About Von Willebrand Disease

Von Willebrand disease (VWD) is a bleeding disorder caused by insufficient or poor clotting from a faulty gene known as the von Willebrand factor (VWF). This protein helps blood to clot, which is vital for bleeding to stop. Usually milder than hemophilia, von Willebrand disease is genetic in most cases, passed on from parent to child.

Unlike hemophilia, which mainly affects males, VWD occurs equally in males and females but can be more severe in women due to bleeding from menstruation and childbirth.

Von Willebrand Disease Types

There are three types of von Willebrand disease: 1, 2, and 3. Knowing which type a patient has can assist in obtaining appropriate and effective treatment.

Von Willebrand Disease Type 1

Type 1 von Willebrand Disease is the most common type, affecting about 70%-80% of people with the disease. People with Type 1 VWD have the von Willebrand factor and it works normally but there is not enough to adequately control bleeding.

Von Willebrand Disease Type 2

Type 2 von Willebrand Disease is the next most common type, affecting 15%-30% of people with VWD. People with Type 2 von Willebrand Disease have enough von Willebrand factor but it doesn’t work properly.

Von Willebrand Disease Type 3

Type 3 von Willebrand Disease is the rarest and most severe type of VWD. People with Type 3 VWD have very little or no von Willebrand factor in their blood and have low levels of factor VIII (the clotting factor missing in hemophilia A).

Symptoms of Von Willebrand Disease

Symptoms vary greatly from person to person. Some individuals are asymptomatic (they have no symptoms at all), whereas others may have moderate to very severe bleeding complications. When symptoms first appear will also vary, ranging from infancy to adulthood.

Among the symptoms are persistent and frequent nosebleeds, blood in urine or stool, easy bruising, excessive bleeding from an injury, surgery, or minor cut, and bleeding from the gums or dental work (especially tooth extractions).

For women, symptoms include a heavy period (known as menorrhagia), presence of large blood clots, reproductive tract bleeding during pregnancy and childbirth, or hemorrhaging after childbirth. Anemia, fatigue, and shortness of breath are other possible symptoms of von Willebrand disease.

Among the more severe but considerably less common symptoms is internal bleeding such as swelling, gastrointestinal bleeding, and bleeding into the muscles and joints. Left untreated, these symptoms can be fatal.

Von Willebrand Diagnosis

Anyone with the above symptoms should go the doctor immediately. In addition to exploring the family’s medical history, the doctor will perform a physical examination and have blood drawn for laboratory analysis to determine the level and efficacy of von Willebrand factor.

While an individual may not experience symptoms of von Willebrand disease, the faulty gene can still be passed on to children. A child whose parent is a carrier for VWD should be checked for the disease. Early diagnosis is vital for a healthy, active, and normal life.

Treatment for Von Willebrand Disease

Treatment focuses on stopping or preventing bleeding episodes, typically by using medications that assist with blood clotting. Depending on the diagnosis, treatments may be administered orally, through injections, or by nasal spray. Other medicines can be applied topically. In mild cases, medication may only be required for surgery or dental work.

Drugs for Treating von Willebrand Disease

Any treatment regimen will depend upon the severity of symptoms and may include a combination of different drug types and other therapies. Here is a list of common von Willebrand disease treatments:

Hormone replacement to stimulate von Willebrand factor production:

  • Desmopressin (DDAVP, Stimate)

Coagulation factors to replace low or missing VWF:

  • Recombinant von Willebrand factor (Vonvendi)
  • Antihemophilic factor/von Willebrand factor complex (Alphanate, Humate P, Wilate)

Antifibrinolytics to stabilize clots:

  • Aminocaproic acid (Amicar)
  • Tranexamic acid (Cyklokapron, Lysteda)

Contraceptives/estrogen to reduce menstrual bleeding severity:

  • Ethinyl estradiol and levonorgestrel (Levora, Nordette, Lutera, Trivora)

Topical treatments for external injuries:

  • Fibrin sealant (Tisseel VHSD)

Routine vaccinations if human blood infusions are received to prevent diseases passed on through blood

Managing Von Willebrand Disease Symptoms

Actively managing von Willebrand disease symptoms leads to better outcomes for VWD patients. This includes following treatment plans laid out by doctors and other specialists, taking medications consistently, and obtaining infusions when needed.

NCHS is proud to have a team of experienced bleeding disorder specialists who function as an extension of your care team. We provide discreet delivery services, help coordinate care, navigate health insurance matters, and provide support to individuals with von Willebrand disease and their families. To learn how we can best care for you, contact a patient support specialist.