Von Willebrand or EvW disease is a genetic and inherited disease characterized by decreased or absent von Willebrand factor (FvW) production, which plays an important role in the clotting process.According to the amendment, von Willebrand disease can be classified into three main categories:
This factor is important for promoting platelet adhesion to the endothelium, decreasing and stopping bleeding, and transporting coagulation factor VIII, which is important for preventing platelet degradation in plasma and is necessary for X-factor activation and continuation of the coagulation cascade.to form the platelet cap.
- This disease is genetic and hereditary.
- I.
- E.
- It can be passed down from one generation to the next.
- However.
- It can also be acquired during adult life when the person has a type of autoimmune disease or cancer.
- For example.
Von Willebrand’s disease has no cure, but a lifelong control, which must be done on the doctor’s advice, type of disease, and symptoms.
Symptoms of von Willebrand disease depend on the type of disease, however, the most common include:
In general, these symptoms are more severe in patients with von Willebrand type 3 disease, as there is an increased protein deficiency that regulates clotting.
Diagnosis of Von Willebrand disease is made by laboratory tests verifying the presence of FvW and plasma factor VIII, in addition to testing bleeding time and the number of platelets in circulation, it is normal to repeat the test 2 to 3 times to have a correct diagnosis of the disease , avoiding false negatives.
Because it is a genetic disease, genetic counseling may be recommended before or during pregnancy to check the risk of the baby being born with the disease.
For laboratory tests, low levels or absence of VWF and factor VIII and prolonged TCA are generally identified.
Treatment of Von Willebrand disease is done on the advice of the hematologist and the use of antifibrinolytics, capable of controlling bleeding from the oral mucosa, nose, bleeding and dental procedures, is generally recommended.
In addition, the use of desmopressin or aminocaproic acid may be indicated to regulate coagulation in addition to Von Willebrand factor concentrate.
During treatment, people with Von Willebrand disease are advised to avoid risky situations, such as extreme sports and taking aspirin and other nonsteroidal anti-inflammatory drugs, such as ibuprofen or diclofenac, without medical advice.
Women with Von Willebrand disease may have a normal pregnancy without medication, however, the disease can be passed on to their children because it is a genetic disease.
In these cases, treatment of the disease during pregnancy is done only 2 to 3 days before delivery with desmopressin, especially when delivery is done by C-section, it is important to use this medicine to control bleeding and preserve a woman’s life.It is also important that this medicine is used up to 15 days after delivery, as factor VIII and VWF levels decrease again, at risk of postpartum bleeding.
However, this care is not always necessary, especially if factor VIII levels are usually 40 IU/dL or more, so it is important to have regular contact with the hematologist or obstetrician to check for medication and whether there is any risk to women and babies.
The use of von Willebrand disease-related medications during pregnancy is not harmful to the baby and is therefore a safe method, however, the baby needs to perform a genetic test after birth to check whether or not the baby has the disease and, if necessary, begin treatment.