A Lifetime of Von Willebrand Factor and What it Can Look Like
As a child, I was a bit of a tomboy. I was reckless and rough on the playground and loved playing baseball during recess. Typically on the team we created, my position was back catcher, and as much as I enjoyed it, I never truly understood the risks that I put myself into.
During a ballgame, one season when I was around 10 years old, I was hit in the face by a ball. I clearly had missed a hard throw from our pitcher and the next few hours were a blur.
I remember the pain in my face and seeing blood. A LOT of blood.
It ran down my hands and bare arms as I held my face, and I remember a teacher running towards me before I blacked out.
I awoke in a hospital bed and it felt like my face was ten times as large as normal. The excruciating pain in my nose brought tears to my eyes. When I touched my nose, I remember feeling confused, as it was swollen to the point I couldn’t open my eyes all the way. As a young girl, laying in a bed surrounded by strangers, pain and an enlarged face, I was terrified, as I yelled for someone-anyone-to come and tell me what happened. I couldn’t quite recall how I ended up in the hospital and was beyond freaked out.
A nurse came into my room and asked me questions about my head. She wanted to know if I had pain, but all I could do is nod or shake my head. Because my nose was so enlarged, my voice was different and only loud whispers seemed to come out.
Before I knew what was happening, the nurse looked at my face and tugged on something attached to my nose. She pulled and pulled a long streamer of what looked like bloody toilet paper out of my face. I swear there were well over 100 feet of the odd paper shoved up my sinus cavity. I could feel the sensation in the back of my throat and behind my eyes as she pulled the blood-soaked tissue out of my face.
That should have been the beginning of an understanding of what was wrong with me. But, it wasn’t.
My very first menstrual cycle started on my 13th birthday. I recall waking up in a sleeping bag, after having a few girls over for a sleepover, and I was soaked. At first, I thought it was sweat, but was horrified to realize that I was covered from my breasts to my ankles in blood. I honestly don’t know how I slept through the events that occurred during the night. I looked like Carrie, covered in pig’s blood on prom night, but from toe to breast, instead of head to toe.
My mother was rarely present in my life during my childhood and teen years, so I never had anyone to discuss periods and such with. My sister and I were never close, and by this time, she had moved out of the house.
The only people I could ask secretly for advice were my peers. I was too shy to ask about the amount of blood or other concerns I had, so I simply dealt with it. I hid in the bathroom to tidy up and shyly told my friends that I started my cycle. Most of them had at that time, so I felt like I belonged, never thinking to ask them questions about the severity of my bleeding.
I also didn’t understand that it wasn’t “normal” when my periods lasted 6 or more weeks, every time my cycle began. One would end, and a week or so later, another one would begin. There was rarely a time during my teens years when I wasn’t having my period. I was always tired and felt weak, and as time passed, I finally asked my mother about it. I always had a sweater or jacket and spare pants in my school locker, because inevitably through the day my pants would be soiled in blood. I became a pro at wrapping clothes around my waist to hide the mess.
“Mom,” I stammered, “How long are periods supposed to last, and is it normal to bleed through three maxi pads and a tampon at the same time?”
Bear in mind, this was the beginning of the ’80s and it wasn’t “proper” to discuss such personal issues. My mother was very old school when it came to “lady talks”. She would always whisper her part of the conversation, in case a man was within earshot of our private topic.
“My periods are long too. You just deal with it,” she whispered. “I will buy you more supplies when I go to the store.”
Periods and nosebleeds seemed to take over my teen years. I had my sinuses cauterized more times than I care to remember. If I cut myself or scraped a knee, the amount of bleeding that ensued seemed to always be excessive. Often times I would bleed through multiple band-aids for the smallest cut. If I told my mom or dad that I bled a lot, they brushed it off as me “whining” or being dramatic.
It was a different world during that time. Kids were tougher, and always outside playing. I had a lot of minor injuries that bled a lot, but over time learned to just suck it up and not make a big deal of it.
At the age of 17, I started dating a young man, who I would later marry. In our first 3 years together, I had 3 rough, very dangerous miscarriages. The second one caused me to stay in the hospital for days, as they worked on ways to try and stop me from bleeding. I had cauterization done internally, they attempted a D & C, which made the bleeding worse, and life-threatening, and finally, they decided to try and give me coagulation therapy and medication to stop me from hemorrhaging. I was so weak, I could barely get out of bed to use the washroom, and when they tested my blood they determined that I was extremely anemic. There was very little iron or proteins in my blood.
That was when a specialist within the hospital was brought in.
As he stood over my bed, he asked about a few large bruises I had on my arms and legs. I was always covered in bruises and chalked it up to being a klutz. It was a running joke with my boyfriend/husband that I could literally find anything to bang my legs against or bruise my arms on. I was rarely without dark black bruises.
I told the doctor about my clumsiness and instead of responding to my description of myself, he told the nurse to order blood work for me. He then explained that my bruising did not appear “normal”. He asked me if I was being physically abused by anyone, and didn’t seem shocked when I told him no.
Shortly after the visit from the doctor, a nurse came in with a tray carrying a syringe with about 15 vials for my blood samples. She asked me if I was feeling all right before poking me, and told me I looked extremely pale. I just wanted to get the blood work over and said I was fine. I watched as she drained my vein, vial after vial. After the 5th or 6th, I blacked out and woke up to machines connected to me and was told I needed to stay still while they tried to stop my vein from surging blood. The nurse was very kind and had a furrowed brow of concern, thinking that she had done something wrong. I ended up requiring a blood transfusion within the next few hours.
Before being discharged from the hospital, following miscarriage number 2, a hematologist appointment was made for me. I was told that I had VWF and the doctor asked me if I had heard of it before.
He then went on to tell me that I was at least a Type 1, but most likely a Type 2 category level hemophiliac, under the Von Willebrand Factor.
Once he explained that this is why I bleed so much and bruise so easily, I sobbed. I wasn’t crying because of the fact that I had been diagnosed. I sobbed because at that moment, I KNEW I wasn’t crazy, and that I wasn’t just being “dramatic” about it. It was such a relief to give this oddity a name.
I finally felt like “someone” understood what I had been going through with all of the extra blood that came out of me.
The explanation he gave me was vague, however, and since then I haven’t done a ton of research on the “disease” for fear of freaking myself out. If I feel concerned, I try to avoid Googling questions, as there is limited and often skewed information about VWF.
Von Willebrand disease (VWD) is a genetic disorder caused by missing or defective von Willebrand factor (VWF), a clotting protein. VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process. The condition is named after Finnish physician Erik von Willebrand, who first described it in the 1920s.
VWD is the most common bleeding disorder, affecting up to 1% of the US population. It is carried on chromosome 12 and occurs equally in men and women.
The thing about this factor is that is unpredictable. It is different from one day to the next. It can also go into what can only be described as “remission” where I have times that I don’t bleed as much. This is due to the change in platelets in my blood, that can be affected by variables such as diet, exercise, stress and overall health.
I have had two blood infections in my life, which were most likely caused by sickness that leaked into my blood stream. I have yet to find a specialist in my area who knows enough about VWD to answer my questions in easy terms to understand.
VWD or VWF is not exactly the same as Hemophilia, but the symptoms are very much the same. Typically VWD shows up while women have surgeries, miscarriages, nosebleeds or other types of injuries. It is rare that a doctor in an ER looks for VWD as the cause of bleeding. I was one of the “lucky” ones, who was referred due to my excessive bruising and because of the blood loss that occurred during my second miscarriage.
Since then, I had a third miscarriage which was much easier, due to my VHD being in remission. It is one of the blessings that the factor entails. Hemophilia doesn’t go into remission, however, VWF can.
I have also had a baby, over 25 years ago, and although childbirth was fairly normal, I bled for a year following her delivery. I underwent D & Cs and Laparoscopic surgeries as well as cauterization to eventually stop the blood loss. It was determined when my daughter was a year old that the iron levels in my blood had dropped to dangerous levels and I was bedridden for almost two weeks, and having iron injections every 3 days.
With the help of an injected medication called Lupron, my periods stopped 10 years ago. It put me in chemical menopause and it made the quality of my life improve beyond measure.
As well as no longer having periods, my nose bleeds seemed to calm down. I had one that sent me to hospital by ambulance 7 years ago, but since then, after my last cauterization, they have seemed to be less often, with less blood loss.
I do worry about injuries and am much more careful than I used to be. I still bruise easily, however, they are less often, as I am less reckless than I once was.
So far, my daughter has shown a few symptoms of VWD, but to date, she has not been diagnosed with the Factor. I wasn’t diagnosed until I was in my 20’s and my symptoms were much more severe. She doesn’t want to know, at this point in her life if she carries any of my genetics in her blood and I respect that, but worry often. She was tested as a child, but the factor was not present. I hope that she will never be diagnosed with it.
People do call it a disease, yet it is more of “syndrome” or a factor. For me, referring to it as a disease carries the determination that it is a sickness. It cannot be healed and is only treated in cases of Type 3, or in severe cases of active bleeds in Type 2 patients. There are medications that can be prescribed to help blood clot, but I have not been prescribed them for regular self-care. My factor seems to go into remission more often as I age. And that is a good thing.
I know that it freaks people out when I tell them my story, yet, knowing it gives me comfort. Knowing that it can go into remission also gives me comfort.
Since I have been on my own, and in a new circle of people in my life, I have made the decision to have a tattoo placed on my wrist to alert that I carry the factor. In case of an accident where I am badly injured, it is important for EMS people and hospital staff to understand that I have VWF and that I cannot be cut open without proper preparation and treatment.
For more information on Von Willebrand Factor (Disease) here are a couple of links.
A Lifetime of Von Willebrand Factor and What it Can Look Like
Research & References of A Lifetime of Von Willebrand Factor and What it Can Look Like|A&C Accounting And Tax Services
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