Von Willebrand's disease is an inherited disease that makes sufferers bleed more easily. In sufferers, the blood-clotting protein called von Willebrand factor is small or does not work normally. Von Willebrand's disease is an incurable condition. However, with proper treatment, patients can live a normal life.
Symptoms of von Willebrand's disease
Symptoms of von Willebrand's disease can be mild or not appear at all, so the sufferer is not aware of it. The severity of this disease is different for each patient.
The following are symptoms of von Willebrand's disease:
- Heavy bleeding after tooth extraction or surgery
- Nosebleeds that don't stop in 10 minutes
- There is blood in the urine or stool
- Easy bruising skin
Symptoms of von Willebrand's disease will be more easily seen in women, especially during menstruation, including:
- Menstruation lasts a long time, or blood that comes out very much.
- Two pads are needed at the same time to restrain the rate of menstrual blood.
- Change pads or tampons more than once an hour.
- Symptoms of anemia appear, such as easily tired, weak, or short of breath.
Causes of von Willebrand's Disease
Von Willebrand disease is caused by a mutation in the VWF gene, the gene that instructs the body to produce von Willebrand factor. Mutations in the VWF gene can reduce the amount of von Willebrand factor, or cause disturbances in the function of von Willebrand factor.
Von Willebrand factor itself functions to form blood clots, by attaching platelet cells (platelets) to the walls of injured or bleeding tissue, to form blood clots. The formation of this blood clot is important to stop bleeding. However, in patients with this disease, there is only a small amount of von Willebrand factor in the body, or there is a disturbance in the function of this factor. This condition causes platelets to not stick together, resulting in blood clots not forming and bleeding becoming uncontrollable.
These gene abnormalities are usually inherited from parents. However, this gene abnormality can appear on its own when a person is an adult, even though they do not have parents with the same disease.
Diagnosis of von Willebrand's disease
At the initial examination, the doctor will ask the patient and family history of the disease. Then, the doctor will perform several blood tests, such as:
- Von Willebrand factor antigen. Through this test, doctors can determine the level of von Willebrand factor in the blood, by measuring the levels of a number of proteins in the blood.
- Ristocetin cofactor activity. In this test, the doctor will measure how well von Willebrand factor performs in the blood clotting process.
Please note that test results may vary due to a number of factors such as infection, stress, pregnancy, or taking medication. So the patient can be advised to do a repeat blood test.
Treatment of von Willebrand's disease
Although von Willebrand's disease has no cure, medication can help prevent and stop bleeding. The treatment method chosen depends on the patient's condition, type, severity of the disease, and the patient's response to previous treatment methods.
Some of the types of treatment for Von Willebrand's Disease are:
- Tranexamic acid. Tranexamic acid is used to slow down the process of breaking down blood clots that have already formed, so that bleeding can stop.
- Family planning pills. In female patients, birth control pills can be used to control heavy bleeding during menstruation. The content of the hormone estrogen in birth control pills can also encourage the production of von Willebrand factor.
- Desmopressin.Desmopressin is a synthetic hormone that works to stimulate the body to release more von Willebrand factor which is stored in the walls of blood vessels.
Complications of von Willebrand's disease
If not treated properly, people with Von Willebrand's disease can experience complications, such as:
- Pain and swelling. This condition occurs when there is abnormal bleeding in the soft tissues or joints.
- Anemia. Excessive menstrual bleeding can lead to iron deficiency anemia.
Von Willebrand's disease can cause uncontrolled bleeding that can lead to death. Therefore, medical treatment should be given immediately in case of severe bleeding.
