Clinical Case Database / Category: Case Based Discussion

A 4-year-old boy with sickle cell disease & acute chest syndrome

Publication details

Dr Florian Gahleitner, Dr Ben Cambers, Dr Kim Sykes
Foundation Years Journal, volume 8, issue 6, p.46 (123Doc Education, London, June 2014)


A 4 year old boy with sickle cell disease (SCD) presented with abdominal and leg pain to his local district general hospital (DGH). He was treated for a presumed vaso-occlusive crisis (VOC) receiving antibiotics, fluids and analgesia. During the fist 24 hours, he rapidly deteriorated with respiratory distress, increasing oxygen requirement and left lower lobe consolidation on chest X-ray (CXR). He was intubated, ventilated and retrieved by PICU with a working diagnosis of acute chest syndrome (ACS). Multi-organ dysfunction syndrome (MODS) ensued over the subsequent 24 hours. He required high frequency oscillation ventilation (HFOV), inhaled nitric oxide (iNO), inotropes, and broad-spectrum antibiotic cover. Exchange transfusion decreased his HbS fraction from 45% to 15%. Over the next 12 days he required mechanical ventilation, inotropic support, haemofitration, antibiotics and antifungal treatment. Ongoing consolidation of one lung prompted a bronchoscopy revealing plastic bronchitis. Rigid bronchoscopy with instillation of DNAse and saline was required to remove the plastic casts. He was extubated two days later making a swift recovery thereafter.

This article discusses the management of a child with SCD with reference to the current literature. Important aspects include the recognition, rapid progression, clinical management and outcome of ACS. Our discussion highlights the pitfalls of managing an unusual complication of ACS and the complexities of the decision making process.

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Dr Florian Gahleitner (Corresponding author)

ST5 Paediatrics, Paediatric Department, Southampton,
General Hospital, Tremona Road, Southampton, SO16 6YD

Dr Ben Cambers

ST2 Paediatrics, Paediatric Department, Southampton,
General Hospital, Tremona Road, Southampton, SO16 6YD

Dr Kim Sykes

Consultant Paediatric Intensivist, Paediatric Department,
Southampton General Hospital, Tremona Road, Southampton, SO16 6YD


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