Infusions for Inclusion: Don't Let Hemophilia Keep You Sidelined

A few days before my 6^th grade class field trip to the Washington State Fair, I was standing in my yard when a falling tree branch glanced off my head. Although I suffered only a minor bruise, my mother sent a note to my group's chaperone to watch me for signs of concussion and other symptoms of head trauma that can be lethal to someone with hemophilia. The chaperone, fearful of injury, stood by me for the entire field trip while all the other kids had fun on the roller coaster and other rides.

That experience helped me develop my "teaching mode," my "Let me explain what hemophilia is" mode: "Let me tell you what I'm doing to make sure I'm like anyone else and can take care of myself."

I was diagnosed at birth with hemophilia A, and for several years of my childhood I experienced complications from an inhibitor. I spent a lot of time watching TV because of the perceived need to be "inactive" while waiting to recover from joint bleeds. That kept me from participating in activities like other kids. Fortunately, I was born in the early 1970s, not long after the discovery of cryoprecipitate -- the only adequate fibrinogen concentrate (i.e., full of clotting factors) available for intravenous use. Cryoprecipitate allowed me to transition to an in-home prophylactic infusion regimen, which enabled me to be more active. I even started going on weekend overnighters with the Boy Scouts -- and eventually attained the rank of Eagle Scout – as a prophylactic infusion on a Friday morning would last throughout the weekend.

Later in life, as a young adult with a B.A. in Microbiology from Brigham Young University, I continued to cultivate my inclination to teach, first by working at hemophilia summer camps, and later as a middle school biology teacher. By 2000 I was married with two kids and had accepted a teaching job back home "Living with a condition like hemophilia, especially as a child, you recognize quickly that you may not be able to do everything, and that your experiences may not be the same as your best friends. But I never let that hold me back." near Seattle. I began moonlighting as a guest speaker at the University of Washington Medical School, where I realized that my experience as a patient helped me augment medical students' education, as aspiring doctors typically spend approximately one week learning about hemophilia. I would tell them, "The hemophilia patient who walks in your door will know more about the disease than you do."

Now I'm 46 years old, and our family includes three daughters and one son. Because hemophilia is a gender-linked recessive condition, I've passed on the "hemophilia X chromosome" to each of our daughters, making them what's known as "obligate carriers" of the disease. More than ever, I appreciate that knowledge is power – not just for the person with hemophilia, but for carriers and other family members as well.

I also appreciate the importance of adherence to a prophylactic infusion regimen, especially for teenagers with hemophilia, who, left to their own devices, will try to infuse as infrequently as they can get away with. They may not understand that non-compliance can lead to joint damage, with far-reaching health implications later in life. Whenever I talk about hemophilia, I say, "Don't wait for a bleed to occur; do what the doctor says." Every bleed into a joint only makes the disease worse. A five-minute infusion is worth the health of your joints.

My experience also enhances my understanding of how a person’s activity level can affect his or her prophylactic infusion schedule. A highly active person may be at higher risk of joint bleeds and injury and therefore should speak to their doctor about the right dosing frequency for them. In short, you and your doctor need to find the right therapy to understand what works best for you based on your level of activity.