# 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?

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## 문제

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.

## 보기

1. Administer aspirin 30 minutes before the procedure to prevent pain
2. Apply ice packs to the jaw area for 15 minutes before the procedure
3. Coordinate with the physician to administer desmopressin (DDAVP) before the procedure **✔ 정답**
4. Encourage the child to rinse with mouthwash containing alcohol before the procedure

**정답: 3**

## 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.

## 심화 해설

Understanding the Condition

The child has von Willebrand disease (VWD), the most common inherited bleeding disorder. In this condition, there is a quantitative or qualitative defect in von Willebrand factor (vWF), a protein critical for platelet plug formation and for carrying Factor VIII (FVIII) in the blood. A history of prolonged bleeding after minor injuries and nosebleeds lasting longer than 20 minutes signals a clinically significant hemostatic defect. Any surgical procedure, including a dental extraction, poses a serious risk for hemorrhage because the initial injury disrupts the vasculature, and without functional vWF, the platelets cannot properly adhere and aggregate to form a stable clot [1,3,4].

Analyzing the Options

1.  Administer aspirin 30 minutes before the procedure to prevent pain

This intervention is contraindicated. Aspirin irreversibly inhibits platelet aggregation by blocking the cyclooxygenase pathway. In a patient with an existing platelet adhesion defect due to VWD, administering an antiplatelet drug would compound the hemostatic impairment and dramatically increase the risk of severe perioperative bleeding [3].

2.  Apply ice packs to the jaw area for 15 minutes before the procedure

Applying ice causes local vasoconstriction, which can be a useful adjunct for superficial bleeding or post-procedural swelling. However, it does not address the underlying systemic deficiency of von Willebrand factor. This mechanical measure alone is insufficient to prevent bleeding from a surgical wound in a patient with a moderate to severe bleeding disorder.

3.  Coordinate with the physician to administer desmopressin (DDAVP) before the procedure

This is the correct preoperative strategy. Desmopressin (DDAVP) is a synthetic analog of vasopressin that stimulates endothelial cells to release stored vWF and FVIII into the circulation. For many patients with VWD (particularly Type 1 and some Type 2 subtypes), a DDAVP trial is a standard first-line intervention to transiently correct their hemostatic defect before elective surgery. A systematic review on perioperative bleeding management confirms that pharmacological prophylaxis to raise factor levels is a cornerstone of risk stratification and management for patients with inherited bleeding disorders undergoing invasive procedures . The nurse's role is to anticipate this need and coordinate its timely administration, typically 30 to 60 minutes before the procedure.

4.  Encourage the child to rinse with mouthwash containing alcohol before the procedure

Alcohol-containing mouthwashes can irritate and dry the oral mucosa and have no hemostatic benefit. More importantly, alcohol can interfere with platelet function and coagulation, making this an inappropriate and potentially harmful recommendation for a child with a bleeding disorder.

Clinical Reasoning and Perioperative Management

The core principle in managing a patient with VWD for a dental extraction is to temporarily correct the hemostatic defect. The systematic review on bleeding risk assessment emphasizes that management strategies must be tailored to the specific bleeding disorder, focusing on prophylactic factor elevation and careful monitoring . The case reports on surgical interventions in VWD patients reinforce that optimizing vWF and FVIII levels is critical to preventing perioperative bleeding, and this often requires a multidisciplinary approach involving the nurse, dentist, and hematologist [1,4]. Administering DDAVP preoperatively is a direct application of this principle, aiming to boost the child's own factor levels to achieve adequate hemostasis during and after the extraction. The nurse's most appropriate intervention is to facilitate this pharmacological preparation, ensuring the child is protected from the significant bleeding risk identified in the history and diagnosis.References (research sources)

- [3]Immune Thrombocytopenia and Type 1 von Willebrand Disease in a Patient With a Femoral Fracture: A Case Report.Case reportFerreira A, Roberto A. (2025) · DOI: 10.7759/cureus.98215

## 임상 시나리오

Perioperative Management of von Willebrand DiseasePreventing Hemorrhage During Dental Extraction
The primary goal before any invasive procedure is to correct the hemostatic defect. For mild to moderate von Willebrand disease, desmopressin (DDAVP) is the first-line agent. It stimulates the release of stored von Willebrand factor and factor VIII from endothelial cells, typically raising levels within 30 to 60 minutes.

A test dose of DDAVP is often administered prior to the scheduled surgery to confirm the patient's response. The peak effect lasts for approximately 6 to 12 hours. For major procedures or severe subtypes, replacement therapy with vWF/FVIII concentrates is required instead.

CautionNever administer aspirin or other antiplatelet agents to patients with bleeding disorders, as this further impairs platelet function and can precipitate catastrophic hemorrhage. Local measures like ice are only adjuncts and do not replace systemic hemostatic therapy.

## 핵심 개념

- **von Willebrand disease (VWD)** — The most common inherited bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor, leading to impaired platelet adhesion and prolonged bleeding.
- **Desmopressin (DDAVP)** — A synthetic analog of vasopressin that stimulates the release of stored von Willebrand factor and factor VIII from endothelial cells, used to prevent or treat bleeding in mild to moderate VWD.
- **von Willebrand factor (vWF)** — A plasma glycoprotein that mediates platelet adhesion to the subendothelium at sites of vascular injury and serves as a carrier protein for factor VIII.
- **Platelet plug formation** — The initial phase of hemostasis where platelets adhere, activate, and aggregate at the site of vessel injury to form a temporary seal, a process critically dependent on vWF.
- **Cyclooxygenase (COX) pathway** — An enzymatic pathway in platelets that produces thromboxane A2; aspirin irreversibly inhibits this pathway, blocking platelet aggregation.

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