Sickle Cell Anemia: Alterations in Health - Pediatric Nursing, Cardio Disorders | @LevelUpRN

Summary

In this educational video, Kathy from Level Up RN provides a thorough overview of sickle cell anemia, covering its genetic basis, pathophysiology, risk factors, and key signs and symptoms. She explains the different types of crises—vaso-occlusive, splenic sequestration, and aplastic—and the appropriate interventions, including pain management with opioids, IV fluids, oxygen, and medications like hydroxyurea. The video also emphasizes essential nursing care and family teaching, such as infection prevention and proper hydration. To reinforce learning, Kathy concludes with a short quiz on hemoglobin electrophoresis, lab findings, and pain management strategies, helping viewers solidify their understanding of this complex condition.

Takeaways

• 😀 Sickle cell anemia is an autosomal recessive genetic disorder that leads to chronic anemia, pain, infections, and organ damage.
• 😀 The disorder occurs when normal hemoglobin (HbA) is replaced with sickle hemoglobin (HbS), causing red blood cells to sickle and block blood flow.
• 😀 Sickle-shaped red blood cells can obstruct blood vessels, leading to tissue hypoxia and potential organ damage.
• 😀 Key risk factors for sickle cell anemia include family history, African American descent, and individuals of Middle Eastern or Mediterranean origin.
• 😀 Symptoms of sickle cell anemia include pain, fatigue, swollen hands and feet, jaundice, and the occurrence of painful 'crises' triggered by stress, infections, or dehydration.
• 😀 The most critical crisis to understand is the vaso-occlusive crisis, characterized by severe pain and the need for opioid analgesics for pain relief.
• 😀 Other types of crises include splenic sequestration (sickled cells trapped in the spleen) and aplastic crisis (bone marrow stops producing red blood cells, often due to viral infection).
• 😀 Lab findings typically include decreased hematocrit, increased reticulocytes (immature red blood cells), increased white blood cells, and elevated bilirubin levels due to the breakdown of sickled cells.
• 😀 Sickle cell anemia is diagnosed using hemoglobin electrophoresis, which identifies abnormal hemoglobin types.
• 😀 Treatment includes opioid pain management, antibiotics for infection, IV fluids, blood products, oxygen therapy, and ongoing medication like hydroxyurea to reduce sickling and prevent crises.

Q & A

What is sickle cell anemia and how is it inherited?

Sickle cell anemia is an autosomal recessive genetic disorder that results in chronic anemia, pain, infection, and organ damage. It is inherited when a child receives two abnormal hemoglobin genes (HBS), one from each parent.

What type of hemoglobin is abnormal in sickle cell anemia and what effect does it have on red blood cells?

Abnormal hemoglobin in sickle cell anemia is called HBS. It causes red blood cells to form a sickle or C-shape, making them prone to getting stuck in blood vessels and leading to tissue hypoxia.

Which populations are at higher risk for sickle cell anemia?

Individuals with a family history of sickle cell anemia, as well as those of African-American, Middle Eastern, or Mediterranean descent, are at higher risk.

What are common signs and symptoms of sickle cell anemia?

Common signs and symptoms include pain, fatigue, swollen hands and feet, and jaundice. Patients may also experience crises triggered by infection, stress, or dehydration.

Describe the main types of sickle cell crises and their characteristics.

1) Vaso-occlusive crisis: severe pain treated with scheduled opioid analgesics. 2) Splenic sequestration crisis: sickled RBCs trapped in the spleen causing enlargement and severe anemia. 3) Aplastic crisis: bone marrow stops producing RBCs, often caused by viral infection, leading to severe anemia.

What lab findings are typically seen in a patient with sickle cell anemia?

Typical lab findings include decreased hematocrit, increased reticulocytes, increased white blood cell count, and increased bilirubin due to the breakdown of abnormal RBCs.

How is sickle cell anemia diagnosed in patients?

Sickle cell anemia is diagnosed using hemoglobin electrophoresis, a blood test that identifies abnormal hemoglobin types. All newborns are screened for the disorder.

What are the main treatment strategies for managing sickle cell anemia?

Treatment includes managing crises with scheduled opioid analgesics, IV fluids, blood products, and oxygen therapy. Antibiotics are used for infections, and hydroxyurea can be taken to reduce sickling of RBCs and prevent complications.

What family teaching is important for a child with sickle cell anemia?

Families should ensure adequate hydration, practice infection prevention (hand hygiene, vaccinations, avoiding crowds), and seek immediate medical attention for fever or signs of infection.

Which lab findings would you expect to see in a patient with sickle cell anemia? Select all that apply: increased bilirubin, increased reticulocytes, increased hematocrit, increased white blood cell count.

Expected lab findings include increased bilirubin, increased reticulocytes, and increased white blood cell count. Hematocrit is typically decreased.

Why are NSAIDs or Tylenol insufficient for pain management in vaso-occlusive crises?

NSAIDs or Tylenol are insufficient because the pain during a vaso-occlusive crisis is severe and requires strong opioid analgesics administered on a scheduled basis to effectively manage it.

What triggers can precipitate a sickle cell crisis?

Sickle cell crises can be triggered by infection, stress, or dehydration, which increase the likelihood of red blood cells sickling and obstructing blood flow.