NCLEX Review Guide: Pediatric Hemophilia

Pathophysiology & Types

• Hemophilia is an X-linked recessive bleeding disorder affecting blood clotting factors. Males are primarily affected since they only have one X chromosome, while females are typically carriers.
• Hemophilia A (Classic) involves Factor VIII deficiency and accounts for 85% of cases. Hemophilia B (Christmas Disease) involves Factor IX deficiency and represents 15% of cases.
• Severity is classified as mild (5-40% factor activity), moderate (1-5% factor activity), or severe (<1% factor activity) based on clotting factor levels.

Signs & Symptoms

• Hemarthrosis (joint bleeding) is the most common and serious complication, typically affecting knees, ankles, and elbows. Repeated bleeding leads to joint deformity and chronic arthritis.
• Intracranial hemorrhage is the leading cause of death in hemophiliacs and requires immediate emergency intervention with factor replacement therapy.
• Other manifestations include prolonged bleeding after trauma, surgery, or dental procedures, easy bruising, and deep muscle hematomas that can compress nerves and blood vessels.

Factor Replacement Therapy

1. Immediate factor replacement is the primary treatment for bleeding episodes, administered IV as soon as bleeding is suspected.
2. Factor concentrates are preferred over cryoprecipitate due to reduced risk of viral transmission and more precise dosing.
3. Prophylactic factor infusions may be given 2-3 times weekly for severe hemophilia to prevent spontaneous bleeding.
4. Monitor for development of inhibitors (antibodies against clotting factors) which occur in 20-30% of patients with severe hemophilia A.

Supportive Care & Safety

• RICE protocol (Rest, Ice, Compression, Elevation) for acute joint bleeding, along with immobilization until bleeding stops.
• Avoid aspirin and NSAIDs which impair platelet function and increase bleeding risk; acetaminophen is the preferred analgesic.
• Encourage appropriate physical activity like swimming while avoiding contact sports and activities with high injury risk.

Safety Measures

• Teach families to recognize early signs of bleeding including joint pain, swelling, tingling, or decreased range of motion before visible bleeding occurs.
• Medical alert identification should be worn at all times, and emergency action plans must be readily available for schools and caregivers.
• Dental care requires prophylactic factor replacement before procedures, and intramuscular injections should be avoided due to hematoma risk.

Check Your Knowledge:

• ☐ Can identify inheritance pattern of hemophilia
• ☐ Knows difference between Hemophilia A and B
• ☐ Understands priority nursing interventions for bleeding
• ☐ Can teach family safety measures
• ☐ Recognizes signs of joint bleeding