In this clinical scenario, we have a 19-year-old female with extremely long heavy periods. So now let's define that. That's bleeding that lasts more than seven days. Bleeding that soaks through one or more tampons or pads every hour for several hours in a row. Needing to wear more than one pad at a time to control menstrual flow. Needing to change pads or tampons during the night. Or even larger. So this patient has extremely long, heavy periods, as we discuss what the definition is. Bleeds after brushing teeth, okay? Bleeds after brushing teeth, which we and we examine this patient, she doesn't have poor dentation. Sometimes we have to look at that extremely poor dentation. You'll bleed after brushing teeth. Infrequent noseblees, infrequent nose bleeds. So what does this patient have? So the answer to the clinical scenario is a 19-year-old female had von Williambrand's disease. And so what is von Wilbrian's disease? It's caused by quantitative or qualitative defects in the von Wilbrand factor. That's a large multimeric glycoprotein essential for hemostasis. It's inherited in an autosomal pattern, so it affects both male and female equally. But you know, women more commonly seek medical attention due to the G OIN bleeding. Now you can also have mucocataneous bleeding, which is hepataxis, easy bleeding from uh from minor wounds, heavy menstrual bleeding, which we talked about, excessive bleeding after dental work. So that can be a diagnosis sometimes. You can always ask patients, hey, when you had your wisdom, if you had your wisdom teeth removed, did your uh surgeon andor dentist say that you were bleeding very heavily? Also noted for heavy bleeding after surgeries and or childbirth. What is von Wilbrand's factor? Now, von Wilbrand's factor acts as a bridging molecule for normal platelet adhesion and aggregation as a carrier for factor eight in the circulation, increasing the half-life of factor eight. I want you to think von Wilbrand's factor and factor eight as being cousins. They just hang to each other. They're close cousins that hang with each other. So these are very important. Van Wolbrand factor is a cousin with factor VII, they hang with each other. They increase the half-life of Von Wilburn factor increased the half-life of factor eight. Very important. Now, Van Wolbrand's disease, as we discuss, is a defect in von Wolbrand's factor. And it's the most common inherent bleeding disorder in the United States. It affects about 1% of the population, so greater than 3 million people in the United States alone. Now let's talk about the different types of von Wolbrand's disease. Type 1 is the most common. So we have there's rare where there's various rare defects. These are one in a million. Type 2, where it's quant qualitative, it just doesn't work right. The von Wolbrand factor just doesn't work right. Type 3, where there's near or total absence. No, type 1 is the most common, and it's about 70 to 80% of the cases. It's a quantitative defect, less von Wolbrand factors, but it works. Okay, this is the most frequent less von Wolbrand factor, but it works. So when does von Wolbrand's disease occur or manifest? So we have a primary deficiency or defect in von Wolbrand factor, and this has contributed with low levels of factor VI, and we'll talk why this occurs. Van Wolbrand factor carries and protects factor VIII in the blood. So a von Wolbrand factor problem often leads to lower factor VIII levels. Okay? They're a team, they work together. So in normal hemostasis, inside the blood vessel, we have functional von Wolbrand factor, and it works. And it gets together with its cousin factor VIII. They go hand in hand together and they do a great job and they form a platelet plug and flyber and clot. Now, if impaired homostasis, we have a deficiency or defect in von Wolbrand factor. We talked about one, two, and three. And we have a dysfunctional or low levels or no levels of von Wollbrand factor, and we have reduced platelet adhesion, weak delayed clot, and you have bleeding symptoms. Now, remember von Wolbrand factor protects and carries his cousin factor eight. Okay, so then you have reduced factor eight, and you have unstable rapid clearance due to insufficient von Wahbrand carrier, and you have impaired fiber and clot. So they work together as a team, but if they're not together as a team or there's reduced factor eight, they are not gonna control the bleeding symptoms. So who gets von Wahlbrand's disease? Both men and women both get it, but because women will present with menoragia, they're gonna be come to the providers more often. So let's go again and discuss what is heavy bleeding, because that's so important. Bleeding that lasts more than seven days, bleeding that soaks through more, one or more tampons or pads an hour for several hours in a row, need to wear more than one pad at a time to control menstrual flow, need to change pads or tampons during the night, menstrual flow blood clots are as big as a quarter or larger. Remember, about 10 to 30 percent of women with menorage will have Van Wolbrand's disease. So this is important when you're taking care of your patients to think about. Let's talk about the treatment of von Wolbrand's disease. Now, remember is this that the treatment for von Wolbrand's disease, it's an individualist approach, so it's going to be based on the disease subtype, the disease phenotype in clinical context. Now, desmopressin can be used because what does it do? It induces endothelial release of stored von Wolbrand factor and factor 8, the two cousins that work together. Now, this won't work for someone who has von Wolbrand's disease type 3 because why? They have none. They're not producing any, so they're not going to release anything. In these types of patients, you would need von Wolbrand factor concentrates. So, and there are some patients where desired present doesn't work or is ineffective or they just cannot use it due to their subtype. Now, let's talk specifically about treatment for poor women who have very heavy menses or menoragia, which can be treated with transamic acid or amicabroic acid. Now, these uh these, if you as a clinical point, if you're doing a lot of procedures, maybe you're doing central lines, is some places will have a liquid formin and you can put on a sterile gauze and put it at the site. Sometimes that does help uh help stop bleeding. Now let's go back to our clinical context of this case with this 19-year-old female with heavy menses. So it can be used and inhibits, so transinoic acid and amylocaproic acid, they inhibit the lysis of fibrin and it blocks plasmid during menses. So it reduces bleeding for these poor women who have horrible menoragia.