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Von Willebrand’s Disease

A 12-year-old child with von Willebrand's disease is scheduled for a dental extraction. Which nursing intervention is most appropriate to prepare this child for the procedure?

A 12-year-old child diagnosed with von Willebrand's disease requires a dental extraction due to severe tooth decay. The child has a history of prolonged bleeding after minor injuries and nosebleeds that last longer than 20 minutes.
해설
Children with von Willebrand's disease have a bleeding disorder that requires specific preparation before invasive procedures like dental extractions.

Von Willebrand disease is the most common inherited bleeding disorder, affecting about 1% of the population. This condition is caused by a deficiency or dysfunction of von Willebrand factor (vWF), a protein essential for normal blood clotting. This protein helps platelets stick together and adhere to blood vessel walls when bleeding occurs.

For children with von Willebrand disease, invasive procedures like dental extractions pose a serious risk of prolonged bleeding. A key nursing intervention is to collaborate with physicians to administer desmopressin (DDAVP) before the procedure. DDAVP is a synthetic hormone that stimulates the release of von Willebrand factor and factor VIII from storage sites, temporarily improving the child's clotting ability.

DDAVP is typically administered 30-60 minutes before the procedure and can be given intravenously, subcutaneously, or as a nasal spray depending on the severity of the condition and the invasiveness of the procedure. This medication increases von Willebrand factor levels by 2-5 times above baseline, significantly reducing the risk of bleeding.

Nurses should also ensure proper communication between the dental and hematology teams, confirm that appropriate blood products are available if needed, and educate the family about post-procedure bleeding prevention measures. Monitoring for signs of excessive bleeding during and after the procedure is essential.

These interventions directly address the underlying pathophysiology of von Willebrand disease and most effectively prevent complications during dental procedures.
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