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Children's experiences of cystic fibrosis: a systematic review of qualitative studies

Nathan Jamieson1, Dominic Fitzgerald2, Davinder Singh-Grewal2

  • 1Kids Research Institute.

Pediatrics
|May 21, 2014
PubMed

Insights

Children and adolescents with cystic fibrosis (CF) experience vulnerability and loss of independence. Patient-centered care is crucial for improving their quality of life and treatment outcomes.

Area of Science:

  • Pediatric Health
  • Genetic Disorders
  • Psychosocial Outcomes

Background:

  • Cystic fibrosis (CF) is a prevalent, life-shortening genetic disorder.
  • CF significantly impacts psychosocial well-being and quality of life in young patients.

Purpose of the Study:

  • To explore the lived experiences and perspectives of children and adolescents with CF.
  • To inform care strategies by focusing on patient-identified priorities.

Main Methods:

  • Systematic literature search across major health databases (MEDLINE, Embase, PsycINFO, CINAHL) up to April 2013.
  • Thematic synthesis methodology applied to analyze findings from 43 included articles.

Main Results:

  • Six key themes emerged: resilience, lifestyle restrictions, treatment resentment, temporal limitations, emotional vulnerability, and transplant considerations.
  • Participants reported challenges including limited independence, social isolation, treatment burden, and emotional strain.
  • Experiences varied across age groups (4-21 years) and countries, highlighting diverse challenges.

Conclusions:

  • Children and adolescents with CF often feel vulnerable, disempowered, and isolated.
  • Multidisciplinary, patient-centered care models are vital.
  • Promoting shared decision-making, self-efficacy, and support for educational/social needs enhances outcomes.
Abstract

Keywords:
adolescentcystic fibrosispediatricsqualitative researchsystematic review

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