disease?

VWD is caused by a problem in one of the genes that tells the body to make VWF. Unlike hemophilia, which mainly affects males, VWD affects both men and women equally. A man or woman with VWD has a 50% chance of passing the disease on to their child.

What are the types of von Willebrand disease?

There are 3 main types of VWD and several subtypes. The 3 main types are:

  • VWD type 1: The most common form. A person with type 1 has about 20% to 50% of normal levels of VWF. Levels of the blood clotting factor VIII might also be reduced
  • VWD type 2: People with type 2 produce enough VWF, but the protein is defective and affects the way that FVIII binds, as well as the way that platelets clump together
  • VWD type 3: The least common but the most severe form of VWD. People with type 3 might have a total absence of VWF, and factor VIII levels are often very low

In people with VWD types 2 and 3, bleeding episodes may be severe and potentially life-threatening.

What are signs and symptoms of von Willebrand disease?

  • Bruising easily
  • Bleeding gums
  • Frequent nosebleeds
  • Excessive bleeding after losing a tooth or oral surgery
  • Women can have increased menstrual bleeding
  • Severe internal or joint bleeding (with type 3 VWD)

How is von Willebrand disease diagnosed?

  • Appearance of the above symptoms
  • Diagnosing VWD can be difficult. Laboratory blood tests can be performed to determine the amount, structure, and function of VWF
  • A family history of VWD. Because VWD is an inherited condition, a child of people who have the VWD gene needs to be tested

What happens in von Willebrand disease blood clotting?

There are 4 stages in VWD blood clotting:

  1. The blood vessel constricts, reducing blood flow through the wounded area
  2. The body does not produce enough VWF, so platelets don't stick together and the platelet plug does not form as effectively. Bleeding continues
  3. People with VWD often do not produce enough clotting factor VIII either. Factor VIII is crucial in forming the mesh that holds the platelet plug in place. Without enough factor VIII, the mesh does not as effectively hold the platelet plug in place
  4. When a person with VWD uses plasma-derived FVIII/VWF complex, enough VWF and factor VIII are temporarily added to the blood, allowing platelets to effectively form a platelet plug and blood to form the mesh that holds the platelet plug together; bleeding can be stopped or prevented

Indications

ALPHANATE® (antihemophilic factor/von Willebrand factor complex [human]) is indicated for:

  • Control and prevention of bleeding episodes and perioperative management in adult and pediatric patients with factor VIII (FVIII) deficiency due to hemophilia A
  • Surgical and/or invasive procedures in adult and pediatric patients with von Willebrand disease (VWD) in whom desmopressin (DDAVP) is either ineffective or contraindicated. It is not indicated for patients with severe VWD (type 3) undergoing major surgery
Important Safety Information

ALPHANATE is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product or its components.

Anaphylaxis and severe hypersensitivity reactions are possible with ALPHANATE. Discontinue use of ALPHANATE if hypersensitivity symptoms occur, and initiate appropriate treatment.

Development of procoagulant activity-neutralizing antibodies (inhibitors) has been detected in patients receiving FVIII-containing products. Carefully monitor patients treated with AHF products for the development of FVIII inhibitors by appropriate clinical observations and laboratory tests.

Thromboembolic events have been reported with AHF/VWF complex (human) in VWD patients, especially in the setting of known risk factors.

Intravascular hemolysis may occur with infusion of large doses of AHF/VWF complex (human).

Rapid administration of a FVIII concentrate may result in vasomotor reactions.

Because ALPHANATE is made from human plasma, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, despite steps designed to reduce this risk.

Monitor for development of FVIII and VWF inhibitors. Perform appropriate assays to determine if FVIII and/or VWF inhibitor(s) are present if bleeding is not controlled with expected dose of ALPHANATE.

The most frequent adverse drug reactions reported with ALPHANATE in >1% of infusions were pruritus, headache, back pain, paresthesia, respiratory distress, facial edema, pain, rash, and chills.

Please see full Prescribing Information for ALPHANATE.

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.