Chest infections and syndromes in sickle cell disease of childhood

Sickle cell lung disease occurs in all of the more common sickle hemoglobinopathies. Of the acute pulmonary diseases, acute chest syndrome (ACS) is most common. The risk of recurrence after one episode of ACS ranges from 20% to 80%. Repeated episodes of ACS contribute to the development of earlier and potentially rapid pulmonary deterioration particularly in young adults. ACS is the second most common cause for hospital admission and has been reported to be responsible for 25% of sickle cell deaths. The exact etiology of ACS may be unclear and is often caused by the interaction of a number of factors. Although infection is most likely in the young child, infarction and thromboembolism are the more likely causes in older patients. Outcome is dependent on immediate recognition and rapid institution of therapy. Maintenance of adequate oxygenation treatment of possible underlying infection, and adequate hemoglobin delivery are essential. Simple or exchange transfusion is vital to improve hemoglobin delivery and decrease hemoglobin S concentration. Caution is advised when administering drugs such as morphine for pain control because of risk of exacerbation of pulmonary symptomatology.