Dear Tesstee,
I started having bad nosebleeds about six years ago. They were treated with the tampon stuffing and that worked sort of. After the second nosebleed (epistaxis to the medically minded) I did the same thing you did, got my teeth cleaned and wound up sitting in the ER for eight hours looking like a vampire after dinner. I don’t remember any family member having coagulation issues, but on my mother’s side there were only one handful of first and second cousins. That would have been about the time vWD was discovered, in a Finnish family, whose family tree looked like Queen Victoria’s greater family and Hemophilia.
After the gum bleed, which was stopped by ddavp infusion,I went to a Hematologist who passed the Doctor Google background check, (that is very important, vWD is sufficiently rare, especially some of the 2 subtypes and type 3, so that many Hematologists only have seen one or two patients. If they stick mostly to Oncology, as many of them do, their up-front knowledge of coagulopathy may be in a dark place in their head. They also won’t be reading the right journals or going to the right meetings.) My Doc did all the usual tests for VWF antigen, Ristoceticn C co-factor. I have type three vWD complicated by very very low factor eight (probably caused by successive uses of Alphanate VWF and Factor VIII factors leading to my body producing antibodies to the alphanate factor infusion. This would be an “autoimmune” response" in Med. Jargon. People who get transfusions or factor infusions often develop this type of response which leads to the search for other paths to stop bleeding.
Another complicating issue for people who go anemic from blood loss and have vWD or Hemophilia A or B, is that Hospitals usually prefer to use packed red blood cells (PRBC). PRBC come from the blood bank with a big chunk of anti-coagulants in them to help preserve them from going bad, (hematolysis) . As I write this, my Hgb has come up to 8.0 g/dL from 7.6 g/dL(grams of Hemoglobin per deciliter of liquid) A deciliter is 100 milliters or a little less than half a 12 0z can. Six weeks ago I had lost about 1.5 Liters of blood after a minor surgery. Desmopressin (ddavp a vaspressor substitute) did nothing for me as it only brings my VWF and Factor VIII up to about 20%. Most type I people can use ddavp effectively in a small spray bottle marketed under the name Stimate. I keep a bottle in the fridge all the time. it will work on a small bleed in my case but appears to do nothing for say a surgical wound or a GI bleed from a vein/artery bundle called an AVM or atrerial-venal-malformation. Baically they look like a bundle of blood vessel coming together. Since veins are low pressure construction and arteries are high-pressure systems, going directly from artery to vein creates a chance for a leak to develop. I have had several of these in the stomach and the first part of the small bowel (the duodenum). An upper GI endoscopy, where they stick a tube down your throat that is capable of being wiggled around, take pictures and cauterize any bleeders they find. Yesterday they found bleeders in the stomach and duodenum and successfully cauterized them. OVernight my Hgb started up again because my body was making Hemoglobin faster than losing it. Hurray!!! At Hgb around 8.0, anemia justifies transfusion. Luckily, there is a blood product ,called apherized blood platelets, that only contains a little anti-coagulant.
My Doctor and I decided to hold off on the apherized blood and keep watch on my Hgb for a few days. Since I didn’t use the new type blood, I won’t develop antibodies for it and it will work the first time they use it in the future. Kind of like keeping one bullet on a necklace, just in case.
The Pharmacist, at my Hospital was able to locate Factor that the company would loan him and only charge on use. At about 5000$ US/dose. I feel very lucky to be close to the only Hospital in Florida that keeps it on the shelf. Just like your experience in Honduras.
As for my day to day activities, I avoid driving more than fifty miles from the hospital. I keep a large box of “pressure bandage” making material in each car and in the house.
I wear heavy leather high top boots around the yard and I always wear gloves. I no longer use gas chain saws ,(DUH!!!) and I am extremely safety-first minded about “sharps”.
So after I get my Hgb above 10.0 I will go back to my four acres and my gardens and have fun, like you, I have a kayak. I also am conscious of pygmy rattlesnakes as they us anti-coagulants to kill you.
So trust your proven good doctors but always verify! That’s what Dr. Google does for you. Keep on the NIH and Mayoclinic sites where there are summary articles about vWD and Hemohilia A. I also google things like “thrombocytopoenia” so I can understand the professional journals jargon.
So here I sit in the Hospital, with my laptop, netflix, google and lots and lots of music. You meet a lot of nice people here and many, if not most, will listen.
I still plant perennials and buy green bananas!!! So don’t get morbid on us!!!Fight the beast with all your might by prior preparation. Learn how to be assertive in Hospitals also. There are ombudsman in many hospitals that help you negotiate with the staff. They are on your side. There is also a Medicare “problem” line that helps you appeal early discharge or incorrect treatment. Use it, it works quickly and Hospital middle management turn pale when Medicare drops a wake-up call on them. make sure you get the Medicare discharge appeal two page handout when you are registered. The number is on the second page.
Love you all,
John the elder