Why access to treatment is crucial with von Willebrand disease

Originally published on Hemophilia News Today by BioNews, Inc.

A columnist's friend faces medical setbacks due to an urgent need for treatment

I’ve previously written about fellow Floridian Kayla Mack, a woman in her 50s who has Type 3 von Willebrand disease (VWD). Her severe bleeding disorder is similar to those who deal with severe hemophilia, as her blood clotting factor VIII levels are nearly negligible. Her von Willebrand factor levels are also significantly low, a hallmark of the disease.

When I interviewed Kayla last January, she noted that, “My VWD has complicated every single thing that has gone wrong in my body. Surgeries stretch out for weeks. My patience has been constantly tested. I do everything in my power to stop a bleed on my own. The ER is absolutely my last line of defense.”

Kayla’s words could easily be my own.

She resides in the same part of the state as I do, where the prospect of seeking medical care in hospitals can evoke feelings of panic. She told me about her constant dread of encountering an unexpected bleeding episode and the unsettling thought of not receiving the necessary treatment. Regrettably, a concerning tendency exists among emergency room physicians to underestimate or overlook women who deal with bleeding disorders.

Emergency intervention

I connected Kayla with my hematologist at a federally funded hemophilia treatment center in Tampa. Following their consultation, doctors recommended that Kayla use prophylactic treatment to prevent her bleeding rather than relying on reactionary measures.

But Kayla encountered obstacles with her insurance provider when attempting to access her medication. Additionally, her attempts to self-administer the infusions were challenging, particularly when trying to locate a suitable vein. When she pursued the possibility of having a port placed to facilitate the infusions, her insurance company denied the request, further complicating the situation. The inability to implement an effective treatment plan put Kayla’s life at risk.

Earlier this month, Kayla’s husband, Terence, discovered her lying on the bathroom floor, nearly unconscious, in the middle of the night. His swift actions saved her life. Kayla says that, had he not been present, she doubts she could have mustered the energy to dial 911.

When the paramedics arrived, her blood pressure was a worrisome 50 over 30, underscoring the severity of the situation. At the emergency room, she underwent two blood transfusions.

“Things were really touch and go for a while,” she told me in a Facebook message.

Doctors found an active bleed in the lining of her stomach and performed surgery to cauterize those areas. At the same time, a port was placed so Kayla could finally get the prophylactic treatment she had long needed.

Later, she was discharged from the hospital and went home. But the bleeding started again. She was rushed back to the hospital and admitted into intensive care.

“My fear is that I will die shopping at Publix,” Kayla said. “My bleeding disorder has become very dangerous over the past three years. I am no longer safe without [prophylactic treatment]. I don’t want to ever have to do this again.”

Kayla plans to start using her port to receive prophylactic treatment as soon as she is home from the hospital.

In the face of adversity, Kayla’s unwavering spirit shines through. Her determination to embrace a new treatment approach symbolizes hope for a life less defined by the challenges of her condition. As a friend and witness to her journey, I stand alongside her, united in the wish for her swift and complete recovery.