Von Willebrand syndrome in children causes types and the latest treatment methods

Von Willebrand disease in children is a rare bleeding disorder that affects the blood’s ability to clot normally. A child with this condition may experience frequent nosebleeds, bleeding gums, easy bruising, or unusually prolonged bleeding after minor cuts or simple medical procedures. Understanding the causes of Von Willebrand syndrome, recognizing its symptoms and types, and learning about the available diagnostic methods and treatments can help parents protect their children from serious complications. In this Dailylie Medical guide, we will explain in detail everything you need to know about the condition: its causes, types, symptoms, prevention strategies, and the latest treatment options — whether through medication, nutritional support, or medical interventions.

❓ What happens if a child has Von Willebrand disease?

Children with Von Willebrand disease (VWD) have a higher tendency to bleed than normal. They may experience:

Frequent nosebleeds.
Prolonged bleeding even from small cuts.
Easy bruising without a clear reason.
In girls, heavy menstrual periods or excessive bleeding after childbirth.
In severe cases, bleeding into joints or soft tissues causing pain and swelling.
Some children may develop anemia due to repeated blood loss.

❓ How common is Von Willebrand disease?

Von Willebrand disease is the most common inherited bleeding disorder worldwide.

In the U.S., it affects about 1% of the population.
Globally, it appears in 23 to 110 cases per million people.
Many cases go undiagnosed for years because symptoms can look like “normal” bleeding.

❓ Is Von Willebrand disease hereditary?

✅ Yes. In most cases, the condition is inherited from one of the parents.
❌ Rarely, it can be acquired later in life due to autoimmune conditions or other health problems.

❓ Is Von Willebrand disease life-threatening for children?

In most cases, VWD is not life-threatening if diagnosed early and managed properly. The main risk is during severe bleeding, especially after surgery or major injuries. This makes regular medical follow-up very important.

❓ Can a child with Von Willebrand disease live a normal life?

✅ Yes. With proper treatment, lifestyle adjustments, and regular check-ups, most children can live a completely normal life.

❓ Is Von Willebrand disease contagious?

❌ No. It is either inherited or rarely acquired, but it is not contagious.

❓ What’s the difference between Von Willebrand disease and hemophilia?

Von Willebrand disease: caused by a problem with the Von Willebrand factor (VWF protein) and sometimes platelets.
Hemophilia: caused by a deficiency of clotting factors VIII or IX.

Both cause bleeding issues, but the root cause is different.

❓ Can children with VWD receive vaccines?

✅ Most vaccines are safe.
If the vaccine is usually given intramuscularly, doctors may prefer a subcutaneous injection to lower the risk of bleeding.

❓ Can a child with VWD play sports?

✅ Safe sports: walking, swimming, and light activities.
❌ Avoid contact or high-risk sports (like football, wrestling, or boxing) to reduce the chance of injury and bleeding.

❓ Does diet affect Von Willebrand disease?

There are no specific foods that trigger bleeding. However, children should avoid medications like aspirin or certain painkillers that can affect platelets and worsen bleeding.

❓ Is there a cure for Von Willebrand disease?

❌ No permanent cure exists.
But with medications, plasma infusions, and preventive care, children can manage symptoms and live normally.

❓ Does Von Willebrand disease go away with age?

❌ No. VWD is a lifelong condition.
But with proper treatment, symptoms can become so mild that the child can lead a normal life.

✅ Types of Von Willebrand disease in children

Type 1 (most common – 70–80% of cases):
- Partial deficiency of the VWF protein.
- Symptoms: mild to moderate bruising, nosebleeds, gum bleeding, prolonged bleeding after cuts.
Type 2 (about 15–30% of cases):
- VWF protein is present but doesn’t function properly.
- Has 4 subtypes: 2A, 2B, 2M, 2N.
- Symptoms: moderate bleeding, frequent nose/mouth bleeds, prolonged bleeding after injuries or surgery.
Type 3 (rare and most severe – 5–10% of cases):
- Almost no VWF protein in the blood.
- Symptoms: severe bleeding, internal bleeding into joints and muscles (like hemophilia), severe anemia.

✅ Causes of Von Willebrand disease in children

Genetic causes (most common)

Caused by mutations in the gene responsible for producing VWF protein.
Role of VWF:
- Helps platelets stick to the wound site.
- Protects clotting factor VIII from breaking down.
Usually inherited in an autosomal dominant pattern (if one parent is affected, the child has a high chance of inheriting it).

Acquired causes (very rare in children)

Sometimes develops later due to:

Autoimmune diseases (body attacks the VWF protein).
Certain blood disorders or cancers (like lymphoma or myeloma).
Heart conditions (more common in adults, e.g., aortic stenosis).
Some medications that affect VWF function.

✅ Diagnosis and tests for Von Willebrand disease

Step 1: Medical history and symptoms

Doctor will ask about frequent bleeding, easy bruising, or family history of bleeding disorders.

Step 2: Laboratory tests

Complete Blood Count (CBC): usually normal, but may show anemia if bleeding is severe.
Platelet function tests: check how well platelets clump together.
APTT (Activated Partial Thromboplastin Time): measures clotting time and factor VIII activity.
PT (Prothrombin Time): checks additional clotting factors.
Fibrinogen test: evaluates another clotting protein.
VWF antigen test: measures the amount of VWF in the blood.
Ristocetin cofactor test: checks VWF activity and function.
VWF multimer test: analyzes the structure of the protein.

Note: Tests may need to be repeated because VWF levels can fluctuate due to stress, physical activity, or hormones.

Determining the Type of von Willebrand Disease

After medical tests, doctors identify the type of the disease:

Type 1

The most common form (60% – 80% of cases).
Reduced levels of von Willebrand factor, but symptoms are usually mild.

Type 2

The protein is present but does not function properly.
Accounts for about 15% – 30% of cases.
Symptoms: mild to moderate bleeding.

Type 3

The rarest and most severe type (5% – 10% of cases).
Almost complete absence of von Willebrand factor.
Symptoms: severe and potentially life-threatening bleeding similar to hemophilia.

✅ Symptoms of von Willebrand Disease in Children

The symptoms vary depending on the severity of the protein deficiency and the type of the disease. Some children may carry the condition without showing obvious signs.

1. Common bleeding symptoms

Frequent bruising, even with minor injuries.
Nosebleeds (epistaxis) lasting for long periods.
Gum bleeding, especially during toothbrushing or teething.
Prolonged bleeding from small cuts or scratches.

2. Gastrointestinal symptoms

Bleeding in the stomach or intestines (rare but possible).
Black or blood-stained stools.

3. After medical procedures

Prolonged bleeding after tooth extraction.
Severe bleeding following surgery or even minor wounds.

4. Specific to girls (after puberty)

Very heavy menstrual bleeding (menorrhagia).
Periods that last longer than usual.

5. General symptoms

Anemia due to frequent blood loss.
Fatigue, general weakness, and pale skin.

The severity of symptoms depends on the type of von Willebrand disease (Type 1, 2, or 3).

Mild cases: Symptoms may only appear after injuries or surgeries.
Severe cases: Bleeding can occur spontaneously and be dangerous even without a clear injury.

Stages of von Willebrand Disease in Children

Unlike cancer, von Willebrand disease is not classified into stages, but symptoms progress depending on the severity and type of the condition.

Stage 1: Mild symptoms (common in Type 1)

Occasional nosebleeds.
Scattered bruises.
Longer-than-normal bleeding after small cuts or dental extraction.

Stage 2: Moderate symptoms (common in Type 2)

Frequent nose and mouth bleeding.
Noticeable bleeding after minor injuries or surgery.
In girls at puberty: heavy and prolonged menstrual bleeding.

Stage 3: Severe symptoms (common in Type 3)

Internal bleeding in joints and muscles (similar to hemophilia).
Severe anemia from recurrent blood loss.
Uncontrollable bleeding after injuries or surgery.

⚠️ Complications of von Willebrand Disease in Children

The condition affects the blood’s ability to clot normally, leading to both short- and long-term complications depending on severity.

Complications due to frequent bleeding

Recurrent nosebleeds.
Prolonged bleeding after dental work or minor cuts.
Gum bleeding during toothbrushing.

Complications affecting general health

Anemia from constant blood loss.
Fatigue and general weakness.
Pale skin and shortness of breath during physical activity.

Serious complications in severe cases

Internal bleeding in joints and muscles, causing:
- Severe pain and swelling.
- Reduced mobility over time.
Gastrointestinal bleeding.
Major bleeding after surgery or trauma, which can be life-threatening.

Psychological and Social Impact

Constant fear and anxiety of sudden bleeding episodes.
Restriction from certain activities such as contact sports.
Negative effect on self-confidence and social participation.

Medical Treatment of von Willebrand Disease in Children

The treatment depends on the severity of the condition (mild – moderate – severe) and the type of von Willebrand disease. The main treatment options include:

1. Desmopressin (DDAVP)

Most commonly used for mild to moderate cases.
Stimulates the release of von Willebrand factor and clotting factor VIII.
Administered as an intravenous injection or nasal spray.
Suitable for most children but not effective in all types.

2. von Willebrand Factor (VWF) Concentrates

Used in severe cases or when desmopressin is not effective.
Given as an intravenous infusion of ready-made clotting protein.
Essential before surgeries or during major bleeding episodes.

3. Antifibrinolytic Medications

Examples: Tranexamic acid and Aminocaproic acid.
Prevent the breakdown of existing clots.
Very helpful in gum bleeding, nosebleeds, or after dental procedures.

4. Iron Supplements

Not a direct treatment for the disease, but important in managing anemia caused by frequent blood loss.
Increase hemoglobin levels and improve energy in children.

Vitamins and Minerals in Treatment

While vitamins and minerals do not treat the root cause, they support overall health and reduce the impact of frequent bleeding.

Key nutrients:

Iron: To treat anemia caused by blood loss.
Vitamin C: Improves iron absorption and strengthens blood vessels.
Vitamin K: Essential for clotting, sometimes prescribed as a supplement.
Vitamin D and Calcium: Support bone health, especially with long-term treatment.

Important notes:

Supplements are supportive, not a main treatment.
Always taken under medical supervision.
Balanced diet is key: vegetables, fruits, protein, dairy, and whole grains.

Surgical Treatment of von Willebrand Disease in Children

Surgery is not a primary treatment but may be necessary in special cases:

1. Dental or surgical procedures

Example: tonsillectomy or tooth extraction.
Requires preparation with desmopressin or plasma/VWF concentrates.
Goal: reduce bleeding risk during and after the procedure.

2. Splenectomy (removal of the spleen)

Very rare.
Considered in specific cases related to platelet problems or severe complications.

3. Emergency surgery

For life-threatening internal bleeding (e.g., in the brain or abdomen).
Requires urgent surgical intervention with rapid blood and plasma transfusion.

Coping with von Willebrand Disease in Children

Managing the condition goes beyond medical treatment. It also involves lifestyle adjustments and psychological support to help children live safely and confidently.

1. Medical care under doctor supervision

Desmopressin (DDAVP) to raise VWF and factor VIII levels.
VWF concentrates for moderate to severe cases, especially before surgeries.
Antifibrinolytics (Tranexamic acid, Aminocaproic acid) for gum or post-dental bleeding.
Blood or plasma transfusions for severe emergencies.

2. Daily care and lifestyle adjustments

Avoid medications that weaken clotting, like aspirin and ibuprofen, unless prescribed.
Prevent injuries:
- Avoid high-risk sports.
- Use protective gear during physical activity.
Maintain good oral hygiene to reduce gum bleeding.
Follow an iron-rich diet to prevent anemia from frequent blood loss.

3. Psychological and social support

Explain the condition to the child in simple terms so they can understand and manage themselves.
Train parents on how to handle sudden bleeding.
Inform the school about the child’s condition to ensure a safe environment.
Provide emotional support to help the child feel included and not different from peers.

4. Regular medical follow-ups