RESUMEN
Background Sickle cell disease (SCD) is a genetic disease of public health concern. Improved quality healthcare has increased the life expectancy of these patients; however, they also face an increased frequency of vaso-occlusive crises and other SCD complications. These complications affect their quality of life, an area of care, which healthcare providers often overlook. We sought to determine the health-related quality of life among patients living with sickle cell disease in Lagos, Nigeria. Materials and methods We conducted a cross-sectional study of 198 patients with sickle cell disease who attended the adult sickle cell clinic at a tertiary hospital in Lagos, Nigeria, during the period from October 1, 2018, to February 28, 2019. A self-administered questionnaire was used to obtain the clinical and socio-demographic characteristics of the patients and the 35-item Short-Form Health Survey (SF-36) questionnaire was used to determine their health-related quality of life (HRQoL). Determinants of HRQoL were established using bivariate and multivariate regression analysis. Results The mean age of the 198 patients who participated in the study was 28.4±9.1 years, mean steady-state hemoglobin was 8.2 ± 1.3 g/dl, and 85 (42.9%) patients had a monthly income of 150 USD or less. In the previous year, 65 (32.1 %) and 33 (16.6%) patients, respectively, suffered one to two episodes (s) of acute bone pain crises and acute chest syndrome, and 43 (24.7%) had blood transfusion. Using the scoring system for SF-36 provided by RAND Health, role limitation due to physical health had the lowest median score of 50 (interquartile range {IQR}: 0-100). On bivariate analysis, bone pain crisis was associated with statistically significant low scores across all the 8 HRQoL domains of the SF36 questionnaire. Other variables, including having received blood transfusion, recent hospitalization, acute chest syndrome, lower level of income, and younger age, were also associated with significantly low scores. On regression analysis, bone pain crisis, level of income, and acute chest syndrome were found to be independent determinants of quality of life in the patients. Conclusion Sickle cell disease has a negative impact on the health-related quality of life of those affected. The presence of bone pain crisis is an important predictor of health-related quality of life in sickle cell disease patients. To improve patient outcomes, healthcare providers should take a holistic approach in evaluating and managing this disease, taking into cognizance how the complications and the financial burden of this disease impact the quality of life of affected patients.
RESUMEN
PURPOSE: To determine the neurological complications associated with sickle cell anaemia (SCA) in Nigerians and evaluate the relative frequencies. METHODOLOGY: Six-hundred-thirteen patients with SCA attending outpatient clinics of Lagos University Teaching Hospital and 616 control subjects were evaluated using a uniform structured questionnaire to determine the occurrence of neurological complications. The relative frequencies of neurological abnormalities in patients and controls were compared. RESULTS: Neurological abnormalities occurred in a significantly higher percentage of patients (76%) compared to controls (32.1%). Among children, these abnormalities included stroke, febrile seizures and headache. Among adolescents and adults, the abnormalities included paraplegia, epileptic seizures and localized sensory neuropathy. Headache occurred in a significantly higher percentage in children and adolescents compared to controls, but not in adults. CONCLUSIONS: We conclude that SCA is associated with neurological complications: stroke and febrile seizures in children, epileptic seizures, paraplegia and localized sensory neuropathy in adolescents and adults, headache in children and adolescents. Detailed studies of each of these complications would be required to provide further insight into their significance.
