20 years ago I was diagnosed with Von Willebrands in Canada. Now to make a long story short despite many issues when I found myself moving around the globe, I never got treatment of any kind and since leaving Canada I didn't need any kind of surgery. Until 2 years ago. I was sent to a hematologist when I was pregnant. And he said results were normal. Unfortunately I was bleeding continuously and eventually it just didn't work out. I was advised to try the Mirena as therapy for VWD and 3 weeks after the start of that, the hematologist took more blood. The ONLY times he has done blood tests for VWD were when I was pregnant and when I was subsequently getting synthetic hormones. Both can make VWD difficult to detect. He refused to review my medical files from Canada because they were too dated. A few months later he sent a letter to me and my GP saying that sometimes VWD just disappears, as it had apparently done with me. "Please tell her she has no risk of bleeding." A couple months after that I couldn't cope with the side effects of the Mirena anymore. I had way too many. I had never been told what they could be despite having told the nurses in the hematology department that I had really bad side effects to the combo pill. I had it removed after having to get tested for thyroidim first. When I did I bled for 30 days straight. And so my GP sent me for blood test.... it turn out only Full blood count!!! LOL. I got a new GP almost a year later and showed him my medical records from Canada and he looked at all the hematologist had done and explained that he had only run quantitative tests and all my records showed that my VWD is qualitative and there is an abnormality in my platelets. So, he sent me for more blood tests. Two weeks later the GPs office informed me that all had come back normal. Well that's great, so the symptoms of bleeding have never gone away all my life and yet somehow my body forgot to tell me?? A few months later I went to a dental surgeon. He called my GP and learned that the hematologist refused to do any more tests. His lab at another hospital received my samples and he responded that he had already run tests and discharged me.
I have since moved and my latest GP said this is insanity...and got me an appointment at another hospital, not associated in any way with the other one as far as I know. AND because my work has noticed I am bruised all over, all the time, I have broken blood vessels everywhere (and those are just the visible things!), my knees are always covered in little bruises...especially when I exercise...so they're working on making sure our private insurance will cover this.
Has anyone else had an issue in any way like this because of the quantitative v qualitative issue?
Quantitative sounds like the variation of type one vWD based on the measurement of VWF and Factor VIII levels in percent of "normal" where 10% activity is severe and 90% is mild with all possibilities in-between possible. I, personally, am type three with much less than 10% activity. Qualitative sounds like the type two vWD categories where the VWF factor molecules have the wrong shape to funtion correctly in coagulation or they don't clump together in "multimeres" with the correct number of partners.My analogy is that multimeres are like a number of jellyfish stuck together, Coagulation won't be effective unless a clump of VWF molecules of the right size sticks to the wounded edge of the veins or capillaries. Some type two people have too small multimeres, some too large and some the wrong shape to work.
Unfortunately, the multimere size distribution test, the VWF and Factor VIII activity tests and the antigen tests have to be sent out to big labs for analysis and the transport and handling of the blood degrades the quality of the tests. One sample is not enough. Three or more over time should be more reliable.Some of my tests come back with opposite results. Especially the multimere tests.
Most Hematologists only see a few or less severe vWD patients in a career. The treatment for some of the type two patients is different than the treatment for type one and three patients. Stimate (ddavp) doesn't work for all and the same for aminocaproic acid tablets. Look for a compassionate Hematologist who will not dismiss your concerns because you have an orphan disease. Contact the patient advocate and case manager immediately on entry to the Hospital so they will alert the others to your special needs.
Today, I am awaiting the call to go to the Hospital for VWF and Factor VIII (alphanate) IV infusion to stop internal bleeding from an Arterio-Venosal Malformation in my GI tract. No GI specialist will look for the bleeder with endoscopy because of the risk of scratching my mouth or throat and causing more bleeding. Alphanate is expensive so the doctor and I have to firmly insist it be ordered- it;s the only response that works for me.
My son is type 3 severe with 1% VIII.we infuse every other day with humate -p which seems to work for us when bleed does occur for him we find that humate-p and aminocaproic oral solution works great together for him.i too know how hospitals /nurses/some doctors don’t get it even when explained it usually takes a call to our hematologist. I wish you well sir and hope you recover quickly.
Finally yesterday I went to see the hematologist at a different hospital. The Dr seemed surprised be the sequence of tests I had received previously. So to make a long story short he said that looking at the results from the hospital in Canada he would say it is borderline VWD. But he said that their focus is not classifying or giving a name. He said they will do more tests such as FBC, coagulations test, vwd antigen, and platelets. He seemed surprised that my blood type was not listed anywhere... So they're doing that one too. @Johntheelder he told me that they will do all the work there as they are the 'largest' hematology center in the UK. At the end of the day I was treated so different yesterday then when I was in north london. I asked the doctor about having kids and in the event that all my bleeding issues are a figment of my imagination (no sarcasm...no no) and as the other doc says...there is no risk of bleeding...shouldn't there at least be something in my file that says I have this history for when I have kids etc. He answered by explaining that they have seen it all, mild bruising to unimaginable bruising and everything in between. And as they are focused on providing care, when I am pregnant they will decide exactly what I need and who...and not rely on just writing VWD in my medical file. In fact he said they try not to do that because in many ways it is unspecific but leads to specific treatments etc which are not always what is required. At the end of the day, I felt better already.
I see this is an older post but you and I seem to be in the same situation. I,too, am Canadian but now living in the USA. Also, your condition sounds a lot like mine. I have had bleeding issues my whole life and had been tested for bleeding disorders since I was a teenager with everything coming back “normal”. Most problems have been the usual like being covered in bruises, bleeding gums, constant nosebleeds, chronic anemia and heavy periods lasting up to 3 weeks. Then there were the more serious problems. Once in high school after a gym volleyball game my arms bruised and swelled up so badly I was sent to the emergency room because they thought I had broken both my arms. Again, I was tested, everything came back normal and the only thing they told me was to avoid contact sports ( seriously!). As I grew older I just accepted all the bruising and bleeding as an inconvenience and never thought much more about it.
Then came the day I had abdominal surgery. After surgery I bled into my abdomin suffered a blood clot and had to have a blood transfusion. All was good until a week later I developed a hematoma near my hip joint that was so bad I couldn’t walk. My surgeon was perplexed since she had my platelets tested a few days before the surgery and they were normal, so she sent me to a hematologist. Based on my current experience, recounting my childhood experiences and family history (father with low platelets, mother, grandmother and sister with bleeding issues, notably with childbirth) he sent my blood for a full panel Von Willebrand test.
When my test results came back my follow-up appointment went like this: The hematogist looked at me and said “you are a very unusual case. I’m going to need to do some explaining about what’s going on here with your tests and why you are having all of the bleeding problems you are having. Most doctors would not pick-up on what’s happening based on your numbers.” He went on to explain that I have False Negative Von Willebrands (he listed it as +/- on the report). He showed me the results of my blood work and explained how he came to that conclusion. On the report it lists your results in one column, the expected normal range for the parameter tested next to it, and a column which states if your result was In Range or Out of Range. He pointed out that everything on my report was in the normal range but there was a “but”. Although factor VIII activity, vWF A, and vWF activity, and platelets were all normal, they were all in the low range of normal. Anyone looking solely at numbers might assume everything is OK when in actuality it is not. He equated my ability to clot to a car with an engine that runs but the oil needs changing, the gas pan leaks, hoses need replacing and the spark plugs aren’t fully firing. He said your car may run but it isn’t doing it very well and will eventually breakdown and it certainly couldn’t handle another stressor like a surgery. He told me to tell all of my doctors I had Von Willebrands and if they had a problem with it to tell them to phone him. Maybe you too are false negative in your testing. Sometimes my levels are really low and other times not so much.
Also, I am having the same issues with pregnancy. We had tried to conceive naturally for 5 years before turning to IVF. It was really difficult going off birth control pills and having to deal with the long heavy periods associated with VWD. Since doing IVF I have had five miscarriages in the past couple of years. Each time the pregnancy is progressing normally and then I have a major bleeding episode from a subchorianic hematoma and approximately a week later I will miscarry. IVF involves lots of testing and monitoring. I know most miscarriages are due to chromosomal abnormalities but in my case I have had all of the embryos tested and they were all normal. Every doctor I have been to said SCHs are normal in pregnancy and most don’t cause miscarriage and my VWD is not a factor but I keep thinking that it is. I asked my hematologist and he said it is only a factor at time of delivery and not in early pregnancy. My own research shows there has been little investigation by the medical research community into miscarriage and VWD but what is there does suggest there is a link. I don’t know what else to do and I don’t know if there is anything that can be done if even my hematologist says I don’t needed treatment during the early stages of pregnancy. Are you experiencing any of the same? You said you were doing hormonal supplementation which is given by REs for IVF treatment. Are you doing IVF, as well? I think some of the meds they give you for IVF can interfere with you VWD testing.
Oh, I forgot to add that if you get any testing done while you are pregnant or have recently had a miscarriage your numbers may be higher than they are normally for you. This is because pregnancy naturally causes your body to produce more Von Willebrands factor. Just something else to be aware of if your doctor didn’t tell you already!