- Discussion:
- replacement of missing clotting factor remains the essential method of treatment;
- many hemorrhages are treated w/ home infusions;
- this allows bleeding to be controlled earlier;
- major hemorrhagic emergencies are reduced;
- Activity of Tranfused Factor VIII:
- administration of one unit of factor VIII / kg body wt will raise level by 2%;
- Prophylaxis for Elective Surgery:
- factor VIII levels be maintained at 100-130% of nl for first 2-3 days after surgery;
- levels < 80% are not acceptable;
- after 3 days, levels are maintained at 50-60% for first 14 days;
- for six wks pts are maintained between 30-50% of nl, depending on their physical therapy and response to treatment;
- contraindications to surgery:
- pts known to have inhibitors to factor VII should not undergo elective surgery;
- Prophylaxis for:
- acutes hemarthrosis: 30- 40%
- fracture: 30- 40% for 2-4 days upto 1 wk (100% w/ ORIF)
- compartment syndrome: 40% (100% if surgery is needed);
- seriour muscle bleeds: 40- 50%
- serious neural bleed: 80-100%
- surgery: 100%
- Elbow:
- w/ moderate destructive changes of hemophilic arthropathy, including marked hypertrophy of the radial head;
- radial head resection w/ synovectomy:
- indicated even in presence of advanced disease;
- will maintain joint function and will reduce frequency of hemarthrosis;
- Hip Joint:
- there is a high rate of loosening of cemented femoral components (35% over 8 yrs);
- higher rate of infection when there is concomitant HIV infection (11%)