What I Wish I Had Known

by Pam WIlliams

December 1997 forever changed my family’s life.

That was when hemophilia B entered our lives. My oldest son, Joshua, was 11, had just undergone ear reconstruction surgery at Cincinnati Children’s Hospital. As we were leaving the ENT’s office for his post-operative check, his ear began to bleed profusely. Lucky for us, his ENT often worked closely with the hematology department and knew exactly which lab tests to order.

The next day, we were contacted by the hospital to return, where my son was admitted for additional testing and treatment. I remember telling my husband at the time, “You better not lose your job - we’re going to need really good healthcare.” Thinking back on that conversation, it probably wasn’t my best moment or the best reaction to have.

Back in 1991, Joshua began waking up every morning covered in bruises. Having had a young cousin who died of leukemia, I knew one of the symptoms was unexplained bruising. I instantly took Joshua to his pediatrician at Dartmouth Medical Center. A pediatric resident saw him and ordered bloodwork, but it was obvious he thought the unexplained bruising could only mean one thing – child abuse.

As we stood in the hospital hallway, Joshua’s pediatrician came walking towards us, “What’s up, Joshua?” Joshua replied, “I have bruises. I haven’t been playing King of the Mountain at recess, I promise.”

The doctor immediately began checking out the bruises, then hurried him back to an examination room where she found the resident getting ready to write “child abuse” in Joshua’s chart. She grabbed the pen from his hand and informed him there was no way Joshua was being abused, and that she had known him since he was born. She reviewed the bloodwork list, added more tests, and sent us home to wait for the results.

Several days later, she called to say that Joshua did not have leukemia. That he has a type of blood disorder, but it was nothing to worry about. We didn’t question anything and for the next six years, while there were many signs that pointed to something significant, we just chalked it up to life with a boy and moved on.

As a female, I had abnormal menstrual cycles, but no one questioned them. The typical response to my concerns was, “It’s normal, every woman has heavy bleeding and long cycles, you’re fine.” Since it wasn’t known at the time that my dad had hemophilia B, I was none the wiser. Even after a miscarriage and giving birth to Joshua and his younger brother, Thomas, no one was concerned about the amount of blood accompanying those births. Even when I mentioned to my OB/GYN that I bled continually for 6-8 weeks following childbirth, sometimes passing clots the size of quarters, she brushed me off as a new mom who hadn’t taken time to recuperate before returning to a full-time job.

Back to 1997, Thomas, as well as my dad, were diagnosed with mild factor IX deficient hemophilia.

My dad was 62 years old, had been raised on a farm and spent time in the Army during the Korean War. He never knew; however, his siblings had been told by their father that, “Fred is a bleeder.” No one knew what that meant exactly, which meant that Dad had never been treated for hemophilia B and thus, had no idea having a daughter would mean the disorder would be passed on genetically and the circle would continue.

So, what do I wish I had known? If I had known I had hemophilia B or at least, known I was an obligate carrier, I would not have married the man I did. I would have been able to tell those I dated that I had a bleeding disorder and what it would mean for our future lives and childbearing. They would have known from the beginning giving birth to sons would mean a 50-50 chance the son would have hemophilia B.

When my now ex-husband found out Joshua and Thomas had a bleeding disorder, he said, “This is all your fault. My sons will never be ‘real boys,’ you know, the kind that play football or join the military.” It was at that moment I realized had been aware of my genetic history, he would not have been my choice for a spouse. It took me three years to leave the marriage.

Hemophilia B has brought so many wonderful people into the lives of my boys and my life. I met so many families who have approached hemophilia together, no one blaming anyone, and couples staying together.

Sure, some families split, but most often for reasons unrelated to hemophilia. While our marriage dissolved for many reasons, I do feel the diagnosis of hemophilia played a part in the demise of our union. I noticed most hemophilia families stuck it out. They do what they need for insurance and even pursue employment based on the needs of their child/children.

Both of my sons entered their relationships upfront and honest with their significant others regarding their bleeding disorder. They both entered marriage knowing that having a daughter would mean she was an obligate carrier and as we now know from our advocacy and education, would possibly be diagnosed as a female with mild hemophilia B.

I would have entered marriage knowing my mate was fully aware what having children would mean for us... specifically, what having boys could potentially mean. I would have been able to determine in advance how the potential mate would respond to the possible genetic complications and whether he would have what it takes to go the long haul knowing the treatment and costs.

I would have made different choices. I would have put all the cards on the table and watched his expression when I explained everything. I would have made sure my mate understood what a bleeding disorder meant for my health as well. We would have entered a relationship and marriage with eyes wide open and been more prepared for our future.

Over the past 26 years, I have learned a great deal about bleeding disorders and hemophilia B. I’ve learned that being a female with hemophilia is a long road to a diagnosis and treatment. Sometimes, it takes trial and error to find the hematologist who will listen to you, order the proper testing, and agree to treat with the necessary factor replacements.

As women, we still have a long road ahead of us to reach equality in these areas. With other hemophilia women, we will continue to advocate and push for diagnosis and treatment until we are taken as seriously as our male counterparts. It is an ongoing mission and trek.

Shoulda. Woulda. Coulda. What I wish I had known.