Health Related Quality of Life and Psychosocial Profile of Patients with Sickle Cell Disease and Their Caregivers

Abstract Background Sickle cell disease (SCD) is a chronic, debilitating disorder can negatively affect health-related quality-of-life (HRQoL) outcomes in SCD. Aim of the Study Primary objective was to assess the prevalence of impaired psychosocial profile and poor HRQoL among SCD patients and their caregivers in comparison to age and sex matched controls, while secondary objective was to determine the association of such impairment if any with parameters of disease severity. Methods An observational, case control study recruited sixty-five children and adolescent with confirmed SCD, conducted at Paediatric Haematology Oncology clinic, Ain Shams University, Egyptfrom1st of August 2020 to 28th of February 2021. Demographic and clinical data were collected, and thorough clinical and psychiatric assessments were conducted. Quality-of-life (QoL) of both children and their caregivers were performed compared to 65 matched healthy control. Results sixty five children and adolescents with confirmed SCD were included; there were 34 (52.3%) boys and 31 (47.7%) girls and their mean age was 11.40 ± 3.55. Most patients 44(67.7%) had sickle HbSβ+-thalassemia, the most frequent manifestations were vasoooclusive crises in 24 (36.9%) patients, The most common causes for hospital admission were pain crisis in 14 (33.3%) patients and vasoocclusive crisis in 14 (33.3%). Results indicated that patients with SCD and their caregivers had depression and anxiety symptoms scores more than reported in control group.Sickle children had significantly less self-esteem and quality of life than control children, least scores in s ick le c hild re n Q O L wer e in co mmunica t io n do ma in. These were s ignific a nt ne ga t ive consequences, advanced age, duration of illness, number and duration of hospitalizations, disease severity score and occurrence of complications were associated with psychological disorders. Conclusion The QOoL of children suffered from SCD and their caregivers is adversely affected necessitate implementation of interventions which focus on reducing depressive symptoms, enhancing self-esteem and QoL.