Sickle cell disease is an inherited hemoglobin disorder in which red cells become stiff and sickle shaped, block small blood vessels and break down early. This causes chronic anemia, sudden pain crises and damage to organs such as the lungs, brain, spleen and kidneys.
Key symptoms
- Sudden severe pain in the back, chest, arms or legs (vaso-occlusive crisis)
- Painful swelling of the hands and feet in young children
- Fatigue, pallor and yellow eyes
- Frequent infections
- Chest pain, cough and fever in acute chest syndrome
What you find on exam
- Vital signs, oxygen saturation and temperature
- Pallor and jaundice
- Lung exam for crackles or reduced breath sounds
- Spleen size in children (splenic sequestration)
- Neurologic exam and check for priapism
Tests
- Blood count and reticulocyte count compared with the patient's baseline
- Hemoglobin electrophoresis (and newborn screening) for diagnosis
- Bilirubin, LDH, kidney function, type and screen
- Chest X-ray with chest pain, cough, fever or low oxygen; blood cultures with fever
Treatment basics
- Fast pain control with IV opioids within 1 hour, then scheduled doses
- IV fluids if dehydrated, oxygen if saturation is low, incentive spirometry
- Acute chest syndrome: ceftriaxone with azithromycin, and transfusion if severe
- Long-term hydroxyurea; penicillin prophylaxis and vaccines in children
- Transcranial Doppler screening in children; transfusion for stroke
Red flags
- Chest pain, fever or low oxygen: acute chest syndrome
- Sudden weakness, trouble speaking or seizure: stroke
- Fever of 38.5 C or more, a fast-growing spleen or a prolonged painful erection
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.