Von Willebrand Disease

Program Summary: Hemophilia and Related Bleeding Disorders

What is the Hemophilia and Related Bleeding Disorders Clinical Management program?

If you use first.dba Specialty Pharmacy for your factor product, you may be enrolled into the Hemophilia and Related Bleeding Disorders Clinical Management (CM) program. The goal of this program is to help you and/or caregivers manage hemophilia or other types of bleeding disorders, and to promote health and wellness through education. This program is provided to you at no cost.

What does the program involve?

  • You will get one-on-one consultations with a pharmacist or nurse who is trained in bleeding disorders. The pharmacist or nurse can:
    • Answer any questions you may have about hemophilia or another type of bleeding disorder and its treatments
    • Provide information to help you manage hemophilia or another type of bleeding disorder
    • Teach you how to manage any side effects
  • You will also receive education materials in the mail to help you to learn more about hemophilia and related bleeding disorders

A phone appointment will be scheduled when you receive a call to coordinate your medicine delivery. You may also call us at 1-855-855-8754 (TTY: 711) if you would like to schedule the phone appointment.You will also receive education materials in the mail to help you to learn more about hemophilia and related bleeding disorders.

How will this program benefit me?

In this program, you will:

  • Have access to knowledgeable pharmacists or nurses
  • Gain a better understanding of hemophilia or another type of bleeding disorder and the medicines used to treat it
  • Have the knowledge and necessary tools to take an active part in the management of your health
  • Receive information about other resources, such as support groups and financial assistance

Contact Us
If you have questions or would like more information, please call 1-855-855-8754 (TTY: 711), Monday through Friday, from 5 a.m. to 7 p.m. PST.

­ WHAT IS VON WILLEBRAND DISEASE?

Von Willebrand disease (VWD) is a bleeding disorder. In VWD, a blood protein called von Willebrand factor is either not working or there are low levels of it. VWD is the most common bleeding disorder. It takes longer for people with VWD to form a clot and stop bleeding.

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­ WHAT IS VON WILLEBRAND FACTOR?

The von Willebrand factorVWD is a glue-like protein needed to stop bleeding. When there is a bleed, the VWF sticks to platelets in the bloodstream to create a clot. This blood clot prevents bleeding. Factor VIII is another blood protein needed to stop a bleed. The VWF also carries factor VIII (eight) in the blood.

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­ WHO GETS VWD?

Both males and females may get VWD. VWD is an inherited disease. Parents may pass it down to their children.

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­ WHAT ARE THE DIFFERENT TYPES OF VWD?

Listed below are the 3 different types of VWD

Table 1. Types of VWD

Type 1
  • The most common, but mildest form of VWD
  • People with type 1 do not have enough VWF.
  • They may have lower than normal levels of factor VIII
Type 2
  • People with type 2 have enough VWF, but the VWF does not work  properly.
  • Bleeding tends to be more severe in type 2 than type 1.
Type 3
  • The least common but most severe form of VWD. 
  • People with type 3 VWD have no VWF. 
  • They also have low levels of factor VIII. 
  • Severe bleeding into the joints and muscles can occur.

 

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­ WHAT ARE THE COMMON SYMPTOMS OF VWD?

Common symptoms of VWD including the following:

  • Easy bruising
    • Often large in size (more than 2-3 inches)
    • Can occur without known injury
  • Many and long-lasting nosebleeds 
    • More than once or twice a month 
    • Does not stop within 10-15 minutes
  • Heavy or prolonged menstrual bleeding
    • Menstral pad or tampon needs to be changed more often than hourly
    • Lasts longer than 6 to 8 days
  • Long-lasting bleeding after surgery, dental work, childbirth or injury
    • Lasts more than a few hours
  • Blood in the urine or stool
    • From bleeding in the kidneys, bladder, intestines, or stomach

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­ WHAT ARE SOME GENERAL TIPS FOR ME OR MY CHILD WITH VWD?

  • Physical activity and exercise may help raise. VWF levels and strengthen muscles. Avoid certain activities like football or boxing. Talk to your doctor before doing any sports.
  • Avoid medications, vitamins, and herbs that prevent platelets from working, such as aspirin and ginko biloba.
  • Avoid using Non-Steroidal Anti-Inflammatory Drugs (NSAIDs), such as ibuprofen (Advil®, Motrin®) and naproxen (Aleve®). They may increase the risk of bleeds.
  • Be careful when using sharp objects such as knives or scissors. Avoid straight razors. Use safety razors or electric shavers instead.
  • Prevent nosebleeds by keeping your nostrils moist. Use petroleum jelly (e.g., Vaseline®) or vitamin E oil in your nostrils.
  • Get immunized against hepatitis A and hepatitis B.
  • Maintain good dental hygiene.
    • Talk to your doctor before all dental procedures.
    • Talk to your doctor about keeping some Amicar® (aminocaproic acid) at home. This medication helps preserve a blood clot once it forms in the mouth.
    • Tell the dentist that you or your child has VWD.
  • Keep a log of bleeding events
    • Include the name of the factor medication used, how much used, the lot number, and the expiration date.
    • Talk to your other.dba pharmacist for a log to help you keep track.

 

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­ HOW DO YOU TREAT VWD?

VWD is a lifelong condition with no cure. Recommended treatment is to prevent or stop a bleed. Listed below are the most commonly used treatments for VWD.

 

Table 2. Treatments Commonly Used for VWD.

Treatment

Description

Desmopressin (DDAVP®, Stimate®, Minirin®)

  • Given as an injection or nasal spray.
  • Used for most people with type 1 VWD and certain people with type 2 VWD.
  • Helps your body release more VWF and factor VIII into your bloodstream.

VWF and Factor VIII Replacement Therapy

  • Alphanate®
  • Humate-P ®
  • Wilate®

 

  • Given as an infusion into a vein in your arm.
  • Used if you:
    • Can’t take desmopressin or need longer treatment
    • Have type 1 VWD that doesn’t respond to desmopressin
    • Have type 2 or type 3 VWD
  • These products replace VWF and factor VIII in your body.

Oral Contraceptives

  • Taken by mouth.

  • Control heavy menstrual bleeding.

  • The hormones in birth control pills may boost levels of VWF and factor VIII activity.

Antifibrinolytics

  • Amicar® (aminocaproic acid)
  • Cyklokapron®, Lysteda® (Tranexamic acid)

 

  • Taken by mouth or injected into a vein.
  • Help stop bleeding after minor surgery or tooth extraction.
  • Help prevent blood clots from breaking down.

Fibrin Glue

  • Applied on the wound like glue to stop bleeding.

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­ CAN I GIVE VWF AND FACTOR VIII REPLACEMENT THERAPY AT HOME?

Some people give the products at home to themselves or to their children. You will need special instructions before using the medications at home. Contact your doctor for special instructions

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­ HOW DO YOU STORE THE MEDICATIONS?

Some medications may need to be refrigerated or stored in a certain way. Please read the medication guide that came with your medication to find out where to store it. Or you can contact your other.dba pharmacist if you have any questions about where to keep your medications.

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­ DO I NEED TO BE CONCERNED ABOUT GETTING INFECTIONS FROM FACTOR PRODUCTS?

The risk for viral infection has decreased because blood donors are checked. Donated blood products are tested and treated to destroy viruses. There is a smaller chance of getting an infection from recombinant factor products than plasma-derived products. Register for the Patient Notification System free of charge. This program tells people about recalls on any of the factor products.

 

                            Patient Notification System

                            Phone: 1.888.UPDATE.U (1.888.873.2838)

                            Web site: www.patientnotificationsystem.org 

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­ WHAT ARE HEMOPHILIA TREATMENT CENTERS (HTC)?

Hemophilia treatment centers (HTC) are resources for families and people with bleeding disorders. Medical experts at HTCs provide treatment, education and support. They teach people how to do home treatments and can also provide information to your provider. People who get care at HTCs are:

  • Less likely to have bleeding complications and hospitalizations.
  • More likely to have a better quality of life.

How often a person visits a HTC will vary. Some people are followed closely by their health care providers and only go to a HTC once or twice a year for check-ups, while others may go more often. For a listing of HTCs near you, please visit the Centers for Disease Control and Prevention Website (https://www2a.cdc.gov/ncbddd/htcweb/Dir_Report/Dir_Search.asp).

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­ WHERE CAN I FIND OUT MORE INFORMATION ABOUT VWD?

other.dba can give you more information about VWD. Some of the topics include:

  • Dental care
  •  First aid
  • Infusion log
  • Nosebleeds
  • Physical therapy
  • Prevention

For more information on VWD, please contact the following resources:

other.dba

Phone: 1.855.855.8754
Web site: www.optumrx.com[CMN1] 

National Heart Lung and Blood Institute
Phone: 1.301.592.8573
Web site: http://www.nhlbi.nih.gov/health/public/blood/index.htm[CMN2] 

National Hemophilia Foundation Information Resource Center
Phone: 1.800.42.HANDI
Web site: www.hemophilia.org[CMN3] 

National Institutes of Health Medline Plus
Web site: www.nlm.nih.gov/medlineplus[CMN4] 

 

 WHERE CAN GO TO GET AN INFUSION LOG? 

Please click here  to download an infusion log.

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References: 

  1. National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html. Accessed December 22, 2009.
  2. National Hemophilia Foundation Web site. Von Willebrand disease. http://www.hemophilia.org/NHFWeb/MainPgs/ Accessed December 22, 2009.
  3. Paper R, Kelley LA. A Guide to Living with von Willebrand Disease. Georgetown, MA: LA Kelley Communications, Inc.; 2003.
  4. Patient Notification System Web site. http://www.patientnotificationsystem.org/. Accessed September 1, 2010.

The information in this educational handout doesn’t substitute the medical advice, diagnosis, or treatment provided by your doctor. Always seek the help of your doctor or a qualified health provider for any questions about your medical condition.

 


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