Sickle Cell Anemia Lecture Notes

Introduction

• Presenter: Kathy from Level Up RN
• Topic: Sickle Cell Anemia
• Includes a quiz at the end to reinforce key facts
• Reference: Level Up RN Pediatric Nursing Flashcards

Overview of Sickle Cell Anemia

• Type: Autosomal recessive genetic disorder
• Results in:
  • Chronic anemia
  • Pain
  • Infection
  • Organ damage

Pathophysiology

• Normal hemoglobin (HBA) is replaced with abnormal sickle hemoglobin (HBS)
• Results in:
  • Sickling of red blood cells (C-shape instead of round)
  • Obstruction of blood flow to organs
  • Tissue hypoxia

Risk Factors

• Family history of sickle cell anemia
• Higher risk in:
  • African-Americans
  • Individuals of Middle Eastern or Mediterranean descent

Signs and Symptoms

• Pain
• Fatigue
• Swollen hands and feet
• Jaundice
• Crisis triggers: Infection, stress, dehydration

Types of Crises

1. Vasoocclusive Crisis:
   • Severe pain
   • Requires opioid analgesics, not NSAIDs or acetaminophen
2. Splenic Sequestration Crisis:
   • Sickled cells trapped in spleen
   • Leads to spleen enlargement and severe anemia
3. Aplastic Crisis:
   • Bone marrow stops producing red blood cells
   • Caused by viral infections (e.g., fifth disease)

Laboratory Findings

• Decreased hematocrit
• Increased reticulocytes
• Increased white blood cells
• Increased bilirubin

Diagnosis

• Test: Hemoglobin electrophoresis
  • Measures hemoglobin levels
  • Identifies abnormal types
• All newborns are screened for sickle cell anemia

Treatment

• Crises:
  • Opioid analgesics on schedule
  • Antibiotics for infections
  • IV fluids, blood products, and oxygen therapy
• Ongoing:
  • Hydroxyurea to reduce sickling and prevent complications

Family Teaching

• Ensure adequate fluid intake to prevent crises
• Prevent infections with hygiene, avoiding crowds, vaccinations
• Seek immediate medical attention for fever or infection signs

Quiz Questions

1. Question 1: What blood test identifies abnormal hemoglobin?
   • Answer: Hemoglobin electrophoresis
2. Question 2: How should pain be treated during a vasoocclusive crisis?
   • Answer: Opioid analgesics on a schedule
3. Question 3: Expected lab findings (select all that apply):
   • Answers: Increased bilirubin, increased reticulocytes, increased white blood cell count (note: decreased hematocrit, not increased)

Conclusion

• Encourages subscribing and sharing videos with classmates
• Reminder to like the video if found helpful