Hemophilia: A Patient’s Perspective

VAL BIAS, CEO of the National Hemophilia Foundation (NHF), has lived with hemophilia his entire life, beating odds almost from the start. At the time of his birth, every male member of his family with hemophilia had already died. When Val was in the fourth grade, he came across a hemophilia section in a school textbook and learned the average life expectancy for someone with his condition was 20 years. At age 10, Val came to the shocking realization his life was already half over. Despite predictions to the contrary, Val survived well past the anticipated two decades, only to find his life threatened again by an equally formidable foe: human immunodeficiency virus (HIV).

Understanding Hemophilia More than three million Americans have a bleeding disorder such as hemophilia, von Willebrand disease or rare factor deficiencies. These disorders prevent the blood from clotting normally, can result in extended bleeding after injury, surgery or trauma, and can be fatal if not treated effectively. Because of this, people with hemophilia depend heavily on clotting factor replacement therapy derived from human blood or a recombinant developed in the lab.

It was through an infusion of clotting factor concentrate derived from human plasma that Val contracted HIV and hepatitis C. It’s well documented from the late 1970s to the mid-1980s (prior to more stringent blood safety measures) HIV, hepatitis C and hepatitis B from infected blood donors made its way into blood products. As a result, nearly half of all people with hemophilia became infected with HIV, many developed acquired immunodeficiency syndrome and thousands died, including many of Val’s friends, colleagues and his beloved first wife, Katie, who unknowingly contracted the virus from Val.

The year Katie died, Val attended the NHF annual meeting, and his subsequent involvement led to him being elected chairman of the board. “We began a crusade to help people who developed HIV from tainted blood products,” he remembers. The work culminated in the passage of the Ricky Ray Hemophilia Relief Act of 1998. Ten years later, in 2008, Val became NHF’s CEO.

Since stepping into the role of CEO, Val has led the organization to the forefront of the healthcare reform debate on such issues as the elimination of lifetime caps on insurance benefits and coverage for pre-existing conditions with insurers. In addition, he has greatly expanded NHF’s research agenda and created the Women’s Health and Bleeding Disorders Institute to address the growing need for awareness and treatment for women with bleeding disorders. “In recent years, great strides have been made in treatment, public policy and advocacy for the bleeding disorders community,” says Val. “When I came on board, we had a three-quarter million [dollar] deficit. Today, we have a $25 million budget and $14 million in reserves. We had 35 chapters in 2008, and now we have 52 and a staff of almost 80.”

Advocating for Quality of Life

Today, this seasoned survivor and advocate is focused on helping NHF expand its research agenda, with an emphasis not just on bleeding disorder cures but also quality of life for this diverse community. “Life expectancy for people with bleeding disorders is normal today,” he explains. “But, it’s not just about how long you live but also about the quality of your life. You might live to be 70 years old, but if you get up and go to work every day and come home to your family and have no energy to engage socially, your quality of life is not what it could be.”

Val has also concentrated his efforts on raising national awareness regarding bleeding disorders. In 2016, he led a successful effort to have the month of March designated by the U.S. Department of Health and Human Services as the first-ever Bleeding Disorders Awareness Month. He also helped launch the Red Tie Challenge, a movement intended to start a national conversation about bleeding disorders.

Val notes current research is shifting its focus to products in the pipeline that can potentially increase people’s ability to live fuller and more active lives. “Some of the most promising trials are demonstrating the possibility that many patients could live very normal lives,” he explains. “There are questions, of course, about the costs. What remains to be seen is if these products will be accessible and reimbursable for everyone who needs them.”