Treatment of Hemophilia



- 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%)



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, August 22, 2012 12:37 pm