Nokea
Therapeutic management of a 6-year-old child with hereditary spherocytosis (HS) should include which therapeutic intervention?
- A. Perform a splenectomy.
- B. Supplement the diet with calcium.
- C. Institute a maintenance transfusion program.
- D. Increase intake of iron-rich foods such as meat.
Correct Answer: A
Rationale: Splenectomy corrects hemolysis in hereditary spherocytosis, typically for children over 5 with symptomatic anemia. Calcium doesn?t affect HS, transfusions suppress RBC production and carry risks, and iron supplementation is ineffective for this condition.
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A school-age child is admitted in vasoocclusive sickle cell crisis (pain episode). The childs care should include which therapeutic interventions?
- A. Hydration and pain management
- B. Oxygenation and factor VIII replacement
- C. Electrolyte replacement and administration of heparin
- D. Correction of alkalosis and reduction of energy expenditure
Correct Answer: A
Rationale: Vasoocclusive sickle cell crisis requires hydration to reduce blood viscosity and pain management to alleviate discomfort. Oxygen may prevent further sickling but doesn?t reverse it, factor VIII and heparin are irrelevant, and acidosis, not alkalosis, needs correction.
What statement best describes b-thalassemia major (Cooley anemia)?
- A. It is an acquired hemolytic anemia.
- B. Inadequate numbers of red blood cells (RBCs) are present.
- C. Increased incidence occurs in families of Mediterranean extraction.
- D. It commonly occurs in individuals from West Africa.
Correct Answer: C
Rationale: B-thalassemia major has a higher incidence in Mediterranean populations and is inherited, not acquired. It involves overproduction of unstable RBCs, not inadequate numbers, and is distinct from sickle cell anemia, which is common in West African populations.
In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxia-ischemia cycle. What information should the nurse share with parents in a teaching plan?
- A. Encourage drinking.
- B. Keep accurate records of output.
- C. Check for moist mucous membranes.
- D. Monitor the concentration of the childs urine.
Correct Answer: C
Rationale: Checking for moist mucous membranes assesses hydration in SCA, where impaired kidney function prevents urine concentration. General encouragement to drink is vague, output records don?t reflect fluid needs, and urine concentration is unreliable due to kidney dysfunction.
The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. What statement most accurately reflects inheritance of SCA?
- A. SCA is not inherited.
- B. All siblings will have SCA.
- C. Each sibling has a 25% chance of having SCA.
- D. There is a 50% chance of siblings having SCA.
Correct Answer: C
Rationale: SCA is an autosomal recessive disorder, so each sibling has a 25% chance of inheriting SCA, 25% chance of being unaffected, and 50% chance of having the trait. SCA is inherited, not all siblings will have it, and 50% applies to the trait, not the disease.
A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse?
- A. Administer 100% oxygen to relieve hypoxia.
- B. Notify the practitioner because chest syndrome is suspected.
- C. Infuse intravenous antibiotics as soon as cultures are obtained.
- D. Give ordered pain medication to relieve symptoms of pain episode.
Correct Answer: B
Rationale: Symptoms suggest acute chest syndrome, a medical emergency in SCA, requiring immediate practitioner notification. Oxygen may be needed but doesn?t reverse sickling, antibiotics follow evaluation, and pain management is secondary to addressing the emergency.