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New insights into the pathophysiology of acute chest syndrome (ACS) have highlighted potential therapeutic strategies including the use of nitric oxide. A multidisciplinary approach to the management of patients admitted with a sickle cell crisis is needed to prevent progression to ACS and need for mechanical ventilation.
CASE REPORT
A 21 year old Afro-Caribbean man with known sickle cell disease (SCD) was admitted to hospital with painful chest, thighs, and generalised abdominal pain. This was his second admission to hospital with a sickle cell crisis. He was on no regular medications apart from analgesia taken at home during crises. Apart from anaemia, there was nothing abnormal to find on examination. The haemoglobin (Hb) was 7.5 g/dl (normal for him), white cell count 22.5 × 109, and the electrolytes were normal apart from a slightly raised C-reactive protein (CRP) level of 30 mg/l. Radiographs of his abdomen and thighs were normal, but his chest radiograph showed a small degree of basal atelectasis bilaterally. His oxygen saturation was 96% on air. He was treated with a subcutaneous opiate infusion using a syringe pump, intravenous fluids, oxygen, and encouraged to drink.
Over the next 24 hours his pain was not well controlled and required an increasing dose of opiates. He became pyrexial (38.5°C), his oxygen saturation fell to 92% on air, and antibiotics to cover community acquired pneumonia were commenced. On the third day, however, he became more drowsy with arterial blood gases of pH 7.35, Pao2 13.5 kPa, and Paco2 7.5 kPa on 40% oxygen. A repeat chest radiograph showed new infiltrates in both lower zones and a diagnosis of acute sickle chest syndrome (ACS) was made. Despite an exchange transfusion, he continued to deteriorate and was eventually intubated and ventilated. On intensive care his Fio2 …