Hemophilia

Hemophilia A/B
- Hemophilia A much more common than hemophilia B
- present with delayed bleeding
- severity: severe( spontaneous bleeding), moderate( occasional bleeding), mild
- inheritance: X linked recessive, 30% spontaneous

- Dx- often done early in life, can get PT, PTT, BT, platelet count if no Dx and bleeding

Hemathroses:
- ankles, elbow, knee
- 15-40x risk septic arthritis, arthrocentesis only if concern for septic joint or no improvement in 24 hr- Treat with major dose factor if obvious effusion (if not, just routine dose), RICE, crutches, follow up with hematology

Muscle bleeds: 
- Dx with US, CT, MRI
- Watch out for compartment syndrome (especially iliopsoas) or airway compromise 

CNS hemorrhage:
- Give factor prior to getting CT. 

Therapies:
- Factor 8 major dose: 50 U/kg, Routine dose 25 U/kg
- Factor 9 major dose: 80-100 U/kg, Routine dose 50 U/kg

     - talk to family and patient, use their factor if available, needs it's own IV, avoid IM injections
     - Factor must be reconstituted, 30 minute turnaround call pharmacy ahead of time

- DDAVP release Von willebrand ( 0.3 mcg/kg)

- cryo, antifibrinolytics, fibrin glue

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