Pediatric Von Willebrand Disease Follow-up

  • Author: John D Geil, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: May 3, 2010
 

Inpatient & Outpatient Medications

The following may be used in patients with von Willebrand disease (VWD):

  • Epsilon amino caproic acid (Amicar)
    • Inhibition of fibrinolysis
    • Useful in mucous membrane bleeding
    • Dose: 100 mg/kg/dose orally every 4-6 hours
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Deterrence/Prevention

Avoid medications with known antiplatelet effects. Although aspirin is rarely taken by children, over-the-counter compounds containing acetylsalicylic acid often are used by adolescents. Ibuprofen and other nonsteroidal anti-inflammatory drugs (NSAIDs) are reversible cyclooxygenase inhibitors and may cause intestinal bleeding. The risks of these and other medicines with antiplatelet effects should be considered in light of the severity of the von Willebrand disease. Provide patients with von Willebrand disease a list of prescription and nonprescription medications to avoid. This list should include the following:

  • Over-the-counter medications
    • Aspirin
    • Ibuprofen
    • Naproxen
    • Antihistamines
    • Ethanol
  • Antiplatelet agents
    • Dipyridamole
    • Ticlopidine
    • Prescription nonsteroidal anti-inflammatory compounds
  • Antimicrobials
    • High-dose penicillins
    • Cephalosporins
    • Nitrofurantoin
    • Hydroxychloroquine
  • Cardiovascular medications
    • Propranolol
    • Furosemide
    • Calcium channel blockers
    • Quinidine
  • Others
    • Caffeine
    • Tricyclic antidepressants
    • Phenothiazines
    • Valproate
    • Heparin
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Prognosis

Individuals with von Willebrand disease have a lifelong tendency toward easy bruising, frequent epistaxis, and menorrhagia.

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Patient Education

  • Avoid medications with antiplatelet activity.
  • Mild activity restrictions may be necessary.
  • For excellent patient education resources, visit eMedicine's Skin, Hair, and Nails Center. Also, see eMedicine's patient education article Bruises.
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Contributor Information and Disclosures
Author

John D Geil, MD  Associate Professor of Pediatrics, Division of Hematology/Oncology, University of Kentucky College of Medicine; Consulting Staff, Department of Pediatric Hematology/Oncology, University of Kentucky Children's Hospital

John D Geil, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

J Martin Johnston, MD  Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Pediatric Hematology/Oncology, Backus Children's Hospital; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Bowman M, Hopman WM, Rapson D, Lillicrap D, Silva M, James P. A prospective evaluation of the prevalence of symptomatic von Willebrand disease (VWD) in a pediatric primary care population. Pediatr Blood Cancer. Mar 8 2010;[Medline].

  2. Tosetto A, Castaman G, Rodeghiero F. Evidence-based diagnosis of type 1 von Willebrand disease: a Bayes theorem approach. Blood. Apr 15 2008;111(8):3998-4003. [Medline].

  3. Rodriguez KD, Sun GH, Pike F, et al. Post-tonsillectomy bleeding in children with von Willebrand disease: a single-institution experience. Otolaryngol Head Neck Surg. May 2010;142(5):715-21. [Medline].

  4. [Guideline] Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia. Mar 2008;14(2):171-232. [Medline].

  5. [Guideline] Nichols WL, Rick ME, Ortel TL, et al. Clinical and laboratory diagnosis of von Willebrand disease: A synopsis of the 2008 NHLBI/NIH guidelines. Am J Hematol. Mar 16 2009;[Medline].

  6. Batlle J, Torea J, Rendal E, Fernandez MF. The problem of diagnosing von Willebrand's disease. J Intern Med Suppl. 1997;740:121-8. [Medline].

  7. Carcao MD, Blanchette VS, Dean JA, et al. The Platelet Function Analyzer (PFA-100): a novel in-vitro system for evaluation of primary haemostasis in children. Br J Haematol. Apr 1998;101(1):70-3. [Medline].

  8. Federici AB, Mannucci PM. Management of inherited von Willebrand disease in 2007. Ann Med. 2007;39(5):346-58. [Medline].

  9. [Guideline] Lee CA, Brettler DB. Guidelines for the diagnosis and management of von Willebrand disease. Haemophilia. 1997;3:1-25.

  10. Nichols WC, Ginsburg D. von Willebrand disease. Medicine (Baltimore). Jan 1997;76(1):1-20. [Medline].

  11. Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost. Oct 2006;4(10):2103-14. [Medline].

  12. Werner EJ. von Willebrand disease in children and adolescents. Pediatr Clin North Am. Jun 1996;43(3):683-707. [Medline].

  13. Zhang Z, Blomback M, Anvret M. Understanding von Willebrand's disease from gene defects to the patients. J Intern Med Suppl. 1997;740:115-9. [Medline].

 
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Structure and domains of von Willebrand factor.
 
 
 
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