Health-related Quality of Life in Children and Adolescents With Sickle Cell Disease

Abstract

Objective-To assess health-related quality of life (HRQOL) in children and adolescents with sickle cell disease (SCD).Design, Setting, and Participants-The PedsQL™ 4.0 Generic Scales, a multidimensional self-report instrument that has been shown to be valid and reliable for use in children and adolescents with chronic illness, consists of 23 items that assess physical, emotional, social, and school functioning. Questionnaires were administered to 124 children and adolescents (aged 8-18 years, child self-report) with SCD (100 sickle cell anemia [SS], 24 sickle beta zero thalassemia [Sβ 0 thal]) and their parents (parent-proxy report). Summary scores for children's and parents' ratings of overall HRQOL and psychosocial health and subscale scores for physical, emotional, social, and school functioning were compared to published data for healthy children. Both summary and subscale scores for children with SCD were also compared to those of their parents.Results-Children with SCD and their parents rated overall HRQOL and all sub-domains of HRQOL lower than ratings of healthy children and their parents (p < .001). Children with SCD rated their own HRQOL significantly better than their parents for overall HRQOL and all subdomains (p <.001) except emotional functioning (p = .06).Conclusions-Children with SCD and their parents perceived overall HRQOL and all HRQOL sub-domains to be lower than scores reported in healthy children. Therefore, successful therapeutic efforts to improve HRQOL could represent important advances in the health of children with SCD. KeywordsQuality of Life; Health Related Quality of Life; Sickle Cell Disease; Chronic Illness; Children and Adolescents None of the authors report any financial interest or potential conflicts of interest.Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Sickle cell disease (SCD) is one of the most common genetic disorders affecting 1 in every 500 African-American births and 1 in 1000 to 1400 Hispanic-American births in the United States (Human Genome Project, 2005). Children and adolescents with SCD may require hospitalization for acute complications such as painful episodes, acute chest syndrome, splenic sequestration, infection, stroke, aplastic crisis, and priapism. Common chronic complications of SCD include also pigment gallstones, delayed growth and development, avascular necrosis, pulmonary hypertension, and renal disease. Management of these complications may require hospitalization or treatment at home, in an ambulatory setting, or in the emergency department (ED), thus affecting attendance at school and normal p...

Health Related Quality of Life of Patients with Sickle Cell Disease

“…Prior studies confirmed that children with SCD had significantly impaired health related quality of life when compared to healthy children [2,5,8]. The quality of life of our patients were not only lower than healthy children but also very similar to those found in patients suffering from other chronic conditions such as cancer receiving treatment and rheumatoid arthritis [2,5,8].…”

“…It is a severe chronic diseases associated with continuous treatment over an extended period of time, and significant human suffering [2]. It is well known that all chronic and acute complications of SCD could be expected to have a negative impact on quality of life of the patients, especially for children and adolescent [2,3].…”

“…It is a severe chronic diseases associated with continuous treatment over an extended period of time, and significant human suffering [2]. It is well known that all chronic and acute complications of SCD could be expected to have a negative impact on quality of life of the patients, especially for children and adolescent [2,3]. Therefore, the evaluation of patients' health based almost exclusively on the clinical severity of the disease has certainly created distortions between the medical approach and expectation of individuals, especially in chronic diseases such as SCD [2][3][4].…”

“…It is well known that all chronic and acute complications of SCD could be expected to have a negative impact on quality of life of the patients, especially for children and adolescent [2,3]. Therefore, the evaluation of patients' health based almost exclusively on the clinical severity of the disease has certainly created distortions between the medical approach and expectation of individuals, especially in chronic diseases such as SCD [2][3][4]. Health-related quality of life (HRQoL) is a concept that may provide the subject's own view of his/her wellbeing and not the view of a health professional about the disease [4].…”

“…Health-related quality of life (HRQoL) is a concept that may provide the subject's own view of his/her wellbeing and not the view of a health professional about the disease [4]. In spite of the importance of HRQoL in medical evaluation, few studies in this field have been focused on SCD, and rarer still are those that were dedicated to the pediatric age-range [2][3][4][5]. For this reason, the aim of the present study was to evaluate the health-related quality of life in pediatric patients with Sickle Cell Disease.…”