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Physiological AdaptationSickled RBCs → vaso-occlusion

Sickle Cell CrisisNCLEX cheat sheet

A sickle cell crisis occurs when abnormal hemoglobin S causes red cells to sickle, clump, and occlude blood vessels, blocking oxygen delivery to tissues.

24 key facts 3 practice questions Reviewed Oct 2026
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Triggers

  • hypoxia, dehydration, infection
  • cold, stress, high altitude, exertion

Signs

  • SEVERE pain (vaso-occlusion)
  • swelling joints, jaundice, fever

Do

  • HOP: Hydration, Oxygen, Pain
  • IV fluids, O2, opioids (PCA), rest
  • warm compress (NOT cold)
  • hydroxyurea prevents crises

Report

  • acute chest syndr → fatal
  • stroke, splenic sequestration
Red flag to remember

Report signs of acute chest syndrome: chest pain, fever, dyspnea, or falling oxygen saturation.

Before you read

Test yourself first: 3 questions

Question 1 of 3

A client with sickle cell disease is admitted in vaso-occlusive crisis. Which intervention is the nurse's priority?

What is sickle cell crisis?

Triggers include hypoxia, dehydration, infection, cold, stress, and high altitude. It matters because severe pain and ischemic organ/tissue damage result, and untreated crises can be life-threatening. Memory aid for prevention: avoid the things that deoxygenate or dehydrate.

Sickle Cell Crisis illustrated

Sickle Cell Crisis — medical illustration
Sickle Cell Crisis — medical illustration. Illustration: BruceBlaus via Wikimedia Commons, CC BY-SA 4.0.

Key points to remember

  • Vaso-occlusion causes severe pain, most often in the chest, abdomen, back, and joints.
  • Dehydration, hypoxia, infection, and cold are the main precipitating triggers.
  • Acute chest syndrome (chest pain, fever, hypoxia, infiltrate) is a leading cause of death.
  • Splenic sequestration causes a sudden drop in hemoglobin and circulatory collapse.
  • Priapism, swelling of hands and feet (dactylitis), and jaundice can occur.
  • This is an autosomal recessive disorder seen most in patients of African descent.

Signs & symptoms

  • SEVERE pain (vaso-occlusion)
  • swelling joints, jaundice, fever

Nursing priorities

  1. Provide oxygen to relieve hypoxia and reduce further sickling.
  2. Hydrate aggressively with IV fluids to reduce blood viscosity.
  3. Manage pain with scheduled opioids (often morphine) and avoid undertreating pain.
  4. Keep the patient warm and at rest to lower metabolic and oxygen demand.
  5. Monitor for infection and administer antibiotics promptly when ordered.

Labs & values

Hemoglobin (normal12-16 g/dL female, 14-18 g/dL male) decreased, often baseline 6-9 g/dL
Reticulocyte count elevated
Hemoglobin electrophoresis confirms hemoglobin S
Oxygen saturation decreased during crisis
1216g/dL Normal

Patient teaching

  • Drink plenty of fluids daily and avoid dehydration, especially in hot weather or with exercise.
  • Avoid known triggers: high altitude, unpressurized flights, cold exposure, smoking, and strenuous exertion.
  • Get recommended vaccines and seek care early for any fever, since infection can trigger a crisis.

Red flags: report now

  • Report signs of acute chest syndrome: chest pain, fever, dyspnea, or falling oxygen saturation.
  • Report a sudden enlarging spleen with falling hemoglobin (splenic sequestration) or signs of shock.
  • Report new neurologic deficits suggesting stroke, or priapism lasting over a few hours.
  • Never apply cold; cold promotes sickling and worsens the crisis.

References

Clinical references for this topic

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