Von Willebrand Disease

Question 1

Pathophysiology of Von Willebrand Disease

Answer 1

Genetically transmitted bleeding disorder (deficiency in von Willebrand factor)

Von Willebrand serves two functions:
1. to find with factor VIII, protecting it from breaking down
2. Serves as a glue that attaches platelets to the site of injury

Results in a mild bleeding disorder

Question 2

Risk Factors for Von Willebrand Disease

Answer 2

Family history of the disease

Question 3

Signs and Symptoms of Von Willebrand Disease

Answer 3

1. Ecchymosis
2. Hematomas
3. Mucosal Bleeding
4. Longer bleeding time from cuts/surgical sites
5. Menorrhagia

Question 4

Nursing Assessment: Health History

Answer 4

1. Determine the nature of the bleeding episode or bruise
2. Include any hemorrhagic episodes in other systems, such as GI (black, tarry stools, hematemesis) or as a result in joint hemorrhage or hematuria
3. Inquire about the length of bleeding and amount of blood loss

Question 5

Nursing Assessment: Physical Examination

Answer 5

1. Assess circulation (heart sounds, pulses if severe prolonged bleeding occurs)
2. Note chest pain or abdominal pain

Without intervention, hypovolemia could follow, leading to shock

Question 6

Lab and Diagnostics

Answer 6

1. Decrease hemoglobin and hematocrit if bleeding is prolonged or severe
2. Factor levels may be quantified with blood testing
3. PT normal
4. PTT normal or prolonged
5. Platelet count normal or slightly low

Question 7

Complications of Von Willebrand Disease

Answer 7

Hemorrhage

Question 8

Preventing Bleeding in the Child

Answer 8

1. Protect toddlers with soft helmets, padding of the knees, carpet in the home, and softened or covered corners
2. Children should stay active: swimming, baseball, basketball and bicycling (wearing a helmet) are good physical activities
3. Avoid intense contact sports such as football, wrestling, soccer and high diving
4. Avoid trampoline use and riding ATVs
5. Arrange premedication with Amicar if oral surgery is indicated

Question 9

Nursing Management: Preventing Bleeding Episodes

Answer 9

1. Pre-medication prior to procedures

Question 10

Nursing Management: Managing a Bleeding Episode

Answer 10

1. Administer Desmopressin nasal spray, via IV infusion or subcutaneously (less common)
2. Desmopressin is an antidiuretic hormone, so closely monitor fluid balance
3. 24 hours should relapse between doses, as lessening of the response (tachyphylaxis)
4. Can be treated with infusion of vWF

Question 11

Nursing Assessment: Providing Education

Answer 11

1. Child should wear medical alert bracelet
2. Notify school nurse and teachers of child’s diagnosis
3. Instruct all school personnel to call parents immediately if the child sustains a head, abdominal or orbit injury at school
4. Teach parents and caregiver how to administer Desmopressin or vWF
5. Teach the family access, care and flushing of the implanted port