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Sickle cell anaemia (SCA) is a severe, inherited haemoglobinopathy characterised by abnormal haemoglobin S (HbS), which distorts red blood cells into rigid, crescent shapes. These sickle cells are fragile, breaking down prematurely and causing chronic haemolytic anaemia, while their stiffness obstructs small blood vessels, triggering ischaemic pain crises and multi-organ complications.

Symptoms typically emerge in infancy, beginning with dactylitis (swollen hands and feet), followed by fatigue, pallor, jaundice, and recurrent infections due to splenic dysfunction. Painful vaso-occlusive crises—sudden, severe episodes in the chest, back, or limbs—are hallmark features, often requiring hospitalisation. Children may experience delayed growth and puberty, while adults face risks of stroke, acute chest syndrome, priapism, and retinopathy.

Diagnosis is confirmed via newborn screening or haemoglobin electrophoresis, identifying HbS homozygosity. Management focuses on symptom relief and prevention: hydration, analgesics, antibiotics, vaccinations, and hydroxyurea to reduce crisis frequency. Blood transfusions treat severe anaemia or stroke risk, while emerging gene therapies offer potential cures for select patients.

SCA predominantly affects individuals of African, Mediterranean, or South Asian descent, reflecting its evolutionary link to malaria resistance. Lifelong multidisciplinary care is essential to mitigate complications and improve quality of life.