Today’s Morning Report is presented by Dr. Kendall
HEMOPHILIA
Hemophilia A – Factor VIII deficiency
Hemophilia B – Factor IX deficiency
History:
Type and severity of factor deficiency?
Nature of the hemorrhage?
Previous treatments with blood products?
Presence of inhibitors, probable titer?
For hemophilia A, history of DDAVP use?
Severity of Illness (factor level)
Mild: > 5%
Moderate: 1-5%
Severe: <1%
Severity of Injury
Minor Hemorrhage: early hemarthrosis, epistaxis, gingival bleeding, mild hematuria
Major Hemorrhage: hemarthrosis/muscle bleed with swelling and pain
Life-threatening Hemmorhage: CNS, major trauma, advanced/recurrent hemarthrosis
Lab studies
-CBC
-Coags
-Factor VIII or IX assay
-Inhibitor level?
How much factor?
Small cut/abrasion: conservative measures?
Minor Hemorrhage: 30%
Major Hemorrhage: 50%
Life-threatening Hemorrhage: 100%
Units of factor VIII = (wt in kg)(50mL/kg)(1 U fVIII/mL)(desired fVIII level – native fVIII level)
Units of factor IX = (wt in kg)(100mL/kg)(1 U fIX/mL)( desired fIX level – native fIX level)
VIII: (wt)(50)(% correction)
IX: (wt)(100)(% correction)
40kg kid with 1% factor activity, head bleed
VIII: (40)(50)(0.99) = 1980 units
IX: (40)(100)(0.99) = 3960 units
40kg kid with 1% factor activity, hemarthrosis
VIII: (40)(50)(0.49) = 980 units
IX: (40)(100)(0.49) = 1960 units
Further ED management
Imaging
Pain control
Joint immobiliziation?
Hematology consult
Other considerations
-DDAVP (only for mild or moderate hemophilia A)
-Antifibrinolytics (aminocaproic acid and TXA)
-Treating pts with inhibitors (PCC, Recombinant FVIIa)
-Second dose of factor VIII at 12 hours
-Second dose of factor IX at 24 hours
-Continuous infusion?
Jay Khadpe MD
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