NCLEX Review Guide: Von Willebrand's Disease in Pediatric Patients

Disease Overview

• Von Willebrand's Disease (vWD) is the most common inherited bleeding disorder, affecting 1-3% of the population with deficiency or dysfunction of von Willebrand factor protein.
• Von Willebrand factor helps platelets stick together and carries factor VIII, making it essential for primary hemostasis and clot formation.
• Three main types exist: Type 1 (mild, most common), Type 2 (moderate, several subtypes), and Type 3 (severe, rare).

Clinical Manifestations

• Mucocutaneous bleeding including frequent nosebleeds, easy bruising, prolonged bleeding from cuts, and heavy menstrual periods in adolescent girls.
• Bleeding symptoms typically appear early in childhood but may be overlooked as "normal" childhood injuries until severe bleeding episodes occur.
• Excessive bleeding after dental procedures, surgery, or trauma that requires medical intervention or blood products.

Laboratory Findings

• Prolonged aPTT (activated partial thromboplastin time) due to decreased factor VIII levels, while PT (prothrombin time) remains normal.
• Decreased ristocetin cofactor activity and von Willebrand factor antigen levels confirm diagnosis through specialized coagulation studies.
• Platelet count is typically normal, but platelet function is impaired leading to prolonged bleeding time.

Acute Care Management

1. Apply direct pressure and cold compresses to bleeding sites for immediate hemostasis control.
2. Administer DDAVP (desmopressin) as ordered to temporarily increase von Willebrand factor and factor VIII levels.
3. Prepare for factor replacement therapy with von Willebrand factor concentrates for severe bleeding episodes.
4. Monitor vital signs closely for signs of hypovolemic shock during active bleeding episodes.

Preventive Care & Education

• Teach families to avoid aspirin and NSAIDs which further impair platelet function and increase bleeding risk.
• Educate about proper dental hygiene using soft-bristled toothbrushes and regular dental care to prevent gum bleeding.
• Encourage participation in low-contact sports while avoiding high-risk activities like football or hockey that increase trauma risk.
• Ensure child wears medical alert identification and family knows when to seek immediate medical attention for bleeding episodes.

NCLEX Success Strategies

• Remember that vWD affects both males and females equally unlike X-linked hemophilia which primarily affects males.
• Focus on bleeding prevention strategies rather than just treatment when answering NCLEX questions about vWD.
• Understand that DDAVP effectiveness varies by vWD type and may not work for Type 2B or Type 3.