Bleeding Disorders 101

Although there are many types of bleeding disorders, Hemophilia and von Willebrand disease are two of the most well known. Both are inherited disorders that cause prolonged bleeding episodes.

Hemophilia affects approximately 20,000 persons in the United States. This genetic disorder is characterized by a missing or deficient clotting protein in the plasma, causing delayed clotting (prolonged bleeding). Hemophilia occurs almost exclusively in males. While there is an incidence of mutation, the genetic defect is carried on the X chromosome. As a result, women are carriers of hemophilia but rarely have the disorder.

carierThere are two major types of hemophilia – A and B. About 80-85% of all persons with hemophilia have hemophilia A (factor VIII deficiency); 15-20% have hemophilia B (factor IX deficiency). Each type has different levels of severity. A person with mild hemophilia may not have problems with bleeding except for surgeries or injuries. A person with severe hemophilia may have spontaneous bleeds for no apparent reason. Treatment for all bleeding episodes involves replacement, by IV injection, of the clotting protein missing from the blood.

autosomalDominantvon Willebrand disease (vWD) is much more common, found in approximately 1-2% of the U.S. population. Like hemophilia, vWD is inherited from parent to child. Unlike hemophilia, vWD occurs equally in men and women.

Persons with vWD have a deficiency of the blood protein called von Willebrand factor. As with hemophilia, there are different levels of severity. Common symptoms are prolonged nosebleeds, easy bruising, and abnormal bleeding after surgery, dental work, or injury. Women are more likely to be diagnosed with the disease because of symptoms of heavy or abnormal bleeding during their menstrual periods and after childbirth. Treating for vWD bleeds is varied and based on severity and type, along with the person’s response to the different treatments.