Hemophilia Flashcards
What causes hemoiphilia A and B?
Common thing in their history:
both are x lined recessive disorders:
- A, deficiency of factor 8
- B, deficiency of factor 9
Common thing in history: bleeding into joints, can also happen with CNS
what do hemophiliacs have that can neutralize infused clotting factors?
IgG Abs
Which comorbidities are you expecting hemophiliacs to have?
HIV, hep c, HTN, chronic pain
DDX for prolonged aPTT?
Hemophilia, vWF, lupus anticoagulants, or administered meds (heparin and activated protein C)
Do not perform elective surgical procedures in hemophilia if what?
if abnormal coagulation studies
What do you want to ask hemophilac?
Hx of bleeding, hematomas, hemarthroses, major bleeding with surgery?
Comorbidities-Hep C, chronic pain (meds for that)
Treatment of hemophilia-what are they taking for that?
Physical exam of hemophiliac?
bleeding stuff-gums, joints
what labs do you want for Hemophilia a and b? what would they say?
A: normal PT, normal bleedign time, prolonged PTT; assay for factor 8 coagulant activity
B: normal PT, normal bleeding time, prolonged aPTT
CBC
Do you have to give hemophiliacs anything before surgery/How can you optimize these pts? Hemophilia A
hemophilia A: Greater than or equal to 30% factor activity for hemostasis, but for major surgery-100% factor activity before surgery
For hemophilia-DDAVP is first line tx for pre-surgery. Active bleeding-cryo (FFP-kim that there is a large volum required)
Hemophiliia B:
recombinant factor 9 concentrates
What to avoid in Hemophiliacs?
Avoid aspirin and NSAIDS; avoid IM injections
Options for anesthesia in hemophilia?
GA, MAC, or local as required
REgional or PNB-usually avoided-require factor replacement and close monitoring of factor levels
Who would you consult for hemophiliacs?
Hematology
induction/maintenance/emergence in hemophiliacs
care with intubation, avoid nasal intubation if possible-careful positioning, routine emergence
