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Lakshmi Venkateswaran, MD

Pediatric Hematologist / Oncologist
Houston, Texas

Lakshmi Venkateswaran, MD is a Pediatric Hematologist / Oncologist in Houston, TX. They attended medical school at Kilpauk Medical College in 1991. They completed their residency at University Of Tn College Of Medicine.

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von willebrand disease

Understanding von Willebrand Disease: Do You Know the Signs?

Your Health

This article has been archived. We will no longer be updating it. For our most up-to-date information, please visit our blood disorders information here.

Bleeding disorders occur more frequently than many people realize. The most common type of bleeding disorder, von Willebrand disease (VWD), is found in up to 1 percent of the population. Although VWD is equally common in men and women, symptoms can be more noticeable in women because of heavy bleeding during their menstrual periods and after childbirth.

What is VWD?

When you have VWD, your blood refuses to clot properly. Blood contains numerous proteins that help the body stop bleeding following a medical procedure or when you have an injury; one of these proteins is named von Willebrand factor (VWF). People with VWD have low levels of VWF or VWF that doesn't work properly. As a result, blood clots might take longer to form and bleeding may take longer to stop. VWD is almost always hereditary, meaning that it is passed down from a parent to a child.

There are three types of VWD. Type 1, in which people have unusually low levels of VWF, is the most common and least serious form of the disease. People with type 1 VWD may also have low levels of another kind of blood-clotting protein called factor VIII.

In type 2, the body makes the right amount of VWF but the protein doesn't work properly. Type 2 is divided into four subtypes, depending on the specific problem with your VWF. It is important to know which subtype you have, because each may require different treatment.

People with type 3 VWD have little or no VWF and low levels of factor VIII. This is considered the most severe form of the disease.

Diagnosis

Early diagnosis of VWD is important to ensure that you receive proper treatment and continue to live an active and fulfilling life. However, VWD can be tricky to diagnose. Those with type 1 VWD may never experience major bleeding problems unless they are in an accident, undergo surgery or dental procedures or have heavy bleeding with menstruation or childbirth. Women who experience heavy and prolonged menstrual bleeding, and who might have VWD, are often misdiagnosed with gynecological problems.

If your health care professional suspects that you have VWD, he or she will take a comprehensive medical history and perform a physical exam and a variety of blood tests. Your health care professional also might refer you to a hematologist (a doctor specializing in blood disorders) to confirm the diagnosis and provide follow-up care.

5 signs and symptoms

The signs of VWD vary depending on the type and severity of the disease. However, any of the following symptoms can be a signal to you and your provider.

  • Frequent or hard-to-stop nosebleeds that:
    • happen spontaneously
    • occur often (five times or more a year)
    • last longer than 10 minutes
  • Bruising that:
    • occurs after little or no trauma or injury
    • happens often (1-4 times per month)
    • is larger than a quarter
    • has a raised lump
  • Heavy menstrual periods that:
    • last more than seven days
    • include clots larger than a quarter
    • soak through a pad or tampon hourly or more often
    • result in a diagnosis of anemia
  • Longer than normal bleeding after injury, surgery, childbirth or dental work, such as:
    • bleeding lasting more than five minutes following a cut to the skin
    • heavy or extended bleeding during or after dental or other surgery
    • surgical bleeding that stops then starts again hours or days later
    • heavy bleeding during or after childbirth

Other bleeding events in individuals with VWD include:

  • blood in the stool from bleeding into the stomach or intestines
  • blood in the urine from bleeding into the kidneys or bladder
  • rarely, bleeding into joints or internal organs

Treatment options

Most cases of VWD are mild and require treatment only if you are in an accident, undergo surgery or dental procedures or have heavy bleeding with menstruation or childbirth. When the condition is more serious, there are several medications your health care professional might suggest.

One common treatment for VWD is an artificial hormone called desmopressin, usually taken by injection or nasal spray. Desmopressin encourages your body to release more VWF and factor VIII. It has been proven successful in most people with type 1 VWD and some people with type 2 VWD.

Factor replacement therapy provides medicines rich in VWF and factor VIII to replace the missing factors in the blood. Typically, these are used to treat people with more severe forms of VWD or with milder forms that need prolonged treatment or don't respond well to desmopressin. The medicines are given through a vein in the arm.

Antifibrinolytic medications help prevent the breakdown of blood clots. They're mostly used to stop bleeding from the nose or oral cavity after minor surgery or injury and for heavy menstrual bleeding. They may be prescribed alone or in conjunction with desmopressin and factor replacement therapy. Birth control pills also may increase the levels of VWF and factor VIII in the blood and can reduce menstrual blood loss.

Pregnancy can prove challenging for women with VWD because you run an increased risk of bleeding problems during delivery and for an extended time after it. However, there are ways to lower the risk of such complications. If you have VWD and are considering having a child, it's a good idea to talk with a hematologist and an obstetrician who specializes in high-risk pregnancies before you become pregnant.

Living with VWD

Though VWD isn't curable, it's manageable with appropriate treatment. There are also a number of steps you can take to ensure a full and rewarding life:

  • Avoid over-the-counter medicines that affect blood clotting, such as aspirin, ibuprofen and other nonsteroidal anti-inflammatory drugs (also known as NSAIDs). Always check with your doctor before taking any medicines.
  • Tell your doctor, dentist and pharmacist that you have VWD.
  • Consider telling friends, colleagues, gym trainers and sports coaches about your condition. Such knowledge will allow them to act quickly if you get injured.
  • If you have a more severe form of VWD, wear a medical ID bracelet or necklace. In case of accident or injury, this can provide valuable information for your health care team.
  • Stay physically active and maintain a healthy weight. Exercise helps keep you flexible and prevents damage to muscles and joints. Safe physical activities include swimming, biking and walking. Avoid contact sports like football, hockey and wrestling. Always check with your doctor before starting any exercise program.

This resource was developed with the support of CSL Behring.

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