eMedicine Specialties > Pediatrics: General Medicine > Hematology

Von Willebrand Disease: Follow-up

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
Contributor Information and Disclosures

Updated: May 21, 2009

Follow-up

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

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

Prognosis

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

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.

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize bleeding tendency prior to elective surgery
  • Failure to recognize associated symptoms (eg, menorrhagia)
  • Failure to inform patient and family of activity restrictions
  • Other concerns: Excessive bruising suggestive of child abuse frequently instigates an evaluation to rule out a coagulopathy. Borderline abnormal laboratory results may mislead an investigator/caregiver and prevent recognition of actual abuse. Because von Willebrand disease (VWD) is a common disorder, its coexistence with nonaccidental trauma needs to be considered if the evidence of abuse is strong. Alternatively, the medical professional must recognize that the caregivers of a child with von Willebrand disease may be falsely suspected of abuse, with obvious stressful ramifications.

Special Concerns

  • The dose of intranasal desmopressin used in VWD is about 15 times the dose used to treat patients with diabetes insipidus. A more concentrated nasal spray (1.5 mg/mL) is available for patients with VWD compared to the spray used for individuals with diabetes insipidus (0.1 mg/mL).
  • Restrict free-water intake to avoid hyponatremia, especially in very young or elderly patients.
  • May repeat desmopressin at intervals of 12-24-hours; however, depletion of tissue stores eventually develops.
 


More on Von Willebrand Disease

Overview: Von Willebrand Disease
Differential Diagnoses & Workup: Von Willebrand Disease
Treatment & Medication: Von Willebrand Disease
Follow-up: Von Willebrand Disease
Multimedia: Von Willebrand Disease
References
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References

  1. 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].

  2. [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].

  3. [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].

  4. 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].

  5. 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].

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

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

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

  9. 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].

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

  11. 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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Further Reading

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Keywords

von Willebrand disease, VWD, von Willebrand factor, VWF, congenital bleeding disorder, intramuscular bleeding, hemarthrosis, bruising, menorrhagia, wound bleeding, excessive bleeding, Wilms tumor, congenital heart disease, systemic lupus erythematosus, angiodysplasia, hypothyroidism, treatment, diagnosis

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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 and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Medical Editor

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 Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
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.

 
 
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