Acute painful crisis in adults with sickle cell disease

A 54 year old woman with sickle cell disease presents to the emergency department with severe back and chest pain after swimming in the sea. On examination, she is tachycardic and afebrile, and has no other clinical signs apart from pallor of the mucous membranes. She has had similar episodes of pain previously which have responded to oral analgesia and hydration at home but these interventions have been ineffective on this occasion. What is the next stage of acute management and what advice would you give her to prevent future episodes?

Sickle cell disease (SCD) is a multisystem disease that shortens life, and acute painful crisis is the hallmark of the condition. The clinical course is usually one of well compensated chronic anaemia interspersed with episodes of pain.1 With advances such as hydroxyurea therapy, chronic transfusion programmes, and stem cell transplantation, the life expectancy of patients in high income countries has risen from the mid 40s in the 1980s to the late 50s today.2 Clinicians working in primary and secondary care are therefore more likely to encounter patients with sickle cell disease and manage acute painful crises.

This article aims to help readers identify and assess patients with suspected acute painful crisis, and how to recognise and manage the complication of acute chest syndrome, optimise management in the inpatient and outpatient settings, and deliver care compassionately.

Management principles are largely similar for …