Von Willebrand disease is an inherited bleeding disorder. People with this disease have problems controlling their bleeding. You can find more information below.
What is von Willebrand disease?
Von Willebrand disease is a lifelong bleeding disorder that affects the blood’s ability to clot well. People with the disease have low levels of a protein called Von Willebrand factor that helps blood clot, or the protein doesn’t work as it should.
Most people with the disease are born with it, having inherited the disease from one or both parents. However, the warning signs of the disease may not appear for years unless there is bleeding from a surgical operation or dental treatment.
Von Willebrand disease cannot be cured definitively. But with symptomatic treatment and self-care, most people with this condition can have a normal life.
What causes von Willebrand disease?
The root cause of von Willebrand disease is an inherited and abnormal gene that controls Von Willebrand factor in the blood. This gene is a protein that plays an important role in blood clotting.
In individuals who have low levels of this protein or don’t work properly, small blood cells called platelets are not able to stick together properly or attach themselves to blood vessel walls normally when an injury has occurred. This condition interferes with the clotting process and can sometimes cause uncontrolled bleeding.
Many people with von Willebrand disease have low levels of factor 8, another protein that helps with clotting. Factor 8 plays a role in another genetic blood disorder called hemophilia . But unlike hemophilia, which mainly affects men, Von Willebrand disease affects both men and women and is usually milder in symptoms.
Although rare, Von Willebrand disease can develop later in people who do not inherit an abnormal gene from a parent. This is known as acquired Von Willebrand syndrome and is likely caused by a medical condition.
Who is at risk?
The main risk factor for von Willebrand disease is having a genetic predisposition. Parents pass on the abnormal gene, which plays a role in the formation of the disease, to their children. Rarely, the disease may not appear for long between generations.
The disease is usually inherited by “autosomal dominant inheritance”. This means that it is sufficient to inherit the abnormal gene from a single parent to be affected by the disease. If you have the von Willebrand gene, the probability of passing it on to your children is 50%, or half.
The most severe form of the disease is inherited by “autosomal recessive inheritance”. In this case, it means that both of your parents passed on an abnormal gene to you.
What are the symptoms of von Willebrand disease?
Many people with von Willebrand disease do not know this because the signs are mild or absent. The most common symptom of the condition is abnormal bleeding.
There are three main types of the disease. The amount of bleeding varies from person to person, depending on the type and severity of the disease.
If a person has Von Willebrand disease, they may have the following symptoms:
- Excessive bleeding after injury, surgery, or dental treatment
- Nosebleeds that don’t stop within 10 minutes
- heavy or prolonged menstrual bleeding
- Blood in the urine or blood in the stool
- easy bruising of the body
Menstrual symptoms may include:
- Blood clots 2.5 centimeters in diameter or larger in the menstrual flow
- The need to change the menstrual pad or tampon more than once an hour
- Signs of anemia, such as tiredness, weakness, or shortness of breath
When should you see a doctor?
If you have prolonged bleeding or if your body bruises easily, it is a good idea to see a doctor as soon as possible.
How is von Willebrand disease diagnosed?
Mild Von Willebrand disease can be difficult to diagnose because the bleeding condition is common and is not considered a symptom of vWH for most people. However, if the doctor suspects a bleeding disorder, they may refer the person concerned to a blood disorders specialist (hematologist).
To evaluate the person for Von Willebrand disease, the doctor will likely ask detailed questions about their medical history and check for bruising or other signs of recent bleeding.
The doctor will likely also recommend the following blood tests:
- Von Willebrand factor antigen: This determines the level of Von Willebrand factor in the blood by measuring a particular protein.
- Von Willebrand factor activity: There are several tests to measure how well von Willebrand factor works in the clotting process.
- Factor 8 coagulation activity: This indicates whether the person concerned has abnormally low coagulation levels and factor 8 activity.
- Von Willebrand factor multimers: This evaluates the structure of Von Willebrand factor in the blood, its protein complexes and how its molecules are broken down.
The results of these tests may fluctuate over time in the same person due to factors such as stress, exercise, infection, pregnancy and medications. Therefore, it may be necessary to repeat some tests.
If someone has Von Willebrand disease, the doctor may recommend testing to find out if other family members also have the disease.
How is von Willebrand disease treated?
Although there is no cure for von Willebrand disease, current treatment can help prevent or stop bleeding episodes. Treatment depends on:
- Type and severity of the condition
- how it responded to previous treatment
- Other drugs and conditions used
The doctor may recommend one or more of the following treatments to increase Von Willebrand factor, strengthen blood clots, or control heavy menstrual bleeding in women :
- Desmopressin: This medication is available as an injection or nasal spray. It is a synthetic hormone that controls bleeding by stimulating the body to release more of Von Willebrand factor stored in the lining of blood vessels.
- Substitution treatments: These include infusions of concentrated blood clotting factors containing Von Willebrand factor and factor 8. If the above treatment is not an option or is ineffective, the doctor may recommend them.
- Oral contraceptives: For women, these can be useful for controlling heavy bleeding during menstrual periods. The estrogen hormones in birth control pills can increase the activity of Von Willebrand factor and factor 8. This effect could possibly be used with birth control patches, but more studies are needed to confirm.
- Clot-stabilizing drugs: Anti-fibrinolytic drugs, such as aminocaproic acid and tranexamic acid, can help stop bleeding by slowing the breakdown of blood clots. Doctors usually prescribe these medications before or after a surgical procedure or tooth extraction.
- Medications applied to cuts: A fibrin sealant placed on a cut helps reduce bleeding. This is applied like glue using a syringe. There are also over-the-counter products to stop nosebleeds.
If the condition is mild, the doctor may recommend treatment only when performing surgery or dental operations, or in cases of trauma such as a traffic accident.
Remember, your doctor will decide which medicine to take and how.
Lifestyle and home remedies
The following self-care tips can help manage the condition:
- Switching pain relievers: To help prevent bleeding episodes, talk to a doctor before taking blood-thinning medications such as aspirin , ibuprofen , or naproxen sodium. The doctor may recommend pain and fever relievers such as acetaminophen instead.
- Telling Doctors and Dentists Before Procedures: Doctors or dentists should be told that you have Von Willebrand disease before starting a new medication or giving birth before surgery. It should also be noted whether anyone in the family has a history of excessive bleeding.
- Warning card: The patient should be aware of the Von Willebrand disease warning on it to assist healthcare personnel in the event of an accident or being taken to the emergency room.
- Being active and safe: Exercise can be done as part of maintaining or maintaining a healthy weight. Activities that can cause bruising, such as football, wrestling, and hockey, should be avoided.