Hemophilia

WHAT WENT WRONG?
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.

PROGNOSIS
The most common sites of bleeding are into the joints, muscles, or from the GI
tract. Mild forms of the disease will only cause bleeding after surgery or trauma,
whereas severe forms of the disease will cause bleeding without any prior cause.

HALLMARK 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)

INTERPRETING TEST RESULTS
• PTT prolonged.
• PT normal.
• Bleeding time normal.
• Fibrinogen level normal.
• Decrease in clotting factor VIII found in blood serum in Hemophilia A.
• Decrease in clotting factor IX found in blood serum in Hemophilia B.

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.