Hemophilia is the rare genetic bleeding disorder that strikes only men.
But a bleeding problem that is 100 times more common–von Willebrand’s disease–and causes debilitating problems for women is virtually unheard of and just now commanding the attention of the medical community.
Millions of women with the disease go undiagnosed, suffering heavy menstrual periods that can last for weeks, making them anemic, exhausted and in extreme cases, even requiring transfusions.
It took years for Tamara Bledsoe to learn that she had the debilitating condition. When she was a teenager, her periods lasted 30 days–any woman’s definition of hell. Now that’s she has been diagnosed, her periods are under control but they are still so heavy she pops iron pills to fight off anemia.
“You just get so tired after so many days,” said Bledsoe, 30, of Pembroke Pines, Fla. “It’s like, `OK, I’ve had enough of this.’ ” Yet before she was diagnosed some 10 years ago, Bledsoe never suspected a problem because her mother and family all had similarly heavy periods. Her story is familiar to doctors who say that because the disease is genetic, many women assume their bleeding is normal. They never realize they have a treatable disorder.
Von Willebrand’s disease is caused by problems with a clotting factor that helps form and reinforce a plug the body creates to stop up the tear in a cut blood vessel. Von Willebrand’s patients either don’t produce enough of that clotting factor or produce a factor that doesn’t work right. When they sustain a cut, their bodies are slow to form that plug and they are unable to stop the bleeding quickly.
The disease is diagnosed by a blood test, but it can be hard to find because the body naturally varies the levels of von Willebrand factor so much that people with the disease can test normal. It is common to repeat the test several times.
The problem is compounded by a medical community that has been slow to pick up the disorder.
Von Willebrand’s disease strikes an estimated 1 percent of the population, and it is almost always far milder than hemophilia. Many symptoms, which include nosebleeds and a tendency to bruise easily, are often shrugged off by both patients and doctors. In most instances, the disorder is never suspected until it causes serious bleeding after surgery, childbirth, or a traumatic injury.
The disease affects women and men at the same rate, but because men don’t menstruate, they tend to be far less bothered by it. Women plagued by the problem are rarely diagnosed even when they seek treatment.
There are a number of reasons for that, experts say. For many years, von Willebrand’s disease was thought to be extremely rare–so doctors didn’t think of it when treating women for heavy menstrual bleeding. There was a widespread myth that bleeding disorders only affected men. Also, some gynecologists assume that it’s normal to have such heavy menstrual bleeding in a certain percentage of the population. The problem is often masked because gynecologists frequently control heavy bleeding with birth control pills–which will work in many von Willebrand’s patients. That can be dangerous because the pills won’t be enough to control the bleeding after surgery or childbirth.
Many women with the problem are frustrated by a medical profession they say has ill served them.
“I hate to always bring gender into it, but I think one of the reasons it’s been underserved is because it’s a female disorder,” said Renee Paper, a nurse and an activist who has the disease and has pushed to bring more attention to it on the national level. “Women are suffering in silence because when you go to the doctor they tell you, `It’s all in your head’ or `Live with it.’ “
The women’s complaints and an increased interest in women’s health has led to stepped-up research and funding for von Willebrand’s disease in the 1990s. The Centers for Disease Control and Prevention is conducting several studies–including some that have shown the disorder to be far more common than previously thought. Several studies have found that 10 percent to 15 percent of women who suffer heavy periods may actually have von Willebrand’s disease, said Anne Dilley, an epidemiologist with the CDC’s Hematologic Diseases branch.
Studies also indicate that the disease goes undiagnosed in about 95 percent of women who have it. The women often go through unnecessary, and sometimes counterproductive, treatments. Some go through a procedure in which the uterus is scraped, which may accelerate bleeding. Some take ibuprofen to control the heavy cramping associated with the condition, unaware that the drug can accelerate the bleeding. Others opt for hysterectomies at a young age. One estimate puts the number of unnecessary hysterectomies in women with bleeding disorders at about 25,000 a year.
“That’s why the CDC is interested in this,” said Dilley. “We know a substantial number of hysterectomies are done for this kind of uterine bleeding, and upwards of 10 percent of these women may have von Willebrand’s disease that could be managed with drugs, thereby preserving fertility and sexual function and preventing other complications.”
Studies have also shown that women with von Willebrand’s lose time from work and school, suffer significant stress and anxiety and have other ailments, from anemia to chronic fatigue and cramps.
The suffering is so unnecessary, because the disease is easily treatable. A simple nasal spray that releases the body’s stores of von Willebrand factor can control the bleeding.
“You don’t just have to live with this,” said Dr. Joanna Davis, a pediatric hematologist and director of the Comprehensive Hemophilia Center at the University of Miami. “It’s a manageable problem. Once you’re aware of it, you can live a normal life.”
