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Pediatric Sickle Cell Management

Pediatric Sickle Cell Management

A 2-month-old is identified during newborn screening with sickle cell anemia.

  • How would you manage this patient at 2 months, 2 years, 6 years, and at 13 years old?
  • What are the issues for each stage in development?
  • Where would you refer this child?
  • How would you coordinate the care of this child?

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Pediatric Sickle Cell Management

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APA

Pediatric Sickle Cell Management

Sickle Cell Anemia (SCA) is a genetic hemoglobinopathy characterized by the presence of hemoglobin S (HbS), leading to sickle-shaped red blood cells that cause vaso-occlusion, hemolysis, and multi-organ complications. Early diagnosis through newborn screening allows for timely interventions to prevent complications and improve quality of life. This discussion outlines the management plan for a child with sickle cell anemia at key developmental stages (2 months, 2 years, 6 years, and 13 years old), highlighting specific concerns at each stage, referrals, and coordination of care.

1. Management at 2 Months Old

Key Issues at This Stage:

  • High risk of infections due to functional asplenia.
  • Parental education on sickle cell disease (SCD) and signs of complications.
  • Nutritional support and immunization planning.

Management Plan:

  1. Initiation of Penicillin Prophylaxis

    • Start penicillin VK 125 mg BID to prevent pneumococcal infections (continue until at least 5 years old).
    • If allergic, use erythromycin as an alternative.

  2. Immunizations:

    • Ensure administration of routine vaccines per CDC guidelines.
    • Additional vaccines:
      • Pneumococcal conjugate vaccine (PCV13)
      • Pneumococcal polysaccharide vaccine (PPSV23) at 2 years old
      • Meningococcal vaccines (MenACWY and MenB)
      • Annual influenza vaccine.

  3. Parental Education:

    • Teach parents about:
      • Early signs of infection (fever >101°F requiring immediate medical attention).
      • Hydration and pain management.
      • Signs of splenic sequestration (rapid abdominal enlargement, pallor, lethargy).

  4. Specialist Referral:

    • Refer to a pediatric hematologist for ongoing management.
    • Connect family with genetic counseling and sickle cell support groups.

2. Management at 2 Years Old

Key Issues at This Stage:

  • Increased risk of splenic sequestration crisis and stroke.
  • Neurodevelopmental monitoring and early education interventions.
  • Preventive care for…