Von Willebrand Disease Video

Ludovico Guarini MD Pediatric Hematology
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Male: Sometimes kids going for relatively minor surgeries like maybe a tonsillectomy or tubes or something, something pops up and they are concern about bleeding and what are those concerns, how can we address it so we can minimize our risk from minor surgical procedures. Doctor: This is actually one of the most common problems we deal with in our practice; the typical stories, the kid that goes to the EENT doctor because they need a tonsillectomy and they get the blood test done before the surgery. And the blood tests come back really abnormal and the EENT doctor says I cannot do this surgery; you need to go to the hematologist and that creates all kinds of worries in the family. And what often occurs is a disorder that is very, very common. The condition we are talking about is Von Willebrand Disease, again a big name, medicine is front with big names. This is actually refers to the physician who initially found this condition of all places in a small island of the coast of Finland. And it tends out; this is actually a very common condition, so common that probably in the average causation populations is about 1 in a 100 by it so, so common in all population. There are people who have African ancestry have it, oriental habit. So, it is not just the European population but just about everybody can have it. And what this does is actually creates a slight abnormality in the blood vessels that is routinely done as a screening for bleeding disorders, so that is where the light bulb goes off, that is where the trigger of the investigation. But in terms of what it does to the patient is actually relatively minimal. This is a condition which I generally defined as if one bleeds, one bleeds more and the bleeding more is very difficult to qualify. But because we had ways to modify that or we have way to treat this, we generally go through a work up, we generally try to define the condition that in kids did required for instance the surgery like tonsillectomy. Once we defined that and that is done through blood test, what we do is we actually challenge that particular patient with a small molecules or DDAVP, which is actually a close relative to a hormone that is produce by the human body that is involve in the number of things including how much urine reproduced. But this hormone in particular can also release the factor that is missing that has been formed deliberant, in fact from what is stored in the body. So by giving this medication, we can increase the presence of this factor in the blood stream and essentially make the person normal for a period of time. Male: If you go and have surgery minor and your concern about, what test would you recommend so and get before the procedure? Doctor: Well, the screening tests are the so called PT and PTT and this are really screening test that serve to like turn in the key in the car and if the car runs well its serves to okay, but once one of this has some slight abnormality, then what we need to do is to look at the different pieces of the car to see which one is missing. So, what we do in these circumstances is we look at all the factors that we can measure in the clotting cascade including this from delivering a test and this generally involves 2 or 3 specific test that measure this condition. Now, one interesting piece of this and I always caution my patient as I see them. You said this test are a little bit finicky in part because the test themselves are finicky but also because the simplest stress of the venipuncture can actually change the level in the blood stream of some of the clotting factor. Rejection if you think about it, you know if you go to battle, if you go to be stressed and you think you are going to risks bleeding, it is actually intriguing that the body prepare itself by that. Adrenalin, essentially which is the stress armament by extra line raises all the kinds of clotting factor in the blood stream. So we know kids do not like it to stuck unlike lot of adults and so t

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