In sickle cell disease, red blood cells turn stiff and curved and block small vessels, cutting off oxygen to bones and organs and causing sudden, severe pain. Triggers include cold, dehydration, infection and stress, and fast pain relief is a priority.
Key symptoms
- Severe pain in the back, chest, limbs or belly
- Painful swelling of the hands and feet in young children
- Fatigue and fever
- Pain like the patient's usual crisis, or pain that feels different
What you find on exam
- Pain score and vital signs, including oxygen saturation
- Lungs for crackles or reduced air entry
- Spleen size and signs of anemia
- Neurologic check and any priapism
Tests
- Blood count and reticulocyte count compared with baseline
- Chest X-ray if chest pain, cough, fever or low oxygen
- Blood cultures if fever is above 38.5 °C (101.3 °F)
- Type and screen, kidney and liver tests
Treatment basics
- An opioid such as morphine or hydromorphone within 60 minutes of arrival, reassessed every 15 to 30 minutes
- Intravenous or oral fluids without overloading
- Incentive spirometry to prevent acute chest syndrome
- Oxygen only if saturation is low
- Hydroxyurea long term to reduce crises
Red flags
- Chest pain, cough, fever and a new lung infiltrate (acute chest syndrome)
- Weakness, facial droop or trouble speaking (stroke)
- Pale, weak child with an enlarging spleen (splenic sequestration)
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Related guides
Sources: NCCPA PANCE Content Blueprint; NCSBN 2026 NCLEX-RN Test Plan; NREMT psychomotor skill sheets (EMT and Paramedic). Written by Making Synapses. Making Synapses is not affiliated with these organizations.