Homozygous sickle cell disease in patients above 20 years of age:: follow-up of 108 patients in Dakar

Context. - The progress realized in the treatment of sickle cell anemia has resulted to a better and longer life for these patients in developed countries. Ongoing challenge are to manage complication and social living for these patients. The purpose of this study was to determine morbidity, mortality and socioprofessional insertion of homozygous sickle cell patients, followed up in Dakar university hospital. Patients and methods. - We performed a longitudinal and prospective study including 108 homozygous sickle cell anemia patients, whose age were above 20 years, followed in a regular basis (at least 3 times per year), during 5 years on average (3 to 12 years). All patients had a quarterly hematological check-up and a annual statement to detect chronic complications. Clinic and paraclinical data, as information in socioprofessional insertion were noted in medical records for analysis. Results. - Mean age was 27 years (20-51 years). The age between 20-29 years was represented by 67.5%, 26.9% aged 30-39 years and 5.6% were above 40 years of age. Men were slightly predominant with a sex-ratio of 1.25. Concerning morbidity, 96.3% of patients had at least 1 vaso-occlusive crisis per year, 26.9% were transfused and 64.8% had been hospitalized during the follow-up. A chronic complication was found in 49% of patients (53/108). The more frequent of these complications were gall stone (10%), femoral head necrosis (9.2%), priapism (11.6% of men), chronic leg ulceration (4.6%), ophthalmic involvement (3.7%), renal and cardiac complications (2.7% for each one). The presence of complications was not significantly influenced by patient's sex. Five patients died during a mean follow-up of 5 years. Concerning school attendance, 13% reach university level whereas 47% stop their education on the secondary level and 40% on primary level. Professionally, 36.2% of patients had no exercise any activity. Conclusion. - Our results emphasize that life expectancy can be prolonged in sickle cell anemia patients in Africa, when they have a benefit of a regular follow-up. We show also the respective frequency of chronic complications and then, the necessity of multidisciplinary teams to optimize the take care of sickle cell anemia patients in Africa. (C) 2003 Editions scientifiques et medicales Elsevier SAS. Tous droits reserves.