Quality of life and behavioral functioning in Dutch pediatric patients with hereditary spherocytosis

Myra Teunissen, Channa T. Hijmans, Marjon H. Cnossen, Madelon B. Bronner, Martha A. Grootenhuis, Marjolein Peters

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)

Abstract

The objective of this study was to evaluate health-related quality of life (HRQoL) and behavioral functioning in pediatric patients with hereditary spherocytosis (HS). A cross-sectional study was conducted in 132 Dutch children and adolescents with HS and aged 8-18 years of whom 48 underwent splenectomy prior to the study. HRQoL was assessed using the KIDSCREEN-27, and behavioral functioning was evaluated using the strength and difficulties questionnaire (SDQ). Scores of pediatric patients with HS were compared to a Dutch norm population. Additionally, the effects of three factors were assessed: fatigue, self-image, and parents' perceived vulnerability (measured with the checklist individual strength, the self-perception profile for children and adolescents, and the child vulnerability scale). Both unsplenectomised and splenectomised pediatric patients reported lower HRQoL on the domain physical well-being (KIDSCREEN-27) compared to Dutch peers. For behavioral functioning, parents of both groups reported more emotional problems (SDQ) compared to the norm population. Pediatric patients with lower scores on physical well-being experienced more fatigue. The patients' perceived social acceptance and parents' perceived vulnerability appeared as determinants of emotional problems. Conclusion: Pediatric patients in the current study generally report few complaints, and the results suggest that these patients overall have a strong ability to cope with HS. Despite these few complaints, fatigue and parents' perceived vulnerability seem to be important determinants for lower HRQoL and more emotional problems. Therefore, screening on these factors could serve as an addition to the treatment of HS, to help pediatric patients who are at risk for lower HRQoL or more emotional problems.

Original languageEnglish
Pages (from-to)1217-1223
Number of pages7
JournalEuropean Journal of Pediatrics
Volume173
Issue number9
DOIs
Publication statusPublished - Sept 2014
Externally publishedYes

Keywords

  • Behavioral functioning
  • Hereditary spherocytosis
  • HRQoL
  • KIDSCREEN
  • SDQ

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