What is Von Willebrand Disease?
Von Willebrand Disease (VWD) is a common inherited bleeding disorder that affects the blood's ability to clot properly. It is caused by a deficiency or dysfunction of the
von Willebrand factor (VWF), a protein crucial for platelet adhesion.
How Common is VWD in Children?
VWD is the most common inherited bleeding disorder, occurring in approximately 1 in 100 individuals. However, many cases are mild and go undiagnosed. In the pediatric population, the prevalence is similar, but awareness and diagnosis can be more challenging due to the variability in symptoms.
Type 1: The most common and mildest form, characterized by a partial quantitative deficiency of VWF.
Type 2: Involves a qualitative defect in VWF. It is divided into several subtypes (2A, 2B, 2M, 2N) based on the specific functional abnormality.
Type 3: The rarest and most severe form, characterized by an almost complete absence of VWF.
Frequent nosebleeds (
epistaxis)
Easy bruising
Prolonged bleeding from cuts or injuries
Excessive bleeding during dental procedures
Heavy menstrual bleeding in adolescent girls (
menorrhagia)
How is VWD Diagnosed?
Diagnosis of VWD involves a combination of clinical evaluation and laboratory tests. Important diagnostic tests include:
Complete Blood Count (CBC): To check for anemia due to blood loss.
VWF Antigen Test: Measures the amount of VWF in the blood.
Ristocetin Cofactor Activity: Evaluates the functioning of VWF.
Factor VIII Activity: Since VWF stabilizes factor VIII, this test can help identify VWD.
Multimer Analysis: Differentiates between different subtypes of VWD.
Desmopressin (DDAVP): A synthetic hormone that can boost levels of VWF and factor VIII in mild cases.
VWF Replacement Therapy: For moderate to severe cases or when DDAVP is ineffective.
Antifibrinolytic Agents: Such as
tranexamic acid or aminocaproic acid, to prevent breakdown of clots.
Avoid medications that affect platelet function, such as aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs).
Inform healthcare providers, including dentists, about the child's condition.
Ensure the child wears a medical alert bracelet indicating their diagnosis.
Teach the child about their condition and how to manage minor bleeding episodes.
What is the Prognosis for Children with VWD?
With proper diagnosis and management, most children with VWD can lead normal, healthy lives. Regular follow-up with a hematologist is essential to monitor the disease and adjust treatment as needed.
Conclusion
Von Willebrand Disease is a manageable condition with appropriate care and treatment. Awareness and education are key to ensuring children with VWD receive the necessary support and live active, fulfilling lives.
