A 7-year-old child with hemophilia A is brought to the emerg… | 마이메르시 MyMerci
Child Health
문제

A 7-year-old child with hemophilia A is brought to the emergency department after falling from a bicycle and hitting his head. The child is alert and oriented, with no visible external bleeding. What is the most appropriate immediate nursing intervention?

Priority intervention for a child with hemophilia A following head trauma
해설
Children with hemophilia A have a deficiency in factor VIII, making them prone to prolonged bleeding, especially after trauma. Head injuries in hemophilic patients require immediate factor replacement therapy to prevent potentially life-threatening intracranial hemorrhage, even when no external bleeding is visible.

심화 해설

Core Nursing Explanation Key Concept Analysis: This question tests the priority nursing action for a patient with a bleeding disorder following trauma, specifically head trauma. The core theme is life-threatening complication prevention. In Hemophilia A, there is a deficiency of Factor VIII, a crucial protein in the intrinsic pathway of the coagulation cascade. Any trauma, especially to the head, carries a high risk of internal bleeding, which may not be immediately apparent. An intracranial hemorrhage (ICH) is a medical emergency. The priority is to prevent this by rapidly correcting the underlying clotting factor deficiency.

Answer Rationale: Key Point! The most appropriate immediate intervention is to Administer factor VIII concentrate as prescribed by the physician. This is a definitive treatment that directly addresses the pathophysiology. For a hemophiliac with head trauma, factor replacement is considered a prophylactic or emergency treatment to stop potential bleeding before it causes neurological damage or becomes life-threatening. The nurse's role is to anticipate this need, prepare for administration, and carry out the physician's order promptly.

Distractor Analysis:
Watch out for confusion! Option ① (Apply ice and monitor) is a supportive measure for minor injuries but is not the priority for a high-risk patient with a known coagulopathy. Monitoring is essential, but it must occur concurrently with definitive treatment, not instead of it.
Option ② (Obtain labs) is an important diagnostic step, but it should not delay life-saving treatment. Drawing blood for coagulation studies (PT, aPTT) confirms the diagnosis but does not treat the immediate threat. Treatment is based on the patient's known history and the mechanism of injury.
Option ④ (Trendelenburg position) is incorrect and potentially harmful. The Trendelenburg position (head lower than feet) can increase intracranial pressure (ICP), which is dangerous if intracranial bleeding is suspected. The appropriate position for a head injury patient is flat or with the head of the bed slightly elevated, if spinal injury is ruled out.

Related Concepts: This scenario integrates knowledge of hematology, emergency nursing, and pediatric care. The nursing process here prioritizes Implementation of a critical intervention over further Assessment because the risk is known and imminent. Understanding the difference between supportive care and definitive, life-saving treatment is crucial for clinical judgment. Concept Summary
ConceptKey Takeaway
Hemophilia A PathophysiologyX-linked recessive disorder causing Factor VIII deficiency, leading to prolonged bleeding.
Priority in TraumaFor hemophiliacs, assume internal bleeding is occurring until proven otherwise. Head trauma = highest risk.
Definitive TreatmentFactor replacement therapy (Factor VIII concentrate) is the cornerstone of emergency management.
Nursing RoleRapid recognition, preparation, and administration of factor concentrate; vigilant neurological monitoring.

Side-by-Side Comparison!
ScenarioPriority Nursing InterventionRationale
Head Trauma in HemophiliaAdminister Factor VIIIPrevent life-threatening intracranial hemorrhage. Treatment precedes detailed assessment.
Head Trauma (General Population)Stabilize C-spine, Assess Neurological Status (GCS), obtain CT scan.Rule out fracture and hemorrhage. Assessment guides treatment.
Joint Bleed (Hemarthrosis) in HemophiliaAdminister Factor VIII, RICE protocol (Rest, Ice, Compression, Elevation).Stop bleeding and minimize joint damage and pain.

Anatomy, Physiology & Pharmacology Points
  • Coagulation Cascade: Factor VIII works with Factor IX in the intrinsic pathway. Its deficiency prolongs the aPTT (activated Partial Thromboplastin Time), while PT (Prothrombin Time) remains normal.
  • Factor VIII Concentrate: Can be plasma-derived or recombinant. Administration is typically IV (Intravenous injection). The goal is to raise factor levels to hemostatic levels (often 80-100% for major trauma).
  • Intracranial Space: The skull is a fixed container. Any bleeding increases pressure, which can lead to brain herniation—a rapid, fatal event.

Memory Tips
  • Acronym: For Hemophilia emergencies: FACTOR First (Factor replacement, Assess for bleeding, Call for help, Trauma protocol, Observe neuro status, Record everything).
  • Association: Think of hemophilia as a car with no brakes (clotting factors). After a crash (trauma), you need to install brakes (give factor) immediately before the car crashes again (internal bleeding worsens).

High-Frequency NCLEX Topics NCLEX frequently tests priority-setting for patients with chronic conditions who experience acute complications. Hemophilia + trauma is a classic combo. Remember: In bleeding disorders, replacing what's missing is almost always a higher priority than diagnostic tests or comfort measures.
Watch Out for Question Variations!
  • If the question asks for the next action after administering factor: The answer would shift to continuous neurological monitoring (assessing Glasgow Coma Scale (GCS), pupil checks).
  • If the trauma was to a joint (knee) instead of the head: The priority is still factor administration, but the answer may also include immobilization and application of ice (RICE).
  • If the patient has Hemophilia B (Christmas Disease): The correct answer would be to administer Factor IX concentrate.

임상 시나리오

Nursing Clinical Practice Guide Clinical Scenario: You are the triage nurse in the Pediatric ED. A frantic mother arrives with her 7-year-old son, who has a known history of severe Hemophilia A. He fell off his bike 30 minutes ago, hitting the right side of his forehead on pavement. He has a small abrasion but no active bleeding. He is talking and says his head hurts.

Nursing Intervention Strategy:
  1. Immediate Action (Seconds): Bring the child to a treatment room. While a colleague takes vital signs, you immediately notify the physician and the hematology team. Verbally relay: "Known Hemophilia A, head strike, alert and oriented, requesting order for Factor VIII bolus."
  2. Concurrent Assessment (1-2 minutes): Perform a rapid, focused assessment: Glasgow Coma Scale (GCS), pupil size and reaction, motor strength, and presence of any other injuries. Inquire about loss of consciousness, nausea, or vomiting.
  3. Definitive Treatment (5-10 minutes): Once the order is received, prepare and administer Factor VIII concentrate per protocol. Verify the dose, check the product, and use aseptic technique for IV administration. Document the lot number and time.
  4. Ongoing Monitoring & Diagnostics: After factor administration, obtain blood for CBC (Complete Blood Count) and coagulation panel (PT, aPTT). Continue frequent neuro checks (every 15-30 minutes initially). Prepare for a stat head CT scan.

Patient Safety and Precautions:
  • Never Delay Treatment for Diagnostics: Do not wait for lab results or a CT scan to give factor. The risk of waiting is catastrophic neurological injury.
  • Positioning: Keep the head of the bed flat or elevated to 30 degrees if the cervical spine is cleared. Avoid Trendelenburg.
  • Medication Caution: Factor concentrates are expensive and blood-derived (for some products). Handle with care, confirm the order, and monitor for any signs of an allergic reaction during infusion.
Nursing Procedure & Medication Flow Administering Factor VIII Concentrate:
  1. Verify: Patient identity, physician order (dose in units/kg), product name (recombinant or plasma-derived), and expiration date.
  2. Reconstitute: Use provided diluent. Gently swirl or roll the vial; do not shake vigorously to avoid denaturing the protein.
  3. Administer: Use a filtered needle for drawing up and a filtered IV administration set. Infuse at the rate prescribed (often over 5-15 minutes).
  4. Monitor: Observe for flushing, tachycardia, chest tightness, or fever – potential signs of a reaction.
  5. Document: Time started/ended, total dose (units), lot number, any adverse reactions, and post-infusion assessment.
A Word from Your Senior Nurse "In the ED, time is brain. For our hemophilia patients, we operate on a different clock. A 'simple' fall that would result in a bump for another child can be a ticking time bomb for them. Your knowledge and swift action in initiating factor replacement are what stand between that child and a devastating brain bleed. Always think: 'What is the one thing I can do right now that will most directly stop the worst possible outcome?' That's your priority. This mindset saves lives."

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