Women Can Have Hemophilia, Too

Originally published on Hemophilia News Today by BioNews, Inc.

Imagine you are in an emergency room with a life-threatening bleeding problem. How would you convince an ER doctor that you are a woman with hemophilia if they don’t think women with hemophilia exist?

I have both von Willebrand disease (VWD) and hemophilia B, a less common form of the disease that arises when a blood-clotting protein called factor IX is missing or defective.

When I tell doctors I have VWD, they nod their heads. They are somewhat familiar. When I reveal I have hemophilia, they shake their heads and raise their eyebrows. Most have learned that hemophilia is not supposed to happen in women. They assume I am confused.

Hospitals are scary for women with hemophilia. I once overheard an ER doctor say to his medical team, “Hemophilia happens to men; von Willebrand disease happens to women.” My blood boiling, I shouted from the exam room, “Excuse me! Excuse me! That is not true.” Both bleeding disorders can occur in either gender.

Being a woman with hemophilia requires me to be an expert about my condition so that I can educate others. I wear a medic alert bracelet and carry an emergency letter validating my diagnosis. I’ve learned to infuse clotting factors and bypass my local hospital whenever possible.

The plan

I needed a radical hysterectomy and hernia repair in 2019. My hematologist wrote a treatment plan for this major surgery. The plan tells the surgeons what dose of medications I need before, during, and after surgery. It’s like a surgery cookbook for bleeding disorders.

After several life-threatening complications, I scrutinized the plan and realized it said it was “to support his disease process.” The plan for my hysterectomy assumed I was a man.

Carrier and label confusion

This has been a longtime issue for me. More than 20 years ago, my primary care doctor called to let me know that my factor IX level was 38. He said, “Don’t worry, it means you are a carrier of hemophilia. Women can’t have hemophilia.”

This doctor didn’t realize factor levels below 50% are diagnostic of hemophilia. Fortunately, I was already working with a hemophilia treatment center.

Women with hemophilia need to be labeled consistently based on factor levels. Carrier. Symptomatic carrier. Mild hemophilia. Factor IX deficient. I’ve had all these labels based on my factor IX level through the years. It makes a difference. Access to care and treatment requires a proper diagnosis. The clotting factor used to treat hemophilia is costly. Labels are important to insurance carriers.

Also, graphic illustrations in medical literature and elsewhere that are intended to show how hemophilia occurs need to be updated for women. Oversimplified images of the genetics of hemophilia do a disservice to women. Most of these images label women as carriers. Women with hemophilia, like me, are left out. When people see images like these, they may assume incorrectly that women can only be carriers and not hemophiliacs.

Let’s stop questioning

A huge step forward would be for women to not be questioned about their hemophilia diagnosis. For example, last month, my primary care doctor told me, “I don’t have any patients with hemophilia.” I blurted out, “But you have me!”

I’ve heard stories of women using their son’s clotting factor to treat themselves because they struggle to receive a diagnosis. Bloody noses, heavy menstrual bleeding, and surgical complications are symptoms of a bleeding disorder. Women with these problems deserve to be tested and offered treatment before a life-threatening emergency arises.

Yes, women with hemophilia exist. It’s time to hear our voices.