PedsQL™ sickle cell disease module: Feasibility, reliability, and validity

Abstract

Background Sickle cell disease (SCD) is an inherited chronic disease that is characterized by complications such as recurrent painful vaso-occlusive events that require frequent hospitalizations and contribute to early mortality. The objective of the study was to report on the initial measurement properties of the new PedsQL™ SCD Module for pediatric patient self-report ages 5-18 years and parent proxy-report for ages 2-18 years. Procedure The 43-item PedsQL™ SCD Module was completed in a multisite study by 243 pediatric patients with SCD and 313 parents. Participants also completed the PedsQL™ 4.0 Generic Core Scales and PedsQL™ Multidimensional Fatigue Scale. Results The PedsQL™ SCD Module Scales evidenced excellent feasibility, excellent reliability for the Total Scale Scores (patient self-report α = 0.95; parent proxy-report α = 0.97), and good reliability for the nine individual scales (patient self-report α = 0.69-0.90; parent proxy-report α = 0.83-0.97). Intercorrelations with the PedsQL™ Generic Core Scales and PedsQL™ Multidimensional Fatigue Scales were medium (0.30) to large (0.50) range, supporting construct validity. PedsQL™ SCD Module Scale Scores were generally worse for patients with severe versus mild disease. Confirmatory factor analysis demonstrated an acceptable to excellent model fit. Conclusions The PedsQL™ SCD Module demonstrated acceptable measurement properties. The PedsQL™ SCD Module may be utilized in the evaluation of SCD-specific health-related quality of life in clinical research and practice. In conjunction with the PedsQL™ Generic Core Scales and the PedsQL™ Multidimensional Fatigue Scale, the PedsQL™ SCD Module will facilitate the understanding of the health and well-being of children with SCD.

A multicenter randomized controlled trial of intravenous magnesium for sickle cell pain crisis in children

“…29,30 The PedsQL sickle cell disease module, the only valid and reliable disease-specific measure for children with sickle cell disease, was also used. 31 HRQL assessments were measured at all 8 sites by paper report (parent proxy for all patients 18 years old or younger, self-report for patients 8 years old or older, and self-report with aid of the research coordinator for patients aged 5-7 years) before the first study drug infusion, after the sixth dose of medication (or immediately before discharge if fewer than 6 doses were administered), and by telephone 8 to 10 days postdischarge. Biomarkers were measured 1 hour after the fourth dose of medication and analyzed as previously described.…”

Validated instruments for children and adolescents with chronic disease: an integrative review

“…Finally, the authors showed clinical potential and focusing the scale research of purposes to assess the severity of symptoms of fatigue [10] and said that the use of it will facilitate the understanding of health and wellbeing of children with the disease [9]. In Portugal, a version of PedsQL was also validated to be applied to children aged between 5-7 years old and 8-12 years old diagnosed with diabetes mellitus type 1 or spina bifida [8].…”

“…The synthesis build of the selected studies in the review show that the motivation to develop an instrument or quiz in the theme of chronical is strictly linked to factors, as: handling of a specific disease such as diabetes [6,7,8], sickle cell anemia [9,10], asthma [7,11,12], chronic kidney disease [13], Cystic fibrosis [14]; children and/or adolescents family inclusion in the validation process or as subject of the instrument application [6,10,15,16,17]; measurement of subjective dimensions of life experience with chronic disease such as: quality of life [7,13], satisfaction with care [17], chronic pain experience [18] as well as the limitations to develop common activities [19]. The use of statistical measures as tools to support the validity, reliability and reproducibility of instruments built was present in all studies.…”

“…The content validation was used in some studies [10,11,16,17] as a method to check reliability to the purpose that each instrument proposed to measure. Asthma was also cited as the target of research, in this the authors developed and validated a set of illustrations representing the asthma control levels in order to improve the care provided by parents to children.…”

“…Among the experiments with sickle cell anemia [9,10] researchers used an already validated questionnaire called PedsQL (Pediatric Quality of Life inventory) and built a version to evaluate the fatigue caused by sickle cell anemia using the MFS scale (Multidimensional Fatigue Scale). Finally, the authors showed clinical potential and focusing the scale research of purposes to assess the severity of symptoms of fatigue [10] and said that the use of it will facilitate the understanding of health and wellbeing of children with the disease [9].…”