Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
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Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
© 2010 Pharmaceutical Education Consultants, Inc. unless otherwise noted. All rights reserved.
Reproduction in whole or in part without permission is prohibited.
Page 1
Kristen Jaworski, RN, BSN, CCRC
Hemophilia Center of Western PA
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
Accreditation Pharmacists: 0798-0000-12-046-L01-P Nurses: N-771
Faculty
Kristen Jaworski RN, BSN, CCRC Hemophilia Center of Western PA
CE Credit(s) 1.0 contact hour(s)
Faculty Disclosure Dr. Jaworski has no actual or potential conflicts of interest in relation to this program.
Learning Objectives •Define the epidemiology of Von Willebrand Disease •Describe the pathophysiology, including types, of Von Willebrand Disease •Provide an overview of the tiered treatment regimen in type I Von Willebrand Disease
Legal Disclaimer The material presented here does not necessarily reflect the views of Pharmaceutical Education Consultants (PharmCon) or the companies that support educational programming. A qualified healthcare professional should always be consulted before using any therapeutic product discussed. Participants should verify all information and data before treating patients or employing any therapies described in this educational activity.
Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• Lack of function of a blood protein called von
Willebrand factor
• Von Willebrand factor works with factor VIII to
create blood clots
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• Injury
• Vessel constricts
• Endothelial cells
• VW binds to collagen at injury site
• Platelet rush to injury
• VW works as the glue
Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
© 2010 Pharmaceutical Education Consultants, Inc. unless otherwise noted. All rights reserved.
Reproduction in whole or in part without permission is prohibited.
Page 2
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
Available from: http://medlibes.com/entry/von-willebrands-disease [accessed May 2012]
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• Inherited bleeding disorder
• Occurs in 1 in every 100 people.
• 1-2% of general population
• Equal in males and females
Treatment of von Willebrand's Disease Pier Mannuccio Mannucci, M.D. N Engl J Med 2004; 351:683-694
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• Genetic database in early stages
• Inherited as a dominated pattern
• Phenotype vs. Genotype
• Family history
• Laboratory testing
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
Available from: http://bleedingdisorder.files.wordpress.com/2010/01/vw
d-generations-chart.jpg [accessed May 2012]
Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
© 2010 Pharmaceutical Education Consultants, Inc. unless otherwise noted. All rights reserved.
Reproduction in whole or in part without permission is prohibited.
Page 3
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• Nosebleeds
• Easy bruising
• Heavy menstrual cycle
• Gastro-intestinal bleeding
• Bleeding after surgery and
childbirth
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• Most common type
• Quantitative (partial) deficiency
of Von Willebrand Protein
• Reduced VWF in circulation
• Treatment Desmopressin
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• Least common type
• Qualitative deficiency of Von Willebrand Protein
• Abnormal binding sites
– Platelets
– Factor VIII
• Treatment Factor Concentrate
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• Rare – 1 in a million
• Quantitative (complete) deficiency of
Von Willebrand Protein
• Treatment Factor Concentrate –
blood plasma product
Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
© 2010 Pharmaceutical Education Consultants, Inc. unless otherwise noted. All rights reserved.
Reproduction in whole or in part without permission is prohibited.
Page 4
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• DDAVP
• Stimate
• Factor concentrate
– Humate P
– Alphanate
– Wilate
– Koate DVI
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• Synthetic vasopressin
• Method of action -
– release of stores from endothelial cells raising factor VIII and vWD serum levels
• Administration -
– Intravenous
– Subcutaneously
–Nasally (Stimate)
• Side effects
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• serious symptoms of a low level of sodium in the blood:
– loss of appetite
– severe nausea
– vomiting
– severe headache
– muscle weakness
– unusual severe drowsiness
– mental/mood changes
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• How supplied
– 1.5 mg./ ml
– 2.5 ml bottle - delivers 25 doses of 150 mcg.
• Dosing
– Every 24-48 hours prn
– <50 kg. body weight - 1 spray (150 mcg.) >50 kg. body weight - 2 sprays (300 mcg.)
Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
© 2010 Pharmaceutical Education Consultants, Inc. unless otherwise noted. All rights reserved.
Reproduction in whole or in part without permission is prohibited.
Page 5
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• DDAVP
– Maximum of 3 consecutive days administration
– Side effects common
– Priming the nasal pump
– Fluid restriction
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• Plasma-derived VWF-containing FVIII concentrates.
– Humate-P®
– Alphanate®
– Wilate®
– Koate DVI®
• Off label use is accepted
Pier M. Mannucci. Blood January 15, 2002 vol. 99 no. 2 450-456
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• Dose 20-80 units/kg • calculated based on the FVIII content of the products
• OR
• dosing based on VWF functional activity [e.g., VWF ristocetin cofactor activity (VWF:RCo)]
• Half life 8-12 hours
• IV push
Batlle, Javier. Blood Coagulation & Fibrinolysis: March 2009 - Volume 20 - Issue 2 - p 89-100
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Median values of plasma levels.VWF:RCof (circles, dash-dot lines) and FVIII:C (triangles, solid lines) (A) and of bleeding times (squares, short dash lines) (B) before and after administration of A-SD (open symbols) and A-SD/HT (closed symbols), 60 IU/kg VW...
Mannucci P M et al. Blood 2002;99:450-456 ©2002 by American Society of Hematology
Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder
© 2010 Pharmaceutical Education Consultants, Inc. unless otherwise noted. All rights reserved.
Reproduction in whole or in part without permission is prohibited.
Page 6
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• Oral contraceptives
• Antifibrinolytics
• Hormonal creams
• In the pipeline…
– IL-11 Neumega
– Recombinant VW factor
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
• VW is a common bleeding disorder
• Symptoms are common within family groups so underreported
• Symptoms managed as an outpatient
• Post operative bleeding results in hematology referral and diagnosis
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
PharmCon is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education
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