In hemophilia A patients with suspected severe or life-threatening bleeding it is important to "Treat first, evaluate second, plan further therapy third"
For potentially serious or life-threatening bleeding, give factor before imaging or other evaluations.
Initial examination findings may be subtle or absent; treat based on the medical history.
If a diagnostic procedure is required (lumbar puncture, arterial blood gas, arthrocentesis), give factor to raise the level to 100% before performing the procedure.
If the patient requires transfer to another facility, give factor before (or during) transport.
For severe disease, assume the factor level is 0%.
Do not waste factor (administer excess rather than discarding).
Use an indwelling central catheter to administer factor if present. If not, the most experienced individuals should perform venipunctures and place an intravenous line if needed. Traumatic venipunctures can cause painful hematomas that limit intravenous access.
The treatment of choice in hemophilia A patients is the patient's own factor VIII product or recombinant human factor VIII. If neither is available, give plasma-derived factor VIII.
For severe bleeding in patients without an inhibitor, give factor VIII at 40 to 50 units/kg as soon as possible to produce a factor VIII level of 80 to 100%.
For less-severe joint or muscle bleeding, a target factor VIII level of 40 to 50% may be used, by giving factor VIII at a dose of 20 to 25 units/kg.
For severe bleeding in patients with an inhibitor, a bypassing product may be indicated (rFVIIa or FEIBA).
For individuals with mild hemophilia A (baseline factor VIII 5 to 50%) who have a documented response to DDAVP and non-life-threatening (or non-limb-threatening) bleeding, DDAVP may be used.
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