HEMOPHILIA

ETIOLOGY

• Lifelong bleeding disorder
• X-linked
• Females to male offspring at birth; can also be acquired
• 1 in 10,000 male births

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CLINICAL MANIFESTATIONS

• Internal and external bleeding
• Hemarthrosis with pain, edema, erythema
• Small cuts can be fatal
• Blood loss from simple dental procedure may be significant

DIAGNOSIS

• Family history
• Coagulation profiles
• Factors 8&9

MEDICAL MANAGEMENT

• Prevention
• Treat bleeding
• Pain relief

NURSING INTERVENTIONS

• Identify cause and source of bleeding
• Assess pain (pain is 1st indicator of joint and tissue bleeding)
• Asses pallor, hematoma, cyanosis, skin
• Monitor vitals
• Monitor PT & INR

NURSING INTERVENTIONS CON'T

• Monitor kidney function & I/O
• Monitor pt receiving fresh, frozen plasma
• Promote fluids & give oxygen
• Pain medications; avoid aspirin & blood thinners
• Pt safety

NURSING INTERVENTIONS CON'T

• Watch wound drainage & perform dressing changes

PT TEACHING FOR DISCHARGE

• Early signs of bleeding
• How to treat minor bleeds
• Administer factor replacement
• Safety precautions (ie; soft bristle toothbrush, avoid extensive exercise, etc)
• Med alert bracelet

PT TEACHING FOR DISCHARGE CON'T

• Let dr know of new meds, pain, bleeding, bruising
• Genetic counseling
• Support groups
• Women monitor menstruation (promote hormone therapy)

WHEN IN DOUBT "DO THE 5"

• 1. Annual comprehensive check at the hemophilia foundation center
• 2. Get vaccinated for Hep A & B
• 3. Treat bleeds early and adequately
• 4. Exercise and maintain healthy weight (protect joints)
• 5. Test regularly for blood born infections

PROGNOSIS

• Before and after HIV
• Good