By Sylvia Foley, AJN senior editor
Quick, what’s the most common inherited bleeding disorder? As you might have guessed from the giveaway title, it’s not hemophilia. It’s von Willebrand disease, and it affects about 1.3% of the U.S. population.
Yet many nurses and other clinicians, as well as the public, are unaware of this disease, its symptoms, and the associated risks. Sequelae can include complications during pregnancy and childbirth, chronic joint disease, even death. In girls and women, menorrhagia is the most common symptom; while the disease is equally prevalent in both sexes, it can be more problematic for menstruating females. In our June CE article, “Just Heavy Menses or Something More? Raising Awareness of von Willebrand Disease,” author Josie Weiss seeks to improve the odds that nurses will know when to suspect that a patient has the disorder and what to do once diagnosis is confirmed.
Simply put, von Willebrand disease is caused by “genetic defects in the concentration, structure, or function of von Willebrand factor,” which is a protein essential to blood clotting. These defects result in episodes of excessive or prolonged bleeding, which can be mild to severe in intensity. Weiss describes the three main types of the disease (which are distinguished by their pathophysiologic mechanisms) and lists suggestive signs and symptoms, which include the following:
- family history of a bleeding disorder
- heavy or prolonged menses since menarche
- heavy menses that don’t respond to conventional management
- bruising (larger than 2 cm) without notable injury
- prolonged bleeding (more than 5 minutes) following minor injury
- one or more prolonged nosebleeds (more than 10 minutes) within the year
- oral bleeding without an obvious cause
- prolonged or excessive bleeding during or after dental procedures
After outlining what the investigation of a suspected case should entail, Weiss explains why diagnosis can be challenging. She goes on to describe management, which “is aimed at preventing excessive bleeding and at stopping it when it occurs.” Pharmacotherapy and patient education are covered in detail. An illustrative patient case is also provided. The article is free online.