Sickle Cell Disease

Sickle cell disease is characterized by vaso-occlusive and hemolysis events that may occur in a variable range of clinical presentations. Patients with this disease, particularly with acute chest syndrome, suffer acute episodes of chest pain caused by vaso-occlusive crisis, that tend to repeat, especially under the influence of lower respiratory infections, dehydration, and any cause of hypoxemia. Children and adolescents with sickle cell disease must be periodically evaluated through oximetry, lung function tests, sleep study, and echocardiograms to detect obstructive and restrictive diseases that frequently worsens the lung condition. Asthma and sleep disorders contribute to morbidity and must be controlled.