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Scoping review of symptoms in children with rare, progressive, life-threatening disorders
  1. Colleen Pawliuk1,
  2. Kim Widger2,
  3. Tammie Dewan3,
  4. Gina Brander4,
  5. Helen L Brown5,
  6. Anne-Mette Hermansen1,
  7. Marie-Claude Grégoire6,
  8. Rose Steele7 and
  9. Harold (Hal) Siden3,8
  1. 1BC Children's Hospital Research Institute, Vancouver, British Columbia, Canada
  2. 2Lawrence S. Bloomberg Faculty of Nursing, University of Toronto, Toronto, Ontario, Canada
  3. 3Department of Paediatrics, The University of British Columbia, Vancouver, British Columbia, Canada
  4. 4Regina Campus Library, Saskatchewan Polytechnic, Regina, Saskatchewan, Canada
  5. 5Woodward Library, The University of British Columbia, Vancouver, British Columbia, Canada
  6. 6Department of Paediatrics, Dalhousie University, Halifax, Nova Scotia, Canada
  7. 7School of Nursing, York University, Toronto, Ontario, Canada
  8. 8Department of Pediatrics, BC Children's Hospital, Vancouver, British Columbia, Canada
  1. Correspondence to Dr Harold (Hal) Siden, Department of Pediatrics, The University of British Columbia, Vancouver, BC V6T 1Z4, Canada; hsiden{at}bcchr.ca

Abstract

Background Q3 conditions are progressive, metabolic, neurological or chromosomal childhood conditions without a cure. Children with these conditions face an unknown lifespan as well as unstable and uncomfortable symptoms. Clinicians and other healthcare professionals are challenged by a lack of evidence for symptom management for these conditions.

Aims In this scoping review, we systematically identified and mapped the existing literature on symptom management for children with Q3 conditions. We focused on the most common and distressing symptoms, namely alertness, behavioural problems, bowel incontinence, breathing difficulties, constipation, feeding difficulties, sleep disturbance, temperature regulation, tone and motor problems and urinary incontinence. For children with complex health conditions, good symptom management is pertinent to ensure the highest possible quality of life.

Methods Scoping review. Electronic database searches in Ovid MEDLINE, Embase and CINAHL and a comprehensive grey literature search.

Results We included 292 studies in our final synthesis. The most commonly reported conditions in the studies were Rett syndrome (n=69), followed by Cornelia de Lange syndrome (n=25) and tuberous sclerosis (n=16). Tone and motor problems were the most commonly investigated symptom (n=141), followed by behavioural problems (n=82) and sleep disturbance (n=62).

Conclusion The evidence for symptom management in Q3 conditions is concentrated around a few conditions, and these studies may not be applicable to other conditions. The evidence is dispersed in the literature and difficult to access, which further challenges healthcare providers. More research needs to be done in these conditions to provide high-quality evidence for the care of these children.

  • symptoms and symptom management
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Footnotes

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  • Contributors CP developed and implemented the search strategy, reviewed abstracts, titles and full articles, and contributed to the writing of the manuscript and revised it for important intellectual content. KW, TD,

    M-CG and RS participated in planning the study, reviewed abstracts, titles and full articles, and revised the manuscript for important intellectual content. GB implemented the search strategy and revised the manuscript for important intellectual content. HLB provided methodological guidance and revised the manuscript for important intellectual content. A-MH reviewed abstracts, titles and full articles and revised the manuscript for important intellectual content. HS conceptualised the study, participated in planning the study, reviewed abstracts, titles and full articles, contributed to the writing and revised it for important intellectual content.

  • Funding This work was supported by the Canadian Institutes of Health Research (CIHR) Knowledge Synthesis Grant #328968.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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