Mild hemophilia patients should monitor their clotting risk after surgery
Five days after surgery, my FVIII levels were still elevated
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I had a lot to learn about hemophilia when I attended my first retreat for women with bleeding disorders in 2017.
Some of the attendees were comparing their levels of clotting factor VIII (FVIII), the protein that’s deficient in hemophilia A, when I was shocked to learn mine were the lowest in the room! One participant told me I was lucky my factor levels were so low. “Hematologists will take you seriously,” she said. I remember thinking how I rarely see a hematologist.
On the second day, we talked about treatment. Another participant challenged me, “You have the lowest levels in the room, but you don’t have a treatment plan? Why?” I didn’t understand it at the time, but I now know it’s important to check in with a hemophilia treatment center to ensure appropriate care and a correct diagnosis, which I received two years later, thankfully.
While I’m “lucky” my FVIII levels are low and I now have access to treatment, I recently had an interesting experience with elevated levels. I don’t think we talk about this enough in the hemophilia community.
Surgery plan
Last December, I had surgery for superior mesenteric artery syndrome, a rare vascular compression disorder. My hematologist created a solid plan for the procedure. As part of it, I requested my FVIII levels be tested before resuming prophylactic therapy, wherein I take FVIII regularly to prevent bleeds before they occur. I use an extended half-life (EHL) FVIII product every other day.
Testing before returning to FVIII prophylaxis was critical because, historically, my FVIII levels have increased with the stress of surgery. I didn’t want to be at risk of forming dangerous blood clots if my levels got too high.
Surgery protocols for hemophilia were first developed for men with severe hemophilia, but protocols for managing surgery with FVIII now consider all people with hemophilia. Still, there are nuances to be aware of.
A person with severe hemophilia A produces little to no FVIII (less than 1% of normal), meaning their levels are still low, even when they’re stressed. People with mild hemophilia have higher factor levels than those with severe disease (more than 5% to 40% of normal). Because their bodies produce some FVIII, the stress of surgery can lead to higher circulating FVIII levels. As the World Federation of Hemophilia’s publication on mild hemophilia notes, “acute phase reaction [a state of low-grade, chronic inflammation] after surgery or in inflammatory disease will temporarily increase the FVIII level.”
Post-surgery realities
Sure enough, this happened to me again after my recent surgery. My normal FVIII baseline levels mostly fluctuate between 11% and 22%. My original surgery plan called for factor replacement therapy immediately after surgery to prevent bleeding. The product used during my surgery and for five days after was a standard half-life product, so it cleared my system faster. This protected my levels from getting too high.
On the fifth day after surgery, I was supposed to return to my normal EHL prophylaxis routine, but my FVIII levels were still elevated — over 70%! Returning to my regular FVIII infusions could have driven my factor levels too high, putting me at risk of clotting. I had to wait until the inflammatory response to surgery decreased and my levels returned to my typical baseline.
That took almost three weeks! I was so glad to be able to take FVIII, as I believe it sped up my healing. That said, I’m also thankful we chose to test to make sure I wasn’t putting myself at risk of blood clots.
We don’t often worry about FVIII levels being too high in patients with hemophilia A, but it can be a concern in certain instances, such as after surgery, for people with mild hemophilia.
Managing this disease is a delicate balance, and part of treating it is ensuring we’re not over-medicating. We do not want to put ourselves in a dangerous position or risk blood clots. I hope that more people with mild hemophilia will consider clotting risks when preparing for surgery. Creating a plan ahead of time will help us identify issues and stay safer.
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 consult your physician or another qualified healthcare 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.
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