Acute chest syndrome is an important complication of sickle cell disease characterised by fever and/or respiratory symptoms and a new pulmonary infiltrate on chest X-Ray. Patients should be monitored for this complication throughout their hospital stay and all hospitals should have a treatment pathway for care.

Essential investigations for diagnosis and prognosis include chest X-Ray, full blood count and basic biochemistry tests. Arterial blood gases analysis should be considered in adults with low oxygen saturations as severe hypoxia is a useful predictor of severity and helps guide treatment.

Aetiology is multifactorial and includes infection so careful investigation for infective causes should be undertaken including blood cultures, sputum for microscopy and culture and sputum and naso-pharyngeal aspiration for viral testing.

Basic management will include prompt pain relief, incentive spirometry and treatment of bacterial or viral infection. Blood transfusion should be considered early in the hypoxic patient. A simple (top-up) transfusion may suffice in early or less severe disease but exchange transfusion will be necessary if there are features of clinical severity or a lack of response to simple transfusion.

The critical care team should be involved in the care of any patients with severe clinical features or in a deteriorating patient to consider non-invasive or invasive ventilation. Following an episode of acute chest syndrome the patient should be offered disease modifying therapy (eg hydroxycarbamide, blood transfusion).

Declaration of Interests

The BSH paid the expenses incurred during the writing of this guidance. None of the authors had conflicts of interest to declare. All authors have made a declaration of interests to the BSH and Task Force Chairs which may be viewed on request.