Hemophilia + von Willebrand Disease Flashcards
What are some characteristics of Hemophilia A?
- Also known as ‘Classic hemophilia’
- Deficiency of factor VIII
- Accounts for 80% of cases of hemophilia
- Occurrence: 1 in 5000
What are characteristics of hemophilia B?
- Also known as ‘Christmas disease’
- Caused by deficiency of factor IX
- Accounts for 15% of cases of hemophilia
- Occurrence: 1 in 50,000
What causes Hemophilia A?
- X-linked recessive trait (mom will be a carrier and will pass on to son)
- Males are affected
- Females may be carriers
- Degree of bleeding depends on amount of clotting factor and severity of a given injury (Table 35-6, p 1443)
What are the different degrees of severity of Hemophilia A?
Clinical Severity, graded by percentage of Factor VIII (100% is average for unaffected children)
Severe =
Moderate = 1%-5%, bleeding with trauma
Mild = >5%-40%, bleeding with severe trauma or surgery
What are the manifestations of hemophilia?
- Bleeding tendencies range from mild to severe
-
Symptoms may not occur until 6 months of age
- Mobility leads to injuries from falls and accidents
- Injuries really allow this to manifest
-
Hemarthrosis
- Bleeding into joint spaces of knee, ankle, elbow leading to impaired mobility
- Ecchymosis
- Bleed longer not faster
- Epistaxis
- Bleeding after procedures
- Minor trauma, tooth extraction, minor surgeries
- Large subcutaneous and intramuscular hemorrhages may occur
- Bleeding into neck, chest, mouth may compromise airway
How would you diagnose a child with hemophillia?
- Can be diagnosed through amniocentesis
- Genetic testing of family members to identify carriers
- Diagnosis on basis of hx, labs, and exam
- Labs: low levels of factor VIII or IX, prolonged PTT
- Normal: platelet count, PT, and fibrinogen
How would you treat a child with hemophilia?
- DDAVP (it is a vasopressive, promotes release of factor 8)
- IV
- Causes 2 to 4 times’ increase in factor VIII activity
- Used for mild hemophilia
- Replace missing clotting factors
-
Transfusions
- At home with prompt intervention to reduce complications
- Following major or minor hemorrhages
What are some interventions for children with hemophilia?
- Close supervision and safe environment – make sure they have soft toys to play with. Give them helmet to protect from falls
- Dental procedures in controlled situation – transfuse with colleting factors before.
- Shave only with electric razor
- Superficial bleeding—apply pressure for at least 15 minutes + ice to vasoconstrict
- If significant bleeding occurs, transfuse for factor replacement
How would you manage hemarthrosis with hemophilic children?
- During bleeding episodes, elevate and immobilize joint
- Ice
- Analgesics – swelling is painful!
- ROM after bleeding stops to prevent contractures
- PT
- Avoid obesity to minimize joint stress
Rest
Ice
Compression
Elevation
RICE
How would a nurse manage a patient with hemophilia?
- Prevent bleeding
- Recognize and control bleeding
- Prevent crippling effects of bleeding
- Support family and prepare for home care
- Can give medications sub q vs IM to prevent bleeding in the muscles
- Teach them good hygiene
What is Von Willebrand Disease?
- A hereditary bleeding disorder, involving deficiency of von Willebrand factor (a plasma protein, and the carrier for factor VIII)
- von Willebrand factor is necessary for platelet adhesion
- Transmitted as autosomal dominant trait
- One person has the trait, and will always pass on to\offspring
- Occurs in males and females
What are the Manifestations of von Willebrand Disease?
- Easy bruising
- Epistaxis MOST COMMON
- Gingival bleeding
- Excessive bleeding with lacerations or surgeries
- Menorrhagia (period is heavy)
What are some lab findings associated with von willebrand disease?
- Decreased von Willebrand factor levels
- von Willebrand antigen levels
- Decreased platelet agglutination
- Prolonged bleeding time
- PTT may be normal or prolonged
How would you treat von willebrand disease?
- Infusion of von Willebrand’s protein concentrate
- DDAVP infusion before surgery or to treat bleeding episode
What are some interventions for von willebrand disease?
- Avoid aspirin or NSAIDs (increase bleeding time and inhibit platelet function) No advil or asprin
- Manage bleeding episodes with prompt infusion therapy
- Children with von Willebrand have normal life expectancy if well managed
-
Educate families and children
- Nosebleeds – teach them to apply pressure at bridge of nose and lean forward. The reason is so that they won’t swallow blood.
- Mennorhagia – Teach adolescent girl to make it less expensive
