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Thursday, June 6, 2013

Von Willebrand's disease

I have just been diagnosed with Von Willebrand's disease (because one rare genetic illness isn't enough for me apparently) and I'm hoping my story might help others.

I got my first period on a class trip to Spain when I was 13 (you can't make this stuff up).

My period lasted 20 some odd days and was truly miserable. I got a few weeks off and Aunt Flo was back in town for another extended stay of 2+ weeks. This pattern repeated for a few months before I finally agreed to see an OBGYN to get checked out for any abnormalities.

Since I have CF, all weird things are often attributed to my genetic disease until proven otherwise - and my horrible periods were no exception. My ultrasounds of my uterus were normal, hormone levels were OK, so the doc, my family (yey to have family involved when you're 13 on the topic of periods.... I was mortified)  and I agreed to wait things out to see if my period "normalized."

Very soon after this agreement I threw up my hands in frustration as a then 14-year-old and demanded some sort of help. The OBGYN prescribed birth control pills and this solved everything. I was overjoyed to not have horrendous cramps or terrible fatigue from losing so much blood. Problem solved.

Fast forward to 27 years old, and I am diagnosed with my 1st superficial venous thrombosis in my left arm after I have a PICC for 2 weeks with abx. Not excited. I threw another superficial venous thrombosis in my right arm (later in 2009) with a PICC (I have this documented in my blog here btw) and naturally red flags go off in my head ; everyone knows estrogen makes you more prone to clots, right?

Fabulous. Off I go to a hematologist to get a work up just in case I'm in a hyper-coagulable state (for various reasons such as an undiagnosed Factor V Liden ) and everything turns out clean. I meet with a new OBGYN to understand my options for birth control and I'm presented with a few options: IUD's (Mirena which is progesterone-based, less clotting possibility; Copper with no hormones at all which can make periods worse), a progesterone pill, or depo privera. I liked the idea of localized (somewhat) hormones of progesterone, one less pill to take, yada yada with Mirena. So in goes the Mirena in December 2010 and off the birth control pill.

Periods begin to return to my 13 year old state- heavy, lasting looooong (2 weeks) for months. I am told by my OBGYN that it can take a few months for the Mirena to kick in. Summer 2011 arrives and periods are still horrendous. A re-visit to the OBGYN and I'm told that gosh, well, literature states in 3% of patients or so it can take a full 12 months for the Mirena to kick in. So I wait. And wait.

January 2012 rolls around and my Mirena has been in for 13 months and periods are still lasting too long and I'm an unhappy camper. I decide to switch OBGYN's to see if something else might be going on. February 2012 we decide on a transvaginal ultrasound to see if anything else might be causing excessive bleeding - fibroids, etc. Ultrasound looks normal but IUD looks slightly out of place ; it's hanging out in my cervix instead of in my uterus.

Feb 2012 I am diagnosed with CFRD so dealing with period issues kind of takes a back burner - CFRD and insulin is a steep learning curve and I need to focus on not destroying my lungs, kidneys, eyes, etc with high blood sugar.

May 2012 we decide, with this new OBGYN, to do a hysteroscopy In June 2012 to make sure nothing else is going on in my uterus that we couldn't see on the transvaginal ultrasound. We also decide to swap out my IUD for a shiny new IUD since who knows, maybe the IUD wasn't in the right place for the past 1.5 years and maybe this is why I'm having crazy bleeding.

Hysteroscopy goes great - nothing to report. No fibroids, no signs of endometriosis, uterine lining looks fine. Fantastic - hopefully this new IUD does the trick and takes away my miserable 1.5 year experience of loooooong periods. Mark in the calendar - June 2012 new IUD.

Our story now brings us to April 2013 - my periods are still lasting 3 weeks and are heavy. I'm exhausted, frustrated, angry and scared. My options for birth control are so limited and clearly Mirena isn't working.

I consult with my OBGYN and she suggests we try Depo. Ugh, that drug name just sends chills up my spine. I've heard so many bad things about the drug (extreme bone loss, infertility, unending hunger) that I am really hesitate to take this next step. I cancel my appointment to get my depo shot with my doc.

On a whim, I mention my struggles to a colleague and she suggests I visit an OBGYN in a fancy part of town who doesn't even take insurance. Great. I spend $1000+/ month on my healthcare as it is, so I'm not eager to drop a few hundreds of dollars on a doc I can't afford. But something tells me to do it.

This 3rd OBGYN in 3 years is very nice. She takes the time to listen to my history and she's eager to help. She suggests a few things that might be going on and recommends we run some blood tests. She also wants records of my blood clots to examine.

First on the list of tests is for a very rare genetic blood disorder. Less than 1% of the population has it and it's probable that I don't have it, but it's possible. She asks if I bleed a lot having teeth pulled (wisdom teeth) - not that I recall. Nose bleeds? Not really. Bruising easily? Why yes, I've definitely noticed that recently. Not my whole life, though. Just the past few years when I bump in to things I bleed easily. And with my insulin shots, I get a bruise very often at the injection site....I always assumed that was normal for shots. Nope. I bruise with my allergy shots in my arm too.... assumed it was normal. Not so much. Long periods are also a symptom of the disease - check.

The blood test would help us to see if the bruising and long periods were an indication of something being off.

Here are the results. 4 Hematologists and 4 OBGYN's later, I have von Willebrand Type I.

A hallmark of von Willebrand's is unexplained loooooong periods. And bruising.

So naturally we ask, why didn't I have long periods from age 14 to 27? Well, estrogen based birth control pills help mitigate the effects of von Willebrand's disease - so I had normal periods and didn't bruise as easily.

This also explains why I didn't have crazy bleeding issues during my many surgeries between 2000-2009 (hernia repair, gallbladder out, appendix out).... I was on an estrogen BC pill.

Great, so now what? Options to treat von Willebrand's are (1) resume estrogen based BC pills - The estrogen hormones present in birth control pills can boost levels of von Willebrand factor and factor VIII activity or (2) Desmopressin (DDAVP) that works by stimulating your body to release more von Willebrand factor already stored in the lining of your blood vessels.

You would imagine I would opt for DDAVP given my history of blood clots. Because both of my PICC-related blood clots were superficial in nature and not deep, my new hematologist feels comfortable putting me on estrogen birth control pills. So we're going to try that out for a few months and fingers crossed it helps!

Lastly, von Willebrand's is genetic (autosomal dominant, unlike CF which is autosomal recessive) which means there's a 99% chance I inherited my gene from one of my parents. I have informed my parents of the consequences of one of them having the disease (trouble during surgery, etc) but they have yet to get tested. Typical parents, right? Not sure about yours, but mine seem to have a lot less sense of urgency about their health than I do. Must be nice :)

I guess a lighter sense of urgency is the luxury of people in relatively good health or of those in denial. Which one my parents are - I think a little of both. 

I'll keep you posted on how my health goes with my new diagnosis and treatment. Wish me luck!


  1. Hi, I came across your blog while searching for information about VWD. I have had the same experience as you but I have not taken BC pills for many years, so I have basically been hemorrhaging on and off for the past 2 decades! I just saw a hematologist today and had blood drawn to check for VWD. I'm amazed that in all these years no one ever thought to refer me to a hematologist; the focus has always been on my gyn/hormonal health, which is normal in all regards- no cysts, fibroids, endo, hormone imbalances, etc. I don't know if I have this disorder but I fit the criteria so well and I have a sister who has the exact same thing, as well as an aunt who had the same thing. Well, just thought I'd drop you a line since I haven't found any other personal blogs that talk about VWD and menorrhagia.

    1. Hi Bex, my heart breaks for you. Having periods for a long time or "hemorrhaging" as you mentioned is so terrible - it really does horrible things to one's life. Sounds like you do have VWD... keep me posted on what results you get. So glad you found a hem and hopefully you'll have answers soon. HUGS!