References:
- Wong’s Nursing Care of Infants and Children, 11th Edition, ISBN 978-0-323-54939-4, by Marilyn J. Hockenberry, David Wilson, and Cheryl C. Rodgers (Ch. 29, pp. 1361-1362)
Von Willebrand Disease (vWD) is a hereditary (autosomal dominant pattern) bleeding disorder characterized by a deficiency of or defect in a protein called von Willebrand factor (vWF). The vWF protein contributes to the adherence of platelets to damaged endothelium and serves as a carrier protein for factor VIII. Most cases of vWD are mild and require intervention only for dental and surgical procedures.
- Clinical Manifestations: this results in prolonged bleeding time because platelets fail to adhere to the walls of the ruptured vessel to form a platelet plug. The most characteristic clinical feature of vWD is an increased tendency toward bleeding from mucous membranes. The most common symptom is frequent nosebleeds, followed by gingival bleeding, easy bruising, and excessive menstrual bleeding (menorrhagia) in females.
- Diagnostic Evaluation: the platelet function analyzer (PFA 100) is a rapid, accurate detection of platelet dysfunction and vWD used by many centers, but its results may be affected by such conditions (e.g., sepsis, pregnancy, certain medications) and therefore requires the need for further platelet aggregation testing.
- Similar to hemophilia, vWD is treated with DDAVP and/or a specially concentrated clotting factor (Humate-P).
Nursing Care Management
- Nosebleeds are a special concern due to their frequency and appearance. They are a frightening experience for both the child and parents.
- Have child sit up and lean forward (not lie down).
- Apply continuous pressure to nose with thumb and forefinger for at least 10 minutes. Most of the nosebleeding originates in the anterior part of the nasal septum. During this time, the child breathes through the mouth.
- Insert cotton or wadded tissue into each nostril and apply ice or cold cloth to bridge of nose if bleeding persists.
- Keep child calm and quiet.
- If local measures are not successful, DDAVP is used to treat mild and moderate vWD as they increase vWF and factor VIII secretion from storage in the endothelial cells.
- Menorrhagia may warrant factor replacement or DDAVP therapy on the first day of the menstrual cycle to lessen flow. Additionally, methods to prevent accidents during menstruation (e.g., double sanitary pads), helps the adolescent adjust to the inconvenience.