Fighting for proper care as a woman affected by bleeding disorders

For Ashley Zebley, a symptomatic carrier, getting treatment has been a battle

At 32, Ashley Zebley from Arkansas is no stranger to bleeding disorders. I recently interviewed her over the phone.

Ashley explained that her father has severe hemophilia B, and both her maternal grandmother and great-grandmother exhibited bleeding problems, although neither was diagnosed with hemophilia. Ashley herself is a carrier of hemophilia and has exhibited bleeding symptoms throughout her life.

As a parent of three daughters, Ashley became concerned when one began displaying troubling symptoms, including excessive bleeding after dental extractions. “As a toddler, she had a giant lump on her head,” Ashley recalled. “It was big, purple, and didn’t go away for months.”

Despite these alarming signs, it wasn’t Ashley’s local hemophilia treatment center (HTC) that tested her daughters. Instead, it was a geneticist who understood the complexities of lyonization (also called X chromosome inactivation) and the possibility of hemophilia in women. “She was fabulous,” Ashley said, reflecting on the geneticist’s comprehensive approach.

When Ashley presented her daughter’s genetic test results to her HTC, further testing revealed her daughter had a factor IX level of 17 units/dl. (Normal levels range from 50 to 150 units/dl.) That led to a diagnosis of mild hemophilia, and her daughter was prescribed factor when needed.

Despite finally having answers for her daughter, Ashley expressed frustration, noting, “We didn’t get the level of education we deserved. Females receive lower-tier healthcare.”

The following are excerpts of our conversation.

JL: When did you first suspect you might have a bleeding disorder?

AZ: Around the age of 12, I started having periods and underwent knee surgery for joint issues. After what should have been a minor procedure, I experienced severe swelling and bruising. My parents took me to the ER [emergency room], and later a hematologist explained I was a carrier and I have a qualitative platelet disorder. My factor [IX] level was 52 or 53 [units/dl], and I was seen every other year. But these visits were more about preparing me for future pregnancies than addressing my bleeding problems. I always felt like they overlooked what was happening in the moment.

What were your periods like?

School was a nightmare. I constantly bled through my clothes and had to keep a jacket on hand to tie around my waist. I carried eight super-plus tampons in my purse and used one every 45 minutes. By my late 20s, I became anemic and started iron infusions, but it wasn’t easy.

Eventually, I had a hysterectomy to stop the bleeding. It was one of the hardest decisions I’ve ever made. I didn’t want a hysterectomy — I didn’t want to lose the option of having more children. But I couldn’t keep living like that.

What has your treatment experience been like?

Honestly, it’s been a roller coaster. I was offered factor several times, only to have it taken away. Once, I was even given a travel letter advising the use of factor in emergencies, but I wasn’t prescribed any.

Earlier this year, I was in a car accident. The ER doctor didn’t know how to handle my bleeding, and that was a turning point. I realized something needed to change, or I would end up dying someday from my bleeding.

What changed for you?

I’d heard about [hematologist] Danielle Nance for years, and I finally decided to see her. The experience was night-and-day different. She had reviewed my records, understood my qualitative factor disorder, and prescribed me factor. She listened, ran genetic tests, and took the time to help. It was life-changing.

What advice do you have for others?

Find a doctor who’s willing to dig deep. Don’t be afraid to switch providers. I’m a deep empath, and I was worried about insulting my previous doctors, thinking they knew better than me. But we know our bodies best, and it’s vital to have someone who listens and takes your concerns seriously.

Most importantly, don’t give up. I won’t lie. I had some dark thoughts. I was ready to quit, but then I reached out to Kim Phelan from the Coalition for Hemophilia B, and others. They didn’t let me give up. Let others support you and help you find a way through.