Von Willebrand disease is a type of haemophilia characterized by a deficiency in a blood protein, known as von Willebrand’s factor, which makes clotting difficult, causing the patient to bleed too much after a cut.
“Von Willebrand disease” is usually a hereditary problem that can be passed from parents to children, but it can also be acquired during adulthood when the patient has an autoimmune disease or cancer, for example.
The von Willebrand disease is not curable but can be controlled with the use of drugs as desmopressin, to control blood clotting lifelong or during surgery.
Treatment for von Willebrand disease
Treatment for Von Willebrand’s disease should be guided by a haematologist and is usually done with the use of medicines such as Desmopressin or Aminocaproic Acid to regulate coagulation.
In milder cases, such as von Willebrand disease type 1, the doctor may recommend these medications only when it is necessary to have surgery or go to the dentist, for example, avoiding excessive bleeding.
During treatment, patients with Von Willebrand disease are advised to avoid risky situations such as extreme sports and the ingestion of aspirin and other non-steroidal anti-inflammatory drugs, such as Ibuprofen or Diclofenac, without medical advice.
Symptoms of von Willebrand disease
Symptoms of Von Willebrand disease depend on the type of the disease, however, the most common include:
Frequent and prolonged nose bleeds;
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 coagulation.
Diagnosis of Von Willebrand’s disease
The diagnosis of von Willebrand disease is made through several blood tests. In these blood tests the plasma factor VIII dosage, assay of the vWF antigen, the dosage of ristocetin cofactor and RIPA are evaluated.
It is normal for these tests to be repeated at least 2 to 3 times for a correct diagnosis of the disease, avoiding false-negative results.
Because it is a genetic disease of a familial nature, genetic counselling during pregnancy can be used to assess the risk of the baby being born with von Willebrand’s disease.
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