I turned 50 this year! As I reflect on how hemophilia has been a very large focus of my life, I am struck by the fact that my focus has always been on the males in my life with severe hemophilia, whom I love. However, I myself have also struggled with bleeding, but as a woman with a bleeding disorder we are often in the shadows of the men around us.
My older brother Jimmy was born with severe hemophilia before factor replacement therapy existed. When I was a baby, he was in the hospital a lot. As we grew up, factor became available and I have clear memories of being awakened in the middle of the night to meet the doctor at the back door of his office, so he could give my brother his medication to stop his bleeding. It was terrible watching Jimmy suffer through the pain of bleeds and the multiple needle sticks that he would receive. Growing up, the focus was naturally on him a lot, and I did my part to keep him company and to care for him when he was sick. He was my hero, suffering bravely. In 1987, when he was diagnosed with HIV, it was a massive blow to our family and others, whose loved ones were also being diagnosed. Jimmy fought valiantly, but lost his battle in 1993, at the age of 28. That left me alone, to be there for my grieving parents and to figure out how to be a parent myself to my son, who was born with hemophilia just 10 months after Jimmy died. My son, Jay, later developed inhibitors after an intracranial hemorrhage at the age of 11 months.
Having a child with hemophilia b and inhibitors is hard. There is no way to sugar coat it. I went from watching my brother suffer, to watching my precious baby boy suffer. My son is now a man of almost 24 and I continue to watch him struggle.
But throughout this journey of being supportive of my brother and my son, I have had many bleeding issues of my own that often went untreated and I suffered in silence. At the ages of 6 and 12, I had teeth removed for a straight smile. After each of these oral surgeries, I bled for at least a month. Dealing with the nausea, bad breath, blood loss and discomfort was hard, but doctors continued to tell us that “girls do not bleed.” When I was 15, I had a major jaw surgery to correct my severe overbite. My jaw was wired shut for 8 weeks and during that time my incisions continued to spurt blood into my mouth every time I laid down on my side and put any pressure on my cheeks. Finally, my brother Jimmy demanded that the oral surgeons test my factor IX level, as I was getting ready to have my wisdom teeth out. I was sent for the bloodwork and the doctors stated that the results came back completely normal. I had my wisdom teeth out and again continued to bleed. I was awake night after night sitting in a chair, so I wouldn’t gag on the large clots that would form down my throat. This time Jimmy took me back to the oral surgeon and asked to see the test results. They had tested my factor VIII level, which of course was normal. Jimmy again demanded the factor IX test, and my level came back at 10%. After that I was considered a symptomatic carrier.
After giving birth to my first child in 1994, I attempted to advocate for my bleeding disorder but I wasn’t taken seriously. I ended up having a massive hemorrhage that went undiagnosed for 6 days, because the male OB decided I was making things up and refused to see me. When my husband called and explained that I was passing out in the shower and passing clots the size of grapefruit, he told my husband over the phone that I was suffering from postpartum depression and not eating enough. Finally, I began to lose feeling in my legs and I insisted that the doctor see me. When I walked in they immediately began taking me seriously. With a hemoglobin of 4, I ended up having emergency surgery and 4 units of blood.
Thankfully, things are beginning to change as more doctors recognize that women do indeed bleed and we have very unique and specific issues. I am so happy that my twin girls can be seen at a Women and Girls Clinic to handle their bleeding issues. I have also found a great team that takes me seriously and makes sure that I get treatment when needed. I am hopeful that research for women with bleeding disorders will continue to advance so that we no longer have to live in the shadows.