[Problems of rehabilitation in patients with sickle cell disease in Cotonou, Benin]

Manifestations of sickle cell disease (SCD) are polymorphous from childhood to adulthood.

The purpose of this study was to assess outcomes of rehabilitation in the patients with SCD.

This retrospective analytical descriptive study included 160 SCD patients rehabilitated from 1998 to 2006 at the National University Hospital in Cotonou, Benin.

Mean patient was 31 years (range, 3 to 73 years). There was a female predominance (62.5%) with a sex ratio of 0.6. The type of SCD was HbAS in 53.1% of patients, HbSS in 21.3%, HbSC in 16.9%, and HbAC in 8.1%. The main clinical manifestations were classified as ischemic/infectious in 40% of patients, rheumatologic in 40%, and neurologic in 10%. Ischemic manifestations were observed in all patients with HbSS, HbSC, and HbAS while manifestations were preferentially neurological in patients with HbSS (p = 4,43.10(-3)) and rheumatologic in patients with HbAS (p<10(-3)). At the end of rehabilitation, persistent deficiencies, disabilities and limitations involved pain in 43.8% of patients, articular stiffness in 43.8%, muscular weakness in 46.9%, gait anomalies in 33.1%, amyotrophy in 21.2%, shortening of lower extremity in 16.9%, and tilting of the pelvis in 6.3%.

This study shows that SCD is a highly debilitating disease. Although rheumatologic manifestations are not specific to SCD, the other complications described including femoral head necrosis, osteomyelitis, and stroke have been extensively documented in the literature. If primary prevention is unfeasible, early multidisciplinary management appears to be the most effective approach to reducing SCD-related disability.