What it’s like managing hemophilia with an inhibitor, part 1

First in a series.

Every person is unique, shaped by the combination of their upbringing, education, cultural influences, values, attitudes, and behaviors. No two people are exactly alike. Even identical twins, while genetically similar, have differences, including their fingerprints.

Medical conditions can also emphasize a person’s individuality. While many may share a diagnosis, how it manifests, progresses, and affects daily living varies from person to person.

For example, no two people experience hemophilia, a rare genetic bleeding disorder, in the same way. And if an inhibitor — a significant complication of hemophilia — is diagnosed, the uniqueness of the disease journey becomes even more pronounced.

As the Hemophilia Federation of America website notes, an inhibitor is an antibody the immune system produces when it identifies infused clotting factor as a foreign threat that must be destroyed. These antibodies neutralize the clotting factor before it can work, making it much harder to control and treat bleeding.

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Some people with an inhibitor may experience more frequent bleeding, severe joint bleeds, mobility problems, and chronic pain. But others with the complication may have milder symptoms that have less impact on their daily life. No single path or treatment plan exists, as each person’s body responds differently to an inhibitor, requiring a unique care approach.

As a mother of two sons with inhibitors, I’ve witnessed firsthand how unpredictable the inhibitor journey can be. My sons, Julian, 28, and Caeleb, 19, have had significantly different experiences with the complication.

My sons’ inhibitors

Julian’s inhibitor was diagnosed when he was 11 months old, and it was present for about four years until we successfully eradicated it through immune tolerance therapy (ITT). Julian was able to start the therapy because his inhibitor level was less than 10 Bethesda units (BUs). Over those four years, daily infusions of high doses of factor VIII overwhelmed his system and brought his inhibitor level to zero. His treatment was a textbook example of ITT. Julian never had a joint bleed or a major bleeding episode. Apart from that early inhibitor, his life has gone untouched by complications of hemophilia.

Caeleb’s inhibitor, also diagnosed at 11 months of age, is still present. While he and Julian were diagnosed at the same age, the similarities stop there. Caeleb’s inhibitor level was over 2,200 BUs, so treating it the same as Julian’s wasn’t possible. My youngest son’s journey has been riddled with one complication after another. I’ll share more about his journey in the coming weeks.

Over the past seven-plus years of writing for Hemophilia News Today, I’ve frequently mentioned inhibitors. However, I haven’t gone into depth about this complication. Over the next few weeks, I’ll take a closer look at inhibitors, their impact, the challenges they present, and my personal experience with my sons. I’m also excited to share insights from my conversation with Janet Brewer, CEO of the CHES Foundation, an organization that focuses on education for women and girls with bleeding disorders, the inhibitor community, and the ultrarare population in the bleeding disorders community.

Education is a powerful tool for managing bleeding disorders. Organizations such as CHES provide vital resources for those affected by inhibitors, empowering patients and caregivers with the knowledge to navigate the complexities of treatment and daily life.

Please take some time to learn about CHES’s educational programs. Visit the foundation’s calendar to learn about its upcoming conference, InhibitCon, where you can gain valuable insights and connect with others in the hemophilia community.

Note: Hemophilia News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Hemophilia News Today or its parent company, Bionews, and are intended to spark discussion about issues pertaining to hemophilia.