…. Von Willebrand’s Disease. Type 1.
Rewind. I was sitting in a hematologist’s office hearing the words ‘You’ve been dicing with death your whole life’. Wait. More information needed. Rewind further.
Birth did not go quite as planned. Actually the birth did – the postpartum period did not. My amazingly skilled OB (we still love Dr. Dryden to pieces, even if she isn’t Dr. Boswell) was torn, she wanted to be believe it was ‘just bad luck’ but could not believe that such extensive loss of blood could not be pathological, so sent me for a battery of tests. ‘On the off chance’. ‘Just In Case’. ‘To tick every box’. 8 vials of blood later (chance of a false positive anyone?) the results were in: they were suggestive of Von Willebrands, a condition where you either don’t produce enough Von Willebrand factor (a factor than carries factor 8, which is necessary for effective clotting) or where you don’t have any (I had some), or where you Von Willebrand’s was there, but it was’t very effective and so can’t recruit platelets into the clotting site.
I now know far more about how blood clots than I am comfortable with.
So, off I went to Dr. Kelty Baker, who has an amazing reputation. And indeed – she was wonderful. Smart, funny, fascinated by hematological conditions and motivated to cure me. Unfortunately, her nurse wasn’t. This was the nurse who walked into the exam room where I was waiting, took my blood pressure and shoved a thermometer in my mouth, took reading and walked out, all without a single word. Lovely. Just lovely.
Dr. Baker felt it was Von Willebrand’s and asked if I minded having a genetic test to confirm. Me? A geneticist – mind? I’m positively enthusiastic. So we made a plan – I would have a genetic test done. This had to be done in Wisconsin for some reason (even though Baylor, just across the road, does this test). I would also (because we don’t know the full mutations contributing to Von Willebrand’s) have had some tests done which looked at whether the little Von Willebrand factor that I did have actually worked (the blood tests were borderline). Aaaaand, because of the fact that regardless of the cause, we would have to treat the fact that I did not have enough Von Willebrands, we would do a challenge to see if I responded to a drug (DDAVP) designed to increase Von Willebrand factor. All good. I agreed. She said that these non-genetic tests were only done in a field by a fairy under the full moon & I even agreed to this. OK, they were only done heinously early in Methodist hospital on Tuesdays and Fridays but that is basically the same thing.
Then we entered the vagaries of the American Healthcare System.
My insurance company said that Wisconsin was ‘out of network’ so I would in essence be responsible for the full cost, minus a teeny-tiny co-pay. Knowing that genetic tests can run into 1000s, I HAD to find out the cost. My insurance company also said that I should find out the name of the tests done at Methodist, to check that they were covered – or again, I’d be liable for the full cost as Methodist is not quite in network but some facilities are. It was complicated, but I called Dr. Baker’s office to get the name of all the tests – genetic and otherwise – to report back to my insurance company. And there I encountered the lovely nurse again. And problems ensued.
The nurse told me that Dr. Baker had ordered the following tests:
Platelet aggregation Panel ($256)
Risocetin co-factor ($184) and
Von Willebrand’s Antigen ($178)
I told the nurse that these were not genetic tests so at least one was missing, and furthermore that I had had already had these tests done for free in-network at Texas Children’s Hospital, so would she explain why they now had to go out-of-network. All she would say is ‘shall I tell Dr. Baker you won’t pay for these tests?’ over and over. Whatever I asked, she came back to that.
Me: ‘Are you sure these are the tests?’ Evil-face: ‘Are you going to pay for these tests?’.
Me: ‘ICould you ask Dr. Baker if I could have the tests done in network?’ Evil-face ‘You have to have these tests where we say. Can I tell Dr. Baker you will pay for these tests or not?’.
Me: ‘There is not a genetic test here – why not?’ Evil-face: ‘Look, are you going to pay for these tests or not?’.
Me: ‘I have test results for these. TWICE! I can read them to you’. Evil-face: ‘You have to have these tests. Can I tell Dr. Baker you will pay for these tests or not?’.
Eventually I had to say ‘well, no, I won’t pay for tests where the cost can’t be justified’ and she hung up and I never heard from the clinic again, until they wrote to my OB and sent me a copy saying that I couldn’t afford medical care and that they would help me when I came to give birth next, if needed.
So, I transferred care to Dr. Mark Udden. First up: his nurse treated me like a human – you know: told me her name, warned me before jabbing things into my mouth… things of that nature. The junior doctor was nice – he had a daughter born 5 days before Sam and had lived in Lewisham (the site of my London flat). OK, so not medical-care necessities, but a good sign nonetheless.
Dr. Udden was the best of all – he was practical and smart. He laid it down: I don’t have enough Von Willebrand factor. Although Type 0 blood naturally has less Von Willebrand factor, he would expect Type 0 to have levels at about 50-60% less than non-Type O, and I was at 32-34%. He suspected that my Von Willebrand’s was working fine, the borderline reduced activity in my tests was just indicative of not having enough. Genetics were fun, he said, but we were still going to have to treat the lack of Von Willebrands so why spend the money? So, I am off for a DDAVP challenge test to see if I respond to DVAPP by making more Von Willebrand’s factor. We assume I will. So from there on, they will check my Von Willebrand levels before giving birth next time, and give me an infusion of the factor while pushing. If I go for minor surgery, I will take DDAVP. If I go into hospital for an emergency: more DDAVP, infusions of the factor if extensive surgery is needed, or I am smashed to bits in a car accident or something.
I objected to all this drug talk. I pointed out that I had got this far and been just fine. Turns out hormonal contraceptive artificially increases Von Willebrand and I have been on those since I was 13 (due to heavy periods – another sign of Von Willebrand) so it had been masking any symptoms. Plus I had avoided any surgery except while pregnant (when I had dental surgery), and pregnancy is a time when you also produce more Von Willebrand, so I made it out without crazy bleeding. Lots of lucky co-incidences have got me thus far in life without incidence. Hence my ‘I am not sure I really need to do anything about this’ was met with ‘You’ve basically been dicing with death your whole life’.
Unfortunately it is a heritable condition, and so we have to get Sam tested. But, my case is so mild, it is likely I am a heterozygote, meaning that Sam only has a 50% chance of contracting the disorder. And if he is not blood type O, he probably will be OK.
And I have to have my IUD out. The extra bleeding that goes with IUD and non-clotting blood is just a bad combo. It is true that things are pretty bad on that front, like today is Day 6 of my period and I still can’t get through the night without a horror show, but as I am allergic to latex and have a bad reaction to hormones, I was sticking it out. I am a bit stuck about what we will do now. Sam no. 2 anyone? 😉
- “My Friend Delaney’s Story” (christophermilo.com)
- Easy Bruising Could Indicate You Have Von Willebrand’s Disease! (zirari6.wordpress.com)