Have you ever had a minor cut that took longer than expected to bleed? Or have you ever had nosebleeds or bruises all over your body? You might be thinking, "Oh... that's just how I am." But there may be a medical reason behind these things that you don't know about. Today we're going to talk about a condition that affects the blood clotting process, one that many people haven't even heard of, but is not that rare. That's von Willebrand Disease.
Simply put, what is von Willebrand disease?
von Willebrand disease is a genetic blood disorder that affects the ability of our blood to clot properly.
Imagine that when we get a wound in our body, there is a small team of workers who stop the bleeding. These workers work together and help to close the wound, like a plaster. That "manager" is a protein called von Willebrand factor (VWF) . This VWF protein is what helps platelets in the blood stick together and form a blood clot that seals the wound.
Now, in a person with von Willebrand disease, the body doesn't produce enough of this VWF protein. Or the protein that is produced doesn't work properly. So because that "manager" isn't there, it takes a long time for platelets to stick together and form a blood clot. That's why even a small wound keeps bleeding.
If this condition becomes severe, bleeding can occur inside the joints or soft tissues, causing severe pain and swelling. In some people, this can also lead to anemia , a condition in which there is a decrease in the amount of blood in the body.
This is not a completely curable disease. But don't worry! Your doctor, especially a hematologist, can help you manage this condition well and live a normal life.
Are there main types of this disease?
Yes, von Willebrand disease is not the same for everyone. There are three main types of it. The severity of symptoms varies depending on the type.
| Disease type | Description |
|---|---|
| Type 1 | This is the most common type . About three out of every four people with the disease have this type. In this case, VWF levels are low, but the symptoms are very mild. Sometimes there may be no symptoms at all. |
| Type 2 | In this type, the body produces the VWF protein, but it doesn't work properly. That is, even though it has a "manager," it doesn't know how to do its job. This can cause mild to moderate bleeding. |
| Type 3 | This is the rarest and most severe type . In this case, the level of VWF protein in the body is very low, sometimes even absent. This puts you at risk of serious bleeding. |
What are the symptoms of von Willebrand disease?
For many people, especially those with Type 1, the symptoms are not that severe. They may live without even knowing they have the disease. However, in severe cases, the following symptoms may occur:
- Frequent nosebleeds: If your nose bleeds from even the slightest thing and it's hard to stop.
- Prolonged bleeding from a minor wound: If a cut or scrape bleeds for more than 10 minutes.
- Bruising easily: If you develop large, swollen bruises even after a minor bump.
- Heavy menstrual bleeding in women: If you bleed more than normal during your period. For example, if you have to change your pads before the end of an hour or two, if you pass large blood clots, or if you bleed for more than 7 days, you should be concerned.
- Excessive bleeding after surgery: If you bleed more than normal, even after something as simple as a tooth extraction.
- Heavy bleeding after childbirth or a miscarriage.
- Blood in the stool or urine.
Why does this disease occur? How is it passed down through generations?
von Willebrand disease is a genetic disorder . Simply put, it is caused by a defect in the gene that controls the production of the VWF protein. We inherit this defective gene from our parents.
There are two ways to inherit this:
1. Autosomal dominant inheritance: This occurs from either the mother or the father.Even if only one person inherits the defective gene, the disease can develop. Type 1 and Type 2 are often inherited this way.
2. Autosomal recessive inheritance: In this case, in order to develop the disease, both parents must inherit the defective gene. Type 3, the most severe type, is most often inherited this way.
Rarely, some cancers, autoimmune disorders, and heart disease can also cause this condition later in life. It is called acquired von Willebrand syndrome .
How does a doctor diagnose this disease?
When you tell your doctor about your symptoms, he or she will first examine you and look for signs of bruising or other signs of bleeding. Then they will ask if anyone in your family has had this type of bleeding problem.
Several blood tests are required to accurately diagnose the disease.
- Complete blood count (CBC): This checks the number of red cells, white cells, and platelets in your blood. If your platelet count is low, or if your hemoglobin is low due to anemia, this condition may be suspected.
- Clotting tests: These are tests that measure how long it takes for your blood to clot.
- von Willebrand factor (VWF) tests: These specialized tests check the level of the VWF protein in your blood and whether it is working properly.
- Factor VIII tests: The VWF protein protects Factor VIII, another important protein that helps blood clot. So when VWF is low, Factor VIII levels can also be low. This test looks for that.
What are the treatments for this?
Treatment depends on the type of disease you have and the severity of your symptoms. Most people only need treatment before surgery or after an injury.
There are several main treatment methods:
- Desmopressin (DDAVP): This is the most commonly used treatment. It is a hormone. It is given as a nasal spray or as an injection. It works by accelerating the release of VWF proteins stored in our bodies into the blood.
- von Willebrand factor infusions: In severe cases, a concentrated form of VWF is given to the body through an injection. This is like giving the body the depleted VWF protein.
- Antifibrinolytics: These medications work by stopping a blood clot from dissolving too quickly. They are given to control bleeding in situations like tooth extractions.
- Birth control pills: These are useful for women with heavy menstrual bleeding. The estrogen hormone in these pills helps increase VWF levels.
When should I see a doctor?
If you bleed easily, bruise easily, or have heavy periods, be sure to see your family doctor. There can be other causes of heavy bleeding, so it's important to get a proper diagnosis.
Most importantly: Any time you can't control the bleeding, go to the hospital's Emergency Treatment Unit (ETU) immediately.
What can I do while living with this disease?
Having von Willebrand disease means you need to be a little more careful to prevent bleeding. Your doctor will advise you on this. In general, it is best to avoid the following:
- Avoid sports that can cause injury: Sports that involve a lot of contact, such as rugby and boxing, are not suitable.
- Avoid taking blood thinners: Do not take painkillers such as aspirin or NSAIDs (e.g., Ibuprofen, Diclofenac) without your doctor's approval.
- Avoid taking certain supplements: Things like vitamin E and fish oil can thin the blood. Ask your doctor before using anything.
Tell all your doctors (including your dentist) that you have this condition so they can take steps to control bleeding before doing anything, such as surgery. It's also a good idea to wear a medical alert bracelet to alert medical staff in case of an emergency.
Take-Home Message
- Von Willebrand disease is an inherited blood clotting disorder caused by a deficiency or malfunction of a protein called von Willebrand factor (VWF).
- The most common type (Type 1) has very mild symptoms and may not show any symptoms at all.
- Frequent nosebleeds, easy bruising, heavy menstruation, and prolonged bleeding from wounds are the main symptoms.
- Although it cannot be completely cured, it can be very well managed with treatments like Desmopressin, VWF infusions, and lifestyle changes.
- If you have these symptoms, don't hesitate to see your doctor and get the necessary tests done. Correct diagnosis and management are the most important things.











💬 Comments (0)
No comments have been posted yet. Add your comment here for the first time.
Add your comment