In sickle cell disease, red blood cells can bend into a sickle shape and block small blood vessels, causing sudden, severe pain called a vaso-occlusive crisis. Some crises turn dangerous fast, such as acute chest syndrome, stroke and infection, so every episode deserves a careful assessment.
Key symptoms
- Severe pain in the bones, back, chest, belly or joints
- Pain like the patient's usual crises, or worse
- Fever, which can signal serious infection
- Shortness of breath or chest pain in acute chest syndrome
- A painful erection lasting hours (priapism)
What you find on exam
- Vital signs, including temperature and oxygen saturation
- Lung sounds and work of breathing
- Neurologic check for stroke signs
- Pale skin or yellow eyes
- Belly exam for an enlarged spleen, mostly in children
Tests
- Pain score with a validated scale
- Hospital: blood count with reticulocytes, compared with baseline
- Chest X-ray for fever, cough or chest pain
- Blood cultures and urinalysis if there is fever
Treatment basics
- Fast pain control, ideally within 60 minutes: IV, intranasal or IM opioid such as fentanyl or morphine
- Oxygen only if saturation is below 94 percent
- IV fluids if dehydrated, without overloading
- Keep the patient warm and reassess pain often
- Transport to a hospital that knows the patient and follow their care plan
Red flags
- Fever of 38.5 C (101.3 F) or higher
- Chest pain, low oxygen or new trouble breathing
- Weakness, slurred speech or severe headache
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Related guides
Sources: NASEMSO National Model EMS Clinical Guidelines; NREMT psychomotor skill sheets (EMT and Paramedic); National EMS Education Standards (2021). Written by Making Synapses. Making Synapses is not affiliated with these organizations.