Hemophilia Basics and ED Management

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?

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• Bio
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Jay Khadpe MD

• Editor in Chief of "The Original Kings of County"
• Assistant Professor of Emergency Medicine
• Assistant Residency Director
• SUNY Downstate / Kings County Hospital

Latest posts by Jay Khadpe MD (see all)

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