When you see the title of this entry, some questions may pop-out in your head.
What is Von Willlebrand?
Is it a person's name?
~etc~
Von Willebrand is a disease.
Few days ago I read a novel about person who suffer this disease.
This disease is like hemofilia but von willebrand is more dangerous.
Can you imagined people cry with blood streaming from their eyes? It's scary~ really.
That's why i searched information about this disease, and share it here.
What is Von Willebrand's disease?
Von Willebrand disease (VWD) is a bleeding disorder that affects most people.
In fact, it's not a single disease, but descent disease. This disease is caused by a problem Von Willebrand Factor (VWF). It is a protein in the blood necessary for blood clotting. VWF gene that makes working on the two cell types, namely:
- Endothelial cells lining the blood vessels and
- Platelets
If there is not enough VWF in the blood, or not working properly, then the blood clotting process takes longer.
How can blood clot in normal circumstances?
Blood in the body is transported in the blood vessels. If there is tissue that get injured, blood vessel damage and will cause leakage of blood through the hole in the wall of blood vessels. Vessels near the surface can be damaged as it is cut off. Or it can be damaged on the inside of the body resulting in bruising or bleeding.
Platelets are tiny cells that circulate in the blood. Each platelet size diameter of less than 1/10, 000 centimeters. There are 150 to 400 billion platelets in 1 liter of normal blood. Platelets have an important role to stop the bleeding and begin repair of injured blood vessels.
If the injured blood vessels, there are four stages to form blood clots are normal.
Figure 1a. Normal blood clotting
Figure 1b. Abnormal blood clotting
Phase 1 The blood vessels were injured and started bleeding.
Phase 2 The blood vessels constrict to slow blood flow to the injured area.
Phase 3 Platelets adhere and spread on the walls of damaged blood vessels. This is called platelet adhesion. Platelet spreading platelets release substances that activate other nearby so it will clot forming platelet plug in place of the injured. This is called platelet aggregation.
Stage 4 Surface of activated platelets to the surface where the blood clot. Blood clotting proteins that circulate in the blood on the surface of activated platelets to form fibrin clot network.
This protein (Factor I, II, V, VII, VIII, IX, X, XI, XII and XIII, and von Willebrand factor) works like dominoes, in a chain reaction. This is called the coagulation cascade (see Figure 2.)
Figure 2a. normal coagulation cascade
Figure 2b. hemophilia coagulation cascade
Von Willebrand Disease How do occur in normal blood clotting process?
VWD can occur in the last two stages in the process of blood clotting.
At stage 3, one can likely do not have enough von Willebrand factor (VWF) in the blood or the factor does not function normally. VWF consequently can not act as a glue to hold the platelets in the vicinity of the damaged blood vessels. Platelets can not coat the walls of blood vessels.
At stage 4, VWF carrying Factor VIII. Factor VIII is one of the proteins needed to form a strong network. In the absence of factor VIII in the normal amounts of blood clotting process will take longer.
How often have Von Willebrand disease is found?
Doctors now think that VWD can be about 1 in 100 people. Because many people - people may get a light bleeding, then only a small number who know they carry this disease.
Who can affect Von Willebrand disease?
Von Willebrand disease can affect men and women. However, because many women with VWD have a lot of menstrual bleeding and bleeding shortly after giving birth, women have more symptoms than men.
Children - children can also suffer from VWD. They are born with this disease. This is because VWD is an inherited disorder.
Can VWD passed down from parents to children?
Yes, if one of the parents have VWD, they can reduce the disease to the child - his son.
Why is it called Von Willebrand disease?
This name is the name of a Finnish physician, Erik von Willebrand, who first described this condition in 1925. He realized that the disease is not the same as hemophilia, which falls on the severity of the condition of men - men.
In what circumstances is said to severe von Willebrand disease?
It depends on the type of disease. Fortunately there are effective treatments for all types of VWD.
What's more, some researchers found that "... a mild VWD can be beneficial to health." The researchers said this in because Visual Studio makes it more difficult for platelets sticking together. So that a person with VWD would be less prone to blockage of blood vessels, as a cause of heart attack or stroke.
