I first became aware of the bleeding disorder hemophilia when I was in high school reading about Nicholas II, the last Russian tsar.
His son had severe hemophilia B, which was untreatable at the time and most frequently led to an early death. Hemophilia became known as “the royal disease” because the faulty gene was carried by many of the nobility.
I found it very interesting, and over the years, I’ve known people with hemophilia A and B, and also a friend who has the lesser known bleeding disorder von Willebrand disease, also known as vWD.
With any bleeding disorder, the person’s blood doesn’t clot properly and without treatment, severe disability and sometimes death can occur. The various types of hemophilia are caused by lack of clotting factors (Factor VII, VIII, and IX), but with vWD, the person has low, or non-existent levels of a certain protein called von Willebrand factor, which also carries Factor VIII. It’s classified into three types, Type 1, Type 2, and Type 3. Type 1 is the mildest form. 75% of people with vWD have this type. They generally have lower than average amounts of von Willebrand factor and Factor VIII in their blood.
Type 2 occurs when the von Willebrand factor doesn’t work well and is broken down into three subtypes, 2A, 2B, 2M, and 2N. Because treatment is specific to each sub, it’s crucial to know what form of vWD the patient has.
Finally, Type 3 occurs when the patient das no detectable von Willebrand factor and low levels of Factor VIII.
Von Willebrand disease is usually not as serious as hemophilia A and B (although women who have seemingly never-ending periods because of it may beg to differ…) and occurs in both males and females, while hemophilia is usually passed down to male offspring.
Today, factor replacement therapy has changed the face of all bleeding disorders. Yet, I feel wistful when I think of Tsar Nicholas and his family. They all worked so hard to protect their only son and brother—to help him prevent bleeds, and to stay alive. In 1918, their efforts came to a sad close when the entire family was executed by the Bolsheviks. History will never know if his four daughters were all carriers of the faulty gene that caused their brother’s hemophilia.
