Management

Pathogens are rarely isolated from homozygous sickle cell adults with the acute chest syndrome, although it is usual practice to give antibiotics (e.g. cefuroxime plus erythromycin) which may at least prevent secondary infection. Oxygen is given and physiotherapy is used to encourage ventilation and to drain infected areas. Pulse oximetry gives reliable information in pigmented subjects and values do not appear to be offset by HbS. However, desaturation is common in the steady state and a quarter of steady state homozygous sickle cell adolescents in the Jamaican Cohort Study had values below 90 per cent. Therefore changes in oxygen saturation are more important than actual levels and continuous monitoring is vital in patients with the acute chest syndrome. Patients demonstrating rapid clinical deterioration and progressive falls in oxygen saturation may indicate acute pulmonary sequestration. A PaO2 below 75 mmHg (10 kPa) has been suggested as an indicator for both intensive care admission and immediate exchange transfusion. This is facilitated by central venous cannulation which also enables central venous pressure monitoring. The aim of exchange transfusion is to reduce levels of HbS; some recommend partial exchange to lower HbS levels below 20 to 30 per cent, whereas others advocate as complete a replacement with adult hemoglobin as possible. The optimal degree of exchange transfusion has yet to be verified by a controlled study, but sufficient evidence exists to confirm that transfusion per se is beneficial. HbS levels should be monitored every 2 to 3 days with further top-up or exchange transfusions as indicated by departmental policy.

Pulmonary artery catheterization is occasionally warranted if hemodynamic compromise is marked or if doubt remains about the adequacy of volume resuscitation. It is important to maintain normovolemia as well as normoxemia. Invasive or non-invasive mechanical ventilation is indicated when there is inability to maintain adequate gas exchange and the patient is fatiguing. It is important to maintain adequate oxygen saturations throughout, although no benefit has been shown in administering oxygen to achieve complete hemoglobin saturation and supranormal arterial oxygen tensions.

Sleep Apnea

Sleep Apnea

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