Disease exposed: Little-known disorder affects millions

Monday, April 8, 2002 | 11:12 a.m.

Von Willebrand disease

It is estimated that 3 million American men and women are afflicted with von Willebrand disease. It is far more common than many other well-known diseases. Here's a look at other ailments for comparison:

Source: Aventis Behring L.L.C. pharmaceutical company.

It is a potentially deadly blood disorder that affects one in every 100 Americans, yet if you asked 500 people on the street about von Willebrand disease, you'd have difficulty finding five who knew anything about it.

Three million Americans have von Willebrand disease, the world's most commonly inherited bleeding disorder, but experts say many have not yet been diagnosed with the ailment that is three times more prevalent than HIV and 150 times more common than the closely related hemophilia, which involves different defects in the blood-clotting process.

However, a Henderson emergency room nurse who has von Willebrand disease, is on a crusade to change that with her new book, "A Guide to Living with von Willebrand Disease." It tells not only of her long struggle, but also shares stories gleaned from interviews with about 100 other sufferers.

"Not knowing you have von Willebrand disease can have potentially fatal consequences, especially for those who have surgery, because they can bleed to death if the blood doesn't clot," said Renee Paper, a 44-year-old nurse at St. Rose Dominican Hospital, who was diagnosed with the disease at age 16.

While hemophilia dates back to Old Testament times, the medical profession did not identify von Willebrand disease until 1926.

In women the disease is usually categorized by heavy menstrual bleeding, but other symptoms include frequent nosebleeds, easy bruising, bleeding gums, gastrointestinal bleeding and excessive bleeding following surgery, childbirth or dental procedures. Men are as likely to get the disease as women.

Getting doctors to identify von Willebrand disease in a patient has been a challenge.

"We as physicians have not been educated about von Willebrand, but things have been improving in recent times," said Dr. Jonathan Bernstein, a Las Vegas pediatric hemotologist and oncologist who treats children and adults with von Willebrand disease.

"Fifteen years ago, von Willebrand disease was thought to be prevalent in one of every 100,000 Americans, and five years ago, one in 10,000. Today we know it affects greater than 1 percent of our entire population."

Bernstein said that an estimated 15,000 to 30,000 Southern Nevada men and women have von Willebrand disease, based on national percentages, but only about 600 have been diagnosed with it.

And misdiagnoses, Paper said, have led to tens of thousands of needless hysterectomies and similar surgeries for young women.

Paper, the director and founder of the 12-year-old Hemophilia Foundation of Nevada, said 600,000 hysterectomies are performed each year in the United States, with 10 to 20 percent done specifically to stop heavy monthly bleeding.

"We don't treat epilepsy by cutting out a piece of a person's brain, so we should not treat a woman with a blood disorder by cutting out her uterus," said Paper, who at age 30 had a hysterectomy.

"I have seen cases where every woman in a particular family had babies in their early 20s and were given hysterectomies by age 24," Bernstein said. Paper's sister has von Willebrand disease and has undergone a hysterectomy.

Von Willebrand disease commonly is treated with medication, including the birth control pill.

Bernstein and Paper plan a series of lectures to local health care officials to educate them about the disease, which is hereditary.

Often von Willebrand disease is not identified until after a surgery, when blood from large incisions oozes through small holes and other wounds that, in a person free of the disease, would routinely clot, Paper said.

Although the presence of the disease can be determined by a simple blood test, such tests are not available at all labs, and test results can take up to two weeks, Bernstein said.

Paper, the 1998 recipient of the National Hemophilia Foundation's Dick James Lifetime Achievement Award, said she hopes her book will raise awareness and encourage people to seek diagnosis and treatment.

But she also hopes it will put a face on von Willebrand disease to show the world that people with blood disorders can lead normal, productive lives.

Paper's book, co-authored by Lauren Kelley, a national consumer expert on bleeding disorders, is free, underwritten by the pharmaceutical company Aventis Behring LLC. It is available at the website www.allaboutbleeding.com, or by calling Aventis Behring at (888) 508-6978.