Chapter 32: Von Willebrand Disease (Children)

Question 1

Von Willebrand’s Disease (vWD)

Answer 1

characterized by a deficiency of von Willebrand factor (vWF)

Question 2

How is it transmitted?

Answer 2

via an autosomal dominant pattern of inheritance

-affecting males and females equally

Question 3

von Willebrand Factor

Answer 3

plays an important role in the early phase of hemostasis by enhancing platelet aggregation and adhesion
-the vWF assist in forming the “platelet plug” to the damaged endothelium and also acts as a carrier protein for coagulation factor VIII

Question 4

Signs and Symptoms

Answer 4

When there is a deficiency of vWF, s/s of prolonged bleeding times may include:

• epistaxis (nosebleed)
• menorrhagia (heavy menstrual flow)
• bleeding from the oral cavity
• easy bruising
• excessive bleeding following minor surgery or dental extraction

Question 5

Diagnosis

Answer 5

diagnosed with vWD when excessive bleeding is present with a simple tooth loss or a minor procedure such as a circumcision

• family hx; reveals similar bleeding manifestations in other family members b/c vWD is an inherited bleeding disorder
• complete physical examination to detect clinical abnormalities for bleeding tendencies such as multiple sites of bruising
• for moderate to severe bleeding: blood samples for PT, PTT, fibrinogen, thrombin time, platelet function assay, CBC, vWF, and vWF antigen

Question 6

Prevention

Answer 6

vWD is a genetically transmitted blood disorder, therefore, the primary strategy= genetic counseling to reduce the transmission of the disease

• teaching priority to parents= controlling and preventing bleeding episodes
• instructed on how to administer desmopressin (DDVAP), administered intranasally; call provider if have an upper respiratory infection such as runny nose or congestion which can decrease the effectiveness of DDVAP
• alert health-care provider if bleeding episodes are not controlled or the patient complains of headaches

Question 7

Desmopressin (DDVAP)

Answer 7

synthetic analog of the antidiuretic hormone vasopressin
>increases the plasma vWF factor and factor VIII by releasing vWF from its endothelial cell storage to produce an immediate increase in plasma levels
-improves platelet function and shortens the bleeding time
-administered intranasally
-contact health-care provider if have a upper respiratory infection such as runny nose or congestion; this can decrease the effectiveness of the intranasal DDVAP
-reduce fluid intake to prevent overhydration and possible hyponatremia
-most effective for mild episodes of bleeding

Question 8

Nursing Care

Answer 8

for the child identified with a bleeding disorder such as hemophilia or vWD, recommend a medical alert bracelet to alert health-care providers and others about the condition

Question 9

Medical Care

Answer 9

tx includes administration of DDVAP, a synthetic analog of the antidiuretic hormone vasopressin
-IV administration of Humate-P and/or the administration of cryoprecipitate or fresh frozen plasma

Question 10

Education/Discharge

Answer 10

• instructing about common sites of bleeding such as the nose, gums, and internal bleeding
• education focus on controlling the bleeding by applying pressure, applying ice, and seeking medical attention
• educate adolescent females on what constitutes excessively heavy menses; include tips to avoid an embarrassing moment during periods of heavy menstrual flow (wearing two maxi pads, no light colored pants or skirts)
• small children instructed to avoid nose picking, vigorous nose blowing, and strenuous activity that may cause a nosebleed
• sneeze with their mouth open and gently blow the nose if needed
• avoid the use of acetylsalicylic acid (Children’s Aspirin) or NSAIDs, which may promote bleeding episodes
• children prone to epistaxis (nosebleeds) can use cool mist humidification
• gentle flossing and usage of a soft bristle tooth-brush