Many women accept excessive, prolonged periods as an annoying but ordinary part of being female—putting up with the monthly stress of periods so heavy that they interfere with their quality of life.
But in some cases, the cause of those heavy periods is von Willebrand disease (VWD), the most common inherited bleeding disorder in the US. While men and women are equally affected by VWD, in women, heavy menstrual bleeding is often the major symptom.
What’s Normal, and What’s Not?
It can be hard to tell if you have heavy or excessive bleeding because you’ve gotten used to it, but in general, periods that last more than seven days is considered heavy menstrual bleeding (HMB).. Other signs of HMB:
- You use more than one pad or tampon every hour
- You need to double up by wearing one pad and a tampon because of heavy bleeding
- You need to change pads or tampons more than once during the night
- You have menstrual flow that repeatedly contains blood clots the size of a quarter or larger
- Your menstrual flow is so heavy that it keeps you from doing the things you would do normally, such as going to work or school
When VWD Is Suspected
Not every woman with heavy periods has VWD, so the diagnostic process for VWD usually begins with a few questions about family and menstrual history, says Andrea Lukes, MD, MHSc, president and CEO of Carolina Women’s Research and Wellness Center in Durham, North Carolina. “If someone has had heavy bleeding since her first period, that raises suspicions. A family history of known bleeding disorders, such as hemophilia, would as well,” says Lukes, who is also the founder and chair of the Ob/Gyn Alliance, a peer-to-peer network educating ob/gyns.
The initial step to determine if a patient has VWD is to run specific tests that measure VW antigen (the amount of VW protein in the blood) and activity levels (how well the VW protein works), and levels of factor VIII (FVIII).
“The range of normal for antigen and activity will vary from 50% to 150%,” says Alice Ma, MD, associate professor of medicine in the Division of Hematology/Oncology at UNC Hospitals in Chapel Hill, North Carolina. “But there’s normal, and then there’s ‘normal.’” The difference is in the patient’s story, according to Ma, a hematologist who specializes in treating women with bleeding disorders.
When Retesting Is Required
Because test results can come back normal for a person with VWD due to several factors, such as stress, elevated estrogen levels, pregnancy, use of oral contraceptives and recent physical exertion, retesting can be required. Test results may also be skewed during periods of acute inflammation, such as the perioperative period, and if infections are present. Any of these can temporarily increase the VW antigen and activity levels into the acceptable range.
“For a patient with a level of 50% to 60%, but in the setting of a really good story—a history of bleeding and a strong family history of bleeding disorders—I am going to repeat those tests, making sure everything is optimized for the best results,” Ma says.
“I may bring a patient back to be tested again right before menstruating [when hormone levels are lower], or take her off birth control pills or HRT [hormone replacement therapy] for a month or two before repeating the test,” Ma says. “Also, if a patient has just run up the stairs or is nervous, [those factors] can increase levels as well. I will bring her back on another day, make sure she takes the elevator and try to keep her calm.”
Another factor that can affect VWD test results is where the testing is done. “It is best to get the tests done by a hematologist, preferably one associated with a hemophilia treatment center,” Ma says. Commercial labs or clinics may not use optimal methods for handling blood. “If it sits outside in a metal box, the sample could be messed up before it gets to the lab.”
Treatment for VWD
Diagnosis of VWD typically takes three to four weeks; the findings will show the type and severity of VWD. There are three different types of VWD: 1, 2 and 3, and several subtypes. In type 1, the VW protein is normal but is produced in decreased amounts—levels range from 20% to 50%. Symptoms are usually, but not always, mild. In type 2, the VW protein is abnormal. Patients with type 3 VWD have very low levels of VWF activity and antigen (typically less than 10%), and low factor VIII levels. They have the most severe symptoms, although people with specific subtypes of either type 1 or type 2 VWD can also have severe VWD.
Type 1 should be treated with DDAVP, or desmopressin acetate, a synthetic hormone released through a nasal spray, according to the National Hemophilia Foundation’s Medical and Scientific Advisory Council (MASAC) recommendations on VWD treatment. DDAVP increases the levels of FVIII and VWF circulating in the blood for up to eight hours after administration.
People with types 2A, 2M and 2N VWD should be treated with DDAVP if they have responded positively to a trial of DDAVP. People with subtypes 2B and type 3 VWD (and those with types 1, 2A, 2M and 2N who don’t respond to DDAVP) should be treated with VWF-containing FVIII concentrates, according to MASAC recommendations. For some women, heavy menstrual bleeding can be controlled with hormone replacement therapy or birth control pills.
Medications called “antifibrinolytics” can also be used to treat heavy periods.
To those undergoing testing or who are newly diagnosed, Lukes offers this assurance: “Once you are diagnosed, it’s the first step to managing the disease. There are lots of options to control its impact. I see a huge sense of relief come over individual women when they finally understand why they have this bleeding disorder and how common it really is.”
For more information on lab testing for VWD and how to prepare for them, visit NHF’s Better You Know website. https://www.betteryouknow.org/women/at-risk/how-to-prepare