Health-Related Quality of Life Assessments by Children and Adolescents with Sickle Cell Disease and Their Parents in Portugal

Clara Abadesso; Susana Pacheco; Maria Céu Machado; G Allen Finley

doi:10.3390/children9020283

Health-Related Quality of Life Assessments by Children and Adolescents with Sickle Cell Disease and Their Parents in Portugal

Children (Basel). 2022 Feb 18;9(2):283. doi: 10.3390/children9020283.

Authors

Clara Abadesso¹, Susana Pacheco¹, Maria Céu Machado², G Allen Finley³

Affiliations

¹ Hospital Prof. Dr. Fernando Fonseca, EPE-Amadora, 2720-276 Amadora, Portugal.
² Pediatrics, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisbon, Portugal.
³ Anesthesia & Psychology, Dalhousie University, Halifax, B3H 4R2 NS, Canada.

Abstract

Health-Related Quality of Life (HRQL) can be used to measure the impact of Sickle Cell Disease (SCD) on the child and their family and is generally reduced. No research has yet measured HRQL in Portuguese pediatric SCD patients.

Objectives: (1) Describe and compare HRQL of children with SCD reported by them and their parents; (2) Compare with a pediatric population with no SCD; (3) Find predictive factors of HRQL in SCD children.

Methods: Descriptive, case-control study that included sixty-eight children and adolescents with SCD (aged 3 to 18 years) and their parents. Control group-children with no SCD, matched by age, gender and ethnic background. HRQL was assessed using the multidimensional self-report PedsQL^® 4.0 Generic Scales. Summary scores for overall HRQL and subscale scores for physical, emotional, social and school functioning were compared within groups (children-parents) and with the control group. Clinical and socio-demographic variables were analyzed to find predictive factors of HRQL in pediatric SCD patients.

Results: Children with SCD and their parents had significantly lower overall and all subdomains of HRQL, compared with the control group. Children with SCD also rated lower when compared with their parents (only significant for social functioning), with low to moderate correlations. Children and parent reports declined with increasing age. Higher pain frequency was associated with worse total and psychosocial domains of HRQL. The number of hospitalizations was a predictor of worse school score, and female gender was a predictor of worse emotional score.

Conclusions: SCD significantly affects children's HRQL. Parents can provide a good proxy report, although both evaluations are beneficial. Disease status, like number of hospitalizations and frequency of pain, influences HRQL. Interventions in SCD should consider improvements in HRQL as an important outcome.

Keywords: HRQL; Health Related Quality of Life; Pediatric Quality of Life InventoryTM; PedsQL; Sickle Cell Disease; adolescent; children.