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

Patients should avoid medications with antiplatelet activity. Mild activity restrictions may be necessary.

For patient education information, see eMedicineHealth's Skin Conditions and Beauty Center, as well as the patient education article Bruises.

Questions

Flood VH, Abshire TC, Christopherson PA, et al. Von Willebrand disease in the United States: perspective from the Zimmerman program. Ann Blood. 2018 Jan. 3:[QxMD MEDLINE Link]. [Full Text].
Keesler DA, Flood VH. Current issues in diagnosis and treatment of von Willebrand disease. Res Pract Thromb Haemost. 2018 Jan. 2 (1):34-41. [QxMD MEDLINE Link]. [Full Text].
Schneppenheim R. The pathophysiology of von Willebrand disease: therapeutic implications. Thromb Res. 2011. 128 Suppl 1:S3-7. [QxMD MEDLINE Link].
Grabowski EF, Curran MA, Van Cott EM. Assessment of a cohort of primarily pediatric patients with a presumptive diagnosis of type 1 von Willebrand disease with a novel high shear rate, non-citrated blood flow device. Thromb Res. 2012 Apr. 129(4):e18-24. [QxMD MEDLINE Link].
Akin M. Laboratory diagnostic approach of the parents-children relationship in differentiating low-level von Willebrand factor from mild type 1 von Willebrand disease. Blood Coagul Fibrinolysis. 2012 Jun. 23(4):351-3. [QxMD MEDLINE Link].
Lindsay H, Bergstrom K, Srivaths L. Co-inheritance of mild hemophilia A and heterozygosity for type 2N von Willebrand disease: a diagnostic and therapeutic challenge. Pediatr Blood Cancer. 2014 Oct. 61(10):1888-90. [QxMD MEDLINE Link].
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. 2010 Mar 8. [QxMD MEDLINE Link].
Holm E, Osooli M, Steen Carlsson K, Berntorp E. Cardiovascular disease-related hospitalization and mortality among persons with von Willebrand disease: a nationwide register study in Sweden. Haemophilia. 2019 Jan. 25 (1):109-15. [QxMD MEDLINE Link]. [Full Text].
Tosetto A, Castaman G, Rodeghiero F. Evidence-based diagnosis of type 1 von Willebrand disease: a Bayes theorem approach. Blood. 2008 Apr 15. 111(8):3998-4003. [QxMD MEDLINE Link].
Sidonio RF Jr, Gunawardena S, Shaw PH, Ragni M. Predictors of von Willebrand disease in children. Pediatr Blood Cancer. 2012 May. 58(5):736-40. [QxMD MEDLINE Link].
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. 2010 May. 142(5):715-21. [QxMD MEDLINE Link].
Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD. Evaluation of the diagnostic utility for von Willebrand disease of a pediatric bleeding questionnaire. J Thromb Haemost. 2009 Aug. 7 (8):1418-21. [QxMD MEDLINE Link]. [Full Text].
Sanders YV, Fijnvandraat K, Boender J, et al. Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric-specific bleeding. Am J Hematol. 2015 Dec. 90 (12):1142-8. [QxMD MEDLINE Link].
Loeffelbein F, Funk D, Nakamura L, Zieger B, Grohmann J, Siepe M, et al. Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. Interact Cardiovasc Thorac Surg. 2014 Sep 16. [QxMD MEDLINE Link].
Sahebkar A, Serban C, Ursoniu S, et al. The impact of statin therapy on plasma levels of von Willebrand factor antigen. Systematic review and meta-analysis of randomised placebo-controlled trials. Thromb Haemost. 2016 Mar. 115 (3):520-32. [QxMD MEDLINE Link].
[Guideline] Committee on Adolescent Health Care, Committee on Gynecologic Practice. Committee opinion: von Willebrand disease in women. The American College of Obstetricians and Gynecologists. Available at https://www.acog.org/Clinical-Guidance-and-Publications/Committee-Opinions/Committee-on-Adolescent-Health-Care/Von-Willebrand-Disease-in-Women. Dec 2013; Accessed: Sep 14, 2018.
Jacobson AE, Vesely SK, Koch T, Campbell J, OʼBrien SH. Patterns of von Willebrand Disease Screening in Girls and Adolescents With Heavy Menstrual Bleeding. Obstet Gynecol. 2018 Jun. 131 (6):1121-9. [QxMD MEDLINE Link].
[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. 2008 Mar. 14(2):171-232. [QxMD MEDLINE Link].
[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. 2009 Mar 16. [QxMD MEDLINE Link].
[Guideline] James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12. 5 (1):280-300. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021 Jan 12. 5 (1):301-25. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Structure and domains of von Willebrand factor.

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Author

Suchitra S Acharya, MD, MBBS Associate Professor of Pediatrics and Pediatrics in Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell; Program Head, Bleeding Disorders and Thrombosis Program, Cohen Children's Medical Center of New York

Suchitra S Acharya, MD, MBBS is a member of the following medical societies: American Society of Hematology, Hemophilia and Thrombosis Research Society, International Society on Thrombosis and Haemostasis, World Federation of Hemophilia

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Novonordisk, Shire <br/>Received research grant from: Bayer Pharmaceuticals .

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

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; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; 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 Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Hassan M Yaish, MD Medical Director, Intermountain Hemophilia and Thrombophilia Treatment Center; Professor of Pediatrics, University of Utah School of Medicine; Director of Hematology, Pediatric Hematologist/Oncologist, Department of Pediatrics, Primary Children's Medical Center

Hassan M Yaish, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

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

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

Disclosure: Nothing to disclose.

Acknowledgements

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.