What if we got rid of the labels 'mild,' 'moderate,' and 'severe'?

In the realm of hemophilia, I believe the current classification system oversimplifies a far more intricate reality. Severe, moderate, and mild hemophilia are classifications based on the amount of clotting factor present in a person’s blood.

Hemophilia is a genetic disorder that impairs the blood’s ability to clot properly. The severity of the condition is determined by the level of clotting factor in the blood, which is necessary for blood to clot and prevent excessive bleeding.

There are several types of hemophilia, but the three most common are hemophilia A, B, and C. According to the Cleveland Clinic, hemophilia A is the most common type. “It happens when you don’t have enough clotting factor 8 (factor VIII). The [U.S. Centers for Disease Control and Prevention, or CDC] estimates about 10 in 100,000 people have hemophilia A. … Hemophilia B happens when you don’t have enough clotting factor 9 (factor IX.) The CDC estimates about 3 in 100,000 people in the U.S. have hemophilia B. … Hemophilia C is also known as factor 11 (factor XI) deficiency. This hemophilia type is very rare, affecting 1 in 100,000 people.”

Women designated as “carriers” of hemophilia A or B face a unique challenge, which is grappling with genuine bleeding issues akin to those with hemophilia. Carriers are categorized as symptomatic or not, adding another layer of complexity to an already nuanced condition. Only recently have women been recognized as having “mild” hemophilia when their factor levels are below 40% of normal levels. (Women have “moderate” or “severe” hemophilia when factor levels are below 5% and 1%, respectively.)

From my observations, the lines between mild, moderate, and severe hemophilia often blur, with people who have been classified as mild experiencing bleeding comparable to those labeled as moderate or severe, and vice versa. In my experience, having more than one “mild” bleeding disorder (hemophilia B and von Willebrand disease) results in an overall classification of “mild” despite encountering bleeding episodes more severe than the label suggests.

My journey

In my personal journey, I’ve been given a variety of labels within the realm of hemophilia B. Over different decades and under the care of different doctors, I’ve been identified as a “carrier,” a “symptomatic carrier,” “factor IX deficient,” and alternatively as having “mild hemophilia B” or “moderate hemophilia B.” The fluctuating terminology highlights the evolving nature of medical understanding and underscores the complexity of individual experiences within the hemophilia spectrum.

I frequently simplify my diagnosis by saying, “I have a bleeding disorder.” While not ideal, it helps avoid the perplexed reactions that often follow when I explicitly mention having “hemophilia.” Unfortunately, many medical practitioners still associate hemophilia predominantly with men.

Sometimes, in response to questioning, I opt for greater specificity, stating, “I am factor IX and VIII deficient,” instead of directly disclosing my diagnoses of hemophilia B and von Willebrand disease. I reserve the detailed explanation provided by my hematologist, in the form of a letter, for situations involving medical procedures or suspected bleeding events. This approach ensures that crucial information is available when needed, without unnecessary complexities and questioning.

A different approach

What if we got rid of the labels “mild,” “moderate,” and “severe”? A potentially better approach might involve specificity as stated above: “I am factor VIII (hemophilia A) or IX (hemophilia B) deficient.”

Emergency situations present a unique challenge, as medical personnel may not always have expertise in distinguishing between treatments for hemophilia A and B. Utilizing precise and specific terminology, such as “factor IX deficiency,” ensures accurate identification and prompt administration of the correct clotting factor.

The key lies in exploring whether a more nuanced and precise classification system could better serve the diverse needs of the hemophilia community. Decisions about medical terminology must involve the entire hemophilia community, including patients, healthcare professionals, and advocacy groups. Collaborative efforts can ensure that any changes are respectful, accurate, and reflective of the lived experiences of those with hemophilia.

Medical terminology evolves in response to our deepening understanding of conditions and the preferences of those directly impacted. Such changes require careful consideration, balancing potential benefits and drawbacks. It’s a conversation that must involve all stakeholders to truly capture the complexity and diversity inherent in the hemophilia experience.