[Sickle cell disease: from childhood to adolescence. Management in 2001]

Abstract

Sickle cell anaemia is a chronic disease which evolves on the background of a basal state punctuated by critical periods, often with complications requiring overall treatment. The management of both periods must be well integrated into the treatment programme. In order to treat occlusive crisis, medical staff must be well trained and equipped for the complicated clinical diagnoses involved in drepanocytosis: bone infarction or osteomyelitis, pneumonia or acute chest syndrome, multifocal crisis or severe infectious disease. Neonatal diagnosis is the best way for early treatment by preventing severe pneumococcal diseases, ensuring sufficient hydration and appropriate surveillance, as well as providing family education. Much progress has been made over the last decade in the early treatment of retinitis, biliary lithiasis, and aseptic hip necrosis. It is also important to ensure accurate prescriptions for hydroxyurea. Specialised centres for drepanocytosis treatment are now a growing necessity and should be encouraged in order to provide treatment for dispersed patients against a relatively little known disease.