1. The Hemophilia Federation of America (HFA) is a national nonprofit organization that assists and advocates for the bleeding disorders community. MISSION [Your Name] [Date] First Responders & von Willebrand Disease © 2015 Hemophilia Federation of America. All rights reserved. Session Titles, Descriptions & Content are copyrighted material belonging to the Hemophilia Federation of America.

2. Objectives Understand the basics of von Willebrand Disease and characteristics of a bleed Understand what to do in case of an emergency with a person living with von Willebrand Disease

3. Definition of Bleeding Disorders A group of hereditary bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of blood.

4. Did you know? von Willebrands Disease is the most common type of bleeding disorder, affecting an estimated 1% of the world's population.

5. von Willebrand Factor (VWF)  VWF is the protein that makes platelets stick together to form a platelet plug  VWF is a carrier protein for factor VIII.  VWF ensures that there is enough factor VIII in the blood stream, and carries it to the site of injury, and protects it from being broken down in the blood stream.

6. Types of von Willebrand Disease  Type 1 – von Willebrand levels are lower than normal  Type 2 – there is a defect in the structure of the von Willebrand protein that causes lower than normal VW factor protein activity  Type 3 – there is very little or no von Willebrand protein produced at all {Insert patient’s name} has von Willebrand Disease Type ___

7. People with von Willebrand Disease bleed longer, not faster.

8. {Insert patient’s name} has ___ vWD Degrees of Severity Type of vWDSeverity Type ISymptoms are usually mild Type IISymptoms are usually moderate Type IIISymptoms are usually severe

9. Symptoms of von Willebrand Disease  Nose bleeds  Prolonged bleeding from minor cuts  Heavy of longer than usual menstrual bleeding  Blood in the urine  Blood in the stools  Large bruises  Hematomas  Gums bleed easily  Heavy periods and/or periods lasting more than 7 days

10. Types of Bleeds  Joint bleeding  Muscle hemorrhage  Soft tissue - bruising  Life threatening bleeding  Others - mouth, nose, scrapes, minor cuts

11. .

12. Photo Credit: National Hemophilia Foundation Joint Bleeding

13. Muscle Bleeding  Signs and symptoms similar to joint bleeding  can include tightness  shininess of skin  is very painful  usually happens in the arms and legs  swelling  Significant blood loss can happen quickly

14. Muscle Bleeding Someone with a bleed can feel a muscle bleed LONG before anyone sees any outward symptoms.

15.  Primarily characterized by bruising and hematomas (raised bruises)  Many with von Willebrand Disease have bruises all over their bodies  Treatment is generally not needed, ice can help with comfort Soft Tissue Bleeding Graphic Credit : Wilkes family

16. Life-Threatening Bleeding  Head/Intracranial  Nausea, vomiting, headache, drowsiness, confusion, visual changes, loss of consciousness  Neck and Throat  Pain, swelling, difficulty breathing/swallowing  Abdominal/GI  Pain, tenderness, swelling, blood in the stools Iliopsoas Muscle  Back pain, thigh tingling/numbness, decreased hip range of motion

17. Other Bleeding Episodes Mouth bleeding  Looks like more than it is, as it is mixed with saliva  Child may vomit  Feces may be black (from swallowed blood) Nose bleeding  Sit up, pinch bridge of nose, cool pack on back of neck  If longer than 20 minutes, a trip to ER may be required Scrapes and/or minor cuts  Wash, pressure, dressing  Call parents if bleeding persists

18. Treatment of von Willebrand Disease  Early and appropriate treatment of each bleeding episode is critical to minimize complications  Treatment may vary depending on the type and severity of the disorder, as well as response to previous therapy and other medications the person may be taking

19. Treatment Administration  In {insert patient’s name}, is administered {insert procedure}  Treatment is kept {insert location in your home} and should accompany {insert patient’s name} if transported to the hospital

20. In an Emergency Setting  Notify the appropriate medical staff locally  Tell your local first responders to flag home and address as a precaution  Create an emergency plan and complete any necessary documentation

21. Things to Know  Some older bruises may become lumpy or hard  Patient may have excessive bruising  Medication is kept in {insert where medication is located in your home}

22. Meet {Insert Your/Child’s Name}  Age  LIST DIAGNOSIS i.e. Has Type II vWD  Explain how treatment is given  Developmentally on target

23. Summary  von Willebrand patients know his/her own body and are experts about their condition.  Have an emergency plan in place for those with bleeding disorders.  Be proactive!

24. © 2015 Hemophilia Federation of America. All rights reserved. Session Titles, Descriptions & Content are copyrighted material belonging to the Hemophilia Federation of America.

25. Thank You! Contact us: hfaprograms@hemophiliafed.org info@hemophiliafed.org