Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder

Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder

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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.



• Lack of function of a blood protein called von

Willebrand factor

• Von Willebrand factor works with factor VIII to

create blood clots


• Injury

• Vessel constricts

• Endothelial cells

• VW binds to collagen at injury site

• Platelet rush to injury

• VW works as the glue




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Available from: http://medlibes.com/entry/von-willebrands-disease [accessed May 2012]


• 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


• Genetic database in early stages

• Inherited as a dominated pattern

• Phenotype vs. Genotype

• Family history

• Laboratory testing


Available from: http://bleedingdisorder.files.wordpress.com/2010/01/vw

d-generations-chart.jpg [accessed May 2012]




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• Nosebleeds

• Easy bruising

• Heavy menstrual cycle

• Gastro-intestinal bleeding

• Bleeding after surgery and

childbirth


• Most common type

• Quantitative (partial) deficiency

of Von Willebrand Protein

• Reduced VWF in circulation

• Treatment Desmopressin


• Least common type

• Qualitative deficiency of Von Willebrand Protein

• Abnormal binding sites

– Platelets

– Factor VIII

• Treatment Factor Concentrate


• Rare – 1 in a million

• Quantitative (complete) deficiency of

Von Willebrand Protein

• Treatment Factor Concentrate –

blood plasma product




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• DDAVP

• Stimate

• Factor concentrate

– Humate P

– Alphanate

– Wilate

– Koate DVI


• 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


• 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


• 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.)




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• DDAVP

– Maximum of 3 consecutive days administration

– Side effects common

– Priming the nasal pump

– Fluid restriction


• 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


• 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


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




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• Oral contraceptives

• Antifibrinolytics

• Hormonal creams

• In the pipeline…

– IL-11 Neumega

– Recombinant VW factor


• 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



Documents

Von Willebrand Disease: Recognizing a Prevalent Bleeding Disorder