Von Willebrand Disease

What is Von Willebrand Disease? This is the explanation

what is von willebrand disease

Von Willebrand Disease

What is Von Willebrand Disease?

What is Von Willebrand Disease? Von Willebrand is a congenital or hereditary disease in which the sufferer is disturbed in the process of blood clotting. In people with Von Willebrand disease, a blood protein called the Von Willebrand factor does not work normally or is too small.

 

Von Willebrand’s disease is a lifelong illness, and most of the sufferers have had the disease since birth. However, the initial symptoms are usually new will appear after a few years. Most people will begin to recognize it when extracting teeth for the first time, or during menstrual periods first, where they will experience excessive and prolonged bleeding.

Until now, there is still no treatment to cure this disease. But with proper and quick handling, Von Willebrand sufferers can live a normal life.

Symptoms of Von Willebrand Disease

Von Willebrand’s disease can cause symptoms at any time from birth. But for most people, the symptoms are mild so the patient will not notice it. The degree of severity will vary from one person to another.

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Some of the symptoms that can be felt by people with Von Willebrand’s disease are:

  1. Excessive bleeding after the procedure of tooth extraction or surgery.
  2. The nosebleed that does not stop within 10 minutes.
  3. Easy bruising.

Von Willebrand’s symptoms will be more easily seen in women, especially during menstruation. Signs and symptoms of menstruation that indicate you have Von Willebrand include:

  1. There is a blood clot with a diameter of more than 2.5 cm at the time of menstruation.
  2. You need two pads at a time to withstand the menstrual blood rate.
  3. Bandages or tampons need to be changed more than once in an hour.
  4. There are symptoms of anemia such as easy fatigue, weakness, or shortness of breath.

If not treated properly, people with Von Willebrand’s disease may experience complications such as:

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  1. Pain and swelling, this occurs when abnormal bleeding occurs in soft tissue or joints.
  2. Patients with women may experience anemia due to excessive menstrual bleeding.
  3. Although rare, death can occur if the bleeding is uncontrolled.

Causes of Von Willebrand’s Disease

The gene abnormality that controls the Von Willebrand factor protein is a major cause of Von Willebrand’s disease. Gene abnormalities are usually passed on by one or both parents to their child.

The Von Willebrand factor is like a glue, where it will glue the platelets (one type of blood cell) to form a clot to stop the bleeding. In addition, this protein also works with other blood clotting factors called factor VIII (factor 8). Therefore, any disturbance in the Von Willebrand factor will cause the blood is not able to clot completely so that the sufferer is at risk for excessive bleeding in the long term.

A person may have Von Willebrand’s disease without any hereditary factors, but because of certain medical conditions. However, this is rare. Some of the medical conditions associated with Von Willebrand’s disease are lupus, hypothyroidism, aortic stenosis, and some cancers.

Von Willebrand’s disease can be divided into four types based on the number and function of Von Willebrand factor in the patient’s blood, namely:

  1. Type 1, is the lightest and most common type of Von Willebrand disease. Type 1 Von Willebrand disease occurs when the level of Von Willebrand factor in the blood decreases. Usually, the patient does not bleed spontaneously, but they can suffer severe bleeding during surgery, injury, or tooth extraction.
  2. Type 2. In this type, the abnormality lies in the function of the Von Willebrand factor, where this factor is not working properly. Patients with this type will experience more severe symptoms than people with type 1.
  3. Type 3. Patients of this type have very low levels of Von Willebrand factor or none, and factor VIII levels are also low. Although rare, this type of symptoms causes severe, such as spontaneous bleeding in muscles and joints.
  4. Type pseudo or platelet. Just like type 2, but sufferers experience this abnormality in platelets rather than on the Von Willebrand factor.

Diagnosis of Von Willebrand Disease

If the patient is suspected of suffering from Von Willebrand’s disease, the general practitioner will usually advise a follow-up examination by a hematologist.

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In the initial examination, the doctor will usually ask about the history of the illness and family history. After that, your doctor may ask you to do some blood tests, such as:

  1. Von Willebrand factor antigen. From the results of this test, will be able to see the level of Von Willebrand factor in the blood by measuring some specific protein.
  2. Ristocetin cofactor activity. In this test, will be examined how well the performance of Von Willebrand factor in blood clotting process.
  3. Activity freezing factor VIII. This test will show whether you have a factor VIII abnormality or not.
  4. Von Willebrand factor multimers. This test will help determine the type of Von Willebrand suffered.

Doctors may request that patients undergo blood tests more than once for more accurate results. The doctor will also ask a history Patients will also be asked questions about the health history of family members.

Treatment of Von Willebrand Disease

Von Willebrand’s disease is an incurable disease that lasts a lifetime. However, Von Willebrand’s mild disease usually does not require treatment. There are three things that determine the type of treatment for patients of this condition, namely the patient’s body response to the previous handling, treatment, and other medical conditions, and the type and severity of the disease.

Some types of treatment for Von Willebrand’s Disease are:

  1. Tranexamic acid and aminocaproic acid. Both drugs that enter the class of antifibrinolytic this serves to slow the process of decomposition of blood clots.
  2. Contraceptive pill. For women, the contraceptive pill can be used to help control heavy bleeding during menstruation.
  3. Giving concentrate factors of blood clotting consisting of Willebrand factor and VIII through infusion.
  4. Desmopressin. This synthetic hormone will stimulate the body to release more of the Von Willebrand factor stored in the blood vessel wall.

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