Sickle cell disease in the French overseas territories (French West Indies, French Guiana, la Reunion, Mayotte): descriptive data and management organization

Introduction - Sickle cell disease is a major public health concern in French overseas territories with the implementation of a systematic neo- natal screening and a comprehensive management programme in the 1980s. The present article describes sickle cell related epidemiological data and management organization of the different overseas territories. Methods - Data were collected from the 2010's annual report of the French Association for Screening and Prevention of Infant Handicaps (AFDPHE) and from the different hospital medical information system or annual reports, including PMSI. Results - The incidence of major sickle cell disease syndromes at birth was 1/4,551 in la Reunion, 1/633 in Mayotte, 1/343 in Martinique, 1/297 in Guadeloupe, and 1/227 in French Guiana. The overall frequency of AS carriers was 5.2% for all the overseas territories versus 2.7% in mainland France from 2006 to 2010. The development of medical organization and the different management services identified are also presented. Conclusion - Sickle cell disease is frequent in French overseas territories. Its frequency is variable from one territory to another. Too many patients are lost to follow up due to migratory flows, non compliance to treatment of patients with asymptomatic disease, deprived socioeconomic conditions of foreign patients, and geographical or communication difficulties.