Many people use art, music, or writing to cope with events in their life. Personally, my platform is poetry; I love the way that I can use words to describe how I’m feeling or what I’m struggling with. But for 16-year-old Erin Smith, who has Von Willebrand Disease (vWD), theater became an unlikely reprieve. Admittedly, Erin explains in the Albany Democrat-Herald, she was never extremely interested in theater growing up. But when she heard about “Hemophilia: The Musical,” she knew that she had to join in.
Von Willebrand Disease (vWD)
Von Willebrand Disease (vWD) is caused by either an inherited or spontaneous genetic mutation. Over 500 mutations are linked to this disorder. vWD is named after Erik Von Willebrand, who first discovered the disorder nearly 100 years ago. Patients with vWD don’t have enough Von Willebrand factor (VWF) in their blood, inhibiting clotting. As a result, patients experience heavy and excessive bleeding, easy bruising, and frequent nosebleeds.
vWD is considered the most common inherited bleeding disorder, even over hemophilia. An estimated 1% of the population has this disorder.
Altogether, there are 3 forms of vWD:
- I: Around 3/4 of all patients with vWD have this type, making it both the most common and the mildest form. Generally, people with Type I have lower than average VWF levels.
- II: In Type II, patients experience more severe symptoms. Instead of having low VWF levels, patients with Type II have normal – but non-function – VWF levels. Type II also has subtypes:
- 2A: The most common form, with a protein defect or mutation that prevents blood platelets from binding together.
- 2B: VWF binds to platelets in the blood stream instead of at the injury site. As a result, too many platelets are removed from circulation, causing a shortage.
- 2N: The most rare form. VWF works normally, clotting at the site of injury. However, factor VIII (which also helps clotting) isn’t brought back into circulation. vWB type 2N may be mischaracterized as hemophilia A.
- III: This is the rarest form and also the most severe. Patients with this type of vWB generally have little or no VWF.
- Bleeding gums
- Bloody urine or stool
- Easy bleeding or bruising, even without injury or trauma
- Joint pain and inflammation
- Excessive bleeding following surgery or dental procedures
- A long and painful menstrual flow
- Difficult nosebleeds
Learn more about vWD here.
Growing up, there were a few things that Erin couldn’t do, like contact sports. Sometimes this didn’t bother her. She grew up understanding her condition; even if something happens, she simply gives herself a shot to promote clotting. However, sometimes Erin felt sad or isolated. Like many people in the rare disease community know, it can be discomforting to always be “set aside” from others, or to always have to explain your condition.
So when she first heard about “Hemophilia: The Musical,” Erin was thrilled. The brainchild of director Patrick James Lynch, who has hemophilia A, the musical is designed to empower teenagers with bleeding disorders. The first show occurred in 2018, but has built up since then. The goal isn’t just to uplift acting, says Lynch. Rather, he encourages his actors to:
speak up for yourself as an advocate to coaches, teachers and employers about this [bleeding disorder] you’re going to have for the rest of your life.
Erin applied for the show and was eventually chosen as part of a 24-person cast. Everyone was between 13 and 18 years old and all had experience with bleeding disorders: either their own or a family member’s. Throughout their experience, Erin and her peers explored what it meant to have a bleeding disorder: education, awareness, treatments, advocacy. In doing so, the performance would raise awareness outside of their community.
Although the musical became a Zoomsical thanks to COVID-19, the cast had an amazing time getting to know each other. Hopefully, says Erin, they will all get to meet once lockdowns ease.
“Hemophilia: The Zoomsical” premieres tonight, July 31, on Facebook Live. However, if you miss the first performance, don’t worry! A Youtube video of the musical will be uploaded shortly.