Synopsis
Recognise a new pulmonary sickle syndrome early, stabilise oxygenation and ventilation, treat infection and atelectasis, and select simple or exchange transfusion before respiratory failure.
- Acute chest syndrome is a new pulmonary infiltrate involving at least one lung segment with fever and/or respiratory symptoms in a person with sickle-cell disease.
- Causes overlap: infection, pulmonary vaso-occlusion, bone-marrow fat embolism, atelectasis, hypoventilation and fluid overload may contribute within one episode.
- It can be present on arrival or develop one to three days into a vaso-occlusive pain admission, especially with rib pain, sedation or reduced inspiration.
- Cough, chest pain, fever, tachypnoea, wheeze, hypoxia or falling saturation from personal baseline should trigger chest imaging and repeated assessment.
- A normal early radiograph does not exclude evolving disease; repeat imaging is appropriate when physiology worsens and may lag behind examination.
- Give supplemental oxygen for hypoxaemia, use incentive spirometry or coached deep breathing, treat bronchospasm when present and avoid fluid overload.
- Empirical antibiotics should cover typical and atypical respiratory pathogens according to local antimicrobial guidance after appropriate cultures when feasible.
- Simple transfusion improves oxygen carriage in selected moderate disease, but avoid excessive haemoglobin and viscosity, especially when baseline haemoglobin is high.
- Automated or manual red-cell exchange is considered for severe, rapidly progressive or poorly responsive disease and requires urgent specialist and transfusion coordination.
- Venous thromboembolism, bacterial pneumonia, pulmonary oedema, opioid hypoventilation and asthma may mimic or coexist and need parallel evaluation.
Key red flags
Rapid oxygen escalation, multilobar change, falling platelets, acidosis or altered consciousness predicts dangerous progression and prompts exchange discussion.
Investigation priorities
Detect change from baseline and respiratory deterioration.
Management branches
A sickle-cell patient develops fever, cough, chest pain, tachypnoea or falling saturation.
- Repeat ABCDE assessment, compare oxygenation with baseline, obtain chest radiography and blood tests and contact haematology early.
- Give oxygen for hypoxaemia, start protocol antibiotics, maintain effective ventilation-preserving analgesia and use incentive spirometry or coached deep breaths.
- Review fluid balance, bronchospasm, thrombosis and opioid sedation and repeat imaging if clinical decline outpaces the first radiograph.
Key medicines
Empirical antibacterial therapyStart the acute-chest protocol promptly. One common adult community-onset regimen is ceftriaxone 2 g intravenously every 24 hours plus clarithromycin 500 mg orally or intravenously every 12 hours; adapt for acquisition setting, allergy, renal or hepatic function and local resistance.Obtain cultures without delaying therapy; check severe beta-lactam allergy, macrolide QT prolongation and interactions, broaden for healthcare-associated or resistant infection when indicated, then narrow by results and trajectory.
SalbutamolGive inhaled salbutamol by spacer or oxygen-driven nebuliser at acute-asthma doses when wheeze or reversible bronchospasm is present, repeating according to response.Tachycardia, tremor, hypokalaemia and lactate rise can confuse deterioration; do not prescribe routinely when no bronchospasm is present.