Persons with bleeding disorders need comprehensive care from experienced specialists.
Millions of people in the U.S. have one or more symptoms of a bleeding disorder. The most familiar, hemophilia, affects around 20,000 people—mostly men (because the abnormal gene is carried on the X chromosome)—in the United States. Hemophilia is primarily an inherited disorder.
Approximately one in 5,000 males is born with hemophilia each year. They have little to no clotting factor, which is a protein needed for normal blood clotting. These proteins work with platelets—small blood cell fragments that form in the bone marrow—to help the blood clot. Depending on its severity, individuals with hemophilia may bleed for a longer time than others after an injury, especially in their knees, ankles and elbows, which can damage organs and vital tissues. Bleeding in persons with hemophilia can even be life-threatening if not treated correctly.
Von Willebrand Disease
Many more people have a less severe bleeding disorder called von Willebrand disease. It’s estimated that one percent of the U.S. population (about three million people) has low levels or low-function von Willebrand factor, an important blood protein that helps to start the clotting process. The symptoms of von Willebrand disease include excessive or easy bruising; oozing of blood from the nose, mouth or gums; heavy menstrual bleeding; and hemorrhage after the delivery of a baby. People with low von Willebrand factor have few, if any symptoms, and most don’t even know they have it. However, approximately 20 percent of women with heavy menstrual bleeding actually have von Willebrand disease, and 60 to 75 percent of these women with von Willebrand disease will have heavy periods.
In a recent study, women with heavy menstrual bleeding and von Willebrand disease (or another diagnosed bleeding disorder) had a worse health-related quality of life than other women in the general population. If the bleeding is severe enough, anemia results, causing more troubling symptoms of fatigue, headaches, dizziness, lightheadedness and nausea. In addition, many women with heavy periods have to miss work or school, which can lead to more stress and a diminished quality of life.
Another more common bleeding disorder, immune thrombocytopenic purpura (ITP) also causes abnormal blood clotting due to a low number of platelets. Some outward signs of ITP can include tiny red or purple dots on the skin called petechiae (peh-TEE-kee-ay) and/or purple bruising known as purpura (PURR-purr-ah). These bruises appear on the skin or mucous membranes (for example, in the mouth), caused from bleeding under the skin.
There are two types of ITP, acute (temporary, or short-term) and chronic (long-term). Acute ITP generally lasts less than six to 12 months. It occurs mainly in children—both boys and girls—and is the most common type of ITP. Acute ITP often occurs after a viral infection.
Chronic ITP lasts six months or longer and mostly affects adults. However, some teenagers and children get this type of ITP as well. Chronic ITP affects women two to three times more often than men. For decades, treatment for ITP has been directed at quieting the immune system that is causing premature destruction of platelets. In the last few years, a new class of medicines that stimulates the body to produce more platelets has been very successful in treating ITP in adults and is currently under investigation to treat ITP in children.
Since the early 1980s, the federal government has recognized that persons with bleeding disorders need to be cared for in a comprehensive care center by experienced specialists. In the U.S., there are nearly 140 federally recognized and funded centers of excellence to care for such persons with bleeding and clotting disorders. These centers were originally developed for persons with hemophilia, but they are now the recognized centers of care for men, women, boys and girls with all types of bleeding disorders.
There are five such centers in the state of Illinois: four serve the Chicagoland area, and the other, The Bleeding and Clotting Disorders Institute in Peoria, serves the majority of the state south of Chicago. If you or someone you know suspects you have a bleeding or clotting disorder, or if you have questions, contact The Bleeding and Clotting Disorders Institute by calling (309) 692-5337. iBi
Dr. Michael Tarantino is the medical director of The Bleeding and Clotting Disorders Institute. He was named Physician of the Year by the National Hemophilia Foundation in 2012.