Potential predictors of musculoskeletal manifestations in paediatric patients with sickle cell disease at the University Teaching Hospital, Lusaka

Abstract

Sickle Cell Disease (SCD) is an autosomal recessive inherited condition characterized by the inheritance of two abnormal genes coding for the formation of Haemoglobin and one of them is Haemoglobin s. Musculoskeletal manifestations are the commonest clinical presentations of both acute and chronic cases of vaso-occlusive crisis (VOC). According to the Surgical department (of the University Teaching Hospital, Lusaka) audits done in the last 3 years, patients with SCD have been presenting very late with advanced musculoskeletal manifestations. The objective of this study was to explore the potential predictors of Musculoskeletal Manifestations in Paediatric patients presenting with Sickle Cell Disease seen at the University Teaching Hospital, Lusaka. The specific objectives were: To determine the commonest musculoskeletal manifestations in paediatric patients presenting with Sickle Cell Disease seen at University Teaching Hospital; then to determine the socio-demographic factors associated with these musculoskeletal manifestations of Sickle Cell Disease; and finally, was to establish if there was any relationship between the musculoskeletal manifestations and the potential predictors. This was an unmatched case-control study conducted at the University Teaching Hospital in Lusaka, Zambia. This study was conducted between January and April 2019. A total of 171 patients all with SCD of 16 years or below were recruited. The ‘cases’ had Musculoskeletal Manifestations, while the ‘controls’ did not have. A full assessment of these patients was done to establish these musculoskeletal manifestations and their potential predictors. The commonest musculoskeletal manifestations were found to be Chronic Osteomyelitis (29.82%), Acute Osteomyelitis (21.05%) and Avascular necrosis of the femoral head (14.04%). The median age for the cases was 9.5 years (Interquartile range (IQR), 7 – 12) while for the controls was 7 (IQR, 4 – 11). Increase in age (p=0.003), age at diagnosis (p<0.001), monthly income (p=0.03), percentage of Haemoglobin s (<0.001), frequency of vaso-occlusive crisis (p<0.001) and Aspartate transaminase (p=0.01) had a significant association with the development of musculoskeletal manifestations. However, using multivariable logistic regressions: Increase in age, frequency of vaso-occlusive crisis and the percentage of Haemoglobin s were the only variables with significant association. Musculoskeletal manifestations are common and their main predictors are: Increase in age, frequency of vaso-occlusive crisis and percentage of Haemoglobin s. Chronic Osteomyelitis is the commonest musculoskeletal manifestation in the paediatric population with SCD seen at the University Teaching Hospital, Lusaka. Keywords: Sickle Cell Disease, Sickle Cell Anaemia, Musculoskeletal Manifestation, Vaso- Occlusive Crisis