Von Willebrand's disease or VWD is a genetic and hereditary disease characterized by the decrease or absence of the production of Von Willebrand factor (VWF), which has an important role in the coagulation process. According to the amendment, von Willebrand's disease can be classified into three main types:
- Type 1, in which there is a partial decrease in VWF production;
- Type 2, in which the factor produced is not functional;
- Type 3, in which there is a complete deficiency of Von Willebrand factor.
This factor is important to promote platelet adhesion to the endothelium, decreasing and stopping bleeding, and it is carrying factor VIII of coagulation, which is important to prevent platelet degradation in plasma and is necessary for activation of factor X and continuation of the cascade. coagulation to form the platelet plug.
This disease is genetic and hereditary, that is, it can be passed between generations, however, it can also be acquired during adulthood when the person has some type of autoimmune disease or cancer, for example.
Von Willebrand's disease has no cure, but control, which must be done throughout life according to the doctor's guidance, type of disease and symptoms presented.
Main symptoms
The symptoms of Von Willebrand's disease depend on the type of the disease, however, the most common include:
- Frequent and prolonged bleeding from the nose;
- Recurrent bleeding from the gums;
- Excess bleeding after a cut;
- Blood in the stool or urine;
- Frequent bruising on various parts of the body;
- Increased menstrual flow.
Typically, these symptoms are more severe in patients with von Willebrand type 3 disease, as there is a greater deficiency of the protein that regulates clotting.
How is the diagnosis
The diagnosis of Von Willebrand's disease is made through laboratory tests in which the presence of VWF and plasma factor VIII are checked, in addition to the bleeding time test and the amount of circulating platelets. It is normal for the exam to be repeated 2 to 3 times in order to have a correct diagnosis of the disease, avoiding false-negative results.
Because it is a genetic disease, genetic counseling before or during pregnancy can be recommended to check the risk of the baby being born with the disease.
In relation to laboratory tests, low levels or absence of VWF and factor VIII and prolonged aPTT are usually identified.
How the treatment is done
Treatment for Von Willebrand's disease is done according to the guidance of the hematologist and the use of antifibrinolytics, which are capable of controlling bleeding from the oral mucosa, nose, hemorrhage and in dental procedures, is usually recommended.
In addition, the use of Desmopressin or Aminocaproic acid to regulate coagulation may be indicated, in addition to the Von Willebrand factor concentrate.
During treatment, it is advised that people with Von Willebrand disease avoid risky situations, such as the practice of extreme sports and the intake of aspirin and other non-steroidal anti-inflammatory drugs, such as Ibuprofen or Diclofenac, without medical advice.
Treatment in pregnancy
Women with Von Willebrand's disease can have a normal pregnancy, without the need for medication, however, the disease can be passed on to their children, since it is a genetic disease.
In these cases, the treatment of the disease during pregnancy is done only 2 to 3 days before delivery with desmopressin, especially when delivery is by cesarean section, and the use of this medication to control bleeding and preserve the woman's life is important. It is also important that this medication is used up to 15 days after delivery, as the levels of factor VIII and VWF decrease again, with a risk of postpartum hemorrhage.
However, this care is not always necessary, especially if factor VIII levels are usually 40 IU / dl or more. That is why it is important to have periodic contact with the hematologist or obstetrician in order to verify the need for the use of medications and if there is any risk for both the woman and the baby.
Is the treatment bad for the baby?
The use of drugs related to Von Willebrand's disease during pregnancy is not harmful to the baby, and is therefore a safe method. However, it is necessary for the baby to carry out a genetic test after birth in order to verify whether or not he has the disease, and if so, to start treatment.
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