A child is admitted to the hospital with a suspected diagnosis of von Willebrand's. On assessment of the child, which symptom would most likely be noted?

Appeared in: DHS Staff Nurse - 2018 (Shift-2nd)

Explanation

Von Willebrand disease (vWD) is caused by a deficiency or dysfunction of von Willebrand factor (vWF), a protein essential for platelet adhesion.
This defect in primary hemostasis (platelet plug formation) characteristically leads to mucocutaneous bleeding.
Common manifestations include epistaxis (nosebleeds), gingival bleeding, easy bruising, and menorrhagia, all of which involve mucous membranes or skin.
Unlike hemophilia, deep joint and muscle bleeding is not the typical presentation of vWD.

Option A: Hematuria (blood in urine) can occur in bleeding disorders, but it is not the most common or characteristic symptom of von Willebrand's disease compared to mucous membrane bleeding.
Option B: While easy bruising (ecchymosis) is common in vWD, large, deep hematomas (collections of blood in muscle) are more characteristic of coagulation factor deficiencies like hemophilia.
Option C: Hemarthrosis, or bleeding into the joints, is the hallmark symptom of severe hemophilia (Factor VIII or IX deficiency). It is rare in most types of von Willebrand's disease.

Clinical Relevance

Nursing practice connection: Use the key finding related to Clinical presentation of von Willebrand's disease to guide bedside assessment, documentation, and the next nursing action.
A key nursing role is patient and family education, especially regarding the avoidance of aspirin and NSAIDs, which can significantly increase bleeding risk in patients with vWD.
Nurses should conduct a thorough bleeding history assessment, using specific questions about nosebleeds, gum bleeding, and prolonged bleeding after minor procedures (like dental work) to help identify potential bleeding disorders.
What if? If a child with known vWD presents with severe abdominal pain and guarding, the nurse should consider the possibility of a gastrointestinal or retroperitoneal bleed, which is a medical emergency, even though it's less common than mucosal bleeding.

How to Approach the Question

First, identify the key diagnosis in the question: 'von Willebrand's disease'.
Recall the basic pathophysiology. Is this a problem with platelets or with clotting factors?
Remember that vWD is primarily a disorder of platelet adhesion (primary hemostasis).
Connect the pathophysiology to the clinical signs. Disorders of primary hemostasis typically cause skin and mucous membrane bleeding.
Evaluate the options: Hematuria, hematomas, and hemarthrosis are more associated with deeper bleeding or are less specific. Bleeding from mucous membranes directly matches the expected pattern for vWD.
Therefore, select the option describing mucocutaneous bleeding as the most likely symptom.

Concept Tested & Keywords

Concept Tested: Clinical presentation of von Willebrand's disease
Stem keywords: child, von Willebrand's, symptom
Lead-in keywords: most likely
Clinical cues: The diagnosis is von Willebrand's, which points to a disorder of primary hemostasis (platelet function).
Negative lead-in flag: false

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E6 Nursing Brunner Adult Health 3SA Vol 1 Part 3 p. 157-159

E6 Medicine Harrison 22e Part 1 p. 513-515

E6 Nelson Textbook of Pediatrics(2024) — Volume 2 p. 823-825

Practise the full DHS Staff Nurse - 2018 (Shift-2nd)

Attempt every question from this paper in a timed mock, then review the full solution for each one.