A 7-year-old child with hemophilia A is admitted to the pediatric unit after falling from a bicycle and sustaining a knee injury. The child's knee is swollen, painful, and has limited range of motion. The parents are anxious and asking about treatment options. What is the most appropriate initial nursing intervention?
Priority nursing intervention for a child with hemophilia A experiencing joint bleeding
1Apply ice packs to the affected knee for 20 minutes every hour
2Encourage active range of motion exercises to prevent joint stiffness
3Administer factor VIII concentrate as prescribed immediately✓ 정답
4Position the knee in a dependent position to promote circulation
해설
Factor VIII concentrate administration is the priority intervention for hemophilia A patients experiencing bleeding episodes to restore clotting ability and prevent further bleeding.
Core Nursing ExplanationKey Concept Analysis: This question tests the priority nursing intervention for a child with Hemophilia A experiencing an acute Hemarthrosis (joint bleeding). Hemophilia A is a deficiency of clotting Factor VIII. The pathophysiology is straightforward: without adequate Factor VIII, the intrinsic coagulation pathway is impaired, leading to prolonged and potentially severe bleeding, even from minor trauma. In this scenario, the swollen, painful knee with limited motion is classic for hemarthrosis. The priority is to stop the bleeding to prevent complications like chronic joint damage, contractures, and pain.
Answer Rationale: Key Point! The definitive, life-saving treatment for a bleeding episode in Hemophilia A is the prompt replacement of the missing clotting factor. Administering factor VIII concentrate is the highest priority intervention because it directly addresses the root cause of the problem—the coagulation defect. It will halt the ongoing bleeding into the joint, which is the source of the pain, swelling, and risk for long-term disability. All other supportive measures are secondary to this critical step.
Distractor Analysis:
Watch out for confusion! Option ①, applying ice, is a correct supportive measure to reduce pain and swelling through vasoconstriction, but it does not stop the underlying coagulopathy. It is important, but not the priority.
Option ②, encouraging active range of motion, is contraindicated during an acute bleeding episode. Movement can exacerbate the bleeding and increase damage. Rest and immobilization are key initially.
Option ④, positioning the knee in a dependent position, would increase blood flow to the area due to gravity, potentially worsening the swelling and bleeding. The correct approach is to elevate and immobilize the joint.
Related Concepts: The nursing process guides us here: Assessment (recognizing signs of hemarthrosis) leads to the nursing diagnosis of Risk for Injury (bleeding) or Acute Pain. The planning and implementation are centered on factor replacement (collaborative intervention) and supportive care (independent nursing interventions). Evaluation would monitor for decreased swelling, improved pain, and return of joint function.
Concept Summary
Concept
Key Takeaway
Hemophilia A Pathophysiology
X-linked recessive disorder causing Factor VIII deficiency, impairing the intrinsic coagulation pathway.
Hemarthrosis (Joint Bleeding)
Classic manifestation. Presents with pain, swelling, warmth, and limited ROM (Range of Motion).
Priority Intervention
Immediate factor replacement therapy (Factor VIII concentrate).
Supportive Nursing Care (R.I.C.E.)
Rest, Ice, Compression (with caution), Elevation. Watch out for confusion! Avoid heat and early exercise.
Patient/Family Education
Injury prevention, recognizing early signs of bleeding, importance of prompt treatment, and genetic counseling.
Side-by-Side Comparison!
Hemophilia A (Factor VIII Deficiency)
Hemophilia B (Factor IX Deficiency / Christmas Disease)
von Willebrand Disease (vWD)
Deficiency in Factor VIII.
Deficiency in Factor IX.
Deficiency or defect in von Willebrand factor (vWF), which helps platelets adhere and stabilizes Factor VIII.
Treatment: Factor VIII concentrate.
Treatment: Factor IX concentrate.
Treatment: Desmopressin (DDAVP) (mild cases) or Factor VIII concentrate containing vWF.
Prolonged aPTT, normal PT.
Prolonged aPTT, normal PT.
Prolonged Bleeding time, may have prolonged aPTT.
Anatomy, Physiology & Pharmacology Points
• Coagulation Cascade: Factor VIII is part of the intrinsic pathway. Its deficiency is measured by the Activated Partial Thromboplastin Time (aPTT).
• Pharmacology: Factor VIII concentrate is a blood product. Nursing responsibilities include verifying the order, checking the product, administering it promptly (often IV push or short infusion), and monitoring for adverse reactions like allergic responses or fever.
Memory Tips
• Acronym for Acute Joint Bleeding Care: Factor First! Then R.I.C.E. (Rest, Ice, Compression, Elevation).
• Hemophilia A vs. B: "A comes before B in the alphabet, and Eight (VIII) comes before Nine (IX)." A = Factor VIII, B = Factor IX.
High-Frequency NCLEX Topics
NCLEX loves to test priority-setting in bleeding disorders. They will present a symptom (like hemarthrosis) and ask for the first or most important action. The answer is almost always the intervention that directly treats the underlying cause (factor replacement) or addresses an immediate life threat (like airway in hemorrhage).
Watch Out for Question Variations!
• Variation 1: "The nurse is preparing to administer Factor VIII concentrate. Which action is most important?" (Answer: Verify patient identity and the specific factor product against the order to prevent medication error.)
• Variation 2: Shift from symptom to education: "What is the priority teaching point for the parents upon discharge?" (Answer: How to recognize early signs of bleeding and when to seek immediate treatment.)
• Variation 3: "Which finding indicates a therapeutic response to Factor VIII administration?" (Answer: Reduction in joint swelling and pain, stabilization of vital signs if there was blood loss.)
Nursing Clinical Practice GuideClinical Scenario: You are the nurse on a pediatric unit. A 7-year-old boy with known Hemophilia A is brought in by his anxious parents. He fell off his bike and his right knee is visibly swollen, warm to the touch, and he is guarding it, refusing to bend it. His vital signs are stable.
Nursing Intervention Strategy:
1Immediate Assessment & CollaborationQuickly assess the joint (pain on a scale of 1-10, circumference measurement for baseline), notify the provider, and ensure the order for Factor VIII concentrate is obtained STAT. Simultaneously, initiate supportive care.
2Priority Implementation: Factor AdministrationWhile preparing the factor, provide emotional support to the child and parents. Administer the factor concentrate as soon as it arrives. Use an IV pump for controlled infusion if required. Monitor closely during infusion for any signs of reaction.
3Supportive Care ImplementationAfter or while the factor is being given, implement the R.I.C.E. protocol:
• Rest: Immobilize the knee with a splint or soft wrap as ordered. Educate on non-weight bearing.
• Ice: Apply an ice pack wrapped in a towel to the knee for 15-20 minutes at a time, every 1-2 hours.
• Compression: Apply a gentle elastic bandage (Ace wrap) from distal to proximal, ensuring it's not too tight. Check distal pulses, color, and sensation frequently.
• Elevation: Elevate the leg above the level of the heart using pillows.
4Pain Management & EvaluationAdminister prescribed analgesics (avoid aspirin and NSAIDs like ibuprofen as they can impair platelet function). Reassess pain and swelling every 2-4 hours. Document findings meticulously.
Patient Safety and Precautions:
• Key Point!Never administer intramuscular (IM) injections or perform unnecessary venipunctures. Use subcutaneous or IV routes when needed.
• Handle the child gently. Avoid restraining limbs forcefully.
• Teach parents to watch for signs of bleeding in other areas (bruising, gum bleeding, tarry stools, headache indicating possible intracranial bleed).
Nursing Procedure & Medication FlowAdministering Factor VIII Concentrate:
1VerifyCheck the order, patient identity (two identifiers), and the specific factor product (type, dosage, expiration).
2ReconstituteFollow manufacturer instructions precisely. Gently roll the vial; do not shake vigorously to avoid denaturing the protein.
3AdministerUse a filtered IV tubing set. Administer via IV push over a few minutes or as a short infusion per protocol. Watch out for confusion! Rate is usually not a slow drip like antibiotics; it's often given relatively quickly to achieve a rapid peak level.
4MonitorObserve for adverse reactions during and for 30-60 minutes after: urticaria, fever, chills, headache, tachycardia, or anaphylaxis (rare). Have emergency equipment available.
A Word from Your Senior Nurse
"In pediatrics, seeing a child in pain is tough, and the parents' anxiety is palpable. Your calm, efficient action is everything. Remember, in hemophilia, time is joint. The faster you get that factor on board, the less long-term damage occurs. Your role isn't just to hang the bag; it's to be the reassuring expert who coordinates the care, educates the family for the next time, and advocates for the child's comfort. This holistic, knowledgeable approach is what separates a good nurse from a great one—on the NCLEX and on the unit."
Hemophilia A — An X-linked recessive bleeding disorder caused by a deficiency of clotting Factor VIII.
Hemarthrosis — Bleeding into a joint space, a common and serious complication of hemophilia, causing pain, swelling, and limited motion.
Factor VIII Concentrate — The replacement therapy used to treat bleeding episodes in Hemophilia A by providing the missing clotting factor.
R.I.C.E. Protocol — A supportive care mnemonic for musculoskeletal injuries: Rest, Ice, Compression, Elevation.
Activated Partial Thromboplastin Time — A coagulation test that measures the function of the intrinsic pathway; it is prolonged in Hemophilia A and B.