Women with hemophilia can develop inhibitors, too
A columnist interviews 2 women about the unique challenges they've faced
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In recent years, there has been a massive shift in the understanding that female hemophilia carriers can indeed have the condition themselves. Fellow Hemophilia News Today columnist Jennifer Lynne and I both write regularly about the struggles women with hemophilia face, though more are now being properly diagnosed and treated. As this shift has happened, I have often pondered how many women will develop inhibitors.
An inhibitor is a complication in which a person’s immune system mistakenly attacks factor replacement therapy, rendering it ineffective. Inhibitors can make hemophilia treatment difficult and be stressful to manage.
When I searched the literature, I came across only four journal articles mentioning women with hemophilia and inhibitors, and three were behind a paywall. For the one accessible article I found, researchers reviewed data from the European Haemophilia Safety Surveillance registry, collected from 90 centers over 14 years. Of the 149 patients with inhibitors, only four (2.7%) were female.
Sharing inhibitor experiences
I recently had the pleasure of interviewing Maryann May from the U.S. and Eke Hullegie from the Netherlands via email. Both agreed to share their experiences as women with hemophilia and inhibitors.
When I asked them how common they thought women with inhibitors are, Hullegie shared that her doctor said there were only four known women in the literature, while May believed there were five. The reality is that we don’t actually know how many there are, but we do know it is rare. Both said they had never met another woman with hemophilia A and an inhibitor.
When I asked them when their inhibitors showed up, both women pointed to surgical experiences as the trigger. May explained that she developed inhibitors after receiving factor VIII (FVIII) to help her blood clot during and after hip surgery. Her arms were “a mess” (covered with bruises) from her walker.
Hullegie said, “When I was 41 years old, my uterus was removed. After this surgery, I received a lot of FVIII. About eight years ago, I was diagnosed with breast cancer, and after the surgery, I was given a lot of FVIII again. When everything was over, my hematologist also routinely tested for inhibitors. To her great surprise, it turned out that my blood had developed inhibitors.”
Both women were given Hemlibra (emicizumab-KXWH), an antibody-based therapy designed to mimic the action of FVIII. Hullegie also received immunotherapy twice. “The inhibitor has indeed dropped from >2,000 [units] to around 45, but it is still present,” she said.
Additional challenges inhibitors bring
“The hardest part [of life with an inhibitor] is the uncertainty with which I now live,” Hullegie said. “Not much research has been done on women with an inhibitor; all treatments are based on research and treatment in men. The lack of knowledge among doctors when something happens is also a challenge.”
May mentioned her frustration with bruising easily.
Inhibitors can affect daily life, as both women said that they’re now more careful. They try to stay active in responsible ways to prevent additional issues from surfacing.
Being a woman with an inhibitor presents unique challenges. May said that it is tricky, particularly because she is living in a new state with a new doctor. Hullegie expressed appreciation for her doctor, saying, “My hematologist is very understanding and tries to help me as best as possible.”
While this is a new challenge for Hullegie’s hematologist, she says the specialist is always thinking about new solutions. Hullegie is tested for inhibitors every six months and discusses the results with her hematologist.
Many women with and without inhibitors struggle to access care for hemophilia. May shared a reminder: “Don’t make your child [with hemophilia] the more important one. You count, too.”
Hullegie added, “As women, unfortunately, we still have to stand up for ourselves too much. I am older now, but younger women who are still menstruating have a bleed every month … and it can be treated more easily if prophylaxis [regular treatment to prevent bleeding] is allowed. This applies both to carriers who themselves have too low FVIII levels and to women with an inhibitor. Keep talking to your hematologist.”
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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