The performance of the PedsQL generic core scales in children with sickle cell disease

Area of Science:

• Pediatric Health
• Hematology
• Quality of Life Research

Background:

• Sickle cell disease significantly impacts children's health-related quality of life (HRQL).
• Accurate measurement of HRQL is crucial for managing pediatric sickle cell disease.
• Validated instruments are needed to assess HRQL in this population.

Purpose of the Study:

• To evaluate the feasibility, reliability, and validity of the Pediatric Quality of Life Inventory (PedsQL) generic core scales.
• To assess the PedsQL's utility in distinguishing HRQL between children with and without sickle cell disease.
• To determine if the PedsQL can differentiate between varying severities of sickle cell disease.

Main Methods:

• Cross-sectional study involving children aged 2-18 years from sickle cell disease and primary care clinics.
• Health-related quality of life (HRQL) assessed using the PedsQL questionnaire (parent proxy-report and child self-report).
• Feasibility assessed by missing item analysis; reliability by Cronbach's alpha; validity by comparing groups and disease severity.

Main Results:

• The PedsQL questionnaire demonstrated feasibility and reliability in children with sickle cell disease.
• Both parent proxy and child self-reports effectively differentiated children with and without sickle cell disease.
• Parent proxy-reports accurately distinguished between mild and severe sickle cell disease.

Conclusions:

• The PedsQL questionnaire is a feasible, reliable, and valid instrument for measuring HRQL in children with sickle cell disease.
• The PedsQL facilitates the assessment of HRQL across different disease severities.
• This tool supports comprehensive care and research for pediatric sickle cell disease.