Hemophilia

Introduction

The patient is missing a coagulation factor that is essential for normal blood
clotting and as a result the blood does not clot when the patient bleeds. It is an
X-linked recessive inherited disorder, passed on so that it presents symptoms in
males, and rarely in females. Hemophilia A is the result of missing clotting factor
VIII. Hemophilia B is the result of missing clotting factor IX and is also known as
Christmas disease.

SIGNS AND SYMPTOMS

• Tender joints due to bleeding
• Swelling of knees, ankles, hips, and elbows due to bleeding
• Blood in stool (tarry stool) due to GI blood loss
• Blood in the urine (hematuria)




TREATMENT

• Avoid aspirin.
• For hemophilia A administer factor VIII concentrates.
• Cryoprecipitate.
• DDAVP for patients with mild deficiency.
• For hemophilia B administer factor IX concentrates.

NURSING DIAGNOSES

• Acute pain
• Impaired gas exchange

NURSING INTERVENTION

• No IM injections.
• No aspirin.
• To stop bleeding:
• Elevate site.
• Apply direct pressure to the site.
• Explain to the patient:
• Wear a medical alert identification.
• Contact physician for any injury.
• Avoid situations where injury might occur.