Elsevier

The Journal of Pediatrics

Volume 148, Issue 4, April 2006, Pages 508-516.e1
The Journal of Pediatrics

Original article
Quality-of-life in children and adolescents with cystic fibrosis managed in both regional outreach and cystic fibrosis center settings in queensland

https://doi.org/10.1016/j.jpeds.2005.11.040Get rights and content

Objectives

To assess the health-related quality-of-life (HRQOL) of children/adolescents with cystic fibrosis (CF) and compare HRQOL in children managed by cystic fibrosis outreach service (CFOS) with those treated in a cystic fibrosis center (CFC). To compare HRQOL of children with CF in Queensland with previously published HRQOL data from the United States and examine the relationship between HRQOL scores and pulmonary function.

Study design

Participants were children/adolescents with CF and their parents managed by the Royal Children’s Hospital Queensland at a CFC or CFOS. Two HRQOL surveys were used: PedsQL™ and Cystic Fibrosis Questionnaire (CFQ).

Results

There were 91 CFC and 71 CFOS participants with similar demographics. PedsQL™ total summary score was statistically higher in CFOS, P = .05. There was no significant difference in CFQ scores between groups. Queensland parents reported lower HRQOL for their children compared with US parents (P<.01) despite similar pulmonary function. Declining pulmonary function correlated with worse CFQ scores in adolescents, P <.05.

Conclusions

Children living in regional Queensland reported as good as or slightly better HRQOL compared with children attending a CFC. Parent proxy HRQOL scores were generally low suggesting a reduced perception of HRQOL by parents for their children.

Section snippets

Methods

The participants were children and adolescents, and their parents, living in Queensland and Northern New South Wales who were treated by the Royal Children’s Hospital CF team in a tertiary (CFC) or outreach setting (CFOS). Children were between 2 and 19 years of age and had a proven diagnosis of CF based on genetic and/or sweat testing and compatible clinical features. Geographical location was categorized according to the Accessibility/Remoteness Index of Australia (ARIA). 14 ARIA

Results

The overall survey completion rate was 75% (162 of 217 participants). There was a higher completion rate in the CFC population (88.4%, 91 of 103) compared with the CFOS population (62.28%, 71 of 114), P <.001. More females (53.7%) than males responded, P = .01, but there was no difference between genotype of respondents and nonrespondents. Of the 46 teens, participation was significantly higher for the CFC (24 of 27, 89.0%) compared with those from the CFOS (10 of 19, 53.0%), P = .006. There

Discussion

We report the quality-of-life of children with CF treated in both tertiary and outreach settings and provide an important insight on quality-of-life for children and adolescents with CF in Queensland. Interestingly, 8- to 12-year-olds tended to report a poorer HRQOL on the Psychosocial, Social, Emotional, and School domains compared with younger children and teens on the PedsQL™. Both the child self-report total scores and parent proxy-report data for this age group were similar to values

References (29)

  • J.W. Varni

    The PedsQL™ 4.0 Measurement Model for the Pediatric Quality of Life Inventory™ Version 4.0

    (2000)
  • A.L. Quittner et al.

    Cystic Fibrosis Questionnaire (CFQ): A Health-Related Quality of Life Measure: User’s Manual

    (2000)
  • J. Williams et al.

    Health-related quality of life of overweight and obese children

    JAMA

    (2005)
  • Measuring Remoteness: Accessibility/Remoteness Index of Australia (ARIA). October 2001. rev. ed

    (2001)
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