Sickle cell crises

Sickle cell crises occur most commonly in haemoglobin S homozygotes. Patients with mixed haemoglobinopathies may have sickling episodes e.g. SC disease.

Sickle cell crises do not occur in Hb S heterozygotes.

Most common sites of pain onset are the back or trunk, and pain may then radiate to the limbs (1):

• duration is usually a few hours to days, but in some cases, it may persist for weeks
• consider the complication of acute chest syndrome* in patients with fever and respiratory signs and symptom

* acute chest syndrome

• a combination of pulmonary infarction, infection, and fat embolism
• caused by sickling and ischaemia in deep tissues of the chest wall

The various sickle cell crises and their acute management are described below.

Reference

1. Charles K S, Friday M, Rochford E. Acute painful crisis in adults with sickle cell disease BMJ 2024; 386 :e075099 doi:10.1136/bmj-2023-075099