About
An x-linked genetic bleeding disorder that causes hemorrhages and inability to clot.
Assess
Bleeding, hematomas, joint pain, Decreased hemoglobin and hematocrit, hematuria, GI bleeding
Complications
Bleeding, hemorrhage, transfusion reactions, death.
Diagnosis
- Risk for injury related to decreased clotting ability.
- Fluid volume deficit related to major blood loss.
- Knowledge deficit related to disease process and management
Goals
- The client will identify activity restrictions and measures to reduce the risk of hemorrhage.
- The client will demonstrate proper procedures to take if hemorrhage were to occur.
- The client will identify signs and symptoms that require immediate medical attention.
- The client will verbalize fears and concerns related to disease process.
Interventions
- Assess for active bleeding, joint pain, hematuria, GI bleeding, injury.
- Assess client's current understanding of disease process, treatments, and medications.
- Monitor CBC and clotting factors.
- Avoid injections, invasive procedures, NSAIDS, ASA, OTC medications.
- Administer fluid replacement, factor VIII concentrates, factor IX concentrates, plasma as indicated by physician for an acute bleed.
- Teach client activity restrictions, signs and symptoms of bleeding, injury prevention strategies.
- Teach client how to infuse concentrates during an acute hemorrhage.
- Encourage client to wear a medical alert bracelet.
- Teach client about disease process, genetic link, treatment.
- Encourage opportunities for client to express feelings and concerns regarding diagnosis and treatment.
- Teach client appropriate prophylaxis prior to any invasive procedure to minimize risks of hemorrhage ( administration of blood, plasma, or concentrates).
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